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OTOLARYNGOLOGY DISORDERS
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especially those with existing hearing losses, should wear earplugs when exposed to moderately loud noises and spe­cially designed earmuffs when exposed to explosive noises.
Chen XM et al. The role of genetic variants in the susceptibility
of noise-induced hearing loss. Front Cell Neurosci. 2022;16:946206. [PMID: 35903368]
Fischer T et al. Are smartwatches a suitable tool to monitor noise
exposure for public health awareness and otoprotection? Front Neurol. 2022;13:856219. [PMID: 35432148]
Le Prell CG et al. Noise-induced hearing loss and its prevention:
current issues in mammalian hearing. Curr Opin Physiol. 2020;18:32. [PMID: 32984667]
C. Physical Trauma
Concussive head trauma has effects on the inner ear simi­lar to those of severe acoustic trauma. Some degree of sensory hearing loss may occur following concussion and is frequent after lateral skull base fracture.
Bartholomew RA … Kozin ED et al. Labyrinthine concussion:
historic otopathologic antecedents of a challenging diagnosis. Laryngoscope Investig Otolaryngol. 2020;5:267. [PMID: 32337358]
D. Ototoxicity
Ototoxic substances may affect both the auditory and ves­tibular systems. The most commonly used ototoxic medi­cations are aminoglycosides; loop diuretics; and several antineoplastic agents, notably cisplatin. These medications
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 kidney disease. 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, monitoring of serum peak and trough levels, and substitution of equivalent nonototoxic medications when­ever possible.
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.
Correa-Morales JE et al. Prevention and treatment of cisplatin-
induced ototoxicity in adults: a systematic review. Clin
Otolaryngol. 2024;49:1. [PMID: 37818931]
Dillard LK et al. Global burden of ototoxic hearing loss associ-
ated with platinum-based cancer treatment: a systematic
review and meta-analysis. Cancer Epidemiol. 2022;79:102203.
[PMID: 35724557]
Tan WJT et al. Molecular characteristics of cisplatin-induced
ototoxicity and therapeutic interventions. Int J Mol Sci.
2023;24:16545. [PMID: 38003734]
E. Idiopathic Sudden Sensory Hearing Loss
Idiopathic sudden loss of hearing in one ear may occur at any age, but typically it occurs in persons over age 20 years. In the setting a normal otologic physical examination,
symptoms may include hearing loss, aural fullness, tinni­tus, and dizziness. The cause is unknown; however, idio­pathic sudden hearing loss may result from a viral infection or a sudden vascular occlusion of the internal auditory artery. Obtaining an MRI is essential after the diagnosis to rule out retrocochlear pathology (eg, tumors); however, this should not delay treatment. Prompt treatment with corticosteroids has been shown to improve the odds of recovery. Intratympanic administration of corticosteroids alone or in association with oral corticosteroids has been associated with an equal or more favorable prognosis. Because treatment appears to be most effective as close to the onset of the loss as possible, and appears not to be effec­tive after 6 weeks, a prompt audiogram should be obtained
in all patients who present with sudden hearing loss without obvious middle ear pathology. Prognosis is mixed, with
many patients living with permanent deafness in the involved ear, while others have complete recovery.
Chaushu H et al. Spontaneous recovery rate of idiopathic sudden
sensorineural hearing loss: a systematic review and meta­analysis. Clin Otolaryngol. 2023;48:395. [PMID: 36640119]
Chrysouli K et al. The effectiveness of intratympanic steroid
injection in addition to systemic corticosteroids in the treat­ment of idiopathic sudden sensorineural hearing loss. Am J Otolaryngol. 2023;44:103872. [PMID: 37060782]
Yoon CY et al. Epidemiology of idiopathic sudden sensorineural
hearing loss in the era of big data. Eur Arch Otorhinolaryn­gol. 2023;280:2181. [PMID: 36239782]
F. Autoimmune Hearing Loss
Sensorineural hearing loss that occurs in both ears simulta­neously may be associated with a wide array of systemic autoimmune disorders, such as SLE, granulomatosis with polyangiitis, and Cogan syndrome (hearing loss, keratitis, aortitis). The loss is most often progressive. The hearing level often fluctuates, with periods of deterioration alternat­ing with partial or even complete remission. Usually, there is the gradual evolution of permanent hearing loss, which often stabilizes with some remaining auditory function but occasionally proceeds to complete deafness. Vestibular dys­function, particularly dysequilibrium and postural instabil­ity, may accompany the auditory symptoms.
In many cases, the autoimmune pattern of audioves­tibular dysfunction presents in the absence of recognized systemic autoimmune disease. 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.
Balouch B et al. Use of biologics for treatment of autoimmune
inner ear disease. Am J Otolaryngol. 2022;43:103576. [PMID:
35963108]
Gordis TM et al. Disease-modifying antirheumatic drugs in the
treatment of autoimmune inner ear disease: a systematic
review and meta-analysis of auditory and vestibular out-
comes. Otol Neurotol. 2023;44:2. [PMID: 36509432]
Yuen E et al. Hearing loss in patients with systemic lupus erythe-
matosus: a systematic review and meta-analysis. Lupus.
2021;30:937. [PMID: 33645314]
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CHAPTER 8
2. Tinnitus
ESSENTIALS OF DIAGNOSIS
»
Phantom noise or sounds.
»
Persistent tinnitus often, although not always, indicates the presence of hearing loss.
