5 - Care at the End of Life

Editors: McPhee, Stephen J.; Papadakis, Maxine A.; Tierney, Lawrence M.

Title: Current Medical Diagnosis & Treatment, 46th Edition

Copyright ©2007 McGraw-Hill

> Table of Contents > 8 - Ear, Nose, & Throat

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8

Ear, Nose, & Throat

Robert K. Jackler MD

Michael J. Kaplan MD

Diseases of The Ear

Hearing Loss

Essentials of Diagnosis

  • Three main types of hearing loss: conductive, sensory, and neural.

  • Most commonly due to cerumen impaction or transient auditory tube dysfunction associated with upper respiratory tract infection.

Classification & Epidemiology

A. Conductive Hearing Loss

Conductive hearing loss results from dysfunction of the external or middle ear. There are four mechanisms, each resulting in impairment of the passage of sound vibrations to the inner ear: (1) obstruction (eg, cerumen impaction), (2) mass loading (eg, middle ear effusion), (3) stiffness effect (eg, otosclerosis), and (4) discontinuity (eg, ossicular disruption). Conductive losses in adults are most commonly due to cerumen impaction or transient auditory tube dysfunction associated with upper respiratory tract infection. Persistent conductive losses usually result from chronic ear infection, trauma, or otosclerosis. Conductive hearing loss is generally correctable with medical or surgical therapy—or in some cases both.

B. Sensory Hearing Loss

Sensory hearing loss results from deterioration of the cochlea, usually due to loss of hair cells from the organ of Corti. Sensorineural losses in adults are common. A gradually progressive, predominantly high-frequency loss with advancing age (presbyacusis) is typical. Other than aging effects, common causes of sensorineural loss include excessive noise exposure, head trauma, and systemic diseases such as diabetes mellitus. Sensory hearing loss is not correctable with medical or surgical therapy but often may be prevented or stabilized.

Angeli SI et al: Etiologic diagnosis of sensorineural hearing loss in adults. Otolaryngol Head Neck Surg 2005;132:890.

C. Neural Hearing Loss

Neural hearing loss occurs with lesions involving the eighth nerve, auditory nuclei, ascending tracts, or auditory cortex. It is the least common clinically recognized cause of hearing loss. Causes include acoustic neuroma, multiple sclerosis, and cerebrovascular disease.

Jackler RK: A 73-year-old man with hearing loss. JAMA 2003; 289:1557.

Evaluation of Hearing (Audiology)

In a quiet room, the hearing level may be estimated by having the patient repeat aloud words presented in a soft whisper, a normal spoken voice, or a shout. Tuning forks are useful in differentiating conductive from sensorineural losses. A 512-Hz tuning fork is used, since frequencies below this level elicit a tactile response. In the Weber test, the tuning fork is placed on the forehead or front teeth. In conductive losses, the sound appears louder in the poorer-hearing ear, whereas in sensorineural losses it radiates to the better side. In the Rinne test, the tuning fork is placed alternately on the mastoid bone and in front of the ear canal. In conductive losses, bone conduction exceeds air conduction; in sensorineural losses, the opposite is true.

Formal audiometric studies are performed in a soundproofed room. Pure-tone thresholds in decibels (dB) are obtained over the range of 250–8000 Hz (the main speech frequencies are between 500 and 3000 Hz) for both air and bone conduction. Conductive losses create a gap between the air and bone thresholds, whereas in sensorineural losses both air and bone thresholds are equally diminished. The threshold of normal hearing is from 0 to 20 dB, which corresponds to the loudness of a soft whisper. Mild hearing loss is indicated by a threshold of 20–40 dB (soft spoken voice), moderate loss by a threshold of 40–60 dB (normal spoken voice), severe loss by a threshold of 60–80 dB (loud spoken voice), and profound loss by a threshold of 80 dB (shout). The clarity of hearing is often impaired in sensorineural hearing loss. This is evaluated by speech discrimination testing, which is reported as percentage correct (90–100% is normal).

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The site of the lesion responsible for sensorineural loss—whether it lies in the cochlea or in the central auditory system—may be determined with auditory brainstem-evoked responses.

Every patient who complains of a hearing loss should be referred for audiologic evaluation unless the cause is easily remediable (eg, cerumen impaction, otitis media). Audiologic screening is not recommended for adults with apparently normal hearing unless they are exposed to potentially injurious levels of noise or have reached the age of 65, after which screening evaluations should be done every few years.

Bagai A et al: Does this patient have hearing impairment? JAMA 2006;295:416.

Hearing Rehabilitation

Patients with hearing loss not correctable by medical therapy may benefit from hearing amplification. Contemporary hearing aids are comparatively free of distortion and have been miniaturized to the point where they often may be contained entirely within the ear canal. To optimize the benefit, a hearing aid must be carefully selected to conform to the nature of the hearing loss. Digitally programmable hearing aids are now becoming available that allow optimization of speech intelligibility and may be tuned to deal with difficult listening circumstances.

Currently, there is much interest focused on the development of semi-implantable and even fully implantable hearing aids. A variety of devices are under development that deliver vibrations—usually via either a rare earth magnet or a piezoceramic crystal—directly to the ossicular chain. Some of these devices are in clinical trials. An alternative strategy, the bone-anchored hearing aid, uses an oscillating post drilled into the mastoid. This technology shows promise in surgically uncorrectable conductive hearing loss as well as in unilateral sensorineural deafness.

Aside from hearing aids, many assistive devices are available to improve comprehension in individual and group settings, to help with hearing television and radio programs, and for telephone communication. In persons with profound sensory hearing loss, the cochlear implant—an electronic device that is surgically implanted to stimulate the auditory nerve—offers socially beneficial auditory rehabilitation to most adults with acquired deafness.

Copeland BJ et al: Cochlear implantation for the treatment of deafness. Annu Rev Med 2004;55:157.

Hol MK et al: Bone-anchored hearing aids in unilateral inner ear deafness: an evaluation of audiometric and patient outcome measurements. Otol Neurotol 2005;26:999.

Lesner SA: Candidacy and management of assistive listening devices: special needs of the elderly. Int J Audiol 2003;42 (Suppl 2):2S68.

Middlebrooks JC et al: Cochlear implants: the view from the brain. Curr Opin Neurobiol 2005;15:488.

Mo B et al: Cochlear implants and quality of life: a prospective study. Ear Hear 2005;26:186.

Palmer CV et al: Hearing loss and hearing aids. Neurol Clin 2005;23:901.

Diseases of The Auricle

Disorders of the external ear are for the most part dermatologic. Skin cancers due to sun exposure are common and may be treated with standard techniques. Traumatic auricular hematoma must be recognized and drained to prevent significant cosmetic deformity (cauliflower ear) resulting from dissolution of supporting cartilage. Similarly, cellulitis of the auricle must be treated promptly to prevent development of perichondritis and its resultant deformity. Relapsing polychondritis is a systemic disorder often associated with recurrent, frequently bilateral, painful episodes of auricular erythema and edema. Treatment with corticosteroids may help forestall cartilage dissolution. Respiratory compromise may occur as a result of progressive involvement of the tracheobronchial tree. Chondritis and perichondritis may be differentiated from auricular cellulitis by sparing of involvement of the lobule, which does not contain cartilage.

Silapunt S et al: Squamous cell carcinoma of the auricle and Mohs micrographic surgery. Dermatol Surg 2005;31(11 Pt 1): 1423.

Diseases of the Ear Canal

1. Cerumen Impaction

Cerumen is a protective secretion produced by the outer portion of the ear canal. In most persons, the ear canal is self-cleansing. Recommended hygiene consists of cleaning the external opening with a washcloth over the index finger without entering the canal itself. In most cases, cerumen impaction is self-induced through ill-advised attempts at cleaning the ear. It may be relieved with detergent ear drops (eg, 3% hydrogen peroxide; 6.5% carbamide peroxide), mechanical removal, suction, or irrigation. Irrigation is performed with water at body temperature to avoid a vestibular caloric response. The stream should be directed at the ear canal wall adjacent to the cerumen plug. Irrigation should be performed only when the tympanic membrane is known to be intact.

Use of jet irrigators designed for cleaning teeth (eg, WaterPik) for wax removal should be avoided since they may result in tympanic membrane perforations. Following professional irrigation, the ear canal should be thoroughly dried (eg, by instilling isopropyl alcohol or using a hair blow-dryer on low-power setting) to reduce the likelihood of inducing external otitis. Specialty referral for cleaning under microscopic guidance is indicated when the impaction has not responded to routine measures or if the patient has a history of chronic otitis media or tympanic membrane perforation.

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Hobson JC et al: Use and abuse of cotton buds. J R Soc Med 2005;98:360.

Roland PS et al: Randomized, placebo-controlled evaluation of Cerumenex and Murine earwax removal products. Arch Otolaryngol Head Neck Surg 2004;130:1175.

2. Foreign Bodies

Foreign bodies in the ear canal are more frequent in children than in adults. Firm materials may be removed with a loop or a hook, taking care not to displace the object medially toward the tympanic membrane; microscopic guidance is helpful. Aqueous irrigation should not be performed for organic foreign bodies (eg, beans, insects), because water may cause them to swell. Living insects are best immobilized before removal by filling the ear canal with lidocaine.

Thompson SK et al: External auditory canal foreign body removal: management practices and outcomes. Laryngoscope 2003;113:1912.

3. External Otitis

Essentials of Diagnosis

  • Erythema and edema of the ear canal skin.

  • Often with purulent exudates.

  • Persistent external otitis in the diabetic or immunocompromised patient may evolve into osteomyelitis of the skull base, often called malignant external otitis.

External otitis presents with otalgia, frequently accompanied by pruritus and purulent discharge. There is often a history of recent water exposure or mechanical trauma (eg, scratching, cotton applicators). External otitis is usually caused by gram-negative rods (eg, Pseudomonas, Proteus) or fungi (eg, Aspergillus), which grow in the presence of excessive moisture.

Examination reveals erythema and edema of the ear canal skin, often with a purulent exudate. Manipulation of the auricle often elicits pain. Because the lateral surface of the tympanic membrane is ear canal skin, it is often erythematous. However, in contrast to acute otitis media, it moves normally with pneumatic otoscopy. When the canal skin is very edematous, it may be impossible to visualize the tympanic membrane.

Fundamental to the treatment of external otitis is protection of the ear from additional moisture and avoidance of further mechanical injury by scratching. Otic drops containing a mixture of aminoglycoside antibiotic and anti-inflammatory corticosteroid in an acid vehicle are generally very effective (eg, neomycin sulfate, polymyxin B sulfate, and hydrocortisone). Purulent debris filling the ear canal should be gently removed to permit entry of the topical medication. Drops should be used abundantly (five or more drops three or four times a day) to penetrate the depths of the canal. When substantial edema of the canal wall prevents entry of drops into the ear canal, a wick is placed to facilitate entry of the medication. In recalcitrant cases—particularly when cellulitis of the periauricular tissue has developed—oral fluoroquinolones (eg, ciprofloxacin, 500 mg twice daily for 1 week) are the drugs of choice because of their effectiveness against Pseudomonas species.

Block SL: Otitis externa: providing relief while avoiding complications. J Fam Pract 2005;54:669.

Roland PS et al: Ciprodex Otic AOE Study Group. Efficacy and safety of topical ciprofloxacin/dexamethasone versus neomycin/polymyxin B/hydrocortisone for otitis externa. Curr Med Res Opin 2004;20:1175.

4. Pruritus

Pruritus of the external auditory canal, particularly at the meatus, is a common problem. While it may be associated with external otitis or with dermatologic conditions such as seborrheic dermatitis and psoriasis, most cases are self-induced either from excoriation or by overly zealous ear cleaning. To permit regeneration of the protective cerumen blanket, patients should be instructed to avoid use of soap and water or cotton swabs in the ear canal. Patients with excessively dry canal skin may benefit from application of mineral oil, which helps counteract dryness and repel moisture. When an inflammatory component is present, topical application of a corticosteroid (eg, 0.1% triamcinolone) may be beneficial. It is axiomatic in persistent pruritus that the patient must cease scratching the ear. In stubborn cases, the fingernails must be kept short and the patient may need to wear cotton gloves at night to avoid manipulation during sleep. Symptomatic reduction of pruritus may be obtained by use of oral antihistamines (eg, diphenhydramine, 25 mg orally at bedtime). Topical application of isopropyl alcohol promptly relieves ear canal pruritus in many patients.

5. Malignant External Otitis

Persistent external otitis in the diabetic or immunocompromised patient may evolve into osteomyelitis of the skull base, often called malignant external otitis. Usually caused by Pseudomonas aeruginosa, osteomyelitis begins in the floor of the ear canal and may extend into the middle fossa floor, the clivus, and even the contralateral skull base. The patient usually presents with persistent foul aural discharge, granulations in the ear canal, deep otalgia, and progressive cranial nerve palsies involving nerves VI, VII, IX, X, XI, or XII. Diagnosis is confirmed by the demonstration of osseous erosion on CT and radionuclide scanning.

Treatment is chiefly medical, requiring prolonged antipseudomonal antibiotic administration, often for several months. Although intravenous therapy is often required, selected patients may be managed with ciprofloxacin

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(500–1000 mg orally twice daily), which has proved effective against many of the causative Pseudomonas strains. To avoid relapse, antibiotic therapy should be continued, even in the asymptomatic patient, until gallium scanning indicates a marked reduction in the inflammatory process. Surgical debridement of infected bone is reserved for cases of deterioration despite medical therapy.

Rubin Grandis J et al: The changing face of malignant (necrotising) external otitis: clinical, radiological, and anatomic correlations. Lancet Infect Dis 2004;4:34.

Singh A et al: Skull base osteomyelitis: diagnostic and therapeutic challenges in atypical presentation. Otolaryngol Head Neck Surg 2005;133:121.

6. Exostoses & Osteomas

Bony overgrowths of the ear canal are a frequent incidental finding and occasionally have clinical significance. Clinically, they present as skin-covered mounds in the medial ear canal obscuring the tympanic membrane to a variable degree. Solitary osteomas are of no significance as long as they do not cause obstruction or infection. Multiple exostoses, which are generally acquired from repeated exposure to cold water, often progress and require surgical removal.

Vasama JP: Surgery for external auditory canal exostoses: a report of 182 operations. ORL J Otorhinolaryngol Relat Spec 2003;65:189.

7. Neoplasia

The most common neoplasm of the ear canal is squamous cell carcinoma (SCC). When an apparent otitis externa does not resolve on therapy, SCC should be suspected and biopsy performed. This disease carries a very high 5-year mortality rate because the tumor tends to invade the lymphatics of the cranial base and must be treated with wide surgical resection and radiation therapy. Adenomatous tumors, originating from the ceruminous glands, generally follow a more indolent course.

Devaney KO et al: Tumours of the external ear and temporal bone. Lancet Oncol 2005;6:411.

Yin M et al: Analysis of 95 cases of squamous cell carcinoma of the external and middle ear. Auris Nasus Larynx 2006 [Epub ahead of print].

Diseases of the Auditory Tube

1. Auditory Tube Dysfunction

Essentials of Diagnosis

  • Aural fullness.

  • Fluctuating hearing.

  • Discomfort with barometric pressure change.

The tube that connects the middle ear to the nasopharynx—the auditory tube, or eustachian tube—provides ventilation and drainage for the middle ear cleft. It is normally closed, opening only during the act of swallowing or yawning. When auditory tube function is compromised, air trapped within the middle ear becomes absorbed and negative pressure results. The most common causes of auditory tube dysfunction are diseases associated with edema of the tubal lining, such as viral upper respiratory tract infections and allergy. The patient usually reports a sense of fullness in the ear and mild to moderate impairment of hearing. When the tube is only partially blocked, swallowing or yawning may elicit a popping or crackling sound. Examination reveals retraction of the tympanic membrane and decreased mobility on pneumatic otoscopy. Following a viral illness, this disorder is usually transient, lasting days to weeks. Treatment with systemic and intranasal decongestants (eg, pseudoephedrine, 60 mg orally every 4 hours; oxymetazoline, 0.05% spray every 8–12 hours) combined with autoinflation by forced exhalation against closed nostrils may hasten relief. Autoinflation should not be recommended to patients with active intranasal infection, since this maneuver may precipitate middle ear infection. Allergic patients may also benefit from desensitization or intranasal corticosteroids (eg, beclomethasone dipropionate, two sprays in each nostril twice daily for 2–6 weeks). Air travel, rapid altitudinal change, and underwater diving should be avoided.

An overly patent auditory tube is a relatively uncommon problem that may be quite distressing. Typical complaints include fullness in the ear and autophony, an exaggerated ability to hear oneself breathe and speak. A patulous auditory tube may develop during rapid weight loss, or may be idiopathic. In contrast to a hypofunctioning auditory tube, the aural pressure is often made worse by exertion and may diminish during an upper respiratory tract infection. Although physical examination is usually normal, respiratory excursions of the tympanic membrane may occasionally be detected during vigorous breathing. Treatment includes avoidance of decongestant products, insertion of a ventilating tube to reduce the outward stretch of the eardrum during phonation, and, rarely, surgical narrowing of the auditory tube.

Grimmer JF et al: Update on eustachian tube dysfunction and the patulous eustachian tube. Curr Opin Otolaryngol Head Neck Surg 2005;13:277.

2. Serous Otitis Media

Essentials of Diagnosis

  • Blocked auditory tube remains for a prolonged period.

  • Resultant negative pressure will result in transudation of fluid.

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When the auditory tube remains blocked for a prolonged period, the resultant negative pressure will result in transudation of fluid. This condition, known as serous otitis media, is especially common in children because their auditory tubes are narrower and more horizontal in orientation than those in adults. Serous otitis media is less common in adults, in whom it usually follows an upper respiratory tract infection or barotrauma. In an adult with persistent unilateral serous otitis media, nasopharyngeal carcinoma must be excluded. The tympanic membrane in serous otitis media is dull and hypomobile, occasionally accompanied by air bubbles in the middle ear and conductive hearing loss. The treatment of serous otitis media is similar to that for auditory tube dysfunction. A short course of oral corticosteroids (eg, prednisone, 40 mg/d for 7 days) has been advocated by some in the management of serous otitis media, as have oral antibiotics (eg, amoxicillin, 250 mg orally three times daily for 7 days)—or even a combination of the two. The role of these regimens remains controversial, but they are probably of little lasting benefit.

When medication fails to bring relief after several months, a ventilating tube placed through the tympanic membrane may restore hearing and alleviate the sense of aural fullness. Endoscopically guided laser expansion of the nasopharyngeal orifice of the auditory tube may improve function in recalcitrant cases.

Dogru H et al: Squamous cell metaplasia of the nasopharyngeal epithelium and its association with adult-onset otitis media with effusion. Acta Otolaryngol 2005;125:580.

Kujawski OB et al: Laser eustachian tuboplasty. Otol Neurotol 2004;25:1.

Satre TJ et al: Treatments for persistent otitis media with effusion. Am Fam Physician 2005;71:529.

3. Barotrauma

Persons with auditory tube dysfunction caused by either congenital narrowness or acquired mucosal edema may be unable to equalize the barometric stress exerted on the middle ear by air travel, rapid altitudinal change, or underwater diving. The problem is generally most acute during airplane descent, since the negative middle ear pressure tends to collapse and lock the auditory tube. Several measures are useful to enhance auditory tube function and avoid otic barotrauma. The patient should be advised to swallow, yawn, and autoinflate frequently during descent, which may be painful if the auditory tube collapses. Systemic decongestants (eg, pseudoephedrine, 60–120 mg) should be taken several hours before anticipated arrival time so that they will be maximally effective during descent. Topical decongestants such as 1% phenylephrine nasal spray should be administered 1 hour before arrival.

The treatment of acute negative middle ear pressure that persists on the ground is with decongestants and attempts at autoinflation. Myringotomy (creation of a small eardrum perforation) provides immediate relief and is appropriate in the setting of severe otalgia and hearing loss. Repeated episodes of barotrauma in persons who must fly frequently may be alleviated by insertion of ventilating tubes.

Underwater diving represents even a greater barometric stress to the ear than flying. The problem occurs most commonly during the descent phase, when pain develops within the first 15 feet if inflation of the middle ear via the auditory tube has not occurred. Divers must descend slowly and equilibrate in stages to avoid the development of severely negative pressures in the tympanum that may result in hemorrhage (hemotympanum) or perilymphatic fistulization. In the latter, the oval or round window ruptures, resulting in sensory hearing loss and acute vertigo. Emesis due to acute labyrinthine dysfunction can be very dangerous during an underwater dive. Sensory hearing loss or vertigo, which develops during the ascent phase of a saturation dive, may be the first (or only) symptom of decompression sickness. Immediate recompression will return intravascular gas bubbles to solution and restore the inner ear microcirculation. Patients should be warned to avoid diving when they have upper respiratory infections or episodes of nasal allergy. Tympanic membrane perforation is an absolute contraindication to diving, as the patient will experience an unbalanced thermal stimulus to the semicircular canals and may experience vertigo, disorientation, and even emesis. Finally, persons with only one hearing ear should be discouraged from diving because of the significant risk of otologic injury.

Mirza S et al: Otic barotrauma from air travel. J Laryngol Otol 2005;119:366.

Diseases of the Middle Ear

1. Acute Otitis Media

Essentials of Diagnosis

  • Otalgia, often with an upper respiratory tract infection.

  • Erythema and hypomobility of tympanic membrane.

General Considerations

Acute otitis media is a bacterial infection of the mucosally lined air-containing spaces of the temporal bone. Purulent material forms not only within the middle ear cleft but also within the mastoid air cells and petrous apex when they are pneumatized. Acute otitis media is usually precipitated by a viral upper respiratory tract infection that causes auditory tube edema. This results in accumulation of fluid and mucus,

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which becomes secondarily infected by bacteria. The most common pathogens both in adults and in children are Streptococcus pneumoniae, Haemophilus influenzae, and Streptococcus pyogenes.

Clinical Findings

Acute otitis media is most common in infants and children, although it may occur at any age. Presenting symptoms and signs include otalgia, aural pressure, decreased hearing, and often fever. The typical physical findings are erythema and decreased mobility of the tympanic membrane. Occasionally, bullae will be seen on the tympanic membrane. Although it is taught that this represents infection with Mycoplasma pneumoniae, most cases involve more common pathogens.

Rarely, when middle ear empyema is severe, the tympanic membrane can be seen to bulge outward. In such cases, tympanic membrane rupture is imminent. Rupture is accompanied by a sudden decrease in pain, followed by the onset of otorrhea. With appropriate therapy, spontaneous healing of the tympanic membrane occurs in most cases. When perforation persists, chronic otitis media frequently evolves. Mastoid tenderness often accompanies acute otitis media and is due to the presence of pus within the mastoid air cells. This alone does not indicate suppurative (surgical) mastoiditis.

Treatment

The treatment of acute otitis media is specific antibiotic therapy, often combined with nasal decongestants. The first-choice oral antibiotic treatment is amoxicillin (20–40 mg/kg/d) or erythromycin (50 mg/kg/d) plus sulfonamide (150 mg/kg/d) for 10 days. Alternatives useful in resistant cases are cefaclor (20–40 mg/kg/d) or amoxicillin-clavulanate (20–40 mg/kg/d) combinations.