»
Intermittent periods of mild, high-pitched tinnitus lasting seconds to minutes are common in nor­mal-hearing persons.
» General Considerations
Tinnitus is defined as the sensation of sound in the absence of an exogenous sound source. Tinnitus can accompany any form of hearing loss, and its presence provides no diag­nostic value in determining the cause of a hearing loss. Approximately 15% of the general population experiences some type of tinnitus, with prevalence beyond 20% in aging populations.
» Clinical Findings
A. Symptoms and Signs
Though tinnitus is commonly associated with hearing loss, tinnitus severity correlates poorly with the degree of hear­ing loss. About one in seven tinnitus sufferers experiences severe annoyance, and 4% are severely disabled. When severe and persistent, tinnitus may interfere with sleep and ability to concentrate, resulting in considerable psychologi­cal distress.
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, pulsatile tinnitus may be far more serious and may indicate a vascular abnormality, such as glomus tumor, venous sinus stenosis, carotid vaso-occlusive disease, arteriovenous malformation, or aneurysm.
A staccato “clicking” tinnitus may result from middle ear muscle spasm (middle ear myoclonus) or sometimes 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. Specialized forms of tympanometry may formally diagnose this condi­tion, and it is typically treated surgically.
B. Diagnostic Testing
For routine, nonpulsatile tinnitus, audiometry should be ordered to rule out an associated hearing loss. For unilat­eral tinnitus, particularly associated with hearing loss in the absence of an obvious causative factor (ie, noise trauma), an MRI should be obtained to rule out a retroco­chlear lesion, such as vestibular schwannoma. MRA and MRV and temporal bone CT should be considered for
patients who have pulsatile tinnitus to exclude a causative vascular lesion or sigmoid sinus abnormality.
» Treatment
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. In addition to masking tech­niques, habituation techniques, such as tinnitus retraining therapy and cognitive behavioral therapy, may prove benefi­cial in those with refractory symptoms. Among patients who have emotional distress due to tinnitus, numerous antide­pressant and antipsychotic medications have been tried. Unfortunately, these medications do not treat the tinnitus directly but may allow the patient to cope with it better.
Conlon B et al. Different bimodal neuromodulation settings
reduce tinnitus symptoms in a large randomized trial. Sci Rep. 2022;12:10845. [PMID: 35773272]
Perrotta MV et al. Bimodal stimulation for the reduction of tin-
nitus using vibration on the skin. Int Tinnitus J. 2023;27:1. [PMID: 38050877]
3. Hyperacusis
Excessive sensitivity to sound may occur following hearing loss, such as that due to noise trauma, in patients suscep­tible to migraines, or for psychological reasons. 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.
Kim EH et al. Exploring the origins of decreased sound tolerance
in tinnitus patients. Front Neurol. 2023;14:1273705. [PMID: 38020634]
Pienkowski M. Loud music and leisure noise is a common cause
of chronic hearing loss, tinnitus and hyperacusis. Int J Envi­ron Res Public Health. 2021;18:4236. [PMID: 33923580]
Ren J et al. Prevalence of hyperacusis in the general and special
populations: a scoping review. Front Neurol. 2021;12:706555. [PMID: 34539554]
4. Vertigo
ESSENTIALS OF DIAGNOSIS
»
Either a sensation of motion when there is no motion or an exaggerated sense of motion in response to movement.
»
Duration of vertigo episodes with associated hear­ing loss or other neurologic issues are the keys to diagnosis.
»
Evaluation includes audiogram, electronystag­mography (ENG) or videonystagmography (VNG), and head MRI.
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» General Considerations
Vertigo can be caused by either a peripheral or central eti­ology, or both (Table 8–2).
» Clinical Findings
A. Symptoms and Signs
Vertigo is the cardinal symptom of vestibular disease. Ver­tigo is typically experienced as a distinct “spinning”
Table 8–2. Causes of vertigo (listed in alphabetical
order, within categories).
Peripheral causes
Benign paroxysmal positioning vertigo
Ethanol intoxication
Inner ear barotraumas
Ménière disease
Semicircular canal dehiscence
Vestibular neuritis/labyrinthitis
Central causes
Cerebellar ataxia syndromes
Chiari malformation
Multiple sclerosis
Seizure
Wernicke encephalopathy
Mixed central and peripheral causes
Cerebellopontine angle tumors
Vestibular schwannoma
Meningioma
Endocrinopathies
Hypothyroidism
Pendred syndrome
Hyperviscosity syndromes
Waldenström macroglobulinemia
Infections
Lyme disease
Syphilis
Migraine
Stroke and vascular insufficiency
Anterior inferior cerebellar artery stroke
Posterior inferior cerebellar artery stroke
Vasculitides
Behçet disease
Cogan syndrome
Granulomatosis with polyangiitis
Susac syndrome
Vertebral artery insufficiency
Vascular compression
Table 8–3. Common vestibular disorders: differential
diagnosis based on classic presentations.
Duration of
Typical
Vertiginous
Episodes
Seconds Perilymphatic
Hours Ménière disease,
Days Labyrinthitis,
Auditory
Symptoms
Present
fistula
syphilis
autoimmune inner ear disease, cerebellopontine angle tumor, ototoxicity
Auditory Symptoms
Absent
Benign paroxysmal
positioning vertigo (cupulolithiasis), vertebrobasilar insufficiency, migraine-associated vertigo
Migraine-associated
vertigo
Vestibular neuronitis,
migraine-associated vertigo, multiple sclerosis, cerebellar degeneration
sensation or a sense of tumbling or of falling forward or backward. It should be distinguished from imbalance, light-headedness, and syncope, all of which are nonvestib­ular in origin (Table 8–3).
1. Peripheral vestibular disease—Peripheral vestibulopa­thy may cause vertigo 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 (ie, otologic) origin.
Critical elements of the history include the duration of the discrete vertiginous episodes (seconds, minutes to hours, or days), and associated symptoms (hearing loss). Triggers should be sought, including diet (eg, increased salt intake in the case of Ménière disease), stress, fatigue, and bright lights (eg, migraine-associated dizziness).
The physical examination of the patient with vertigo includes evaluation of the ears, observation of eye motion and nystagmus in response to head turning, cranial nerve examination, and Romberg testing. In acute 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 CNS disease. In benign paroxysmal positioning vertigo, Dix-Hallpike testing (quickly lowering the patient to the supine position with the head extending over the edge and placed 30 degrees lower than the body, turned either to the left or right) will elicit a delayed-onset (~10 seconds) fatigable nystagmus. Nonfatigable nystagmus in this position indicates CNS disease.
Since visual fixation often suppresses observed nystag­mus, many of these maneuvers are performed with Frenzel goggles, which prevent visual fixation, and often bring out subtle forms of nystagmus. The Fukuda test can
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demonstrate vestibular asymmetry when the patient steps in place with eyes closed and consistently rotates in one direction.
2. Central disease—Vertigo arising from CNS disease (Table 8–2) tends to develop gradually and then becomes progressively more severe and debilitating. Nystagmus is not always present but can occur in any direction, may be dissociated in the two eyes, and is often non fatigable, verti­cal rather than horizontal in orientation, without latency, and unsuppressed by visual fixation. ENG is useful in documenting these characteristics. Evaluation of audioves­tibular dysfunction requires MRI of the brain.
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; it 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 tranquilizer medications or of alcohol.
Arshad Q et al. What visuospatial perception has taught us about
the pathophysiology of vestibular migraine. Curr Opin
Neurol. 2024;37:32. [PMID: 38018799]
Chari DA et al. Telemedicine algorithm for the management of
dizzy patients. Otolaryngol Head Neck Surg. 2020;163:857.
[PMID: 32600170]
Tarnutzer AA et al. What’s in a name? Chronic vestibular
migraine or persistent postural perceptual dizziness? Brain
Sci. 2023;13:1692. [PMID: 3813714]
B. Vestibular Testing
Vestibular investigations, such as audiologic evaluation, caloric stimulation, electro- or videonystagmography (ENG or VNG), vestibular-evoked myogenic potentials (VEMPs), and MRI, are indicated in patients with persis­tent vertigo or when CNS disease is suspected. These stud­ies help distinguish between central and peripheral lesions and identify causes requiring specific therapy. ENG 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.
Grove CR et al. Vestibular perceptual testing from lab to clinic: a
review. Front Neurol. 2023;14:1265889. [PMID: 37859653]
Pastras CJ et al. Vestibular testing- New physiological results for
the optimization of clinical VEMP stimuli. Audiol Res.
2023;13:910. [PMID: 37987337]
» Vertigo Syndromes Due to
Peripheral Lesions
A. Ménière Disease
The cause of Ménière disease is unknown. The classic syn­drome consists of episodic vertigo, with discrete vertigo spells lasting 20 minutes to several hours in association with fluctuating, often low-frequency, sensorineural hear­ing loss, tinnitus (usually low-tone and “blowing” in
quality), and a sensation of unilateral aural pressure (Table 8–3). These symptoms in presence of headaches or migraines may suggest migraine-associated dizziness. Pri­mary treatment is aimed at decreasing dizzy episodes. There are no treatments for reduction in hearing loss. Treatment of Ménière disease typically involves preventive measures, including low-salt diet and daily diuretics (eg, acetazolamide). For symptomatic relief of acute vertigo attacks, lorazepam (0.5–1 mg) or diazepam (2–5 mg) can be used. Nausea may be treated with oral meclizine (25 mg). In refractory cases, patients may undergo intra­tympanic corticosteroid or gentamicin injections, endo­lymphatic sac decompression, or surgical or vestibular nerve section. There are increasing reports of imaging, such as CT or MRI, for evaluation of Ménière disease.
Bächinger D et al. Radiological feature heterogeneity supports
etiological diversity among patient groups in Meniere’s disease. Sci Rep. 2023;13:10303. [PMID: 37365255]
B. Labyrinthitis
Patients with labyrinthitis suffer from acute onset of con­tinuous, usually severe vertigo lasting several days, accom­panied by hearing loss and tinnitus. During a recovery period that lasts for several weeks, the vertigo gradually improves. Hearing may return to normal or remain perma­nently impaired in the involved ear. The cause of labyrin­thitis is unknown. Treatment consists of antibiotics, if the patient is febrile or has symptoms of a bacterial infection, oral corticosteroids, and supportive care. Vestibular sup­pressants are useful during the acute phase of the attack (eg, diazepam) but should be discontinued as soon as fea­sible to avoid long-term dysequilibrium from inadequate compensation.
Cohen HS et al. Relationship between clinical measures of
hearing and clinical measures of vestibular function. Am J Otolaryngol. 2024;45:104052. [PMID: 37801744]
Schoo DP et al. New frontiers in managing the dizzy patient.