Tympanocentesis for bacterial (aerobic and anaerobic) and fungal culture may be performed by any experienced physician. A 20-gauge spinal needle bent 90 degrees to the hub attached to a 3-mL syringe is inserted through the inferior portion of the tympanic membrane. Interposition of a pliable connecting tube between the needle and syringe permits an assistant to aspirate without inducing movement of the needle. Tympanocentesis is useful for otitis media in immunocompromised patients and when infection persists or recurs despite multiple courses of antibiotics.

Surgical drainage of the middle ear (myringotomy) is reserved for patients with severe otalgia or when complications of otitis (eg, mastoiditis, meningitis) have occurred.

Recurrent acute otitis media may be managed with long-term antibiotic prophylaxis. Single daily oral doses of sulfamethoxazole (500 mg) or amoxicillin (250 or 500 mg) are given over a period of 1–3 months. Failure of this regimen to control infection is an indication for insertion of ventilating tubes.

Rovers MM et al: Otitis media. Lancet 2004;363:465.

2. Chronic Otitis Media & Cholesteatoma

Chronic infection of the middle ear and mastoid generally develops as a consequence of recurrent acute otitis media, although it may follow other diseases and trauma. Perforation of the tympanic membrane is usually present. This may be accompanied by mucosal changes such as polypoid degeneration and granulation tissue and osseous changes such as osteitis and sclerosis. The bacteriology of chronic otitis media differs from that of acute otitis media. Common organisms include P aeruginosa, Proteus species, Staphylococcus aureus, and mixed anaerobic infections. The clinical hallmark of chronic otitis media is purulent aural discharge. Drainage may be continuous or intermittent, with increased severity during upper respiratory tract infection or following water exposure. Pain is uncommon except during acute exacerbations. Conductive hearing loss results from destruction of the tympanic membrane and ossicular chain. The medical treatment of chronic otitis media includes regular removal of infected debris, use of earplugs to protect against water exposure, and topical antibiotic drops for exacerbations. The activity of ciprofloxacin against Pseudomonas may help dry a chronically discharging ear when given in a dosage of 500 mg orally twice a day for 1–6 weeks.

Definitive management is surgical in most cases. Tympanic membrane repair may be accomplished with temporalis muscle fascia or with homograft middle ear structures. Successful reconstruction of the tympanic membrane may be achieved in about 90% of cases, often with elimination of infection and significant improvement in hearing. When the mastoid air cells are involved by irreversible infection, they should be exenterated through mastoidectomy.

Cholesteatoma is a special variety of chronic otitis media. The most common cause is prolonged auditory tube dysfunction, with resultant chronic negative middle ear pressure that draws inward the upper flaccid portion of the tympanic membrane. This creates a squamous epithelium-lined sac, which—when its neck becomes obstructed—may fill with desquamated keratin and become chronically infected. Cholesteatomas typically erode bone, with early penetration of the mastoid and destruction of the ossicular chain. Over time they may erode the inner ear, involve the facial nerve, and on rare occasions spread intracranially. Physical examination reveals an epitympanic retraction pocket or marginal tympanic membrane perforation that exudes keratin debris. The treatment of cholesteatoma is surgical marsupialization of the sac or its complete removal. This often requires creation of a “mastoid bowl” in which the ear canal and mastoid are joined into a large common cavity that must be periodically cleaned.

Bance M et al: Topical treatment for otorrhea: issues and controversies. J Otolaryngol 2005;34 (Suppl 2):S52.

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Gardner EK et al: Results with titanium ossicular reconstruction prostheses. Laryngoscope 2004;114:65.

Olszewska E et al: Etiopathogenesis of cholesteatoma. Eur Arch Otorhinolaryngol 2004;261:6.

3. Complications of Otitis Media

Mastoiditis

Acute suppurative mastoiditis usually evolves following several weeks of inadequately treated acute otitis media. It is characterized by postauricular pain and erythema accompanied by a spiking fever. Radiography reveals coalescence of the mastoid air cells due to destruction of their bony septa. Initial treatment consists of intravenous antibiotics and myringotomy for culture and drainage. Failure of medical therapy indicates the need for surgical drainage (mastoidectomy).

Agrawal S et al: Complications of otitis media: an evolving state. J Otolaryngol 2005;34(Suppl 1):S33.

Leskinen K et al: Acute complications of otitis media in adults. Clin Otolaryngol 2005;30:511.

Petrous Apicitis

The medial portion of the petrous bone between the inner ear and clivus may become a site of persistent infection when the drainage of its pneumatic cell tracts becomes blocked. This may cause foul discharge, deep ear and retro-orbital pain, and sixth nerve palsy (Gradenigo's syndrome); meningitis may be a complication. Treatment is with prolonged antibiotic therapy (based on culture results) and surgical drainage via petrous apicectomy.

Lee YH et al: CT, MRI and gallium SPECT in the diagnosis and treatment of petrous apicitis presenting as multiple cranial neuropathies. Br J Radiol 2005;78:948.

Otogenic Skull Base Osteomyelitis

Infections originating in the external or middle ear may result in osteomyelitis of the skull base, usually due to P aeruginosa. The diagnosis and management of this disease are discussed in the section on malignant external otitis.

Facial Paralysis

Facial palsy may be associated with either acute or chronic otitis media. In the acute setting, it results from inflammation of the seventh nerve in its middle ear segment, perhaps mediated through bacterially secreted neurotoxins. Treatment consists of myringotomy for drainage and culture, followed by intravenous antibiotics (based on culture results). The use of corticosteroids is controversial. The prognosis is excellent, with complete recovery in most cases.

Facial palsy associated with chronic otitis media usually evolves slowly due to chronic pressure on the seventh nerve in the middle ear or mastoid by cholesteatoma. Treatment requires surgical correction of the underlying disease. The prognosis is less favorable than for facial palsy associated with acute otitis media.

Popovtzer A et al: Facial palsy associated with acute otitis media. Otolaryngol Head Neck Surg 2005;132:327.

Sigmoid Sinus Thrombosis

Trapped infection within the mastoid air cells adjacent to the sigmoid sinus may cause septic thrombophlebitis. This is heralded by signs of systemic sepsis (spiking fevers, chills), at times accompanied by signs of increased intracranial pressure (headache, lethargy, nausea and vomiting, papilledema). Diagnosis can be made noninvasively by magnetic resonance venography. Treatment is with intravenous antibiotics (based on culture results), surgical drainage, and—when embolization is suspected—ligation of the internal jugular vein in the neck.

Manolidis S et al: Diagnosis and management of lateral sinus thrombosis. Otol Neurotol 2005;26:1045.

Central Nervous System Infection

Otogenic meningitis is by far the most common intracranial complication of ear infection. In the setting of acute suppurative otitis media, it arises from hematogenous spread of bacteria, most commonly H influenzae and S pneumoniae. In chronic otitis media, it results either from passage of infections along preformed pathways such as the petrosquamous suture line or from direct extension of disease through the dural plates of the petrous pyramid.

Epidural abscesses arise from direct extension of disease in the setting of chronic infection. They are usually asymptomatic but may present with deep local pain, headache, and low-grade fever. They are often discovered as an incidental finding at surgery. Brain abscess may arise in the temporal lobe or cerebellum as a result of septic thrombophlebitis adjacent to an epidural abscess. The predominant causative organisms are S aureus, S pyogenes, and S pneumoniae. Rupture into the subarachnoid space results in meningitis and often death. (See Chapter 30.)

Migirov L et al: Otogenic intracranial complications: a review of 28 cases. Acta Otolaryngol 2005;125:819.

Penido Nde O et al: Intracranial complications of otitis media: 15 years of experience in 33 patients. Otolaryngol Head Neck Surg 2005;132:37.

4. Otosclerosis

Otosclerosis is a progressive disease with a marked familial tendency that affects bone surrounding the inner ear. Lesions involving the footplate of the stapes result in increased impedance to the passage of sound through the ossicular chain, producing conductive hearing loss. This may be corrected through surgical

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replacement of the stapes with a prosthesis (stapedectomy). When otosclerotic lesions impinge on the cochlea, permanent sensory hearing loss occurs. Some evidence suggests that this level of hearing loss may be stabilized by treatment with oral sodium fluoride over prolonged periods of time (Florical—8.3 mg sodium fluoride and 364 mg calcium carbonate—two tablets orally each morning). Fluorides have minimal adverse effects other than occasional mild gastric irritation, which may be eliminated by ingesting the drug with meals.

Chandarana S et al: Quality of life following small fenestra stapedotomy. Ann Otol Rhinol Laryngol 2005;114:472.

Massey BL et al: Stapedectomy outcomes: titanium versus teflon wire prosthesis. Laryngoscope 2005;115:249.

5. Trauma to the Middle Ear

Tympanic membrane perforation may result from impact injury or explosive acoustic trauma. Spontaneous healing occurs in the great majority of cases. Persistent perforation may result from secondary infection brought on by exposure to water. Patients should be advised to wear earplugs while swimming or bathing during the healing period. Hemorrhage behind an intact tympanic membrane (hemotympanum) may follow blunt trauma or extreme barotrauma. Spontaneous resolution over several weeks is the usual course. When a conductive hearing loss greater than 30 dB persists for more than 3 months following trauma, disruption of the ossicular chain should be suspected. Middle ear exploration with reconstruction of the ossicular chain, combined with repair of the tympanic membrane when required, will usually restore hearing.

Ohlrogge M et al: Temporal bone fracture. Otol Neurotol 2004;25:195.

6. Middle Ear Neoplasia

Primary middle ear tumors are rare. Glomus tumors arise either in the middle ear (glomus tympanicum) or in the jugular bulb with upward erosion into the hypotympanum (glomus jugulare). They present clinically with pulsatile tinnitus and hearing loss. A vascular mass may be visible behind an intact tympanic membrane. Large glomus jugulare tumors are often associated with multiple cranial neuropathies, especially involving nerves VII, IX, X, XI, and XII. Treatment may require surgery, radiotherapy, or both.

Durvasula VS et al: Laser excision of glomus tympanicum tumours: long-term results. Eur Arch Otorhinolaryngol 2005; 262:325.

Earache

External otitis and acute otitis media are the two most common causes of earache. In external otitis, there is often a recent history of swimming, Q-tip use, or physical trauma, while in acute otitis media there is usually an antecedent or concurrent upper respiratory infection. The physical findings also differ. In external otitis, the ear canal skin is erythematous, while in acute otitis media this generally occurs only if the tympanic membrane has ruptured, spilling purulent material into the ear canal. Also, in external otitis the tympanic membrane may be erythematous, but it retains its mobility owing to the normal aeration of the middle ear cavity. Pain out of proportion to the physical findings may be due to herpes zoster oticus, especially when vesicles appear in the ear canal or concha. Chronic otitis media is usually not painful except during acute exacerbations. Persistent pain and discharge from the ear suggest osteomyelitis of the skull base or cancer.

The sensory innervation of the ear is derived from the trigeminal, facial, glossopharyngeal, vagal, and upper cervical nerves. Because of this rich innervation, referred otalgia is quite frequent. Temporomandibular joint dysfunction is a common cause of ear pain. It is often made worse by chewing or psychogenic grinding of the teeth (bruxism) and may be associated with dental malocclusion. Management includes soft diet, local heat to the masticatory muscles, massage, analgesics, and dental referral. Repeated episodes of severe lancinating otalgia may occur in glossopharyngeal neuralgia. Treatment with carbamazepine (100–300 mg orally every 8 hours) often confers substantial symptomatic relief. Severe glossopharyngeal neuralgia, which is refractory to medical management, may respond to microvascular decompression of the ninth nerve. Infections and neoplasia that involve the oropharynx, hypopharynx, and larynx frequently cause otalgia. Persistent earache demands specialty referral to exclude cancer of the upper aerodigestive tract.

Scarbrough TJ et al: Referred otalgia in head and neck cancer: a unifying schema. Am J Clin Oncol 2003;26:e157.

Tuz HH et al: Prevalence of otologic complaints in patients with temporomandibular disorder. Am J Orthod Dentofacial Orthop 2003;123:620.

Diseases of the Inner Ear

1. Sensory Hearing Loss

Diseases of the cochlea result in sensory hearing loss, a condition that is usually irreversible. Most cochlear diseases result in bilateral symmetric hearing loss. The presence of unilateral or asymmetric sensorineural hearing loss suggests a lesion proximal to the cochlea. Lesions affecting the eighth nerve and central auditory system are discussed in the section on neural hearing loss. The primary goals in the management of sensory hearing loss are prevention of further losses and functional improvement with amplification and auditory rehabilitation.

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Presbyacusis

Presbyacusis, the most frequent cause of sensory hearing loss, is the progressive, predominantly high-frequency symmetric hearing loss of advancing age. It is difficult to separate the various etiologic factors (eg, noise trauma) that may contribute to presbyacusis, but genetic predisposition appears to play a role. Most patients notice a loss of speech discrimination that is especially pronounced in noisy environments. About 25% of people between the ages of 65 and 75 years and almost 50% of those over 75 experience hearing difficulties.

Gates GA et al: Presbycusis. Lancet 2005;366:1111.

Noise Trauma

Noise trauma is the second most common cause of sensory hearing loss. Sounds exceeding 85 dB are potentially injurious to the cochlea, especially with prolonged exposures. The loss typically begins in the high frequencies (especially 4000 Hz) and progresses to involve the speech frequencies with continuing exposure. Among the more common sources of injurious noise are industrial machinery, weapons, and excessively loud music. Personal music devices (eg, MP3 and CD players) used at excessive loudness levels may also be potentially injurious. In recent years, monitoring of noise levels in the workplace by regulatory agencies has led to preventive programs that have reduced the frequency of occupational losses. Individuals of all ages, especially those with existing hearing losses, should wear earplugs when exposed to moderately loud noises and specially designed earmuffs when exposed to explosive noises.

Nelson DI et al: The global burden of occupational noise-induced hearing loss. Am J Ind Med 2005;48:446.

Williams W: Noise exposure levels from personal stereo use. Int J Audiol 2005;44:231.

Physical Trauma

Head trauma has effects on the inner ear similar to those of severe acoustic trauma. Some degree of sensory hearing loss may occur following simple concussion and is frequent after skull fracture. Deployment of air bags during an automobile accident has been associated with hearing loss.

Ishman SL et al: Temporal bone fractures: traditional classification and clinical relevance. Laryngoscope 2004;114:1734.

Ototoxicity

Ototoxic substances may affect both the auditory and vestibular systems. The most common ototoxic medications are salicylates; aminoglycosides; loop diuretics; and several antineoplastic agents, notably cisplatin. The latter three categories may cause irreversible hearing loss even when administered in therapeutic doses. When using these medications, it is important to identify high-risk patients such as those with preexisting hearing losses or renal insufficiency. Patients simultaneously receiving multiple ototoxic agents are at particular risk owing to ototoxic synergy. Useful measures to reduce the risk of ototoxic injury include serial audiometry and monitoring of serum peak and trough levels and substitution of equivalent nonototoxic drugs whenever possible. Efforts are underway to develop strategies, known as ototoxic chemoprotection, using drugs that shield the inner ear from damage during ototoxic exposure.

It is possible for topical agents that enter the middle ear to be absorbed into the inner ear via the round window. When the tympanic membrane is perforated, use of potentially ototoxic ear drops (eg, neomycin, gentamicin) is best avoided.

Black FO et al: Permanent gentamicin vestibulotoxicity. Otol Neurotol 2004;25:559.

Roland PS: New developments in our understanding of ototoxicity. Ear Nose Throat J 2004;83(9 Suppl 4):15.

Unal OF et al: Prevention of gentamicin induced ototoxicity by trimetazidine in animal model. Int J Pediatr Otorhinolaryngol 2005;69:193.

Sudden Sensory Hearing Loss

Sudden loss of hearing in one ear may occur at any age but is more common in the elderly. It most probably is the result of sudden vascular occlusion of the internal auditory artery or of a viral inner ear infection. Prognosis is mixed, with many patients suffering permanent deafness in the involved ear while others have complete recovery. Although treatment with oral corticosteroids is controversial, many clinicians believe that such treatment improves the odds of recovery. A common regimen is prednisone, 80 mg/d, followed by a tapering dose over a 10-day period. For patients who have not responded to oral corticosteroid therapy, some clinicians advocate using supplemental intratympanic administration of corticosteroids.

Cadoni G et al: Sudden sensorineural hearing loss: our experience in diagnosis, treatment, and outcome. J Otolaryngol 2005; 34:395.

Mamak A et al: A study of prognostic factors in sudden hearing loss. Ear Nose Throat J 2005;84:641.

Wei B et al: Steroids for idiopathic sudden sensorineural hearing loss. Cochrane Database Syst Rev 2006;(1):CD003998.

Hereditary Hearing Loss

Sensory hearing loss with onset during adult life often runs in families. The mode of inheritance may be either autosomal dominant or recessive. The age at onset,

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the rate of progression of hearing loss, and the audiometric pattern (high-frequency, low-frequency, or flat) can often be predicted by studying family members. In recent years, great strides have been made in identifying the molecular genetic errors associated with hereditary hearing loss. Over 100 genes have now been identified, many of which have been cloned. The connexin-26 mutation, a particularly prevalent form of autosomal recessive nonsyndromic hearing loss may be tested clinically. Hearing loss is also frequently found in hereditary mitochondrial disorders. Progress is being made toward the development of methods to restore lost hair cells in genetic and other forms of deafness. Both insertion of the hair cell regulatory gene Math-1 via gene therapy as well as stem cell-mediated techniques show considerable promise.

Aggarwal R et al: The genetics of hearing loss. Hosp Med 2005;66:32.

Bayazit YA et al: An overview of hereditary hearing loss. ORL J Otorhinolaryngol Relat Spec 2006;68:57.

Finsterer J et al: Nuclear and mitochondrial genes mutated in nonsyndromic impaired hearing. Int J Pediatr Otorhinolaryngol 2005;69:621.

Izumikawa M et al: Auditory hair cell replacement and hearing improvement by Atoh1 gene therapy in deaf mammals. Nat Med 2005;11:271.

Li H et al: Stem cells as therapy for hearing loss. Trends Mol Med 2004;10:309.

Maiorana CR et al: Advances in inner ear gene therapy: exploring cochlear protection and regeneration. Curr Opin Otolaryngol Head Neck Surg 2005;13:308.

Autoimmune Hearing Loss

Sensory hearing loss may be associated with a wide array of systemic autoimmune disorders such as systemic lupus erythematosus, Wegener's granulomatosis, and Cogan's syndrome (hearing loss, keratitis, aortitis). The loss is most often bilateral and progressive. The hearing level often fluctuates, with periods of deterioration alternating with partial or even complete remission. The tendency is for the gradual evolution of permanent hearing loss, which usually stabilizes with some remaining auditory function but occasionally proceeds to complete deafness. Vestibular dysfunction, particularly dysequilibrium and postural instability, may accompany the auditory symptoms. A syndrome resembling Meniere's disease may also occur with intermittent attacks of severe vertigo.

In the majority of cases, the autoimmune pattern of audiovestibular dysfunction presents in the absence of recognized systemic autoimmune disease. Use of laboratory tests to screen for autoimmune disease (eg, antinuclear antibody, rheumatoid factor, erythrocyte sedimentation rate) may be informative. Specific tests of immune reactivity against inner ear antigens (anticochlear antibodies, lymphocyte transformation tests) are available but are currently of interest for research purposes only. Responsiveness to oral corticosteroid treatment is helpful in making the diagnosis and constitutes first-line therapy. If stabilization of hearing becomes dependent on long-term corticosteroid use, steroid-sparing immunosuppressive regimens may become necessary.

Broughton SS et al: Immune-mediated inner ear disease: 10-year experience. Semin Arthritis Rheum 2004;34:544.

Niparko JK et al: Serial audiometry in a clinical trial of AIED treatment. Otol Neurotol 2005;26:908.

Other Causes of Sensory Hearing Loss

There are numerous less common causes of sensory hearing loss. Metabolic derangements (eg, diabetes, hypothyroidism, hyperlipidemia, and renal failure), infections (eg, measles, mumps, syphilis), and physical factors (eg, radiation therapy) are some of the chief examples. Identification of metabolic or infectious sensory hearing losses is especially important, as these may occasionally be reversible with medical therapy. Meniere's syndrome and labyrinthitis are discussed in the section on vestibular disorders.

Kakarlapudi V et al: The effect of diabetes on sensorineural hearing loss. Otol Neurotol 2003;24:382.

2. Tinnitus

Tinnitus is the perception of abnormal ear or head noises. Persistent tinnitus usually indicates the presence of sensory hearing loss. Intermittent periods of mild, high-pitched tinnitus lasting for several minutes are common in normal-hearing persons. When severe and persistent, tinnitus may interfere with sleep and the ability to concentrate, resulting in considerable psychological distress.

The most important treatment of tinnitus is avoidance of exposure to excessive noise, ototoxic agents, and other factors that may cause cochlear damage. Masking the tinnitus with music or through amplification of normal sounds with a hearing aid may also bring some relief. Although intravenous treatment with antiarrhythmic drugs (eg, lidocaine) suppresses tinnitus in some individuals, evidence suggests no benefit with oral agents that are potentially suitable for long-term symptom relief. Among the numerous drugs that have been tried, oral antidepressants (eg, nortriptyline at an initial dosage of 50 mg orally at bedtime) have proved to be the most effective. Habituation techniques, such as tinnitus retraining therapy, may prove beneficial in those with refractory symptoms.

Pulsatile tinnitus—often described by the patient as listening to one's own heartbeat—should be distinguished from tonal tinnitus. Although often ascribed to conductive hearing loss, this symptom may be far more serious and indicates a vascular abnormality such as glomus tumor, venous sinus stenosis, carotid vaso-occlusive disease, arteriovenous malformation, or aneurysm. MR angiography and venography should be considered to establish the diagnosis.

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A staccato “clicking” tinnitus may result from middle ear muscle spasm, sometimes associated with palatal myoclonus. The patient typically perceives a rapid series of popping noises, lasting seconds to a few minutes, accompanied by a fluttering feeling in the ear.

Folmer RL et al: Long-term effectiveness of ear-level devices for tinnitus. Otolaryngol Head Neck Surg 2006;134:132.

Henry JA et al: General review of tinnitus: prevalence, mechanisms, effects, and management. J Speech Lang Hear Res 2005;48:1204.

Herraiz C et al: Long-term clinical trial of tinnitus retraining therapy. Otolaryngol Head Neck Surg 2005;133:774.

Liyanage SH et al: Pulsatile tinnitus. J Laryngol Otol 2006; 120:93.

3. Hyperacusis

Excessive sensitivity to sound may occur in normal-hearing individuals either for psychological reasons or in association with ear disease. Patients with cochlear dysfunction commonly experience recruitment, an abnormal sensitivity to loud sounds despite a reduced sensitivity to softer ones. Fitting hearing aids and other amplification devices to patients with recruitment requires use of compression circuitry to avoid uncomfortable overamplification. For normal-hearing individuals with hyperacusis, use of an earplug in noisy environments is often beneficial.

Baguley DM: Hyperacusis. J R Soc Med 2003;96:582.

4. Vertigo

Essentials of Diagnosis

  • Either a sensation of motion when there is no motion or an exaggerated sense of motion in response to a given bodily movement.

  • Cardinal symptom of vestibular disease.

  • Must differentiate peripheral from central etiologies of vestibular dysfunction.

  • Peripheral: Onset of vertigo is sudden; tinnitus, hearing loss, and horizontal nystagmus may be present.