Otolaryngol Clin North Am. 2021;54:1069. [PMID: 34294438]
C. Benign Paroxysmal Positioning Vertigo
Patients living with recurrent spells of vertigo, lasting a few (10–15) seconds per spell, associated with changes in head position (often provoked by rolling over in bed), usually have benign paroxysmal positioning vertigo (BPPV). 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.
The typical symptoms of BPPV occur in clusters that persist for several days. There is a brief (10–15 seconds) latency period following a head movement before symptoms develop, and the acute vertigo subsides within 10–60 seconds, although the patient may remain imbalanced for several hours. Dizziness that lasts for more than a few seconds (that is several minutes or hours) is not BPPV. Constant repetition of the positional change leads to habituation. Since some CNS disorders can mimic BPPV (eg, vertebro­basilar insufficiency), recurrent cases warrant head MRI/MRA.
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In central lesions, there is no latent period, fatigability, or habituation of the symptoms and signs. Treatment of BPPV involves physical therapy protocols (eg, the Epley maneuver or Brandt-Daroff exercises), based on the theory that it results from cupulolithiasis (free-floating statoconia, also known as otoconia) within a semicircular canal.
Gurberg J et al. Benign paroxysmal vertigo of childhood. Handb
Clin Neurol. 2023;198:229. [PMID: 38043965]
Saishoji Y et al. Epley manoeuvre’s efficacy for benign paroxys-
mal positional vertigo (BPPV) in primary-care and subspe­cialty settings: a systematic review and meta-analysis. BMC Prim Care. 2023;24:262. [PMID: 38042776]
D. Vestibular Neuronitis
In vestibular neuronitis, a paroxysmal, usually single attack of vertigo occurs without accompanying impairment of auditory function and will persist for several days before gradually abating. During the acute phase, examination reveals nystagmus and absent responses to caloric stimula­tion on one or both sides. The cause of the disorder is unclear though presumed to be viral. Treatment consists of supportive care; vestibular suppressants, such as diazepam 2–5 mg every 6–12 hours during the acute phases of the vertigo only; oral corticosteroids may potentially be used; and antiemetics, such as ondansetron and meclizine, fol­lowed by vestibular therapy if the patient does not com­pletely compensate.
Farhat R et al. The “Vestibular Eye Sign”- “VES”: a new radio-
logical sign of vestibular neuronitis can help to determine the affected vestibule and support the diagnosis. J Neurol. 2023;270:4360. [PMID: 37219605]
Lee JY et al. Clinical characteristics of acute vestibular neuritis
according to involvement site. Otol Neurotol. 2020;41:143. [PMID: 31789808]
E. Traumatic Vertigo
Labyrinthine concussion is the most common cause of vertigo following head injury. Symptoms generally 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. Treatment consists of supportive care and vestibu­lar suppressant medication (diazepam) during the acute phase of the attack and vestibular therapy.
Aljabri A et al. The efficacy of vestibular rehabilitation therapy
for mild traumatic brain injury: a systematic review and meta-analysis. J Head Trauma Rehabil. 2024;39:E59. [PMID: 37335202]
Schlemmer E et al. Vestibular rehabilitation effectiveness for
adults with mild traumatic brain injury/concussion: a mini-systematic review. Am J Audiol. 2022;31:228. [PMID: 35077655]
F. Perilymphatic Fistula
Leakage of perilymphatic fluid from the inner ear into the tympanic cavity via the round or oval window is a very rare cause of vertigo and sensory hearing loss. Most cases result from physical injury (eg, blunt head trauma, hand slap to ear); extreme barotrauma during airflight, scuba diving, etc; or vigorous Valsalva maneuvers (eg, during weight lift­ing). Treatment may require middle ear exploration and window sealing with a tissue graft.
Sarna B et al. Perilymphatic fistula: a review of classification,
etiology, diagnosis, and treatment. Front Neurol. 2020;11:1046. [PMID: 33041986]
Sasaki A et al. Prevalence of perilymphatic fistula in patients
with sudden-onset sensorineural hearing loss as diagnosed by Cochlin-tomoprotein (CTP) biomarker detection: its associa­tion with age, hearing severity, and treatment outcomes. Eur Arch Otorhinolaryngol. 2024;281:2373. [PMID: 38123733]
G. Cervicogenic 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; it is also associated 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). Cervical vertigo may often be confused with migraine­associated vertigo, which is also associated with head movement. Management consists of neck movement exercises to the extent permitted by orthopedic considerations.
Han E et al. Predictive model for diagnosing central lesions in
emergency department patients with isolated dizziness who undergo diffusion-weighted magnetic resonance imaging. Acad Emerg Med. 2022;29:15. [PMID: 34414635]
Piromchai P et al. The efficacy of self-exercise in a patient with
cervicogenic dizziness: a randomized controlled trial. Front Neurol. 2023;14:1121101. [PMID: 36864911]
Seemungal BM et al. The Bárány Society position on ‘Cervical
Dizziness’. J Vestib Res. 2022;32:487. [PMID: 36404562]
H. Migrainous Vertigo
Episodic vertigo is frequently associated with migraine headache. Head trauma may also be a precipitating feature. The vertigo may be temporally related to the headache and last up to several hours, or it may also occur in the absence of any headache. Migrainous vertigo may resemble Ménière disease but without associated hearing loss or tinnitus. Accompanying symptoms may include head pressure; visual, motion, or auditory sensitivity; and photosensitivity. Symptoms typically worsen with lack of sleep and anxiety or stress. Food triggers include caffeine, chocolate, and alcohol, among others. There is often a history of motion intolerance (easily carsick as a child). Migrainous vertigo
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may be familial. Treatment includes dietary and lifestyle changes (improved sleep pattern, avoidance of stress) and antimigraine prophylactic medication.
Chu H et al. Prophylactic treatments for vestibular migraine: a
systematic review and network meta-analysis of randomized clinical trials. Front Pharmacol. 2023;14:1332973. [PMID: 38186654]
Mallampalli MP et al. Care gaps and recommendations in ves-
tibular migraine: an expert panel summit. Front Neurol. 2022;12:812678. [PMID: 35046886]
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, straining, and an apparent conductive hearing loss. Autophony is also a common feature. Diagno­sis is with coronal HRCT scan and VEMP testing. Surgi­cally resurfacing or plugging the dehiscent canal can improve symptoms.
Eberhard KE et al. Current trends, controversies, and future
directions in the evaluation and management of superior canal dehiscence syndrome. Front Neurol. 2021;12:638574. [PMID: 33889125]
Eberhard KE et al. Transmastoid surgery for superior canal
dehiscence: prospective longitudinal objective and patient­reported audiovestibular outcomes. Otol Neurotol. 2024;45:
184. [PMID: 38206067]
» Vertigo Syndromes Due to Central Lesions
CNS causes of vertigo include brainstem vascular disease, arteriovenous malformations, tumors of the brainstem and cerebellum, multiple sclerosis, and vertebrobasilar migraine (Table 8–2). 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. ENG 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.
Bassett A et al. Exploring vestibular assessment in patients with
headache and dizziness. Otolaryngol Clin North Am. 2022;55:549. [PMID: 35490043]
Chari DA et al. The efficient dizziness history and exam. Otolar-
yngol Clin North Am. 2021;54:863. [PMID: 34294439]
Dieterich M et al. Central vestibular networking for sensorimo-
tor control, cognition, and emotion. Curr Opin Neurol. 2024;37:74. [PMID: 38032266]
DISEASES OF THE CENTRAL AUDITORY & VESTIBULAR SYSTEMS
Lesions of the eighth cranial nerve and central audioves­tibular pathways may produce hearing loss and dizziness (Table 8–3). 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 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.
The evaluation of central audiovestibular disorders usu­ally requires imaging of the internal auditory canal, cere­bellopontine angle, and brain with enhanced MRI.
1. Vestibular Schwannoma (Acoustic Neuroma)
Eighth cranial nerve schwannomas are among the most common 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. 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. Diagnosis is made by enhanced MRI. Treatment consists of observation, microsurgical excision, or stereo­tactic radiotherapy, depending on such factors as patient age, underlying health, and size of the tumor.
Barrett TF et al. Single-cell multi-omic analysis of the vestibular
schwannoma ecosystem uncovers a nerve injury-like state.
Nat Commun. 2024;15:478. [PMID: 38216553]
Gambacciani C et al. Surgical management of skull base menin-
giomas and vestibular schwannomas. Curr Opin Oncol.
2022;34:713. [PMID: 36093884]
Kalogeridi MA et al. Stereotactic radiosurgery and radiotherapy
for acoustic neuromas. Neurosurg Rev. 2020;43:941. [PMID:
30982152]
2. Vascular Compromise
Vertebrobasilar insufficiency is a common cause of vertigo in the older adults. It is often triggered by changes in pos­ture or extension of the neck. Reduced flow in the verte­brobasilar system may be demonstrated noninvasively through MRA. Empiric treatment is with vasodilators and aspirin.
Clark M et al. A review of carotid and vertebral artery dissection.
Br J Hosp Med (Lond). 2022;83:1. [PMID: 35506728]
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.
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Kattah JC et al. Eye movements in demyelinating, autoimmune
and metabolic disorders. Curr Opin Neurol. 2020;33:111. [PMID: 31770124]
OTOLOGIC MANIFESTATIONS OF AIDS
AIDS may result in many otologic signs and symptoms. The pinna and external auditory canal may be affected by Kaposi sarcoma and by persistent and potentially invasive fungal infections (particularly Aspergillus fumigatus). Serous otitis media due to eustachian tube dysfunction may arise from adenoidal hypertrophy (HIV lymphade­nopathy), recurrent mucosal viral infections, or an obstructing nasopharyngeal tumor (eg, lymphoma). Unfortunately, ventilating tubes are seldom helpful and may trigger profuse watery otorrhea. Acute otitis media is usually caused by typical bacterial organisms, including
Proteus, Staphylococcus, and Pseudomonas, and rarely, by Pneumocystis jirovecii. Sensorineural hearing loss is com-
mon and, in some cases, results from viral CNS infection. In cases of progressive hearing loss, cryptococcal meningi­tis and syphilis must be excluded. Acute facial paralysis due to herpes zoster infection (Ramsay Hunt syndrome) occurs commonly and follows a clinical course similar to that in nonimmunocompromised patients. Treatment is with high-dose acyclovir (see Chapter 34). Corticosteroids may also be effective as an adjunct.
Dawood G et al. Nature and extent of hearing loss in HIV-
infected children: a scoping review. Int J Pediatr Otorhinolar­yngol. 2020;134:110036. [PMID: 32335463]
º
DISEASES OF THE NOSE & PARANASAL SINUSES
INFECTIONS OF THE NOSE & PARANASAL SINUSES
Rhinosinusitis may be classified by duration of symptoms. Rhinosinusitis is called acute rhinosinusitis if less than 4 weeks’ duration or as chronic rhinosinusitis if lasting more than 12 weeks, with or without acute exacerbations. Acute rhino­sinusitis may also be classified by presumed etiology, such as viral rhinosinusitis or acute bacterial rhinosinusitis.