  • Central: Onset of vertigo is gradual; when nystagmus is present, it is usually vertical. Brain MRI helpful in evaluation.

Clinical Findings

A. Symptoms and Signs

Vertigo is the cardinal symptom of vestibular disease. It is either a sensation of motion when there is no motion or an exaggerated sense of motion in response to a given bodily movement. Thus, vertigo is not just “spinning” but may present, for example, as a sense of tumbling, of falling forward or backward, or of the ground rolling beneath one's feet (“earthquake-like”). It should be distinguished from imbalance, light-headedness, and syncope, all of which are usually nonvestibular in origin. The vertigo that results from peripheral vestibulopathy is usually of sudden onset, may be so severe that the patient is unable to walk or stand, and is frequently accompanied by nausea and vomiting. Tinnitus and hearing loss may be associated and provide strong support for a peripheral origin.

A minimal physical examination of the patient with vertigo includes the Romberg test, an evaluation of gait, and observation for the presence of nystagmus. In peripheral lesions, nystagmus is usually horizontal with a rotatory component; the fast phase usually beats away from the diseased side. Visual fixation tends to inhibit nystagmus except in very acute peripheral lesions or with central nervous system disease. The Nylen-Bárány maneuvers are performed as follows: Put the patient in a sitting position on the examination table with the head turned to the right. Quickly lower the patient to the supine position with the head extending over the edge and placed 30 degrees lower than the body. Watch for nystagmus for 30 seconds. Repeat with the head turned to the left. Lastly, perform the maneuver without turning the head.

These maneuvers are intended to induce positioning nystagmus but are of limited use when the patient is able to visually fixate. This objection may be overcome either by placing +2-diopter lenses (Fresnel glasses) over the eyes or by making observations in the dark by means of electronystagmographic recording. The Fukuda test, in which the patient walks in place with eyes closed, is useful for detecting subtle defects. A positive response is observed when the patient rotates, usually toward the side of the diseased labyrinth. Vertigo arising from central lesions tends to develop gradually and then become progressively more severe and debilitating. Nystagmus is not always present but can occur in any direction and may be dissociated in the two eyes. The associated nystagmus is often nonfatigable, vertical rather than horizontal in orientation, without latency, and unsuppressed by visual fixation. Electronystagmography is useful in documenting these characteristics. The evaluation of central audiovestibular dysfunction usually requires imaging of the brain with MRI.

Episodic vertigo can occur in patients with diplopia from external ophthalmoplegia and is maximal when the patient looks in the direction where the separation of images is greatest. Cerebral lesions involving the temporal cortex may also produce vertigo, which is sometimes the initial symptom of a seizure. Finally, vertigo may be a feature of a number of systemic disorders and can occur as a side effect of certain anticonvulsant, antibiotic, hypnotic, analgesic, and tranquilizing drugs or of alcohol.

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B. Laboratory Findings

Laboratory investigations such as audiologic evaluation, caloric stimulation, electronystagmography, CT scan or MRI, and brain stem auditory evoked potential studies are indicated in patients with persistent vertigo or when central nervous system disease is suspected. These studies will help distinguish between central and peripheral lesions and to identify causes requiring specific therapy. Electronystagmography consists of objective recording of the nystagmus induced by head and body movements, gaze, and caloric stimulation. It is helpful in quantifying the degree of vestibular hypofunction and may help with the differentiation between peripheral and central lesions. Computer-driven rotatory chairs and posturography platforms offer improved diagnostic abilities but are not widely available.

Brandt T et al: General vestibular testing. Clin Neurophysiol 2005;116:406.

Guilemany JM et al: Clinical and epidemiological study of vertigo at an outpatient clinic. Acta Otolaryngol 2004;124:49.

Lempert T et al: Episodic vertigo. Curr Opin Neurol 2005;18:5.

Vertigo Syndromes Due to Peripheral Lesions

A. Endolymphatic Hydrops (Meniere's Syndrome)

Meniere's syndrome results from distention of the endolymphatic compartment of the inner ear. The primary lesion appears to be in the endolymphatic sac, which is thought to be responsible for endolymph filtration and excretion. Although a precise cause of hydrops cannot be established in most cases, two known causes are syphilis and head trauma. The classic syndrome consists of episodic vertigo, usually lasting 1–8 hours; low-frequency sensorineural hearing loss, often fluctuating; tinnitus, usually low-tone and “blowing” in quality; and a sensation of aural pressure. Symptoms wax and wane as the endolymphatic pressure rises and falls. Caloric testing commonly reveals loss or impairment of thermally induced nystagmus on the involved side.

Episodic vertigo resembling that of Meniere's syndrome but without accompanying auditory symptoms is known as recurrent vestibulopathy. The pathogenic mechanism of this symptom complex is unknown in most cases, although a few patients suffer from a variant of migraine. Others will go on to develop the classic syndrome of endolymphatic hydrops.

Kim HH et al: Trends in the diagnosis and the management of Meniere's disease: results of a survey. Otolaryngol Head Neck Surg 2005;132:722.

Onuki J et al: Comparative study of the daily lifestyle of patients with Meniere's disease and controls. Ann Otol Rhinol Laryngol 2005;114:927.

B. Labyrinthitis

Patients with labyrinthitis suffer from acute onset of continuous, usually severe vertigo lasting several days to a week, accompanied by hearing loss and tinnitus. During a recovery period that lasts for several weeks, rapid head movements may bring on transient vertigo. Hearing may return to normal or remain permanently impaired in the involved ear. The cause of labyrinthitis is unknown, although it frequently follows an upper respiratory tract infection.

C. Positioning Vertigo

This form of vertigo is usually peripheral in origin. Transient vertigo following changes in head position is a frequent complaint. The term “positioning vertigo” is more accurate than “positional vertigo” because it is provoked by changes in head position rather than by the maintenance of a particular posture. Use of the term “benign positional vertigo” is discouraged except for cases known to be unassociated with central nervous system disorders. True positional vertigo suggests either vertebrobasilar insufficiency or dysfunction of the cervical spine.

The typical symptoms of positioning vertigo occur in clusters that persist for several days. Typically with peripheral lesions, there is a latency period of several seconds following a head movement before symptoms develop, and they subside within 10–60 seconds. Constant repetition of the positional change leads to habituation. In central lesions, there is no latent period, fatigability, or habituation of the sign and symptoms. Single-session physical therapy protocols, based on the theory that peripheral positioning vertigo results from free-floating otoconia within a semicircular canal, have recently been developed. These strive to reposition the offending crystals through a series of head manipulations. New surgical procedures are also being explored which, by interrupting the posterior semicircular canal, attempt to prevent the exaggerated response to angular head motion.

Salvinelli F et al: Benign paroxysmal positional vertigo: diagnosis and treatment. Clin Ter 2004;155:395.

von Brevern M et al: Benign paroxysmal positional vertigo: current status of medical management. Otolaryngol Head Neck Surg 2004;130:381.

D. Vestibular Neuronitis

In vestibular neuronitis, a paroxysmal, usually single attack of vertigo occurs without accompanying impairment of auditory function and may persist for several days to weeks before clearing. Examination reveals nystagmus and absent responses to caloric stimulation on one or both sides. The cause of the disorder is unclear. Treatment is symptomatic.

E. Traumatic Vertigo

The most common cause of vertigo following head injury is labyrinthine concussion. Symptoms generally

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diminish within several days but may linger for a month or more. Basilar skull fractures that traverse the inner ear usually result in severe vertigo lasting several days to a week and deafness in the involved ear. Chronic posttraumatic vertigo may result from cupulolithiasis. This occurs when traumatically detached statoconia (otoconia) settle on the ampulla of the posterior semicircular canal and cause an excessive degree of cupular deflection in response to head motion. Clinically, this presents as episodic positioning vertigo.

Ernst A et al: Management of posttraumatic vertigo. Otolaryngol Head Neck Surg 2005;132:554.

F. Perilymphatic Fistula

Leakage of perilymphatic fluid from the inner ear into the tympanic cavity via the round or oval window is often discussed as a cause of vertigo and sensory hearing loss but is actually very rare. Most cases result from either physical injury (eg, blunt head trauma, hand slap to ear); extreme barotrauma during airflight, scuba diving, etc; or vigorous Valsalva maneuver (eg, during weight lifting). Treatment may require middle ear exploration and window sealing with a tissue graft; however, this is seldom indicated without a clear-cut history of a precipitating traumatic event.

G. Cervical Vertigo

Position receptors located in the facets of the cervical spine are important physiologically in the coordination of head and eye movements. Cervical proprioceptive dysfunction is a common cause of vertigo triggered by neck movements. This disturbance often commences after neck injury, particularly hyperextension. An association also exists with degenerative cervical spine disease. Although symptoms vary, vertigo may be triggered by assuming a particular head position as opposed to moving to a new head position (the latter typical of labyrinthine dysfunction). Management consists of neck movement exercises to the extent permitted by orthopedic considerations.

Endo K et al: Cervical vertigo and dizziness after whiplash injury. Eur Spine J 2006;15:886.

H. Migrainous Vertigo

Episodic vertigo is frequently associated with a migraine type of headache. Most commonly, the vertigo is temporally related to the headache and lasts up to several hours. Some patients experience a positioning vertigo pattern. Occasionally, vertigo occurs independently of the head pain. The importance of recognizing this association is that antimigraine pharmacotherapy often relieves the vestibular symptoms.

Crevits L et al: Migraine-related vertigo: towards a distinctive entity. Clin Neurol Neurosurg 2005;107:82.

Neuhauser HK et al: Diagnostic criteria for migrainous vertigo. Acta Otolaryngol 2005;125:1247.

I. Superior Semicircular Canal Dehiscence

Deficiency in the bony covering of the superior semicircular canal may be associated with vertigo triggered by loud noise exposure and an apparent conductive hearing loss. Diagnosis is with coronal high-resolution CT scan. Symptoms can be improved in selected cases by operatively sealing the dehiscent canal.

Banerjee A et al: Superior canal dehiscence: review of a new condition. Clin Otolaryngol 2005;30:9.

Vertigo Syndromes Due to Central Lesions

Central nervous system causes of vertigo include brainstem vascular disease, arteriovenous malformations, tumor of the brainstem and cerebellum, multiple sclerosis, and vertebrobasilar migraine. Vertigo of central origin often becomes unremitting and disabling. The associated nystagmus is often nonfatigable, vertical rather than horizontal in orientation, without latency, and unsuppressed by visual fixation. Electronystagmography is useful in documenting these characteristics. There are commonly other signs of brainstem dysfunction (eg, cranial nerve palsies; motor, sensory, or cerebellar deficits in the limbs) or of increased intracranial pressure. Auditory function is generally spared. The underlying cause should be treated.

Baloh RW: Episodic vertigo: central nervous system causes. Curr Opin Neurol 2002;15:17.

Bruzzone MG et al: Neuroradiological features of vertigo. Neurol Sci 2004;25(Suppl 1):S20.

Treatment of the Patient with Vertigo

Few specific treatments for labyrinthine disorders have been designed to reverse a known pathogenic mechanism. In Meniere's disease, treatment is intended to lower endolymphatic pressure. A low-salt diet (< 2 g sodium daily), at times supplemented by diuretics, adequately controls symptoms in the great majority of patients. A typical diuretic regimen is hydrochlorothiazide, 50–100 mg orally daily. Other specific treatments are antibiotics as required and surgical repair of perilymphatic fistulas.

Symptomatic treatment is useful in the vertiginous patient to lessen the abnormal sensation and to alleviate vegetative symptoms such as nausea and vomiting. The most common drug classes used are the antihistamines, anticholinergics, and sedative-hypnotics. Ample evidence exists that vestibular suppressant medications adversely affect the process of central compensation following acute vestibular disease. For this reason, these drugs should be used only for brief periods. Generally, they are best administered to patients with prominent vegetative symptoms and are best tapered and halted when symptoms are resolved, usually within 1–2 weeks.

In acute severe vertigo, vestibular suppressants such as diazepam, 2.5–5 mg sublingually, orally, or intravenously, may abate an attack. Relief from nausea and

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vomiting usually requires an antiemetic delivered intramuscularly or by rectal suppository (eg, prochlorperazine, 10 mg intramuscularly, or 25 mg rectally every 6 hours). Less severe vertigo may often be successfully alleviated with antihistamines such as meclizine, 25 mg, or cyclizine or dimenhydrinate, 25–50 mg, orally every 6 hours. Scopolamine, administered in low dosage transdermally (0.5 mg/d), has proved beneficial to many patients with recurrent vertigo, although side effects (dry mouth, blurred vision, urinary obstruction) often limit its usefulness. Sometimes using half or even one-fourth of a patch may allow therapeutic effect without the usual adverse consequences. A combination of drugs sometimes helps when the response to one drug is disappointing.

Bed rest may reduce the severity of acute vertigo. Conversely, in chronic or recurrent vertigo, one of the most important therapies is exercise. Physical activity substantially enhances the central nervous system's ability to compensate for labyrinthine dysfunction and should be encouraged once nausea and vomiting have resolved. In general, the patient should be instructed to repeatedly perform maneuvers that provoke vertigo—up to the point of nausea or fatigue—in an effort to habituate them. Patients with vertigo and imbalance refractory to conventional therapy may benefit from a formal rehabilitation program under the guidance of a physical therapist. Substantial success has been reported in such patients through use of customized habituation protocols and specialized equipment, including tilt tables. A series of head maneuvers (theoretically intended to reposition free-floating otolithic particles) may help in the management of positioning vertigo. Such protocols have been shown to be at least as effective as vestibular habituation exercises, and they are less time-consuming.

For patients with recalcitrant vertigo or clusters of attacks, specialty referral may be useful. Prednisone has been used for clusters refractory to diuretics, low-salt diet, and vestibular suppressants.

Permanent therapy in medically refractory unilateral peripheral vestibular dysfunction is selective chemical destruction of the vestibular hair cell population by infusion of ototoxins transtympanically into the middle ear. Absorption into perilymph occurs via the round window. The most frequently used drug is gentamicin (80 mg/mL diluted 50:50 with bicarbonate), which is injected into the middle ear via a spinal needle. Results in patients with Meniere's syndrome have been impressive, with about 80–90% of patients relieved of severe episodic vertigo. An additional management option of endolymphatic hydrops (Meniere's disease) is a device that applies intermittent pressure pulses to the inner ear via a tympanostomy tube. Preliminary results for this minimally invasive technique have been promising. Surgical remedies are reserved for those who remain substantially disabled despite a prolonged and varied trial of medical therapy and exercises. Selective section of the vestibular portion of the eighth nerve brings relief of vertigo in over 90% of such patients. Surgical removal of the semicircular canals (labyrinthectomy) is also highly effective but is appropriate only for patients with little or no hearing in the involved ear.

Cohen HS: Disability and rehabilitation in the dizzy patient. Curr Opin Neurol 2006;1:49.

Longridge NS: Meta-analysis of intratympanic gentamicin. Otol Neurotol 2005;26:554.

Morales-Luckie E et al: Oral administration of prednisone to control refractory vertigo in Meniere's disease: a pilot study. Otol Neurotol 2005;26:1022.

Steenerson RL et al: Effectiveness of treatment techniques in 923 cases of benign paroxysmal positional vertigo. Laryngoscope 2005;115:226.

Straube A: Pharmacology of vertigo/nystagmus/oscillopsia. Curr Opin Neurol 2005;18:11.

Thomsen J et al: Local overpressure treatment reduces vestibular symptoms in patients with Meniere's disease: a clinical, randomized, multicenter, double-blind, placebo-controlled study. Otol Neurotol 2005;26:68.

Diseases of the Central Auditory & Vestibular Systems (Table 8-1)

Lesions of the eighth cranial nerve and central audiovestibular pathways produce neural hearing loss and vertigo. One characteristic of neural hearing loss is deterioration of speech discrimination out of proportion to the decrease in pure tone thresholds. Another is auditory adaptation, wherein a steady tone appears to the

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listener to decay and eventually disappear. Auditory evoked responses are useful in distinguishing cochlear from neural losses and may give insight into the site of lesion within the central pathways.

Table 8-1. Common vestibular disorders: Differential diagnosis based on classic presentations.

Duration of Typical Vertiginous Episodes Auditory Symptoms Present Auditory Symptoms Absent
Seconds Perilymphatic fistula Positioning vertigo (cupulolithiasis), vertebrobasilar insufficiency, cervical vertigo
Hours Endolymphatic hydrops (Meniere's syndrome, syphilis) Recurrent vestibulopathy, vestibular migraine
Days Labyrinthitis, labyrinthine concussion Vestibular neuronitis
Months Acoustic neuroma, ototoxicity Multiple sclerosis, cerebellar degeneration

The evaluation of central audiovestibular disorders usually requires imaging of the internal auditory canal, cerebellopontine angle, and brain with enhanced MRI.

Jackler RK et al: Neurotology, 2nd ed. Mosby, 2004.

1. Vestibular Schwannoma (Acoustic Neuroma)

Eighth nerve schwannomas are among the most common of intracranial tumors. Most are unilateral, but about 5% are associated with the hereditary syndrome, neurofibromatosis type 2, in which bilateral eighth nerve tumors may be accompanied by meningiomas and other intracranial and spinal tumors. Although recent concern over the role of cell phones in the origin of these tumors has appeared in the popular press, the evidence of an association is in doubt. These benign lesions arise within the internal auditory canal and gradually grow to involve the cerebellopontine angle, eventually compressing the pons and resulting in hydrocephalus. Their typical auditory symptoms are unilateral hearing loss with a deterioration of speech discrimination exceeding that predicted by the degree of pure tone loss. Nonclassic presentations, such as sudden unilateral hearing loss, are fairly common. Any individual with a unilateral or asymmetric sensorineural hearing loss should be evaluated for an intracranial mass lesion. Vestibular dysfunction more often takes the form of continuous dysequilibrium than episodic vertigo. Other lesions of the cerebellopontine angle such as meningioma and epidermoids may have similar audiovestibular manifestations. Diagnosis is made by enhanced MRI, although auditory evoked responses may have a role in screening. Microsurgical excision is often indicated, although small tumors in older persons may be managed with stereotactic radiotherapy or simply monitored with serial imaging studies.

Betchen SA et al: Long-term hearing preservation after surgery for vestibular schwannoma. J Neurosurg 2005;102:6.

Lunsford LD et al: Radiosurgery of vestibular schwannomas: summary of experience in 829 cases. J Neurosurg 2005;102 Suppl:195.

Neff BA et al: Current concepts in the evaluation and treatment of neurofibromatosis type II. Otolaryngol Clin North Am 2005;38:671.

Schoemaker MJ et al: Mobile phone use and risk of acoustic neuroma: results of the Interphone case-control study in five North European countries. Br J Cancer 2005;93:842.

Yoshimoto Y: Systematic review of the natural history of vestibular schwannoma. J Neurosurg 2005;103:59.

2. Vascular Compromise

Vertebrobasilar insufficiency is a common cause of vertigo in the elderly. It is often triggered by changes in posture or extension of the neck. Reduced flow in the vertebrobasilar system may be demonstrated noninvasively through magnetic resonance angiography. Empiric treatment is with vasodilators and aspirin.

Vascular loops that impinge upon the brainstem root entry zone of cranial nerves have been shown to cause dysfunction. Widely recognized examples are hemifacial spasm and tic douloureux. It has been suggested that hearing loss, tinnitus, and disabling positioning vertigo may result from a vascular loop abutting the eighth nerve.

Sauvaget E et al: Vertebrobasilar occlusive disorders presenting as sudden sensorineural hearing loss. Laryngoscope 2004;114: 327.

3. Multiple Sclerosis

Patients with multiple sclerosis may suffer from episodic vertigo and chronic imbalance. Hearing loss in this disease is most commonly unilateral and of rapid onset. Spontaneous recovery may occur.

Frohman EM et al: Benign paroxysmal positioning vertigo in multiple sclerosis: diagnosis, pathophysiology and therapeutic techniques. Mult Scler 2003;9:250.

Otologic Manifestations of Aids

The otologic manifestations of AIDS are protean. The pinna and external auditory canal may be affected by Kaposi's sarcoma as well as persistent and potentially invasive fungal infections, particularly due to Aspergillus fumigatus. The most common middle ear manifestation of AIDS is serous otitis media due to auditory tube dysfunction arising from adenoidal hypertrophy (HIV lymphadenopathy), recurrent mucosal viral infections, or an obstructing nasopharyngeal tumor (eg, lymphoma). For middle ear effusions, ventilating tubes are seldom helpful and may trigger profuse watery otorrhea. Acute otitis media is usually caused by the typical bacterial organisms that occur in nonimmunocompromised patients, although Pneumocystis jiroveci (formerly Pneumocystis carinii) otitis has been reported. Sensorineural hearing loss is common and in some cases appears to result from viral central nervous system infection. In cases of progressive hearing loss, it is important to evaluate for cryptococcal meningitis and syphilis. Acute facial paralysis due to herpes zoster infection (Ramsay Hunt's syndrome) is quite common and follows a clinical course similar to that in nonimmunocompromised patients. Treatment is primarily with high-dose acyclovir (see Chapters 6 and 32). Corticosteroids may also be effective.

Gurney TA: Otolaryngologic manifestations of human immunodeficiency virus infection. Otolaryngol Clin North Am 2003; 36:607.

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Diseases of the Nose & Paranasal Sinuses

Infections of the Nose & Paranasal Sinuses

1. Viral Rhinitis (Common Cold)

The nonspecific symptoms of the ubiquitous common cold are present in the early phases of many diseases that affect the upper aerodigestive tract. Because there are numerous serologic types of rhinoviruses, adenoviruses, and other viruses, patients remain susceptible throughout life. Headache, nasal congestion, watery rhinorrhea, sneezing, and a scratchy throat accompanied by general malaise are typical in viral infections. Nasal examination usually shows reddened, edematous mucosa and a watery discharge. The presence of purulent nasal discharge suggests bacterial infection.

There is no curative treatment for a cold. There is a common misperception among patients that antibiotics are helpful. Supportive measures such as decongestants (pseudoephedrine, 30–60 mg every 4–6 hours or 120 mg twice daily) may provide some relief of rhinorrhea and nasal obstruction.

Nasal sprays such as oxymetazoline or phenylephrine are rapidly effective. They should not be used for more than a few days at a time, since chronic use leads to a rebound congestion that is often worse than the original symptoms. This chronic nasal stuffiness is known as rhinitis medicamentosa. Treatment requires complete cessation of the sprays. This triggers a period of severe nasal congestion that usually lasts 1–2 weeks. Topical intranasal corticosteroids (flunisolide, two sprays in each nostril twice daily) or a short tapering course of oral prednisone may help during the process of withdrawal.

Other than transient middle ear effusion, complications of viral rhinitis are unusual. Secondary bacterial infection may occur and is suggested by persistence of symptoms beyond a week accompanied both by purulent green or yellow nasal secretions and unilateral facial or tooth pain. The most common pathogens are the same as those responsible for acute otitis media, ie, S pneumoniae, other streptococci, H influenzae, S aureus, and Moraxella catarrhalis. (See Acute Sinusitis, below.)

Eccles R: Understanding the symptoms of the common cold and influenza. Lancet Infect Dis 2005;5:718.

Hirschmann JV: Antibiotics for common respiratory tract infections in adults. Arch Intern Med 2002;162:256.

Steinman MA et al: Predictors of broad-spectrum antibiotic prescribing for acute respiratory tract infections in adult primary care. JAMA 2003;289:719.