1. Viral Rhinosinusitis (Common Cold)
» Clinical Findings
Due to the numerous serologic types of rhinoviruses, ade­noviruses, and other viruses, patients remain susceptible to the common cold throughout life. These infections, while generally quite benign and self-limited, have been impli­cated in the development or exacerbation of more serious conditions, such as acute bacterial sinusitis and acute otitis media, asthma, cystic fibrosis, and bronchitis. Nasal con­gestion, decreased sense of smell, rhinorrhea, and sneezing accompanied by general malaise, throat discomfort and, occasionally, headache, are typical in viral infections. Nasal examination usually shows erythematous, edematous mucosa and a watery discharge. The presence of purulent nasal discharge suggests bacterial rhinosinusitis.
Najafloo R et al. Mechanism of anosmia caused by symptoms of
COVID-19 and emerging treatments. ACS Chem Neurosci. 2021;12:3795. [PMID: 34609841]
Vance H et al. Addressing post-COVID symptoms: a guide for
primary care physicians. J Am Board Fam Med. 2021;34:1229. [PMID: 34772779]
» Treatment
The main treatment for viral rhinitis is supportive care, including rest, hydration, and use of over-the-counter anal­gesics and decongestants. There are no effective antiviral therapies for either the prevention or treatment of most viral rhinitis despite a common misperception among patients that antibiotics are helpful. Buffered hypertonic saline (3–5%) nasal irrigation has been shown to improve symptoms and reduce the need for NSAIDs. Other support­ive measures, such as oral 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 but should not be used for more than a few days to prevent rebound congestion. Withdrawal of the medication after prolonged use leads to rhinitis medica- mentosa, an almost addictive need for continuous usage. Treatment of rhinitis medicamentosa requires mandatory cessation of the sprays, and this is often extremely frustrat­ing for patients. Topical intranasal corticosteroids (eg, flu­nisolide, 2 sprays in each nostril twice daily), intranasal anticholinergic (ipratropium 0.06% nasal spray, 2–3 sprays every 8 hours as needed), or a short tapering course of oral prednisone may help during the withdrawal process.
ESSENTIALS OF DIAGNOSIS
»
Associated malaise, headache, and cough.
»
Nasal congestion, facial pressure, rhinorrhea, and hyposmia.
»
Erythematous, engorged nasal mucosa without intranasal purulence.
»
Symptoms are self-limited, lasting typically less than 10 days.
» Complications
Other than mild eustachian tube dysfunction or transient middle ear effusion, complications of viral rhinitis are unusual. Secondary acute bacterial rhinosinusitis is a well­accepted complication of acute viral rhinitis and is sug­gested by persistence of symptoms beyond 10 days with purulent green or yellow nasal secretions and unilateral facial or dental pain.
Dhama K et al. Coronavirus disease 2019-COVID-19. Clin
Microbiol Rev. 2020;33:e00028. [PMID: 32580969]
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2. Bacterial Rhinosinusitis
ESSENTIALS OF DIAGNOSIS
»
Acute onset of symptoms.
»
Purulent yellow-green nasal discharge or expectoration.
»
Facial pain or pressure over the affected sinus or sinuses.
»
Nasal obstruction.
»
Associated cough, malaise, fever, and headache.
» General Considerations
Compared with viral rhinitis, acute bacterial rhinosinusitis infections are uncommon, but they still affect nearly 20 million Americans annually and account for over 2 billion dollars in health care expenditures. Acute bacte­rial rhinosinusitis is believed to be the result of impaired mucociliary clearance, inflammation of the nasal cavity mucosa, and obstruction of the ostiomeatal complex. Edematous mucosa causes obstruction of the complex, resulting in the accumulation of mucus in the sinus cavity that becomes secondarily infected by bacteria. The largest of these ostiomeatal complexes is deep to the middle turbi­nate in the middle meatus. This complex is actually a con­fluence of complexes draining the maxillary, ethmoid, and frontal sinuses. The sphenoid drains from a separate com­plex between the septum and superior turbinate.
The typical pathogens of bacterial rhinosinusitis are S pneumoniae, other streptococci, H influenzae, and less commonly, S aureus and Moraxella catarrhalis. Pathogens vary regionally in both prevalence and drug resistance; about 25% of healthy asymptomatic individuals may, if sinus aspirates are cultured, harbor such bacteria as well.
» Clinical Findings
A. Symptoms and Signs
There are no agreed-upon criteria for the diagnosis of acute bacterial rhinosinusitis in adults. Major symptoms include purulent nasal drainage, nasal obstruction or con­gestion, facial pain/pressure, altered smell, cough, and fever. Minor symptoms include headache, otalgia, halito­sis, dental pain, and fatigue. Many of the more specific symptoms and signs relate to the affected sinus(es). Bacte­rial rhinosinusitis can be distinguished from viral rhinitis by persistence of symptoms for more than 10 days after onset or worsening of symptoms within 10 days after ini­tial improvement. Acute rhinosinusitis is defined as last­ing less than 4 weeks and subacute rhinosinusitis, as lasting 4–12 weeks.