Wright ED et al: Infectious adult rhinosinusitis: etiology, diagnosis, and management principles. J Otolaryngol 2005;34 (Suppl 1): S7.

2. Acute Sinusitis

Essentials of Diagnosis

  • Pain is usually unilateral over the maxillary sinus or is toothache-like.

  • Symptoms usually last for more than 1 week but less than 4 weeks.

  • Change of secretions from mucoid to purulent green or yellow.

  • Occasional visible swelling or erythema over a sinus.

  • Postnasal drainage, headache, and cough may also be present.

  • Diseases that swell the nasal mucous membrane, such as viral or allergic rhinitis, are usually the underlying cause.

General Considerations

Acute sinus infections are uncommon compared with viral rhinitis, but they still affect over 14% of the population, accounting for over 2 billion dollars in health care expenditures for sinusitis annually. Because sinusitis usually has followed an acute respiratory infection and because media advertisements often use the term “sinusitis” when “rhinitis” would be more accurate, it is understandable that patients and clinicians alike sometimes confuse these entities. Sinusitis is suggested when symptoms have persevered for more than a week and pain is reported unilaterally, either as toothache or as pain over the maxillary sinus. Objective signs include a change of secretions from watery or mucoid to purulent green or yellow, or (occasionally) visible swelling or erythema over a sinus.

Sinusitis usually is a result of impaired mucociliary clearance and obstruction of the osteomeatal complex. Diseases that swell the nasal mucous membrane, such as viral or allergic rhinitis, are usually the underlying cause. Edematous mucosa causes obstruction of the sinus drainage tract, resulting in the accumulation of mucous secretion in the sinus cavity that becomes secondarily infected by bacteria. The typical pathogens of bacterial sinusitis are the same as those that cause acute otitis media: S pneumoniae, other streptococci, H influenzae, and, less commonly, S aureus and M catarrhalis. It should be kept in mind that about 25% of healthy asymptomatic individuals may, if sinus aspirates are cultured, harbor such bacteria as well.

Clinical Findings

A. Symptoms and Signs

Because the maxillary sinus is the largest of the paranasal sinuses and its ostia into the nose is superiorly

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placed, thereby failing to take advantage of gravity, it is the most commonly affected sinus. Pain and pressure over the cheek are the usual symptoms. Pain may refer to the upper incisor and canine teeth via branches of the trigeminal nerve, which traverse the floor of the sinus. It is not uncommon for maxillary sinusitis to result from dental infection, and teeth that are tender should be carefully examined for signs of abscess. Discolored nasal discharge and poor response to decongestants may also suggest sinusitis. Other possible causes for facial pain, such as trigeminal neuralgia and optic neuritis, should be kept in mind as well.

Acute ethmoiditis in adults is usually accompanied by maxillary sinusitis. In such cases, the symptoms of maxillary sinusitis generally predominate. Ethmoidal infection presents with pain and pressure over the high lateral wall of the nose that may radiate to the orbit. Periorbital cellulitis may be present.

Sphenoid sinusitis is usually seen in the setting of pansinusitis. The patient may complain of a headache “in the middle of the head” and often points to the vertex. Sixth nerve palsy may occur as the abducens nerve courses just lateral to the sinus.

Acute frontal sinusitis usually causes pain and tenderness of the forehead. This is most easily elicited by palpation of the orbital roof just below the medial end of the eyebrow. Palpation here is more accurate than percussion of the supraorbital area or forehead.

B. Imaging

It is usually possible to make the diagnosis of sinusitis on clinical grounds alone. Although more sensitive than clinical examination, routine radiographs are not cost-effective and are not recommended by the Agency for Health Care Policy and Research. However, they may be helpful when clinically based criteria are difficult to evaluate. The hallmarks of acute sinusitis radiologically are soft tissue density without bone destruction. An air-fluid level may also be seen.

Limited coronal CT scans have replaced conventional sinus films. CT is no more expensive, is more sensitive to both inflammatory changes and bone destruction (which would raise the possibility of an underlying tumor), and identifying anatomic blockage of the ostiomeatal complex may also help guide endoscopic sinus surgery in recurrent or chronic sinusitis. In critically ill intubated patients, where the prevalence of nosocomial sinusitis is as high as 40%, CT identification and subsequent antibiotic treatment appear to reduce the incidence of bronchopneumonia. Occasionally, a CT scan may be indicated to demonstrate that a patient with midface pain does not have sinusitis.

While reasonably sensitive, CT scans are not specific. Sinus abnormalities can be seen in the majority of patients with an upper respiratory infection, while only 2% develop bacterial sinusitis. Thus, sinusitis is a clinical diagnosis for which CT may be helpful in confirming, denying, or monitoring.

If malignancy is suspected, MRI with gadolinium should be ordered instead of CT. MRI will distinguish tumor from inflammation and inspissated mucus far better than CT, as well as better delineating tumor extent with respect to adjacent structures such as the orbit, skull base, and palate. Bone destruction can be demonstrated as well by MRI as by CT.

Treatment

As two-thirds of untreated patients will improve symptomatically within 2 weeks, appropriate criteria for the use of antibiotics are symptoms lasting more than 10–14 days or severe symptoms, including fever, facial pain, and periorbital swelling. Administration of antibiotics does, however, reduce by 50% the incidence of clinical failure and, coupled with clinical criteria-based diagnosis, represents the most cost-effective treatment strategy. Symptoms may be improved with oral or nasal decongestants (or both)—eg, oral pseudoephedrine, 30–120 mg per dose, up to 240 mg/d; nasal oxymetazoline, 0.05%, or xylometazoline, 0.05–0.1%, one or two sprays in each nostril every 6–8 hours for up to 3 days.

Double-blinded studies exist to support any of the following antibiotic choices, with the choice generally based on predicted efficacy, cost, and side effects:

  • Amoxicillin (500 mg orally three times a day), possibly with clavulanate (125 mg three times a day)

  • Trimethoprim-sulfamethoxazole (4 mg/kg TMP and 20 mg/kg SMZ twice daily; available as tablets with 80 or 160 mg TMP and 160 or 800 mg SMZ)

  • Cephalexin (250–500 mg orally four times a day)

  • Cefuroxime (250 mg orally twice daily)

  • Cefaclor (250 mg orally three times a day)

  • Cefixime (400 mg orally daily)

  • Quinolones, such as ciprofloxacin (500 mg twice daily), levofloxacin (500 mg once daily), moxifloxacin (400 mg once daily), and sparfloxacin (200 mg once daily after an initial dose of 400 mg)

  • Macrolides, such as azithromycin (500 mg once daily, possibly for only 3 days) or clarithromycin (500 mg orally twice daily, for 14 days)

Usually, amoxicillin or TMP-SMZ is adequate and most cost-effective and covers the common pathogens discussed earlier. Treatment is usually for 10 days (or as stated above), although longer courses are sometimes required to prevent relapses. Recurrent sinusitis or sinusitis that does not appear to respond clinically warrants evaluation by a specialist. Selection of antibiotics is usually empiric, but if symptoms persist, obtaining a culture endoscopically or via maxillary sinus puncture may help narrow the choice based on culture and sensitivity tests. Resistance by H influenzae and S pneumoniae is a public health concern. Culture may

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also be helpful in nosocomial sinusitis, where the bacterial spectrum may be less typical and the potential complications greater.

Failure of sinusitis to resolve after an adequate course of oral antibiotics may necessitate hospital admission for intravenous antibiotics and possible surgical drainage. Frontal sinusitis that does not promptly respond to outpatient care should be managed aggressively because the posterior sinus wall is adjacent to the dura and because undertreated infection may lead to intracranial extension. If intravenous antibiotics fail to ameliorate symptoms, it may be necessary to surgically drill a small opening into the floor of the frontal sinus to drain and irrigate the sinus. Persistent maxillary empyema may be cultured and relieved with a needle inserted through the lateral wall of the nose or anterior wall of the antrum through the gingivobuccal sulcus.

Complications

Local complications of sinusitis include osteomyelitis and mucocele. Mucoceles, a consequence of longstanding ductal obstruction, are more common in the supraorbital ethmoids and frontal sinuses and may become secondarily infected. They appear radiologically as a smoothly expanded sinus filled with homogeneous soft tissue density. Treatment is surgical, requiring either drainage of the mucocele intranasally or its complete excision with fat ablation of the sinus cavity.

Osteomyelitis requires prolonged antibiotics as well as removal of necrotic bone. The frontal sinus is most commonly affected, with bone involvement suggested by a tender puffy swelling of the forehead. Following treatment, secondary cosmetic reconstructive procedures may be necessary.

Intracranial complications of sinusitis occur either through hematogenous spread, as in cavernous sinus thrombosis and meningitis, or by direct extension, as in epidural and intraparenchymal brain abscesses. Fortunately, they are rare today. Cavernous sinus thrombosis is heralded by ophthalmoplegia, chemosis, and visual loss. Frontal epidural abscess is usually quiescent. It may be detected on CT scan, a study recommended in all cases of atypical or complicated sinusitis.

It should always be kept in mind that paranasal sinus cancer is in the differential diagnosis of sinusitis. The presence of bone destruction radiologically, cranial neuropathies (especially V2), persistent pain, epistaxis, or a prolonged clinical course should raise the suspicion of possible cancer.

Merenstein D et al: Are antibiotics beneficial for patients with sinusitis complaints? A randomized double-blind clinical trial. J Fam Pract 2005;54:144.

Piccirillo JF: Clinical practice. Acute bacterial sinusitis. N Engl J Med 2004;351:902.

Scheid EC et al: Acute bacterial rhinosinusitis in adults: part I. Evaluation. Am Fam Physician 2004;70:1685.

Scheid EC et al: Acute bacterial rhinosinusitis in adults: part II. Treatment. Am Fam Physician 2004;70:1697.

Slavin RG et al; American Academy of Allergy, Asthma and Immunology; American College of Allergy, Asthma and Immunology; Joint Council of Allergy, Asthma and Immunology: The diagnosis and management of sinusitis: a practice parameter update. J Allergy Clin Immunol 2005; 116(6 Suppl):S13.

Werning JW et al: Physician specialty is associated with differences in the evaluation and management of acute bacterial rhinosinusitis. Arch Otolaryngol Head Neck Surg 2002; 128:123.

Williams JW Jr et al: Antibiotics for acute maxillary sinusitis. Cochrane Database Syst Rev 2003;(2):CD00024.

3. Nasal Vestibulitis

Inflammation of the nasal vestibule commonly results from folliculitis of the hairs that line this orifice. Systemic antibiotics effective against S aureus (such as dicloxacillin, 250 mg orally four times daily for 7–10 days) are indicated. Topical mupirocin (applied two or three times daily) may be a helpful addition. If recurrent, the addition of rifampin (10 mg/kg orally twice daily for the last 4 days of treatment) may eliminate the S aureus carrier state. If a furuncle exists, it should be incised and drained, preferably intranasally. Adequate treatment of these infections is important to prevent retrograde spread of infection through valveless veins into the cavernous sinus and intracranial structures.

4. Rhinocerebral Mucormycosis

Although mucormycosis is rare, any clinician seeing patients in a primary care setting must be aware of its presenting signs and symptoms. The fungus (Mucor, Absidia, Rhizopus) spreads rapidly through vascular channels and may be lethal if not detected early. Patients with mucormycosis almost invariably have an underlying disease, often diabetes mellitus or end-stage renal disease. It also occurs following bone marrow transplantation, in patients with lymphoma, in patients who are immunosuppressed for other reasons, and in patients receiving deferoxamine (a metal chelator). Occasional cases have been reported in patients with AIDS, although Aspergillus is more common in this setting. The initial symptoms may be similar to those of bacterial sinusitis, although facial pain is often more severe. Examination of the nasal mucosa is likely to show black, necrotic eschar adherent to the inferior turbinate, although this may not be present in early stages. Cranial neuropathies and black necrotic skin overlying the ethmoid sinuses are advanced signs. Early diagnosis requires suspicion of the disease and nasal or sinus biopsy, which reveals broad nonseptate hyphae within tissues. Because CT or MRI may initially show only soft tissue changes, intervention should be based on the clinical setting and not on radiologic

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demonstration of bony destruction or intracranial changes.

Mucormycosis represents a medical and surgical emergency. Once recognized, prompt wide surgical debridement and amphotericin B by intravenous infusion are indicated. Lipid-based amphotericin B may be used in patients who have renal insufficiency or in those in whom it develops secondary to nephrotoxic doses of nonlipid amphotericin. When there appears to be sufficiently little orbital involvement that orbital preservation is a realistic therapeutic objective, conservative debridement of the orbit may be supplemented with adjunctive amphotericin B irrigation. There is evidence that suggests that iron chelator therapy may also be a useful adjunct. Close management of the underlying disease is also of great importance. Even with early diagnosis and immediate appropriate intervention, the prognosis is guarded. In diabetics, the mortality rate is about 20%; in patients with renal failure, it is over 50%; in AIDS, it is close to 100%.

Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 22–1999. A 68-year-old woman with multiple myeloma, diabetes mellitus, and an inflamed eye. N Engl J Med 1999;41:265.

Mondy KE et al: Rhinocerebral mucormycosis in the era of lipid-based amphotericin B: case report and literature review. Pharmacotherapy 2002;22:519.

O'Neill BM et al: Disseminated rhinocerebral mucormycosis: a case report and review of the literature. J Oral Maxillofac Surg 2006;64:326.

Spellberg B et al: Novel perspectives on mucormycosis: pathophysiology, presentation, and management. Clin Microbiol Rev 2005;18:556.

Allergic Rhinitis

The symptoms of “hay fever” are similar to those of viral rhinitis but are usually persistent and show seasonal variation. Nasal symptoms are often accompanied by eye irritation, which causes pruritus, erythema, and excessive tearing. Numerous allergens may cause these symptoms: pollens are most common in the spring, grasses in the summer, and ragweed in the fall. Dust and household mites may produce year-round symptoms.

On physical examination, the mucosa of the turbinates is usually pale or violaceous because of venous engorgement. This is in contrast to the erythema of viral rhinitis. Nasal polyps, which are yellowish boggy masses of hypertrophic mucosa, may be seen.

Treatment of allergic and perennial rhinitis has improved in recent years. Numerous over-the-counter antihistamines, such as brompheniramine or chlorpheniramine (4 mg orally every 6–8 hours, or 8–12 mg orally every 8–12 hours as a sustained-release tablet) and clemastine (1.34–2.68 mg orally twice daily) offer the benefit of reduced cost though usually associated with higher rates of drowsiness compared with the newer prescription antihistamines. These oral H1-receptor antagonists include cetirizine (10 mg orally once daily), fexofenadine (60 mg orally twice daily or 120 mg once daily), and loratadine (10 mg orally once daily). Fexofenadine appears to be nonsedating; the other two minimally sedating. Also shown to be effective in randomized trials are ebastine (10–20 mg orally once daily) and misolastine (10 mg once daily). Two H1-receptor antagonist antihistamine nasal sprays have also been shown to be effective in randomized trials: levocabastine (0.2 mg twice daily) and azelastine (two sprays per nostril, 1.1 mg/d).

Intranasal corticosteroid sprays are a mainstay of treatment of allergic rhinitis. Evidence-based literature reviews show that these are more effective—and frequently less expensive—than nonsedating antihistamines. Patients should be reminded that there may be a delay in onset of relief of 1–2 weeks. Corticosteroid sprays may also shrink nasal polyps, thereby providing an improved nasal airway and delaying or eliminating the indications for endoscopic sinus surgery. Available preparations include beclomethasone (42 mcg/spray twice daily each nostril), flunisolide (25 mcg/spray twice daily each nostril), mometasone furoate (200 mcg once daily per nostril), and fluticasone propionate (200 mcg once daily per nostril). The latter two synthetic corticosteroids appear to have higher topical potencies and lipid solubility and reduced systemic bioavailability, suggesting possible practical advantages.

In addition to intranasal corticosteroid sprays and antihistamines, including H1-receptor antagonists, literature supports the use of antileukotriene medications such as montelukast (10 mg/d orally) alone or with cetirizine (10 mg/d orally) or with loratadine (10 mg/d orally). There are proinflammatory effects of cysteinyl leukotrienes in upper and lower airway disease, including asthma, allergic rhinitis, and hyperplastic sinusitis and polyposis. Improved nasal rhinorrhea, sneezing, and congestion are seen with the use of leukotriene receptor antagonists, often in conjunction with antihistamines.

Maintaining an allergen-free environment by covering pillows and mattresses with plastic covers, substituting synthetic materials (foam mattress, acrylics) for animal products (wool, horsehair), and removing dust-collecting household fixtures (carpets, drapes, bedspreads, wicker) is worth the attempt to help more troubled patients. Air purifiers and dust filters (such as Bionaire models) may also aid in maintaining an allergen-free environment. When symptoms are extremely bothersome, a search for offending allergens may prove helpful. This can either be done by skin testing or by serum RAST testing. Desensitization by gradually increasing subdermal exposure to identified allergens may be tried in selected patients, with variable results.

Adjunctive options include intranasal anticholinergic agents such as ipratropium bromide 0.03% sprays (42 mcg per nostril three times daily) when rhinorrhea is a major symptom and intranasal cromolyn spray prior to the onset of seasonal symptoms.

For more information on allergic rhinitis, see Chapter 19.

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Gendo K et al: Evidence-based diagnostic strategies for evaluating suspected allergic rhinitis. Ann Intern Med 2004;140:278.

Haberal I et al: The role of leukotrienes in nasal allergy. Otolaryngol Head Neck Surg 2003;129:274.

Nielsen LP et al: Comparison of intranasal corticosteroids and antihistamines in allergic rhinitis: a review of randomized, controlled trials. Am J Respir Med 2003;2:55.

Portnoy JM et al: Evidence-based strategies for treatment of allergic rhinitis. Curr Allergy Asthma Rep 2004;4:439.

Prenner ME et al: Allergic rhinitis: treatment based on patient profiles. Am J Med 2006;119:230.

Simons FE: Advances in H1-antihistamines. N Engl J Med 2004; 351:2203.

Wilson DR et al: Sublingual immunotherapy for allergic rhinitis. Cochrane Database Syst Rev 2003;(2):CD002893.

Olfactory Dysfunction

The physiology of olfaction is less well understood than that of the other special senses. The taste of foods is strongly affected by our sense of smell, and studies have shown a moderate correlation between taste discrimination ability and odor discrimination ability. In the past few years, discovery of the family of odor-receptor genes as well as inositol phosphate and cyclic nucleotide signaling pathways has led to a molecular basis of olfactory reception. Clinically, odorant molecules traverse the nasal vault to reach the cribriform area and become soluble in the mucus overlying the exposed dendrites of receptor cells. Anatomic blockage of the nares is the most common cause of olfactory dysfunction (hyposmia or anosmia). Polyps, septal deformities, and nasal tumors may prevent air from reaching the area of the cribriform plate high in the nose where these receptors are located. Transient olfactory dysfunction often accompanies the common cold, nasal allergies, and perennial rhinitis. About 20% of olfactory dysfunction is idiopathic, although it often follows a viral illness. Some have suggested administering large doses of vitamin A and zinc to such patients, although little evidence supports their use. Central nervous system neoplasms, especially those that involve the olfactory groove or temporal lobe, may affect olfaction. Head trauma accounts for less than 5% of cases of hyposmia. Absent, diminished, or distorted smell or taste has been reported in a wide variety of endocrine, nutritional, and nervous disorders. In particular, olfactory dysfunction in Parkinson's disease and Alzheimer's disease has been the subject of recent research. A great many medications have also been implicated.

Evaluation of olfactory dysfunction should include a thorough history of systemic illnesses and medication use as well as a physical examination focusing on the nose and nervous system. Most clinical offices are not set up to test olfaction, but such tests may at times be worthwhile if only to assess whether a patient possesses any sense of smell at all. Odor identification and discrimination can be tested using standardized choices (see references). Odor threshold can be tested using increasing concentrations of various materials. In permanent hyposmia, counseling should be offered about seasoning foods with spices (eg, pepper) that stimulate the trigeminal as well as olfactory chemoreceptors and about safety issues such as the use of smoke alarms and electric rather than gas home appliances.

Hawkes C: Olfaction in neurodegenerative disorder. Mov Disord 2003;18:364.

Kovacs T: Mechanisms of olfactory dysfunction in aging and neurodegenerative disorders. Ageing Res Rev 2004;3:215.

Moberg PJ et al: Scent of a disorder: olfactory functioning in schizophrenia. Curr Psychiatry Rep 2003;5:311.

Murphy C et al: Prevalence of olfactory impairment in older adults. JAMA 2002;288:2307.

Wrobel BB et al: Smell and taste disorders. Facial Plast Surg Clin North Am 2004;12:459i.

Epistaxis

Essentials of Diagnosis

  • Bleeding from the anterior nasal cavity is by far the most common type of epistaxis encountered.

  • Most cases may be successfully treated by direct pressure on the bleeding site. When this is inadequate, various nasal tamponade methods are usually effective.

Predisposing factors include nasal trauma (nose picking, foreign bodies, forceful nose blowing), rhinitis, drying of the nasal mucosa from low humidity, deviation of the nasal septum, alcohol use, and antiplatelet medications. Most cases of anterior epistaxis may be successfully treated by direct pressure on the bleeding site. The nasal alae should be firmly compressed for at least 10 minutes. Venous pressure is reduced in the sitting position, and leaning forward lessens the swallowing of blood. Short-acting topical nasal decongestants (eg, phenylephrine, 0.125–1% solution, one or two sprays), which act as vasoconstrictors, may also be helpful. When the bleeding does not readily subside, the nose should be examined, using good illumination and suction, in an attempt to locate the bleeding site. Topical 4% cocaine applied either as a spray or on a cotton strip serves both as an anesthetic and as a vasoconstricting agent. If cocaine is unavailable, a topical decongestant (eg, oxymetazoline) and a topical anesthetic (eg, tetracaine) provide equivalent results. When visible, the bleeding site may be cauterized with silver nitrate, diathermy, or electrocautery. A supplemental patch of Surgicel or Gelfoam may be helpful.

Occasionally, a site of bleeding may be inaccessible to direct control, or attempts at direct control may be unsuccessful. In such cases there are a number of alternatives. When the site of bleeding is anterior, a hemostatic

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sealant, pneumatic nasal tamponade, or anterior packing may suffice. There are a number of ways to do this, such as with several feet of lubricated iodoform packing systematically placed in the floor of the nose and then the vault of the nose, or with various manufactured products designed for nasal tamponade.

About 5% of nasal bleeding originates in the posterior nasal cavity. If an anteriorly placed pneumatic nasal tamponade is unsuccessful, it may be necessary to place a pack to occlude the choana before placing a pack anteriorly. Because this is uncomfortable and because it may require oxygen supplementation to prevent hypoxia, hospitalization for several days is indicated. Opioid analgesics are needed to reduce the considerable discomfort and elevated blood pressure caused by a posterior pack. Ligation of the nasal arterial supply (internal maxillary artery and ethmoid arteries) is an alternative to posterior nasal packing, as is endovascular embolization of the internal maxillary artery. This is certainly necessary when packing fails to control life-threatening hemorrhage. On rare occasions, ligation of the external carotid artery may be necessary.