Acute maxillary sinusitis is the most common form of acute bacterial rhinosinusitis because the maxillary is the largest sinus with a single drainage pathway that is easily obstructed. Unilateral facial fullness, pressure, and tender­ness over the cheek are common symptoms, but may not
always be present. Pain may refer to the upper incisor and canine teeth via branches of the trigeminal nerve, which traverse the floor of the sinus. Purulent nasal drainage should be noted with nasal airway obstruction or facial pain (pressure). Maxillary sinusitis may result from dental infection, and teeth that are tender should be carefully examined for signs of abscess. Drainage of the periapical abscess or removal of the diseased tooth typically resolves the sinus infection.
Acute ethmoiditis in adults is often accompanied by maxillary sinusitis, and symptoms are similar to those described above. Localized ethmoid sinusitis may present with pain and pressure over the high lateral wall of the nose between the eyes that may radiate to the orbit.
Sphenoid sinusitis is usually seen in the setting of pan­sinusitis or infection of all the paranasal sinuses on at least one side. The patient may describe a headache “in the middle of the head” and often point to the vertex.
Acute frontal sinusitis may cause 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.
Hospital-associated sinusitis is a form of acute bacte­rial rhinosinusitis that may present without the usual symptoms. Instead, it may be a cause of fever in critically ill patients. It is often associated with prolonged presence of a nasogastric or, rarely, nasotracheal tube causing nasal mucosal inflammation and ostiomeatal complex obstruc­tion. Pansinusitis on the side of the tube is common on imaging studies.
B. Imaging
The diagnosis of acute bacterial rhinosinusitis can usually be made on clinical grounds alone. Although more sensitive
than clinical examination, routine radiographs are not cost­effective and are not recommended by the Agency for Health Care Policy and Research or American Association of Otolaryngology Guidelines. Consensus guidelines recom-
mend imaging when clinical criteria are difficult to evalu­ate, when the patient does not respond to appropriate therapy or has been treated repeatedly with antibiotics, when intracranial involvement or CSF rhinorrhea is suspected, when complicated dental infection is suspected, or when symptoms of more serious infection are noted.
When necessary, noncontrast screening coronal CT scans are more cost-effective and provide more informa­tion than conventional sinus films. CT provides a rapid and effective means to assess all of the paranasal sinuses, iden­tify areas of greater concern (such as bony dehiscence, periosteal elevation, or maxillary tooth root exposure within the sinus), and direct appropriate therapy.
CT scans are reasonably sensitive but are not specific. Swollen soft tissue and fluid may be difficult to distinguish when opacification of the sinus is due to other conditions, such as chronic rhinosinusitis, nasal polyposis, or mucus retention cysts. Sinus abnormalities can be seen in most patients with an upper respiratory infection, while bacterial rhinosinusitis develops in only 2%.
If malignancy, intracranial extension, or opportunistic infection is suspected, MRI with gadolinium should be ordered instead of, or in addition to, CT. MRI will distinguish
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tumor from fluid, inflammation, and inspissated mucus far better than CT, and will better delineate tumor extent (eg, involvement of adjacent structures, such as the orbit, skull base, and palate). Bone destruction can be demonstrated as well by MRI as by CT.
» Treatment
All patients with acute bacterial rhinosinusitis should have careful evaluation of pain. For symptom reduction in viral rhinitis and bacterial rhinosinusitis without com­plication, the European Position Paper on Rhinosinusitis and Nasal Polyps (EPOS) 2012 recommends NSAIDs, saline nasal sprays, and nasal decongestants (pseudo­ephedrine, 30–60 mg every 6 hours, up to 240 mg/day; nasal oxymetazoline, 0.05% or oxymetazoline, 0.05–0.1%, one or two sprays in each nostril every 6–8 hours for up to 3 days). In cases of suspected bacterial rhinosinusitis, intranasal corticosteroids (eg, high-dose mometasone furoate 200 mcg each nostril twice daily for 21 days) have demonstrated efficacy in reducing nasal symptoms and are recommended. Other medications, such as mucolytics, vitamin C, probiotics, and antihistamines, have not demonstrated efficacy in the management of acute rhinosinusitis.
Antibiotic therapy should be reserved for complicated or
protracted acute bacterial rhinosinusitis. Between 40% and
69% of patients with acute bacterial rhinosinusitis improve symptomatically within 2 weeks without antibiotic ther­apy. Antibiotic treatment is controversial in uncompli­cated cases of clinically diagnosed acute bacterial rhinosinusitis because only 5% of patients will note a shorter duration of illness with treatment, and antibiotic treatment is associated with nearly twice the number of adverse events compared with placebo. Antibiotics may be considered when symptoms last more than 10 days or when symptoms (including fever, facial pain, and swelling of the face) are severe or when cases are complicated (such as immunodeficiency). In these patients, administration of antibiotics does reduce the incidence of clinical failure by 50% and represents the most cost-effective treatment strategy.