After control of epistaxis, the patient is advised to avoid vigorous exercise for several days. Avoidance of hot or spicy foods and tobacco is also advisable, as they may cause vasodilation. Avoiding nasal trauma, including nose picking, is an obvious necessity. Lubrication with petroleum jelly or bacitracin ointment and increased home humidity may also be useful ancillary measures.

It is important in all patients with epistaxis to consider underlying causes of the bleeding. Laboratory assessment of bleeding parameters may be indicated, especially in recurrent cases. Other causes of recurrent epistaxis, such as hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome), should also be considered. Similarly, once the acute episode has passed, careful examination of the nose and paranasal sinuses to rule out neoplasia is wise.

Patients presenting with epistaxis often have higher blood pressures than control patients. Continued management of patients with epistaxis should therefore include follow-up investigation of possible hypertension.

Herkner H et al: Active epistaxis at ED presentation is associated with arterial hypertension. Am J Emerg Med 2002;20:92.

Jones GL et al: The value of coagulation profiles in epistaxis management. Int J Clin Pract 2003;57:577.

Klotz DA et al: Surgical management of posterior epistaxis: a changing paradigm. Laryngoscope 2002;112:1577.

Mathiasen RA et al: Prospective, randomized, controlled clinical trial of a novel matrix hemostatic sealant in patients with acute anterior epistaxis. Laryngoscope 2005;115:899.

Singer AJ et al: Comparison of nasal tampons for the treatment of epistaxis in the emergency department: a randomized controlled trial. Ann Emerg Med 2005;45:134.

Nasal Trauma

The nasal pyramid is the most frequently fractured bone in the body. Fracture is suggested by crepitance or palpably mobile bony segments. Epistaxis and pain are common, as are soft tissue hematomas (“black eye”). It is important to make certain that there is no palpable step-off of the infraorbital rim, which would indicate the presence of a zygomatic complex fracture. Radiologic confirmation may at times be helpful but is not necessary in uncomplicated nasal fractures. It is also important to assess for possible concomitant additional facial, pulmonary, or intracranial injuries when the circumstances of injury are suggestive, as in the case of automobile and motorcycle accidents.

Treatment is aimed at maintaining long-term nasal airway patency and nasal aesthetics. Closed reduction, using topical 4% cocaine and locally injected 1% lidocaine, should be attempted within 1 week of injury. In the presence of marked nasal swelling, it is best to wait several days for the edema to subside before undertaking reduction. Persistent functional or cosmetic defects may be repaired by delayed reconstructive nasal surgery.

Intranasal examination should be performed in all cases to rule out septal hematoma, which appears as a widening of the anterior septum, visible just posterior to the columella. The septal cartilage receives its only nutrition from its closely adherent mucoperichondrium. An untreated subperichondrial hematoma will result in loss of the nasal cartilage with resultant saddlenose deformity. Septal hematomas may become infected, with S aureus the predominant organism. Treatment consists of incision and drainage via an intranasal septal mucosal incision. It is important to be sure that both sides of the septal cartilage are adequately drained. A small Penrose drain sutured in place is helpful. Antibiotics should be given and the drained fluid sent for culture.

Alvi A et al: Facial fractures and concomitant injuries in trauma patients. Laryngoscope 2003;113:102.

Green KM: Reduction of nasal fractures under local anaesthetic. Rhinology 2001;39:43.

Gur E et al: Walk-through injuries: glass door facial injuries. Ann Plast Surg 2001;46:613.

Kraus JF et al: Facial trauma and the risk of intracranial injury in motorcycle riders. Ann Emerg Med 2003;41:18.

Ridder GJ et al: Technique and timing for closed reduction of isolated nasal fractures: a retrospective study. Ear Nose Throat J 2002;81:49.

Tumors & Granulomatous Disease

1. Benign Nasal Tumors

Nasal Polyps

Nasal polyps are pale, edematous, mucosally covered masses commonly seen in patients with allergic rhinitis, but compelling evidence argues against a purely allergic

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pathogenesis. They may result in chronic nasal obstruction and a diminished sense of smell. In patients with nasal polyps and a history of asthma, aspirin should be avoided as it may precipitate a severe episode of bronchospasm. The presence of polyps in children should suggest the possibility of cystic fibrosis.

Initial treatment with topical nasal corticosteroids (see Allergic Rhinitis section for specific drugs) for 1–3 months is usually successful for small polyps and may reduce the need for operation. A short course of oral corticosteroids (eg, prednisone, 6-day course using 21 5-mg tablets: 30 mg on day 1 and tapering by 5 mg each day) may also be of benefit. When medical management is unsuccessful, polyps should be removed surgically. In healthy persons, this is a minor outpatient procedure. In recurrent cases or when surgery itself is associated with increased risk (such as in patients with asthma), a more complete procedure, such as ethmoidectomy, may be advisable. In recurrent polyposis, it may be necessary to remove polyps from the ethmoid, sphenoid, and maxillary sinuses to provide longer-lasting relief.

Alobid I et al: Nasal polyposis and its impact on quality of life: comparison between the effects of medical and surgical treatments. Allergy 2005;60:452.

Badia L et al: Topical corticosteroids in nasal polyposis. Drugs 2001;61:573.

Grigoreas C et al: Nasal polyps in patients with rhinitis and asthma. Allergy Asthma Proc 2002;23:169.

Slavin RG: Nasal polyps and sinusitis. Clin Allergy Immunol 2002;16:295.

Stjarne P et al: A randomized controlled trial of mometasone furoate nasal spray for the treatment of nasal polyposis. Arch Otolaryngol Head Neck Surg 2006;132:179.

Inverted Papilloma

Inverted papillomas are benign tumors that usually arise in the common wall between the nose and maxillary sinus. They present with unilateral nasal obstruction and occasionally hemorrhage. Because SCC is seen in about 10% of inverted or schneiderian papillomas, complete excision is strongly recommended. This usually requires a medial maxillectomy, but in selected cases an endoscopic approach may be possible. Because recurrence rates for inverted papilloma are reported to be as high as 20%, subsequent clinical and radiologic follow-up is imperative. All excised tissue (not just a portion) should be carefully reviewed by the pathologist to be sure no carcinoma is present.

Kasa S et al: Endoscopic resection of inverted papilloma: University of Miami experience. Am J Rhinol 2003;17:185.

Lawson W et al: Treatment outcomes in the management of inverted papilloma: an analysis of 160 cases. Laryngoscope 2003;113:1548.

Tomenzoli D et al: Different endoscopic surgical strategies in the management of inverted papilloma of the sinonasal tract: experience with 47 patients. Laryngoscope 2004;114:193.

Juvenile Angiofibroma

These highly vascular tumors arise in the nasopharynx, typically in adolescent males. Initially, they cause nasal obstruction and hemorrhage. Any adolescent male with recurrent epistaxis should be evaluated for an angiofibroma. Although benign, these tumors expand locally from the sphenopalatine foramen to the pterygopalatine fossa at the pterygoid canal and extend to the pterygoid base, the greater wing of the sphenoid, the nasal cavity, and the paranasal sinuses. They may involve the skull base, usually extradurally, and extend into the superior clivus. Treatment consists of preoperative embolization followed by surgical excision via an approach appropriate for the tumor extent. Small angiofibromas that do not involve the infratemporal fossa may be resected endoscopically. Extensive ones may require skull base approaches. Recurrences are not uncommon and should be resected if possible. Unresectable recurrences that do not appear to grow significantly may be followed radiologically with serial MR scans in expectation of possible eventual involution stabilization or involution of tumor. Low-dose (30 Gy) irradiation may be helpful in nonresectable, continually growing tumors.

Hofmann T et al: Endoscopic resection of juvenile angiofibromas—long term results. Rhinology 2005;43:282.

Lee JT: The role of radiation in the treatment of advanced juvenile angiofibroma. Laryngoscope 2002;112(7 Part 1):1213.

Mann WJ et al: Juvenile angiofibromas: changing surgical concept over the last 20 years. Laryngoscope 2004;114:291.

Nicolai P et al: Endoscopic surgery for juvenile angiofibroma: when and how. Laryngoscope 2003;113:775.

Wormald PJ et al: Endoscopic removal of juvenile angiofibromas. Otolaryngol Head Neck Surg 2003;129:684.

2. Malignant Nasopharyngeal & Paranasal Sinus Tumors

Unfortunately, malignant tumors of the nose, nasopharynx, and paranasal sinuses tend to remain asymptomatic until late in their course. Although the prognosis is poor for advanced tumors, the results of treating resectable tumors of paranasal sinus origin have improved with the wider use of skull base resections and intensity-modulated radiation therapy. Cure rates are often 45–60%. Early symptoms are nonspecific, mimicking those of rhinitis or sinusitis. Unilateral nasal obstruction and discharge are common, with pain and recurrent hemorrhage often clues to the diagnosis of cancer. Any patient with unilateral or persistent nasal symptoms should be thoroughly evaluated. A high index of suspicion remains a key to the earlier diagnosis of these tumors. Patients often present with advanced symptoms such as proptosis, expansion of a cheek, or ill-fitting maxillary dentures. Malar hypesthesia,

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due to involvement of the infraorbital nerve, is common in maxillary sinus tumors. Biopsy is necessary for definitive diagnosis, and MRI is the best imaging study to delineate the extent of disease and plan appropriate surgery and radiation.

SCC is the most common cancer found. It is especially common in the nasopharynx, where it obstructs the auditory tube and results in serous otitis media. Nasopharyngeal carcinoma (poorly differentiated SCC, nonkeratinizing SCC, or lymphoepithelioma) is usually associated with elevated IgA antibody to the viral capsid antigen of the Epstein-Barr virus (EBV). It is particularly common in patients of southern Chinese descent. Any adult with persistent serous otitis media, especially when unilateral, requires careful evaluation of the nasopharynx. Adenocarcinomas, mucosal melanomas, sarcomas, and non-Hodgkin's lymphomas are less commonly encountered neoplasms of this area.

Treatment depends on the tumor type and the extent of disease. Nasopharyngeal carcinoma at this time is best treated by concomitant radiation and cisplatin followed by adjuvant chemotherapy with cisplatin and fluorouracil—this protocol significantly decreased local, nodal, and distant failures and increased progression-free and overall survival. Locally recurrent nasopharyngeal carcinoma may in selected cases be treated with repeat irradiation protocols or surgery with moderate success and a high degree of concern about local wound healing. Other SCCs are best treated—when resectable—with a combination of surgery and irradiation. Numerous protocols investigating the role of chemotherapy are under evaluation. Cranial base surgery appears to be an effective modality in improving the overall prognosis in paranasal sinus malignancies eroding the ethmoid roof.

Duthoy W et al: Postoperative intensity-modulated radiotherapy in sinonasal carcinoma: clinical results in 39 patients. Cancer 2005;104:71.

Fee WE Jr et al: Nasopharyngectomy for recurrent nasopharyngeal cancer: a 2- to 17-year follow-up. Arch Otolaryngol Head Neck Surg 2002;128:280.

Ganly I et al: Craniofacial resection for malignant paranasal sinus tumors: Report of an International Collaborative Study. Head Neck 2005;27:575.

Maghami E et al: Cancer of the nasal cavity and paranasal sinuses. Expert Rev Anticancer Ther 2004;4:411.

Myers LL et al: Differential diagnosis and treatment options in paranasal sinus cancers. Surg Oncol Clin N Am 2004; 13:167.

Resto VA et al: Sinonasal malignancies. Otolaryngol Clin North Am 2004;37:473.

3. Wegener's Granulomatosis, NK Cell & T Cell EBV-Positive Lymphoma, & Sarcoidosis

The nose and paranasal sinuses are involved in over 90% of cases of Wegener's granulomatosis. It is often not realized that involvement at these sites is more common than involvement of lungs or kidneys. Examination shows bloodstained crusts and friable mucosa. Biopsy classically shows necrotizing granulomas and vasculitis, but the diagnosis may be difficult. Sarcoidosis also commonly involves the paranasal sinuses and is clinically similar to other chronic sinonasal inflammatory processes. Biopsy shows noncaseating granulomas.

Polymorphic reticulosis (midline malignant reticulosis, idiopathic midline destructive disease, lethal midline granuloma), as the multitude of apt descriptive terms suggest, is not well understood but appears to be a nasal lymphoma. In contrast to Wegener's granulomatosis, involvement is limited to the mid face, and there may be extensive bone destruction. Its progression in time to a lymphoma is being described with increasing frequency.

Many destructive lesions of the mucosa and nasal structures labeled as polymorphic reticulosis are in fact non-Hodgkin's lymphoma of either NK cell or T cell origin. Immunophenotyping, especially for CD56 expression, is essential in the histologic evaluation. Even when apparently localized, these lymphomas have a poor prognosis, with progression and death within a year the rule.

For treatment of Wegener's granulomatosis, see Chapter 20.

Abdou NI et al: Wegener's granulomatosis: survey of 701 patients in North America. Changes in outcome in the 1990s. J Rheumatol 2002;29:309.

Cheung MM et al: Early stage nasal NK/T-cell lymphoma: clinical outcome, prognostic factors, and the effect of treatment modality. Int J Radiat Oncol Biol Phys 2002;54:182.

Cox CE et al: Sarcoidosis. Med Clin North Am 2005;89:817.

Lee J et al: Extranodal natural killer T-cell lymphoma, nasal-type: a prognostic model from a retrospective multicenter study. J Clin Oncol 2006;24:612.

Lloyd G et al: Rhinologic changes in Wegener's granulomatosis. J Laryngol Otol 2002;116:565.

Merkel PA: Current status of outcome measures in vasculitis: focus on Wegener's granulomatosis and microscopic polyangiitis. Report from OMERACT 7. J Rheumatol 2005;32: 2488.

Nagai H et al: Clinical review of Wegener's granulomatosis. Acta Otolaryngol Suppl 2002;(547):50.

Diseases of the Oral Cavity & Pharynx

Leukoplakia, Erythroplakia, Oral Lichen Planus, & Oral Cancer

Essentials of Diagnosis

  • Leukoplakia—A white lesion that, unlike oral candidiasis, cannot be removed by rubbing the mucosal surface.

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  • Erythroplakia—Similar to leukoplakia except that it has a definite erythematous component.

  • Oral Lichen Planus—Most commonly presents as lacy leukoplakia but may be erosive; definitive diagnosis requires biopsy.

  • Oral Cancer—Early lesions appear as leukoplakia or erythroplakia; more advanced lesions will be larger, with invasion into tongue such that a mass lesion is palpable.

  • Ulceration may be present.

The areas of leukoplakia are usually small but may be several centimeters in diameter. Histologically, they are often hyperkeratoses occurring in response to chronic irritation (eg, from dentures, tobacco, lichen planus); about 2–6%, however, represent either dysplasia or early invasive SCC.

Distinguishing between erythroplakia and leukoplakia is important because about 90% of cases of erythroplakia are either dysplasia or carcinoma. SCC accounts for 90% of oral cancer. Alcohol and tobacco use are the major epidemiologic risk factors. The differential diagnosis may include oral candidiasis, necrotizing sialometaplasia, pseudoepitheliomatous hyperplasia, median rhomboid glossitis, and vesiculoerosive inflammatory disease such as erosive lichen planus. This should not be confused with the brown-black gingival melanin pigmentation—diffuse or speckled—common in nonwhites, blue-black embedded fragments of dental amalgam, or other systemic disorders associated with general pigmentation (neurofibromatosis, familial polyposis, Addison's disease). Intraoral melanoma is extremely rare.

Oral lichen planus is a relatively common (0.5–2% of the population) chronic inflammatory autoimmune disease that may be difficult to diagnose clinically because of its numerous distinct phenotypic subtypes. For example, the reticular pattern may mimic candidiasis or hyperkeratosis, while the erosive pattern may mimic SCC. Management begins with distinguishing it from other oral lesions. Exfoliative cytology or a small incisional or excisional biopsy is indicated, especially if SCC is suspected. Therapy is aimed at managing pain and discomfort. Corticosteroids have been used widely both locally and systemically. Cyclosporines and retinoids have also been used. Many think there is a low rate (1%) of SCC arising within lichen planus (in addition to the possibility of clinical misdiagnosis).

Any area of erythroplakia, enlarging area of leukoplakia, or a lesion that has submucosal depth on palpation should have an incisional biopsy or an exfoliative cytologic examination. Specialty referral should be sought early both for diagnosis and treatment. Intraoral staining with 1% toluidine blue may aid in selection of the most suspicious biopsy site. A systematic intraoral examination—including the lateral tongue, floor of the mouth, gingiva, buccal area, palate, and tonsillar fossae—and palpation of the neck for enlarged lymph nodes should be part of any general physical examination, especially in patients over the age of 45 who smoke tobacco or drink immoderately. Indirect or fiberoptic examination of the nasopharynx, oropharynx, hypopharynx, and larynx by an otolaryngologist, head and neck surgeon, or radiation oncologist should also be considered for such patients when there is unexplained or persistent throat or ear pain, oral or nasal bleeding, or oral erythroplakia. Fine-needle aspiration (FNA) biopsy may be indicated if an enlarged lymph node is found.

Early detection of SCC is the key to successful management. Lesions less than 4 mm in depth have a low propensity to metastasize. Most patients in whom the tumor is detected before it is 2 cm in diameter are cured. Small lesions are best treated with surgical excision, sometimes with a laser. Radiation is an alternative but is associated with xerostomia, osteonecrosis of the mandible, and inability to use a curative dose again in the treatment field. Large tumors are usually treated with a combination of resection and irradiation. Reconstruction, if required, is done at the time of resection and can involve the use of myocutaneous flaps or vascularized free flaps with or without bone.

Molecular and genetic analysis of premalignant and malignant tissue has produced increasing evidence of genetic instability (including microsatellite instability, cell cycle-regulatory gene P16 and P14 deletions and hypermethylation, and mutations in P53); and clonal alterations, such as loss of retinoic acid β-receptor expression, occur during the early stage of aerodigestive tract carcinogenesis. These molecular and epidemiologic studies provide the foundation on which clinical trials have been designed to evaluate the role of retinoids and other compounds in the reversal of premalignancy and the possible reduction in the 4–5% annual rate of second primary tumors.

A number of clinical trials have suggested a role for beta-carotene, vitamin E, and retinoids in producing regression of leukoplakia and reducing the incidence of recurrent SCCs. Retinoids suppress head and neck and lung carcinogenesis in animal models and inhibit carcinogenesis in individuals with premalignant lesions. They also seem to reduce the incidence of second primary cancers in head and neck and lung cancer patients previously treated for a primary. Sudbo has demonstrated clearly that aneuploidy in leukoplakic lesions is associated with an increased propensity to develop malignancy. If validated, increased screening for genomic instability may become plausible in the future.

Bromwich M: Retrospective study of the progression of oral premalignant lesions to squamous cell carcinoma: a South Wales experience. J Otolaryngol 2002;31:150.

Eisen D et al: Number V Oral lichen planus: clinical features and management. Oral Dis 2005;11:338.

Hegarty AM et al: Fluticasone propionate spray and betamethasone sodium phosphate mouthrinse: a randomized crossover study for the treatment of symptomatic oral lichen planus. J Am Acad Dermatol 2002;47:271.

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Neville BW et al: Oral cancer and precancerous lesions. CA Cancer J Clin 2002;52:195.

Sudbo J et al: Which putatively pre-malignant oral lesions become oral cancers? J Oral Pathol Med 2003;32:63.

Candidiasis

Oral candidiasis (thrush) is usually painful and looks like creamy-white curd-like patches overlying erythematous mucosa. Because these white areas are easily rubbed off (eg, by a tongue depressor)—unlike leukoplakia or lichen planus—only the underlying irregular erythema may be seen. Oral candidiasis is commonly encountered among denture wearers; in debilitated patients, diabetes patients, anemia patients, patients undergoing chemotherapy or local irradiation; and in patients receiving corticosteroids or broad-spectrum antibiotics. Candidiasis is often seen as the first manifestation of HIV infection. Angular cheilitis is another manifestation of candidiasis, although it is also seen in nutritional deficiencies.

The diagnosis is made clinically. A wet preparation using potassium hydroxide will reveal spores and may show nonseptate mycelia. Biopsy will show intraepithelial pseudomycelia of Candida albicans.

Effective antifungal therapy may be achieved with any of the following: fluconazole (100 mg/d for 7–14 days), ketoconazole (200–400 mg with breakfast [requires acidic gastric environment for absorption] for 7–14 days), clotrimazole troches (10 mg dissolved orally five times daily), or nystatin vaginal troches (100,000 units dissolved orally five times daily) or mouth rinses (500,000 units [5 mL of 100,000 units/mL] held in the mouth before swallowing three times daily). Shorter-duration therapy has proved effective, using fluconazole. In patients with HIV infection, however, longer courses may be needed, and oral itraconazole (200 mg/d) may be indicated in fluconazole-refractory cases. In addition, 0.12% chlorhexidine or half-strength hydrogen peroxide mouth rinses may provide local relief. Nystatin powder (100,000 units/g) applied to dentures three or four times daily for several weeks may help denture wearers.

Lefebvre JL et al: A comparative study of the efficacy and safety of fluconazole oral suspension and amphotericin B oral suspension in cancer patients with mucositis. Oral Oncol 2002;38:337.

Pankhurst C: Candidiasis (oropharyngeal). Clin Evid 2005;(13): 1701.

Patton LL et al: A systematic review of the effectiveness of antifungal drugs for the prevention and treatment of oropharyngeal candidiasis in HIV-positive patients. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;92:170.

Vazquez JA et al: Mucosal candidiasis. Infect Dis Clin North Am 2002;16:793.

Glossitis, Glossodynia, Dysgeusia, & Burning Mouth Syndrome

Inflammation of the tongue with loss of filiform papillae leads to a red, smooth-surfaced tongue (glossitis). Rarely painful, it may be secondary to nutritional deficiencies (eg, niacin, riboflavin, iron, or vitamin E), drug reactions, dehydration, irritants, and possibly autoimmune reactions or psoriasis. If the primary cause cannot be identified and corrected, empiric nutritional replacement therapy may be of value.

Glossodynia is burning and pain of the tongue; it may occur with or without glossitis. It has been associated with diabetes, drugs (eg, diuretics), tobacco, xerostomia, and candidiasis as well as the listed causes of glossitis. Periodontal disease is not apt to be a factor. Treating possible underlying causes, changing chronic medications to alternative ones, and smoking cessation may resolve symptoms. Glossodynia is benign, and reassurance that there is no infection or tumor is likely to be appreciated. Anxiolytic medications and evaluation of possible psychological status may be considered as well. Symptoms that cannot be related to a specific medication or other cause may be neuropathic in origin. An empiric trial of gabapentin for symptom control may be used.

Clark GT et al: Orofacial pain and sensory disorders in the elderly. Dent Clin North Am 2005;49:343.

Grushka M et al: Burning mouth syndrome. Am Fam Physician 2002;65:615.

Lamey PJ et al: Vulnerability and presenting symptoms in burning mouth syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99:48.

Tanaka M et al: Incidence and treatment of dysgeusia in patients with glossodynia. Acta Otolaryngol Suppl 2002;(546):142.

Intraoral Ulcerative Lesions

1. Necrotizing Ulcerative Gingivitis (Trench Mouth, Vincent's Infection)

Necrotizing ulcerative gingivitis, often caused by an infection of both spirochetes and fusiform bacilli, is common in young adults under stress (classically at examination time). Underlying systemic diseases may also predispose to this disorder. Clinically, there is painful acute gingival inflammation and necrosis, often with bleeding, halitosis, fever, and cervical lymphadenopathy. Warm half-strength peroxide rinses and oral penicillin (250 mg three times daily for 10 days) may help. Dental gingival curettage may prove necessary.

Necrotizing ulcerative periodontitis is discussed later in this chapter in the section on AIDS.

2. Aphthous Ulcer (Canker Sore, Ulcerative Stomatitis)

Aphthous ulcers are very common and easy to recognize. Their cause remains uncertain, although an association with human herpesvirus 6 has been suggested. Found on nonkeratinized mucosa (eg, buccal and labial mucosa and not gingiva or palate), they may be single or multiple, are usually recurrent, and appear as

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painful small (usually 1–2 mm, but sometimes 1–2 cm) round ulcerations with yellow-gray fibrinoid centers surrounded by red halos. The painful stage lasts 7–10 days; healing is completed in 1–3 weeks.

Treatment is nonspecific. Topical corticosteroids (triamcinolone acetonide, 0.1%, or fluocinonide ointment, 0.05%) in an adhesive base (Orabase Plain) do appear to provide symptomatic relief. Other topical therapies shown to be effective in controlled studies include diclofenac 3% in hyaluronan 2.5%, doxymycine-cyanoacrylate, mouthwashes containing the enzymes amyloglucosidase and glucose oxidase, and amlexanox 5% oral paste. A 1-week tapering course of prednisone (40–60 mg/d) has also been used successfully. Thalidomide has been used selectively in recurrent aphthous ulcerations in HIV-positive patients.

Large or persistent areas of ulcerative stomatitis may be secondary to erythema multiforme or drug allergies, acute herpes simplex, pemphigus, pemphigoid, bullous lichen planus, Behçet's disease, or inflammatory bowel disease. SCC may occasionally present in this fashion. When the diagnosis is not clear, incisional biopsy is indicated.

Akintoye SO et al: Recurrent aphthous stomatitis. Dent Clin North Am 2005;49:31, vii.

Letsinger JA et al: Complex aphthosis: a large case series with evaluation algorithm and therapeutic ladder from topicals to thalidomide. J Am Acad Dermatol 2005;52(3 Pt 1):500.

Muzio LL et al: The treatment of oral aphthous ulceration or erosive lichen planus with topical clobetasol propionate in three preparations: a clinical and pilot study on 54 patients. J Oral Pathol Med 2001;30:611.

Tilliss TS et al: Differential diagnosis: is it herpes or aphthous? J Contemp Dent Pract 2002;3:1.

3. Herpetic Stomatitis

Herpetic gingivostomatitis is common, mild, and short-lived and requires no intervention in most adults. In immunocompromised persons, however, reactivation of herpes simplex virus infection is frequent and may be severe. Clinically, there is initial burning, followed by typical small vesicles that rupture and form scabs. Acyclovir (200–800 mg five times daily for 7–14 days) may shorten the course and reduce postherpetic pain. Differential diagnosis includes ulcerative stomatitis (see above), erythema multiforme, syphilitic chancre, and carcinoma. Coxsackievirus-caused lesions (grayish white tonsillar and palatal ulcers of herpangina or buccal and lip ulcers in hand-foot-and-mouth disease) are seen more commonly in children under age 6.

Holbrook WP et al: Herpetic gingivostomatitis in otherwise healthy adolescents and young adults. Acta Otol Scand 2001;59:113.

Whitley RJ: Herpes simplex virus infection. Semin Pediatr Infect Dis 2002;13:6.

Pharyngitis & Tonsillitis

Essentials of Diagnosis

  • Sore throat.

  • Fever.

  • Anterior cervical adenopathy.

  • Tonsillar exudate.

  • Focus is to treat group A β-hemolytic streptococcus infection to prevent rheumatic sequelae.

General Considerations

Pharyngitis and tonsillitis account for over 10% of all office visits to primary care clinicians and 50% of outpatient antibiotic use. The most appropriate management continues to be debated because some of the issues are deceptively complex, but consensus has increased in recent years. The main concern is determining who is likely to have a group A β-hemolytic streptococcal infection (GABHS), as this can lead to subsequent complications such as rheumatic fever and glomerular nephritis. A second public health policy concern is reducing the extraordinary cost (both in dollars and in the development of antibiotic-resistant S pneumoniae) in the United States associated with unnecessary and unrecommended antibiotic use. Questions now being asked: Is there still a role for culturing a sore throat, or have the rapid antigen tests supplanted this procedure under most circumstances? Are clinical criteria alone a sufficient basis for decisions about which patients should be given antibiotics? Should any patient receive any antibiotic other than penicillin (or erythromycin if penicillin-allergic)? For how long should treatment be continued? Numerous well-done studies in the past few years as well as increasing experience with rapid laboratory tests for detection of streptococci (eliminating the delay caused by culturing) appear to make a consensus approach more possible.

Clinical Findings

The clinical features most suggestive of group A β-hemolytic streptococcal pharyngitis include fever over 38°C, tender anterior cervical adenopathy, lack of a cough, and a pharyngotonsillar exudate. These four features (the Centor criteria), when present, strongly suggest GABHS, and some would treat regardless of laboratory results. When three of the four are present, laboratory sensitivity of rapid antigen testing exceeds 90%. When only one criterion is present, GABHS is unlikely. Sore throat may be severe, with odynophagia, tender adenopathy, and a scarlatiniform rash. An elevated white count and left shift are also possible.

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Hoarseness, cough, and coryza are not suggestive of this disease.

Marked lymphadenopathy and a shaggy white-purple tonsillar exudate, often extending into the nasopharynx, suggest mononucleosis, especially if present in a young adult. Hepatosplenomegaly and a positive heterophil agglutination test or elevated anti-EBV titer are corroborative. However, about one-third of patients with infectious mononucleosis have secondary streptococcal tonsillitis, requiring treatment. Ampicillin should routinely be avoided if mononucleosis is suspected because it induces a rash. Diphtheria (extremely rare but described in the alcoholic population) presents with low-grade fever and an ill patient with a gray tonsillar pseudomembrane.

The most common pathogens other than group A β-hemolytic streptococci in the differential diagnosis of “sore throat” are viruses, Neisseria gonorrhoeae, Mycoplasma, and Chlamydia trachomatis. Rhinorrhea and lack of exudate would suggest a virus, but in practice it is not possible to confidently distinguish viral upper respiratory infection from GABHS on clinical grounds alone. Infections with Corynebacterium diphtheriae, anaerobic streptococci, and Corynebacterium haemolyticum (which responds better to erythromycin than penicillin) may also mimic pharyngitis due to group A β-hemolytic streptococci.

Treatment

Treatment strategies for pharyngitis and tonsillitis range from “treat all comers” to “test all comers, reserving treatment for those with positive results.” Issues that affect this decision include the reliability of cultures and rapid tests for streptococci (latex agglutination antigen tests and solid-phase enzyme immunoassays [ELISA]), the incidence of pharyngitis not due to group A β-hemolytic streptococci in the community, patient follow-up and medical compliance, and cost. The advantage of the “treat all” approach is the initial short-term cost, savings from elimination of diagnostic tests, and prevention of rheumatic fever, but such an approach necessarily causes the highest rate of antibiotic use and side effects and therefore overall the highest cost. At the other extreme is the “culture all” approach, which is associated with the fewest side effects but is dependent on excellent and rapid follow-up to prevent postinfectious complications. The sensitivity of current GABHS rapid antigen tests is now excellent, exceeding 90% in appropriately selected patients. It thus appears appropriate to screen for the four Centor criteria and use them in one of the following ways: (1) test patients who satisfy two or more criteria, and treat only those with positive results; (2) test those who satisfy two or three criteria, and treat both those with positive results and all patients who satisfy all four criteria without testing them; or (3) test nobody and treat all who satisfy three or four criteria. Routine cultures arc not needed. Webb and colleagues, using this approach, found no increase in rates of complications among 7500 patients annually, about 75% of whom had high-specificity antigen tests without culture confirmation of negative results.

Given the availability of many well-documented studies in recent years, one would think that a consensus might develop as to the most appropriate way to treat a sore throat. The Infectious Diseases Society of America recommends laboratory confirmation of the clinical diagnosis by means of either throat culture or a rapid antigen detection test. The American College of Physicians-American Society of Internal Medicine (ACP-ASIM), in collaboration with the Centers for Disease Control and Prevention, advocates use of a clinical algorithm alone—in lieu of microbiologic testing—for confirmation of the diagnosis in adults for whom the suspicion of streptococcal infection is high. Others examine the assumptions of the ACP-ASIM guideline for using a clinical algorithm alone and question whether those recommendations will achieve the stated objective of dramatically decreasing excess antibiotic use. Convincing clinical trials as well as clinician reminders and patient-based interventions may be needed before clinicians are likely to abandon long-held teachings (even as different clinicians appear to have been taught different strategies) regarding diagnosis and management of group A streptococcal pharyngitis. The Cochrane review concluded that multifaceted interventions where educational interventions occur on many levels were the only interventions whose effects were of sufficient magnitude to potentially reduce the incidence of antibiotic-resistant bacteria.

Thirty years ago, a single injection of benzathine penicillin or procaine penicillin was standard antibiotic treatment. This remains effective, but the injections are painful. If compliance is an issue, it may be the best choice. Oral treatment is also effective. Antibiotic choice aims to reduce the already low (10–20%) incidence of treatment failures (positive culture after treatment despite symptomatic resolution) and recurrences. A review of recent controlled studies suggests that penicillin V potassium (250 mg orally three times daily or 500 mg twice daily for 10 days) or cefuroxime axetil (250 mg orally twice daily for 5–10 days) are both effective. The efficacy of a 5-day regimen of penicillin V appears to be similar to a that of a 10-day course, with a 94% clinical response rate and an 84% streptococcal eradication rate. Erythromycin (active against Mycoplasma and Chlamydia) is a reasonable alternative to penicillin in allergic patients. Cephalosporins are somewhat more effective than penicillin in producing bacteriologic cures; 5-day administration has been successful for cefpodoxime and cefuroxime. The macrolide antibiotics have also been reported to be successful in shorter-duration regimens. Azithromycin (500 mg once daily), because of its long half-life, need be taken for only 3 days.

Adequate antibiotic treatment usually avoids the streptococcal complications of scarlet fever, glomerulonephritis, rheumatic myocarditis, and local abscess formation.

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Antibiotics for treatment failures are also somewhat controversial. Surprisingly, penicillin-tolerant strains are not isolated more frequently in those who fail treatment than in those treated successfully with penicillin. The reasons for failure appear to be complex, and a second course of treatment with the same drug is not unreasonable. Alternatives to penicillin include cefuroxime and other cephalosporins, dicloxacillin (which is β-lactamase-resistant), and amoxicillin with clavulanate. When there is a history of penicillin allergy, alternatives should be used, such as erythromycin. Erythromycin resistance—with failure rates of about 25%—is an increasing problem in many areas. In cases of severe penicillin allergy, cephalosporins should be avoided as the cross-reaction is common (8% or more).

Ancillary treatment of pharyngitis includes analgesics and anti-inflammatory agents, such as aspirin or acetaminophen. Some patients find that salt water gargling is soothing. In severe cases, anesthetic gargles and lozenges (eg, benzocaine) may provide additional symptomatic relief. Occasionally, odynophagia is so intense that hospitalization for intravenous hydration and antibiotics is necessary. (See Chapter 33.)

Arnold SR et al: Interventions to improve antibiotic prescribing practices in ambulatory care. Cochrane Database Syst Rev 2005;(4):CD003539.

Bisno AL et al: Practice guidelines for the diagnosis and management of group A streptococcal pharyngitis. Infectious Diseases Society of America. Clin Infect Dis 2002;35:113.

Carapetis JR et al: The global burden of group A streptococcal diseases. Lancet Infect Dis 2005;5:685.

Colletti T et al: Strep throat: guidelines for diagnosis and treatment. JAAPA 2005;18:38; quiz 45.

Hirschmann JV: Antibiotics for common respiratory tract infections in adults. Arch Intern Med 2002;162:256.

Johnson BC et al: Cost-effective workup for tonsillitis. Testing, treatment, and potential complications. Postgrad Med 2003;113:115.

Tewfik TL et al: Tonsillopharyngitis: clinical highlights. J Otolaryngol 2005;34 Suppl 1:S45.

Peritonsillar Abscess & Cellulitis

When infection penetrates the tonsillar capsule and involves the surrounding tissues, peritonsillar cellulitis results. Peritonsillar abscess and cellulitis present with severe sore throat, odynophagia, trismus, medial deviation of the soft palate and peritonsillar fold, and an abnormal muffled (“hot potato”) voice. Following therapy, peritonsillar cellulitis usually either resolves over several days or evolves into peritonsillar abscess. The existence of an abscess may be confirmed by aspirating pus from the peritonsillar fold just superior and medial to the upper pole of the tonsil. A No. 19 or No. 21 needle should be passed no deeper than 1 cm, because the internal carotid artery may lie more medially than its usual location and pass posterior and deep to the tonsillar fossa. There is controversy regarding the three ways to treat a peritonsillar abscess: needle aspiraton, incision and drainage, or tonsillectomy. Some clinicians incise and drain the area and continue with parenteral antibiotics, whereas others aspirate only and monitor as an outpatient. To drain the abscess and avoid recurrence, it may be appropriate to consider immediate tonsillectomy (quinsy tonsillectomy). About 10% of patients with peritonsillar abscess exhibit relative indications for tonsillectomy. All three approaches are effective and have support in the literature. Regardless of the method used, one must be sure the abscess is adequately treated, since complications such as extension to the retropharyngeal, deep neck, and posterior mediastinal spaces are possible. Bacteria may also be aspirated into the lungs, resulting in pneumonia. While there is controversy about whether a single abscess is sufficient indication for tonsillectomy, most would agree that patients with recurrent abscesses should have a tonsillectomy.

Dunne AA et al: Peritonsillar abscess—critical analysis of abscess tonsillectomy. Clin Otolaryngol 2003;28:420.

Franzese CB et al: Peritonsillar and parapharyngeal space abscess in the older adult. Am J Otolaryngol 2003;24:169.

Johnson RF et al: The contemporary approach to diagnosis and management of peritonsillar abscess. Curr Opin Otolaryngol Head Neck Surg 2005;13:157.

Khayr W et al: Management of peritonsillar abscess: needle aspiration versus incision and drainage versus tonsillectomy. Am J Ther 2005;12:344.

Matsuda A et al: Peritonsillar abscess: a study of 724 cases in Japan. Ear Nose Throat J 2002;81:384.

Tonsillectomy

Despite the frequency with which tonsillectomy is performed, the indications for the procedure remain controversial. Most clinicians would agree that airway obstruction causing sleep apnea or cor pulmonale is an absolute indication for tonsillectomy. Similarly, persistent marked tonsillar asymmetry should prompt an excisional biopsy to rule out lymphoma. Relative indications include recurrent streptococcal tonsillitis, causing considerable loss of time from school or work, recurrent peritonsillar abscess, and chronic tonsillitis.

Tonsillectomy is not an entirely benign procedure. The pros and cons of tonsillectomy need to be discussed with each prospective patient. Postoperative bleeding occurs in 2–4% of cases and on rare occasions can lead to laryngospasm and airway obstruction. Pain may be considerable, especially in the adult. Protracted emesis or fever may also occasionally occur. Secondary bleeding 5–8 days postoperatively is far more common than bleeding in the first 24 hours. There is increasing economic pressure for these procedures to be done as outpatient surgery. At present it seems clear that outpatient tonsillectomy is usually safe when followed by a 6-hour period of uneventful

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observation, but individual circumstances may mandate hospitalization.

Although reports in the 1970s suggested an association of tonsillectomy with Hodgkin's disease, careful review of this literature reveals no conclusively causative association.

Bhattacharyya N et al: Economic benefit of tonsillectomy in adults with chronic tonsillitis. Ann Otol Rhinol Laryngol 2002;111:983.

Darrow DH et al: Indications for tonsillectomy and adenoidectomy. Laryngoscope 2002;112:6.

Dhiwakar M et al: Antibiotics to improve recovery following tonsillectomy: a systematic review. Otolaryngol Head Neck Surg 2006;134:357.

Windfuhr JP et al: Incidence of post-tonsillectomy hemorrhage in children and adults: a study of 4,848 patients. Ear Nose Throat J 2002;81:626.

Deep Neck Infections

Deep neck abscesses usually present with marked neck pain and swelling in a toxic febrile patient. They are emergencies because they may rapidly compromise the airway. They may also spread to the mediastinum or cause septicemia. Most commonly, they originate from odontogenic infections. Other causes include suppurative lymphadenitis, direct spread of pharyngeal infection, penetrating trauma, pharyngoesophageal foreign bodies, cervical osteomyelitis, and intravenous injection of the internal jugular vein, especially in drug abusers. Recurrent deep neck infection may suggest an underlying congenital lesion such as a branchial cleft cyst.

Fundamentals of treatment include securing the airway, intravenous antibiotics, and incision and drainage. In highly selected patients without airway compromise, needle aspiration and catheter drainage or even conservative management without antibiotics alone has been reported to be successful in uniloculated abscesses. The airway may be secured, when indicated, either by intubation or tracheotomy. Tracheotomy is preferable in the patients with substantial pharyngeal edema, since attempts at intubation may precipitate acute airway obstruction. Bleeding in association with a deep neck abscess suggests the possibility of carotid artery or internal jugular vein involvement and requires prompt neck exploration both for drainage of pus and for vascular control.

Contrast-enhanced CT usually augments the clinical examination in defining the extent of the infection. It often will distinguish inflammation (requiring antibiotics) from abscess (requiring drainage) and define for the surgeon the extent of an abscess. CT with MRI may also identify thrombophlebitis of the internal jugular vein secondary to oropharyngeal inflammation. This condition, known as Lemierre's syndrome, may be associated with septic emboli and requires prompt institution of antibiotics appropriate for Fusobacterium necrophorum as well as the more usual upper airway pathogens. The presence of pulmonary infiltrates (or septic arthritis) in the setting of a neck abscess should lead one to suspect Lemierre's syndrome.

Ludwig's angina is the most commonly encountered neck space infection. It is a cellulitis of the sublingual and submaxillary spaces, often arising from infection of the mandible. Clinically, there is edema and erythema of the upper neck under the chin and often of the floor of the mouth. The tongue may be displaced upward and backward by the posterior spread of cellulitis. This may lead to occlusion of the airway and necessitate tracheotomy. Microbiologic isolates include streptococci, staphylococci, Bacteroides, and Fusobacterium. Usual doses of penicillin plus metronidazole, ampicillin-sulbactam, clindamycin, or selective cephalosporins are good initial choices. Culture and sensitivity data will then refine the choice. Dental consultation is advisable. External drainage via bilateral submental incisions is required if the airway is threatened or when medical therapy has not reversed the process.

Brook I: Microbiology and management of deep facial infections and Lemierre syndrome. ORL J Otorhinolaryngol Relat Spec 2003;65:117.

Chirinos JA et al: The evolution of Lemierre syndrome: report of 2 cases and review of the literature. Medicine (Baltimore) 2002;81:458.

Dool H et al: Lemierre's syndrome: three cases and a review. Eur Arch Otorhinolaryngol 2005;262:651.

Lin D et al: Internal jugular vein thrombosis and deep neck infection from intravenous drug use: management strategy. Laryngoscope 2004;114:56.

Parhiscar A et al: Deep neck abscess: a retrospective review of 210 cases. Ann Otol Rhinol Laryngol 2001;110:1051.

Ridder GJ et al: Spectrum and management of deep neck space infections: an 8-year experience of 234 cases. Otolaryngol Head Neck Surg 2005;133:709.

Wang LF et al: Space infection of the head and neck. Kaohsiung J Med Sci 2002;18:386.

Diseases of the Salivary Glands

The salivary glands are divided into the two large parotid glands, two submandibular glands, several sublingual glands, and 600–1000 minor salivary glands located throughout the upper aerodigestive tract.

Acute Inflammatory Salivary Gland Disorders

1. Sialadenitis

Acute bacterial sialadenitis in the adult most commonly affects either the parotid or submandibular gland. It typically presents with acute swelling of the gland, increased pain and swelling with meals, and tenderness

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and erythema of the duct opening. Pus often can be massaged from the duct. Sialadenitis often occurs in the setting of dehydration or in association with chronic illness. Underlying Sjögren's syndrome may contribute. Ductal obstruction, often by an inspissated mucous plug, is followed by salivary stasis and secondary infection. The most common organism recovered from purulent draining saliva is S aureus. Treatment consists of intravenous antibiotics such as nafcillin (1 g intravenously every 4–6 hours) and measures to increase salivary flow, including hydration, warm compresses, sialagogues (eg, lemon drops), and massage of the gland. Usually one can switch to an oral agent based on clinical and microbiologic improvement to complete a 10-day course. Failure of the process to resolve on this regimen suggests abscess formation, ductal stricture, stone, or tumor causing obstruction. Ultrasound or CT scan may be helpful in establishing the diagnosis. Sialography is best avoided in acute cases.

2. Sialolithiasis

Calculus formation is more common in Wharton's duct (draining the submandibular glands) than in Stensen's duct (draining the parotid glands). Clinically, a patient may note postprandial pain and local swelling, often with a history of recurrent acute sialadenitis. Stones in Wharton's duct are usually large and radiopaque, whereas those in Stensen's duct are usually radiolucent and smaller. Those very close to the orifice of Wharton's duct may be palpated manually in the anterior floor of the mouth and removed intraorally by dilating or incising the distal duct. The duct proximal to the stone must be temporarily clamped (using, for instance, a single throw of a suture) to keep manipulation of the stone from pushing it back toward the submandibular gland. Those more than 1.5–2 cm from the duct are too close to the lingual nerve to be removed safely in this manner. Similarly, dilation of Stensen's duct, located on the buccal surface opposite the second maxillary molar, may relieve distal stricture or allow a small stone to pass. In addition to intraoral stone removal, both extracorporeal shock-wave lithotripsy and fluoroscopically guided basket retrieval have been used successfully in recent years, with success rates between 40% and 80%.

Repeated episodes of sialadenitis are usually associated with stricture and chronic infection. If the obstruction cannot be safely removed or dilated, excision of the gland may be necessary to relieve recurrent symptoms.

Baurmash HD: Submandibular salivary stones: current management modalities. J Oral Maxillofac Surg 2004;62:369.

Brook I: Aerobic and anaerobic microbiology of suppurative sialadenitis. J Med Microbiol 2002;51:526.

McGurk M et al: Modern management of salivary calculi. Br J Surg 2005;92:107.

Ziegler CM et al: Endoscopy as minimal invasive routine treatment for sialolithiasis. Acta Odontol Scand 2003;61:137.

Chronic Inflammatory & Infiltrative Disorders of the Salivary Glands

Numerous infiltrative disorders may cause unilateral or bilateral parotid gland enlargement. Sjögren's disease and sarcoidosis are examples of lymphoepithelial and granulomatous diseases that may affect the salivary glands. Metabolic disorders, including alcoholism, diabetes mellitus, and vitamin deficiencies, may also cause diffuse enlargement. Several drugs have been associated with parotid enlargement, including thioureas, iodine, and drugs with cholinergic effects (eg, phenothiazines), which stimulate salivary flow and cause more viscous saliva.

Salivary Gland Tumors

Approximately 80% of salivary gland tumors occur in the parotid gland. In adults, about 80% of these are benign. In the submandibular triangle, it is sometimes difficult to distinguish a primary submandibular gland tumor from a metastatic submandibular space node. Only 50–60% of primary submandibular tumors are benign. Tumors of the minor salivary glands are most likely to be malignant, with adenoid cystic carcinoma predominating.

Most parotid tumors present as an asymptomatic mass in the superficial part of the gland. Their presence may have been noted by the patient for months or years. Facial nerve involvement correlates strongly with malignancy. Tumors may extend deep to the plane of the facial nerve or may originate in the parapharyngeal space. In such cases, medial deviation of the soft palate is visible on intraoral examination. MRI and CT scans have largely replaced sialography in defining the extent of tumor.

When the clinician encounters a patient with an otherwise asymptomatic salivary gland mass where tumor is the most likely diagnosis, the choice is whether to simply excise the mass via a parotidectomy with facial nerve dissection or submandibular gland excision or to obtain an FNA biopsy first. Although the accuracy of FNA biopsy for malignancy has been reported to be quite high, results vary among institutions. If a negative FNA biopsy would lead to a decision not to proceed to surgery, then it should be considered. Poor overall health of the patient and the possibility of inflammatory disease as the cause of the mass are situations where FNA biopsy might be helpful. In otherwise straightforward nonrecurrent cases, excision is indicated. In benign and small low-grade malignant tumors, no additional treatment is needed. Postoperative irradiation is indicated for larger and high-grade cancers.

Bajaj Y et al: Critical clinical appraisal of the role of ultrasound guided fine needle aspiration cytology in the management of parotid tumours. J Laryngol Otol 2005;119:289.

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Bhattacharyya N et al: Nodal metastasis in major salivary gland cancer: predictive factors and effects on survival. Arch Otolaryngol Head Neck Surg 2002;128:904.

Bhattacharyya N et al: Determinants of survival in parotid gland carcinoma: a population-based study. Am J Otolaryngol 2005;26:39.

Guzzo M et al: Mucoepidermoid carcinoma of the salivary glands: clinicopathologic review of 108 patients treated at the National Cancer Institute of Milan. Ann Surg Oncol 2002; 9:688.

Luukkaa H et al: Salivary gland cancer in Finland 1991–96: an evaluation of 237 cases. Acta Otolaryngol 2005;125:207.

Seethala RR et al: Relative accuracy of fine-needle aspiration and frozen section in the diagnosis of lesions of the parotid gland. Head Neck 2005;27:217.

Wahlberg P et al: Carcinoma of the parotid and submandibular glands—a study of survival in 2465 patients. Oral Oncol 2002;38:706.

Witt RL: Minimally invasive surgery for parotid pleomorphic adenoma. Ear Nose Throat J 2005;84:308, 310.

Diseases of the Larynx

Dysphonia, Hoarseness, & Stridor

The primary symptoms of laryngeal disease are hoarseness and stridor. Hoarseness is caused by an abnormal flow of air past the vocal cords. The voice is “breathy” when too much air passes incompletely apposed vocal cords, as in unilateral vocal cord paralysis. The voice is harsh when turbulence is created by irregularity of the vocal cords, as in laryngitis or a mass lesion. Stridor, a high-pitched sound, is produced by lesions that narrow the airway. Airway narrowing above the vocal cords produces predominantly inspiratory stridor. Airway narrowing below the vocal cord level produces either expiratory or mixed stridor.

Evaluation of an abnormal voice begins with obtaining a history of the circumstances preceding its onset and an examination of the airway. Any patient with hoarseness that has persisted beyond a few weeks should be evaluated by indirect fiberoptic laryngoscopy. Especially when the patient has a history of tobacco use, laryngeal cancer or lung cancer (leading to paralysis of a recurrent laryngeal nerve) must be strongly considered. Laryngitis, voice abuse, and vocal cord nodules are among the most common causes of hoarseness.

Altman KW et al: Current and emerging concepts in muscle tension dysphonia: a 30-month review. J Voice 2005;19:261.

Garrett CG et al: Hoarseness. Med Clin North Am 1999;83:115.

MacKenzie K et al: Is voice therapy an effective treatment for dysphonia? A randomized controlled trial. BMJ 2001;323:658.

Merati AL et al: Common movement disorders affecting the larynx: a report from the neurolaryngology committee of the AAO-HNS. Otolaryngol Head Neck Surg 2005;133:654.

Sataloff RT: Professional voice users: the evaluation of voice disorders. Occup Med 2001;16:633.

Common Laryngeal Disorders

1. Epiglottitis

Epiglottitis (or, more correctly, supraglottitis) in adults should be suspected when a patient presents with a rapidly developing sore throat or when odynophagia (pain on swallowing) is out of proportion to apparently minimal oropharyngeal findings on examination. It is more common in diabetics and may be viral or bacterial in origin. Rarely in the era of H influenzae type b vaccine is this bacterium isolated in adults. Unlike in children, indirect laryngoscopy is generally safe and may demonstrate a swollen, erythematous epiglottis. Initial treatment is hospitalization for intravenous antibiotics—eg, ceftizoxime, 1–2 g intravenously every 8–12 hours; or cefuroxime, 750–1500 mg intravenously every 8 hours; and dexamethasone, usually 4–10 mg as initial bolus, then 4 mg intravenously every 6 hours—and observation of the airway. Corticosteroids may be tapered as signs and symptoms resolve. Similarly, substitution of oral antibiotics may be appropriate to complete a 10-day course. Less than 10% of adults require intubation. Indications for intubation are dyspnea or rapid pace of sore throat (where progression to airway compromise may occur before the effects of corticosteroids and antibiotics). If the patient is not intubated, prudence suggests monitoring oxygen saturation with continuous pulse oximetry and initial admission to a monitored unit.

Berger G et al: The rising incidence of adult acute epiglottitis and epiglottic abscess. Am J Otolaryngol 2003;24:374.

Chang YL et al: Adult acute epiglottitis: experiences in a Taiwanese setting. Otolaryngol Head Neck Surg 2005;132: 689.

Cohen B: The death of George Washington (1732–99) and the history of cynanche. J Med Biogr 2005;13:225.

Katori H et al: Acute epiglottitis: analysis of factors associated with airway intervention. J Laryngol Otol 2005;119:967.

Sack JL et al: Identifying acute epiglottitis in adults. High degree of awareness, close monitoring are key. Postgrad Med 2002; 112:81.

2. Recurrent Respiratory Papillomatosis

Papillomas are common lesions of the larynx and other sites where ciliated and squamous epithelia meet. Unlike oral papillomas, recurrent respiratory papillomatosis (RRP) is likely to be symptomatic, with hoarseness that progresses to stridor over weeks to months. The disease

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is more common in children. Repeated laser vaporizations via microdirect laryngoscopy are usually the mainstay of treatment. Tracheotomy should be avoided, if possible, since it introduces an additional squamociliary junction where papillomas appear to preferentially grow. Interferon treatment has been under investigation for over two decades; rare cases of malignant transformation (often in smokers) have been reported. Cidofovir (a cytosine nucleotide analog in use to treat cytomegalovirus retinitis) is also being investigated as intralesional therapy for RRP; its potential for carcinogenesis is being monitored. Antiviral vaccines to HPV 11 and 6 are being investigated as well.

Chadha NK et al: Adjuvant antiviral therapy for recurrent respiratory papillomatosis. Cochrane Database Syst Rev 2005;(4): CD005053.

Derkay CS et al: Recurrent respiratory papillomatosis. Ann Otol Rhinol Laryngol 2006;115:1.

Gerein V et al: Use of interferon-alpha in recurrent respiratory papillomatosis: 20-year follow-up. Ann Otol Rhinol Laryngol 2005;114:463.

Lee JH et al: Recurrent respiratory papillomatosis: pathogenesis to treatment. Curr Opin Otolaryngol Head Neck Surg 2005; 13:354.

Naiman AN et al: Natural history of adult-onset laryngeal papillomatosis following multiple cidofovir injections. Ann Otol Rhinol Laryngol 2006;115:175.

Shehab N et al: Cidofovir for the treatment of recurrent respiratory papillomatosis: a review of the literature. Pharmacotherapy 2005;25:977.

3. Acute Laryngitis

Acute laryngitis is probably the most common cause of hoarseness, which may persist for a week or so after other symptoms of an upper respiratory infection have cleared. The patient should be warned to avoid vigorous use of the voice (singing, shouting) while laryngitis is present, since this may foster the formation of vocal nodules. Although thought to be usually viral in origin, both M catarrhalis and H influenzae may be isolated from the nasopharynx at higher than expected frequencies. Erythromycin may reduce the severity of hoarseness and cough.

4. Gastroesophageal Reflux & Hoarseness

Gastroesophageal reflux into the larynx (laryngopharyngeal reflux) is considered a possible cause of chronic hoarseness when other causes of abnormal laryngeal airflow (such as tumor) have been excluded by indirect or direct laryngoscopy. Gastroesophageal reflux disease (GERD) has also been suggested as a contributing factor to other symptoms such as throat clearing, throat discomfort, chronic cough, a sensation of postnasal drip, and esophageal spasm; as well as in many cases of posterior laryngitis and some cases of asthma. Since less than half of patients with documented laryngopharyngeal reflux have typical symptoms of heartburn and regurgitation, the lack of such symptoms should not be construed as eliminating this cause.

Management should initially exclude other causes of hoarseness, laryngitis, or chronic cough; consultation with an otolaryngologist is advisable. Many clinicians opt next for an empiric trial of a proton pump inhibitor at twice-daily dosing (eg, omeprazole 20 mg twice daily) for 2–3 months as a practical alternative to an initial pH study. If symptoms improve and cessation of therapy leads to symptoms again, then a proton pump inhibitor is resumed at the lowest dose effective for remission, usually daily but at times on a demand basis. Although H2-receptor antagonists are an alternative to proton pump inhibitors, they are generally both less clinically effective and less cost-effective. Nonresponders should undergo pH testing and manometry. Twenty-four-hour pH monitoring of the pharynx should best document laryngopharyngeal reflux and is advocated by some as the initial management step but it is costly, more difficult, and less available than lower esophageal monitoring alone. Lower esophageal pH monitoring does not correlate well with laryngopharyngeal reflux symptoms.

Ahmed TF et al: Chronic laryngitis associated with gastroesophageal reflux: prospective assessment of differences in practice patterns between gastroenterologists and ENT physicians. Am J Gastroenterol 2006;101:470.

Hopkins C et al: Acid reflux treatment for hoarseness. Cochrane Database Syst Rev 2006;(1):CD005054.

Noordzij JP et al: Correlation of pH probe-measured laryngopharyngeal reflux with symptoms and signs of reflux laryngitis. Laryngoscope 2002;112:2192.

Oelschlager BK et al: Typical GERD symptoms and esophageal pH monitoring are not enough to diagnose pharyngeal reflux. J Surg Res 2005;128:55.

Williams RB et al: Predictors of outcome in an open label, therapeutic trial of high-dose omeprazole in laryngitis. Am J Gastroenterol 2004;99:777.

Tumors of the Larynx

1. Benign Tumors of the Larynx

Vocal cord nodules are smooth, paired lesions that form at the junction of the anterior one-third and posterior two-thirds of the vocal cords. They are a common cause of hoarseness resulting from vocal abuse. In adults, they are referred to as “singer's nodules”; in children, “screamer's nodules.” Treatment requires modification of voice habits, and referral to a speech therapist is indicated. Recalcitrant nodules may require surgical excision.

Polypoid changes in the vocal cords may result from vocal abuse, smoking, chemical industrial irritants, or hypothyroidism. Attention to the underlying problem may resolve the polypoid changes. Inhaled corticosteroid spray (eg, beclomethasone, 42 mcg/spray, or dexamethasone, 84 mcg/spray, two or three times a day) may hasten resolution. At times,

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removal of the hyperplastic vocal cord mucosa may be indicated.

A common but often unrecognized cause of hoarseness is contact ulcers on the vocal processes of the arytenoid cartilages secondary to esophageal reflux. Treatment may be with H2-receptor blockers or proton pump inhibitors (see Gastroesophageal Reflux and Hoarseness, above). Intubation granulomas may also be seen posteriorly between the vocal processes.

2. Laryngeal Leukoplakia

Leukoplakia is a frequent cause of chronic hoarseness, most commonly arising in smokers. Direct laryngoscopy with biopsy is advised. Histologic examination usually demonstrates mild, moderate, or severe dysplasia. Cessation of smoking may reverse dysplastic changes. A certain percentage of patients—estimated to be less than 5% of those with mild dysplasia and about 35–60% of those with severe dysplasia—will subsequently develop SCC. In some cases, invasive SCC is present in the initial biopsy specimen.

3. Squamous Cell Carcinoma of the Larynx

Essentials of Diagnosis

  • Persistent (more than 2 weeks duration) hoarseness.

  • Persistent throat pain.

  • Unexplained ear pain (referred otalgia).

  • Neck mass.

  • Hemoptysis.

  • Stridor or other symptoms of a compromised airway.

  • More common in smokers.

  • If a neck mass is present, an FNA biopsy is usually adequate for initial diagnosis.

Clinical Findings

A. Symptoms and Signs

SCC of the larynx, the most common malignancy of the larynx, occurs almost exclusively in patients with a history of significant tobacco use, and often with alcohol. SCC is usually seen in persons age 50–70 years; about 13,000 new cases are seen in United States each year. A change in voice quality is almost always noted, although throat or ear pain, hemoptysis, dysphagia, weight loss, and airway compromise may occur. Neck metastases are not common in early glottic (true vocal cord [TVC]) cancer in which the vocal cords are mobile, but a third of patients in whom there is impaired cord mobility will also have involved nodes. Supraglottic carcinoma (false vocal cords, aryepiglottic folds, epiglottis), on the other hand, often metastasizes to both sides of the neck early. Complete head and neck examination, including indirect or fiberoptic laryngoscopy, by an experienced clinician is mandated for any person with the concerning symptoms listed under Essentials of Diagnosis.

B. Imaging and Laboratory Studies

Radiologic evaluation is helpful in assessing tumor extent. In contrast to other head and neck sites, CT scanning is preferable to MRI for the larynx. CT scan evaluates neck nodes, tumor volume, and cartilage sclerosis or destruction. A chest CT scan is indicated if there are level VI enlarged nodes (around the trachea and the thyroid gland) or IV enlarged nodes (inferior to the cricoid cartilage along the internal jugular vein) or if a chest film is concerning for a second primary lesion or metastases. Laboratory evaluation includes complete blood count and liver function tests. Formal cardiopulmonary evaluation may be indicated, especially if partial laryngeal surgery is being considered. A positron emission tomography (PET) scan or CT-PET scan may be indicated to assess for distant metastases when there appears to be advanced local or regional disease.

C. Biopsy

Diagnosis is made by biopsy at the time of laryngoscopy. At that time, true cord mobility and arytenoid fixation, as well as surface tumor extent, can be evaluated. Most otolaryngologists recommend flexible esophagoscopy and flexible bronchoscopy at that time. Although an FNA biopsy of an enlarged neck node may have already been done, it is generally acceptable to assume radiologically enlarged neck nodes are neck metastases; rarely is an open biopsy necessary.

D. Tumor Staging

Staging is helpful in discussing laryngeal cancer, but a number of factors that are not included in the TNM staging are also important in deciding treatment recommendations. Table 8-2 outlines (slightly abbreviated) the current American Joint Committee on Cancer (AJCC) tumor staging for the glottis and supraglottis.

Treatment

Treatment of laryngeal carcinoma has four goals: cure, preservation of safe effective swallowing, preservation of useful voice, and avoidance of a permanent tracheostoma. Early tumors (T1 and many T2 lesions, without involved nodes) may be treated with either radiation therapy or partial laryngeal surgery assuming at least one cricoarytenoid unit is normal (transoral endoscopic laser resection, classic open vertical hemilaryngectomy for glottic tumors, or classic horizontal supraglottic laryngectomy). Five-year loco-regional cure rates exceed 80–90%, and patient-reported satisfaction

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is excellent. In supraglottic tumors, even when clinically N0, selective neck dissection or irradiation is indicated because of the high risk of neck node involvement.

Table 8-2. Staging of glottis and supraglottis tumors.

Stage Glottis Supraglottis
T1 Limited to true vocal cords One site, with normal true vocal cord mobility
T2 Extends to supraglottis or subglottis; or impaired true vocal cord mobility Two sites, with normal true vocal cord mobility
T3 True vocal cord fixation True vocal cord fixation, or extension to preepiglottic space, medial pyriform sinus, or postcricoid area
T4 Extralaryngeal (eg, invasion of thyroid cartilage or strap muscles)
N1
N0 No nodes (includes information from physical examination, CT and MRI scans, and positron emission tomography)
N1 Ipsilateral node < 3 cm
N2a Ipsilateral node ≥ 3 cm but < 6 cm
N2b Ipsilateral multiple nodes, all < 6 cm
N2c Bilateral (or contralateral nodes only) < 6 cm
N3 Node(s) ≥ 6 cm
1N staging is the same for all head and neck squamous cell carcinoma except nasopharyngeal carcinoma.

For more advanced tumors, cisplatin-based chemoradiation protocols are used. Twenty-five years ago, total laryngectomy was often recommended for such patients. However, the 1994 VA study (with induction cisplatin and 5-fluorouracil followed by irradiation alone in responders) demonstrated that two-thirds of patients could preserve their larynx. Since that study, cisplatin-based chemotherapy concomitant with radiation therapy has been shown to be superior to either irradiation alone or induction chemotherapy followed by radiation. However, chemoradiation is associated with prolonged gastrostomy-dependent dysphagia. This high rate of chemoradiation-associated dysphagia has prompted a reevaluation of the role of extended, but less-than-total, laryngeal surgery for selected advanced laryngeal carcinoma in which at least one cricoarytenoid unit is intact. Supracricoid laryngectomy (with cricoepiglottohyoidpexy for glottic tumors or cricohyoidpexy for supraglottic tumors) as well as endoscopic resection of selected more advanced tumors, should be discussed as an alternative to chemoradiation. Supracricoid laryngectomy and chemoradiation have similar cure rates; the risk of permanent tracheostoma may be less with supracricoid laryngectomy, but the voice quality may be superior without surgery. Patient comorbidities and patient choice, after thorough discussion, play an important role in the choice between surgery and chemoradiation. A patient and his or her physicians must carefully consider different side effects and complications associated with different treatment modalities. These include the risks of chemotherapy, the likelihood of avoiding irradiation side effects, the likelihood of surgical complications, the likelihood of avoiding a permanent tracheostoma, the quality of posttreatment voice, and the likelihood of cure.

The presence of malignant adenopathy in the neck affects the prognosis greatly. Supraglottic tumors metastasize early and bilaterally to the neck, and this must be included in the treatment plans even when the neck is apparently uninvolved. Glottic tumors in which the TVCs are mobile have less than a 5% rate of nodal involvement; when a cord is immobile, the rate of ipsilateral nodal involvement climbs to about 30%. An involved neck is treated by surgery or chemoradiation, or both. This decision will depend on the treatment chosen for the larynx and the extent of neck involvement.

Total laryngectomy is largely reserved for far-advanced resectable tumors with extralaryngeal spread, those with persistent tumor following chemoradiation, and for recurrent disease. Voice rehabilitation via a primary (or at times secondary) tracheoesophageal puncture produces good speech in about 75–85% of patients. Indwelling prostheses that are changed every 3–6 months are a common alternative to patient-inserted prostheses, which need changing more frequently.

Long-term follow-up is critical in head and neck cancer patients. In addition to the 3–4% annual rate of second tumors and monitoring for recurrence, psychosocial aspects of treatment are common. Dysphagia, impaired communication, and altered appearance, may result in patient difficulties adapting to the workplace and

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to social interactions. In addition, smoking cessation and alcohol abatement are common challenges. Nevertheless, about 65% of patients with larynx cancer are cured, most have useful speech, and many resume their prior livelihoods, albeit with adaptations.

Bernier J et al: Postoperative irradiation with or without concomitant chemotherapy for locally advanced head and neck cancer. N Eng J Med 2004;350:1945.

Forastiere AA et al: Concurrent chemotherapy and radiotherapy for organ preservation in advanced laryngeal cancer. N Engl J Med 2003;349:2091.

Gallo A et al: Supracricoid partial laryngectomy in the treatment of laryngeal cancer: univariate and multivariate analysis of prognostic factors. Arch Otolaryngol Head Neck Surg 2005;131:620.

Ganly I et al: Results of surgical salvage after failure of definitive radiation therapy for early-stage squamous cell carcinoma of the glottic larynx. Arch Otolaryngol Head Neck Surg 2006;132:59.

Gilbert J et al: Organ preservation for cancer of the larynx: current indications and future directions. Semin Radiat Oncol 2004;14:167.

Hanna E et al: Quality of life for patients following total laryngectomy vs chemoradiation for laryngeal preservation. Arch Otolaryngol Head Neck Surg 2004;130:875.

Jones AS et al: The treatment of early laryngeal cancers (T1-T2 N0): surgery or irradiation? Head Neck 2004;26:127.

Loughran S et al: Quality of life and voice following endoscopic resection or radiotherapy for early glottic cancer. Clin Otolaryngol 2005;30:42.

Mendenhall WM et al: Management of T1-T2 glottic carcinomas. Cancer 2004;100:1786.

Sessions DG et al: Supraglottic laryngeal cancer: analysis of treatment results. Laryngoscope 2005;115:1402.

Smith JC et al: Quality of life, functional outcome, and costs of early glottic cancer. Laryngoscope 2003;113:68.

van Gogh CD et al: The efficacy of voice therapy in patients after treatment for early glottic carcinoma. Cancer 2006;106:95.

Yamazaki H et al: Radiotherapy for early glottic carcinoma (T1N0M0): results of prospective randomized study of radiation fraction size and overall treatment time. Int J Radiat Oncol Biol Phys 2006;64:77.

Vocal Cord Paralysis

Most cases of vocal cord paralysis result from involvement of a recurrent laryngeal nerve; others arise more proximally along the vagus nerve itself. Common causes of recurrent laryngeal nerve involvement include thyroid surgery (and occasionally thyroid cancer), other neck surgery (anterior discectomy and carotid endarterectomy), and mediastinal or apical involvement by lung cancer. Skull base tumors often involve cranial nerves IX, X, and XI. Occasionally, no cause can be identified. When either no cause is found or the paresis follows surgical trauma in which the nerve was not divided, spontaneous recovery commonly occurs, usually within a year.

Unlike unilateral cord paralysis, which produces a breathy hoarseness, bilateral cord paralysis usually causes inspiratory and expiratory stridor if acute, in which case intervention to create an emergency airway may be needed. If insidious in onset, it may be asymptomatic at rest, including a normal voice, although there is usually dyspnea on exertion. Causes of bilateral cord paralysis include thyroid surgery, esophageal cancer, and ventricular shunt malfunction. Unilateral or bilateral cord immobility may also be seen in cricoarytenoid arthritis secondary to advanced rheumatoid arthritis, intubation injuries, glottic and subglottic stenosis and, of course, laryngeal cancer. The goal of intervention is the creation of a safe airway with minimal reduction in voice quality and airway protection from aspiration. A number of cord lateralization procedures have been advocated as a means of removing the tracheotomy tube.

Surgical management of persistent or irrecoverable symptomatic unilateral vocal cord paralysis has evolved over the last several decades. The primary goal is medialization of the paralyzed cord in order to rehabilitate the voice. Additional goals include eliminating aspiration, improving diet, and aiding in the subsequent decannulation of individuals with glottic insufficiency. Success has been reported for years with injection medialization using predominantly Teflon, but other materials as well have been used, such as fat or Gelfoam. An alternative to injection medialization of the vocal fold is to medialize the soft tissue and arytenoid via an endoscopically monitored transcutaneous injection or a small incision overlying the thyroid cartilage under local anesthesia. A section of the cartilage is removed, providing access to the soft tissue of the larynx and the arytenoid cartilage. An implant is inserted that displaces this soft tissue and arytenoid medially.

Abraham MT et al: Type I thyroplasty for acute unilateral vocal fold paralysis following intrathoracic surgery. Ann Otol Rhinol Laryngol 2002;111:667.

Baron EM et al: Dysphagia, hoarseness, and unilateral true vocal fold motion impairment following anterior cervical diskectomy and fusion. Ann Otol Rhinol Laryngol 2003;112:9.

Bhattacharyya N et al: Dysphagia and aspiration with unilateral vocal cord immobility: incidence, characterization, and response to surgical treatment. Ann Otol Rhinol Laryngol 2002;111:672.

Jung A et al: Recurrent laryngeal nerve palsy during anterior cervical spine surgery: a prospective study. J Neurosurg Spine 2005;2:123.

Nayak VK et al: Patterns of swallowing failure following medialization in unilateral vocal fold immobility. Laryngoscope 2002;112:1840.

Ollivere B et al: Swallowing dysfunction in patients with unilateral vocal fold paralysis: aetiology and outcomes. J Laryngol Otol 2006;120:38.

Urquhart AC et al: Idiopathic vocal cord palsies and associated neurological conditions. Arch Otolaryngol Head Neck Surg 2005;131:1086.

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Tracheostomy & Cricothyrotomy

There are two primary indications for tracheostomy: airway obstruction at or above the level of the larynx and respiratory failure requiring prolonged mechanical ventilation. In an acute emergency, cricothyrotomy secures an airway more rapidly than tracheostomy, with fewer potential immediate complications such as pneumothorax and hemorrhage. Although classically it has been recommended that one should change a cricothyrotomy to a tracheostomy as soon as it is convenient and safe, recent studies have questioned this if decannulation can be expected soon. Percutaneous dilation tracheostomy as an elective bedside (or intensive care unit) procedure has undergone scrutiny in recent years as an alternative to tracheostomy. In experienced hands, the various methods of percutaneous dilation tracheostomy have been documented to be safe, although complications do occur. When a patient is not intubated, simultaneous bronchoscopy may reduce complications. The major cost reduction comes from not using the main operating room. Bedside tracheostomy (in the intensive care unit) achieves similar cost reduction and is advocated by some as slightly less costly than the percutaneous dilation procedure.

The most common indication for elective tracheostomy is the need for prolonged mechanical ventilation. There is no firm rule about how many days a patient must be intubated before conversion to tracheostomy should be advised. The incidence of serious complications such as subglottic stenosis increases with extended endotracheal intubation. As soon as it is apparent that the patient will require protracted ventilatory support, tracheostomy should replace the endotracheal tube. Less frequent indications for tracheostomy are life-threatening aspiration pneumonia, the need to improve pulmonary toilet to correct problems related to insufficient clearing of tracheobronchial secretions, and sleep apnea.

Posttracheostomy care requires humidified air to prevent secretions from crusting and occluding the inner cannula of the tracheostomy tube. The tracheostomy tube should be cleaned several times daily. The most frequent early complication of tracheostomy is dislodgment of the tracheostomy tube. Surgical creation of an inferiorly based tracheal flap sutured to the inferior neck skin may make reinsertion of a dislodged tube easier. It should be recalled that the act of swallowing requires elevation of the larynx, which is prevented by tracheostomy. Therefore, frequent tracheal and bronchial suctioning is often required to clear the aspirated saliva as well as the increased tracheobronchial secretions. Care of the skin around the stoma is important to prevent maceration and secondary infection.

Durbin CG Jr: Early complications of tracheostomy. Respir Care 2005;50:511.

Epstein SK: Late complications of tracheostomy. Respir Care 2005;50:542.

Homewood J et al: Tracheostomy care. Br J Hosp Med (Lond) 2005;66:M72.

Melker JS et al: Melker cricothyrotomy kit: an alternative to the surgical technique. Ann Otol Rhinol Laryngol 2005;114: 525.

Thatcher GW et al: The long-term evaluation of tracheostomy in the management of severe obstructive sleep apnea. Laryngoscope 2003;113:201.

Foreign Bodies in the Upper Aerodigestive Tract

Foreign Bodies of the Trachea & Bronchi

Aspiration of foreign bodies occurs less frequently in adults than in children. The elderly and denture wearers appear to be at greatest risk. Wider familiarity with the Heimlich maneuver has reduced deaths. If the maneuver is unsuccessful, cricothyrotomy may be necessary. Plain chest radiographs may reveal a radiopaque foreign body. Detection of radiolucent foreign bodies may be aided by inspiration-expiration films that demonstrate air trapping distal to the obstructed segment. Atelectasis and pneumonia may occur later.

Tracheal and bronchial foreign bodies should be removed under general anesthesia by a skilled endoscopist working with an experienced anesthesiologist.

Eroglu A et al: Tracheobronchial foreign bodies: a 10 year experience. Ulus Travma Derg 2003;9:262.

Swanson KL et al: Tracheobronchial foreign bodies. Chest Surg Clin N Am 2001;11:861.

Tariq SM et al: Inhaled foreign bodies in adolescents and adults. Monaldi Arch Chest Dis 2005;63:193.

Esophageal Foreign Bodies

Foreign bodies in the esophagus create urgent but not life-threatening situations as long as the airway is not compromised. There is probably time to consult an experienced clinician for management. Patients are likely to have difficulty handling secretions and may be spitting out their saliva. It is a useful diagnostic sign of complete obstruction if the patient is drooling or cannot handle secretions. They may often point to the exact level of the obstruction. Indirect laryngoscopy often shows pooling of saliva at the esophageal inlet. Plain films may detect radiopaque foreign bodies such as chicken bones. Coins tend to align in the coronal plane in the esophagus and sagittally in the trachea. If a foreign

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body is suspected, a barium swallow may help make the diagnosis.

The treatment of an esophageal foreign body depends very much on identification of its nature. In children, swallowed nonfood objects are common. In adults, however, food foreign bodies are more common, and there is the greater possibility of underlying esophageal pathology. Endoscopic removal and examination is usually best, via flexible esophagoscopy or rigid laryngoscopy-esophagoscopy. If there is nothing sharp such as a bone, some clinicians advocate a hospitalized 24-hr observation period prior to esophagoscopy, noting that spontaneous passage of the foreign body will occur in 50% of adult patients. Obstruction may sometimes occur from a metal stent placed in the treatment of esophageal cancer.

Athanassiadi K et al: Management of esophageal foreign bodies: a retrospective review of 400 cases. Eur J Cardiothorac Surg 2002;21:653.

Lam HC et al: Management of ingested foreign bodies: a retrospective review of 5240 patients. J Laryngol Otol 2001; 115:954.

Mosca S et al: Endoscopic management of foreign bodies in the upper gastrointestinal tract: report on a series of 414 adult patients. Endoscopy 2001;33:692.

Tsikoudas A et al: The management of acute oesophageal obstruction from a food bolus. Can we be more conservative? Eur Arch Otorhinolaryngol 2005;262:528.

Diseases Presenting as Neck Masses

The differential diagnosis of neck masses is heavily dependent on the location in the neck, the age of the patient, and the presence of associated disease processes. Rapid growth and tenderness suggest an inflammatory process, while firm, painless, and slowly enlarging masses are often neoplastic. In young adults, most neck masses are benign (branchial cleft cyst, thyroglossal duct cyst, reactive lymphadenitis), although malignancy should always be considered (lymphoma, metastatic thyroid carcinoma). Lymphadenopathy is common in HIV-positive persons, but a growing or dominant mass may well be malignant. In adults over age 40, cancer is the most common cause of persistent neck mass. A metastasis from SCC arising within the mouth, pharynx, larynx, or upper esophagus should be suspected, especially if there is a history of tobacco or significant alcohol use. Especially among patients younger than 30 or older than 70, lymphoma should be considered. In any case, a comprehensive otolaryngologic examination is needed. Cytologic evaluation of the neck mass via FNA biopsy is likely to be the next step if an obvious primary tumor is not visible or palpable on physical examination.

Congenital Lesions Presenting as Neck Masses in Adults

1. Branchial Cleft Cysts

Branchial cleft cysts usually present as a soft cystic mass along the anterior border of the sternocleidomastoid muscle. These lesions are usually recognized in the second or third decades of life, often when they suddenly swell or become infected. To prevent recurrent infection and possible carcinoma, they should be completely excised, along with their fistulous tracts.

First branchial cleft cysts present high in the neck, sometimes just below the ear. A fistulous connection with the floor of the external auditory canal may be present. Second branchial cleft cysts, which are far more common, may communicate with the tonsillar fossa. Third branchial cleft cysts, which may communicate with the piriform sinus, are rare.

Bloch R: Images in emergency medicine. Branchial cleft cyst. Ann Emerg Med 2006;47:291, 308.

Enepekides DJ: Management of congenital anomalies of the neck. Facial Plast Surg Clin North Am 2001;9:131.

Eskey CJ et al: Imaging of benign and malignant soft tissue tumors of the neck. Radiol Clin North Am 2000;38: 1091.

Palacios E et al: Branchial cleft cyst. Ear Nose Throat J 2001; 80:302.

Prakash PK et al: Differential diagnosis of neck lumps. Practitioner 2002;246:252.

Schwetschenau E et al: The adult neck mass. Am Fam Physician 2002;66:831.

2. Thyroglossal Duct Cysts

Thyroglossal duct cysts occur along the embryologic course of the thyroid's descent from the tuberculum impar of the tongue base to its usual position in the low neck. Although they may occur at any age, they are most common before age 20. They present as a midline neck mass, often just below the hyoid bone, which moves with swallowing. Surgical excision is recommended to prevent recurrent infection. This requires removal of the entire fistulous tract along with the middle portion of the hyoid bone.

Ahuja AT et al: Imaging for thyroglossal duct cyst: the bare essentials. Clin Radiol 2005;60:141.

Dedivitis RA et al: Thyroglossal duct: a review of 55 cases. J Am Coll Surg 2002;194:274.

Ewing CA et al: Presentations of thyroglossal duct cysts in adults. Eur Arch Otorhinolaryngol 1999;256:136.

Mohan PS et al: Thyroglossal duct cysts: a consideration in adults. Am Surg 2005;71:508.

Infectious & Inflammatory Neck Masses

1. Reactive Cervical Lymphadenopathy

Normal lymph nodes in the neck are usually less than 1 cm in length. Infections involving the pharynx, salivary

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glands, and scalp often cause tender enlargement of neck nodes. Enlarged nodes are common in HIV-infected persons. Except for the occasional node that suppurates and requires incision and drainage, treatment is directed against the underlying infection. An enlarged node unassociated with an obvious infection should be further evaluated, especially if the patient has a history of smoking or alcohol use (common etiologic factors in head and neck SCC) or a history of cancer. Other common indications for FNA biopsy of a node include its persistence or continued enlargement. Common causes of cervical adenopathy include tumor (SCC, lymphoma, occasional metastases from non-head and neck sites) and infection (eg, reactive nodes, mycobacteria [discussed below], and cat scratch disease). Rare causes of adenopathy include Kikuchi's disease (histiocytic necrotizing lymphadenitis) and autoimmune adenopathy.

Murakami K et al: Cat scratch disease: analysis of 130 seropositive cases. J Infect Chemother 2002;8:349.

Ridder GJ et al: Role of cat-scratch disease in lymphadenopathy in the head and neck. Clin Infect Dis 2002;35:643.

2. Tuberculous & Nontuberculous Mycobacterial Lymphadenitis

Granulomatous neck masses are not uncommon. The differential diagnosis includes mycobacterial adenitis, sarcoidosis, and cat-scratch disease due to Bartonella henselae. Although mycobacterial adenitis can extend to the skin and drain externally (as described for atypical mycobacteria and referred to as scrofula), this late presentation is no longer common. The usual presentation of granulomatous disease in the neck is simply single or matted nodes. FNA biopsy is usually the best initial diagnostic approach: cytology, smear for acid-fast bacilli, culture, and sensitivity test; and polymerase chain reaction (PCR) can all be done.

Mycobacterial lymphadenitis is on the rise both in immunocompromised and immunocompetent individuals. Identification of Mycobacterium tuberculosis can usually be confirmed by a combination of FNA smear and culture, but excisional biopsy of a node may be needed. PCR from FNA (or from excised tissue) is the single most sensitive test and is particularly useful when conventional methods have not been diagnostic but clinical impression remains consistent for tuberculous infection.

Short-course therapy (6 months) consisting of an initial 4 months of streptomycin, isoniazid, rifampin, and pyrazinamide followed by 2 months of rifampin is the current recommended treatment for tuberculous lymphadenopathy. For atypical (nontuberculous) lymphadenopathy, treatment depends on sensitivity results of culture, but antibiotics likely to be useful include 6 months of isoniazid and rifampin and, for at least the first 2 months, ethambutol—all in standard dosages (see Table 9-14). Some would totally excise the involved nodes prior to chemotherapy, depending on location and other factors.

Golden MP et al: Extrapulmonary tuberculosis: an overview. Am Fam Physician 2005;72:1761.

Jawahar MS et al: Treatment of lymph node tuberculosis—a randomized clinical trial of two 6-month regimens. Trop Med Int Health 2005;10:1090.

Koo V et al: Fine needle aspiration cytology (FNAC) in the diagnosis of granulomatous lymphadenitis. Ulster Med J 2006; 75:59.

Pahwa R et al: Assessment of possible tuberculous lymphadenopathy by PCR compared to non-molecular methods. J Med Microbiol 2005;54:873.

Polesky A et al: Peripheral tuberculous lymphadenitis: epidemiology, diagnosis, treatment, and outcome. Medicine (Baltimore) 2005;84:350.

3. Lyme Disease

Lyme disease, caused by the spirochete Borrelia burgdorferi and transmitted by ticks of the Ixodes genus, may have protean manifestation, but over 75% of patients have symptoms involving the head and neck. Facial paralysis, dysesthesias, dysgeusia, or other cranial neuropathies are most common. Headache, pain, and cervical lymphadenopathy may occur. See Chapter 34 for a more thorough discussion.

Bunikis J et al: Laboratory testing for suspected Lyme disease. Med Clin North Am 2002;86:311.

DePietropaolo DL et al: Diagnosis of lyme disease. Am Fam Physician 2005;72:297.

Ljostad U et al: Acute peripheral facial palsy in adults. J Neurol 2005;252:672.

Lorenzi MC et al: Sudden deafness and Lyme disease. Laryngoscope 2003;113:312.

Steere AC: Lyme disease. N Engl J Med 2001;345:115.

Tumor Metastases

In older adults, 80% of firm, persistent, and enlarging neck masses are metastatic in origin. The great majority of these arise from SCC of the upper aerodigestive tract. A complete head and neck examination may reveal the tumor of origin, but examination under anesthesia with direct laryngoscopy, esophagoscopy, and bronchoscopy is usually required to fully evaluate the tumor and exclude second primaries.

It is often helpful to obtain a cytologic diagnosis if initial head and neck examination fails to reveal the primary tumor. An open biopsy should be done only when neither physical examination by an experienced clinician specializing in head and neck cancer nor FNA biopsy performed by an experienced cytopathologist yields a diagnosis. In such a setting, one should strongly consider obtaining an MRI or PET scan prior to open biopsy, as these methods may yield valuable information about a possible presumed primary site or another site for FNA.

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Other than thyroid carcinoma, non-squamous cell metastases to the neck are infrequent. While tumors not involving the head and neck seldom metastasize to the middle or upper neck, the supraclavicular region is quite often involved by lung and breast tumors. Infradiaphragmatic tumors, with the exception of renal cell carcinoma, rarely metastasize to the neck.

Barzilai G et al: Pattern of regional metastases from cutaneous squamous cell carcinoma of the head and neck. Otolaryngol Head Neck Surg 2005;132:852.

Brockstein B et al: Patterns of failure, prognostic factors and survival in locoregionally advanced head and neck cancer treated with concomitant chemoradiotherapy: a 9-year, 337-patient, multi-institutional experience. Ann Oncol 2004;15:1179.

Cooper JS et al: Postoperative concurrent radiotherapy and chemotherapy for high-risk squamous-cell carcinoma of the head and neck. N Engl J Med 2004;305:1937.

Moore BA et al: Lymph node metastases from cutaneous squamous cell carcinoma of the head and neck. Laryngoscope 2005;115:1561.

Piccirillo JF et al: Development of a new head and neck cancer-specific comorbidity index. Arch Otolaryngol Head Neck Surg 2002;128:1172.

Lymphoma

About 10% of lymphomas present in the head and neck. Lymphoma arising in AIDS patients is an increasing concern. Multiple rubbery nodes, especially in the young adult, are suggestive of this disease. A thorough physical examination may demonstrate other sites of nodal or organ involvement. FNA biopsy may be diagnostic, but open biopsy is often required.

Otolaryngologic Manifestations of Hiv Infection (See Also Chapter 31)

Oral Cavity & Pharynx

The evaluation of oral lesions is critically important in HIV-infected individuals and in patients at risk for HIV infection. Oral candidiasis and hairy leukoplakia, each occurring in 10–20% of HIV-infected patients, are frequently the presenting signs of HIV disease. Kaposi's sarcoma (prevalence about 1%) may similarly be the first indication of HIV infection—as may necrotizing ulcerative periodontitis (occurring in 2–5%), but less predictively. The course of candidiasis and hairy leukoplakia in known HIV-infected patients may correlate with the degree of immune suppression and overall disease progression, heralding the subsequent development of AIDS. For these reasons, oral lesions are useful in staging HIV disease and in designing entry criteria and end points for antiretroviral clinical trials. The United States Department of Health Services Clinical Practice Guideline for Evaluation and Management of Early HIV Infection recommends examination of the oral mucosa with each physician visit as well as dental examination at least every 6 months.

When CD4 cell counts drop below 200/mcL, the incidence of intraoral lesions rises dramatically. In the past few years, the severity of these lesions has lessened, but their incidence has increased. Candidiasis is common and may require treatment for longer than the usual 1-week course with fluconazole (100 mg daily) or ketoconazole (200–400 mg daily). Clotrimazole and topical nystatin are less effective. Itraconazole (200 mg daily) is often helpful in fluconazole-resistant cases, or some cases due to non-albicans species, which are frequently azole-unresponsive. Giant intraoral ulcers have been seen in some patients.

Hairy leukoplakia occurring on the lateral border of the tongue is another common early finding. It may develop quickly and appears as slightly raised leukoplakic areas with a corrugated or “hairy” surface. Histologically, parakeratosis and koilocytes are seen with little or no underlying inflammation. Among HIV-positive patients with oral lesions, hairy leukoplakia was seen in 19% in one study. Clinical response following administration of zidovudine or acyclovir has been reported, and treatment is under active investigation. The appearance of hairy leukoplakia may herald subsequent more ominous manifestations of AIDS.

Kaposi's sarcoma is most common on the hard palate but may be seen anywhere in the oral cavity and pharynx. It usually appears as a raised violaceous lesion beneath an intact mucosa, although it may be ulcerated, erythematous, and bleeding. Radiation therapy may control the tumor. A brisk mucositis can be expected following radiation therapy.

In addition to Kaposi's sarcoma, an increased incidence of non-Hodgkin's lymphoma is seen in AIDS. An increase in SCC is also seen in the homosexual population, perhaps related to HIV infection.

Birnbaum W et al: Prognostic significance of HIV-associated oral lesions and their relation to therapy. Oral Dis 2002;8(Suppl 2):110.

Campo J et al: Oral candidiasis as a clinical marker related to viral load, CD4 lymphocyte count and CD4 lymphocyte percentage in HIV-infected patients. J Oral Pathol Med 2002; 31:5.

Cattaneo C et al: Oral cavity lymphomas in immunocompetent and human immunodeficiency virus infected patients. Leuk Lymphoma 2005;46:77.

Challacombe SJ et al: Overview of the Fourth International Workshop on the Oral Manifestations of HIV Infection. Oral Dis 2002;8(Suppl 2):9.

Coogan MM et al: Oral lesions in infection with human immunodeficiency virus. Bull World Health Organ 2005;83:700.

de Faria PR et al: Tongue disease in advanced AIDS. Oral Dis 2005;11:72.

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Eyeson JD et al: Oral manifestations of an HIV positive cohort in the era of highly active anti-retroviral therapy (HAART) in South London. J Oral Pathol Med 2002;31:169.

Kroidl A et al: Prevalence of oral lesions and periodontal diseases in HIV-infected patients on antiretroviral therapy. Eur J Med Res 2005;10:448.

Patton LL et al: Prevalence and classification of HIV-associated oral lesions. Oral Dis 2002;8(Suppl 2):98.

Ramirez-Amador V et al: Synchronous kinetics of CD4+ lymphocytes and viral load before the onset of oral candidosis and hairy leukoplakia in a cohort of Mexican HIV-infected patients. AIDS Res Hum Retroviruses 2005;21:981.

Singh B et al: The epidemiology of the oral lesions of HIV infection in the developed world. Oral Dis 2002;8(Suppl 2):34.

The Neck

Persistent generalized lymphadenopathy is extremely common in HIV infection. A tender or growing node may represent secondary infection, lymphoma, or other tumor. FNA for culture and cytology is the best initial diagnostic step. Open biopsy will often be needed if granulomatous disease or lymphoma is suspected, although FNA biopsy may be diagnostic of M tuberculosis infection in seropositive patients.

Parotid cysts and benign lymphoepithelial lesions in HIV-positive patients may be seen, often in association with cervical adenopathy.

Diamond C et al: Changes in acquired immunodeficiency syndrome-related non-Hodgkin lymphoma in the era of highly active antiretroviral therapy: incidence, presentation, treatment, and survival. Cancer 2006;106:128.

Owotade FJ et al: Clinical experience with parotid gland enlargement in HIV infection: a report of five cases in Nigeria. J Contemp Dent Pract 2005;6:136.

Paranasal Sinuses

Sinusitis is common in HIV infection and the causative organisms are diverse. The same pathogens encountered in nonimmunocompromised patients remain the most common. Early sinus irrigation, with aspirates sent for cytologic examination as well as fungal, viral, Legionella, and aerobic and anaerobic culture may be helpful in severe cases. Guaifenesin (600 mg orally four times daily), a mucolytic agent, may offer some adjunctive symptomatic relief. Functional endoscopic surgery to provide sinus drainage is often helpful.

Invasive Aspergillus sinusitis is an increasingly reported complication in AIDS. Although this infection is more indolent than mucormycosis, most patients with AIDS and Aspergillus sinusitis die as a result of intracranial extension.

Rosen EJ et al: Alterations of nasal mucociliary clearance in association with HIV infection and the effect of guaifenesin therapy. Laryngoscope 2005;115:27.

Scheid DC et al: Head and neck manifestations of HIV infection: a preliminary study. J Indian Med Assoc 2003;101:93.