Selection of antibiotics is usually empiric and based on a number of factors, including regional patterns of antibi­otic resistance, antibiotic allergy, cost, and patient toler­ance. For adults younger than 65 years with mild to moderate acute bacterial rhinosinusitis, the recom­mended first-line therapy is amoxicillin-clavulanate (500 mg/125 mg orally three times daily or 875 mg/125 mg orally twice daily for 5–7 days), or in those with severe sinusitis, high-dose amoxicillin-clavulanate (2000 mg/125 mg extended-release orally twice daily for 7–10 days). In patients with a high risk for penicillin-resistant S pneu- moniae (age over 65 years, hospitalization in the prior 5 days, antibiotic use in the prior month, immunocom­promised status, multiple comorbidities, or severe sinus infection), the recommended first-line therapy is the high-dose amoxicillin-clavulanate option (2000 mg/125 mg extended-release orally twice daily for 7–10 days). For those with penicillin allergy or hepatic impairment, doxy­cycline (100 mg orally twice daily or 200 mg orally once
daily for 5–7 days) or clindamycin (150–300 mg every 6 hours) plus a cephalosporin (cefixime 400 mg orally once daily or cefpodoxime proxetil 200 mg orally twice daily) for 10 days are options. Macrolides, trimethoprim­sulfamethoxazole, and second- or third-generation cepha­losporins are not recommended for empiric therapy. Dupilumab, a monoclonal antibody with inhibition of IL-4 and IL-13, is approved for patients with chronic sinusitis with nasal polyposis.
Hospital-associated infections in critically ill patients are treated differently from community-acquired infec­tions. Removal of a nasogastric tube and improved nasal hygiene (nasal saline sprays, humidification of supplemen­tal nasal oxygen, and nasal decongestants) are critical interventions and often curative in mild cases without aggressive antibiotic use. Endoscopic or transantral cul­tures may help direct medical therapy in complicated cases. In addition, broad-spectrum antibiotic coverage directed at P aeruginosa, S aureus (including methicillin-resistant strains), and anaerobes may be required.
» Complications
Local complications of acute bacterial rhinosinusitis include orbital cellulitis and abscess, osteomyelitis, cavern­ous sinus thrombosis, and intracranial extension.
Orbital complications typically occur by extension of ethmoid sinusitis through the lamina papyracea, a thin layer of bone that comprises the medial orbital wall. Any
change in the ocular examination necessitates immediate CT imaging. Extension in this area may cause orbital cel-
lulitis leading to proptosis, gaze restriction, and orbital pain. Select cases are responsive to intravenous antibiotics, with or without corticosteroids, and should be managed in close conjunction with an ophthalmologist or otolaryn­gologist, or both. Extension through the lamina papyracea can also lead to subperiosteal abscess formation (orbital abscess). Such abscesses cause marked proptosis, ophthal­moplegia, and pain with medial gaze. While some cases respond to antibiotics, such findings should prompt an immediate referral to a specialist for consideration of decompression and evacuation. Failure to intervene quickly may lead to permanent visual impairment and a “frozen globe.”
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 swelling of the forehead (Pott puffy tumor). Fol­lowing treatment, secondary cosmetic reconstructive pro­cedures may be necessary.
Though rare, intracranial complications of sinusitis can occur either through hematogenous spread, as in cavern­ous sinus thrombosis and meningitis, or by direct exten­sion, as in epidural and intraparenchymal brain abscesses. Cavernous sinus thrombosis is heralded by ophthalmo­plegia, chemosis, and visual loss; the diagnosis is most commonly confirmed by MRI. When identified early, cav­ernous sinus thrombosis typically responds to intravenous antibiotics. Frontal epidural and intracranial abscesses are often clinically silent, but may present with altered mental status, persistent fever, or severe headache.
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» When to Refer
Failure of acute bacterial rhinosinusitis to resolve after an adequate course of oral antibiotics necessitates referral to an otolaryngologist for evaluation. Endoscopic cultures may direct further treatment choices. Nasal endoscopy and CT scan are indicated when symptoms persist longer than 4–12 weeks. Any patients with suspected extension of dis­ease outside the sinuses should be evaluated urgently by an otolaryngologist and imaging should be obtained.
» When to Admit
• Facial swelling and erythema indicative of facial cellulitis.
• Proptosis.
• Vision change or gaze abnormality indicative of orbital
cellulitis.
• Abscess or cavernous sinus involvement.
• Mental status changes suggestive of intracranial
extension.
• Failure to respond to appropriate first-line treatment or symptoms persisting longer than 4 weeks.
Hoy SM. Dupilumab: a review in chronic rhinosinusitis with
nasal polyps. Drugs. 2020;80:711. [PMID: 32240527]
Papacharalampous GX et al. Chronic rhinosinusitis with nasal
polyps (CRSwNP) treated with omalizumab, dupilumab, or mepolizumab: a systematic review of the current knowledge towards an attempt to compare agents’ efficacy. Int Forum Allergy Rhinol. 2024;14:96. [PMID: 37394893]
Poto R et al. Imaging of chronic rhinosinusitis with nasal polyps
in the era of biological therapies. Curr Opin Allergy Clin Immunol. 2024 Jan 11. [Epub ahead of print] [PMID: 38205820]
3. Nasal Vestibulitis & S aureus
Nasal Colonization
Inflammation of the nasal vestibule may result from fol­liculitis of the hairs that line this orifice and is usually the result of nasal manipulation or hair trimming. Systemic antibiotics effective against S aureus (such as dicloxacillin, 250 mg orally four times daily for 7–10 days) are indicated. Topical mupirocin 2% nasal ointment (applied two or three times daily) also may be a helpful addition and may pre­vent future occurrences. If recurrent, the addition of rifampin (10 mg/kg orally twice daily for the last 4 days of dicloxacillin 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.
S aureus is the leading nosocomial pathogen, and nasal
carriage is a well-defined risk factor in the development and spread of nosocomial infections. Nasal and extranasal methicillin-resistant S aureus (MRSA) colonizations are associated with a 30% risk of developing an invasive MRSA infection during hospital stays. While the vast majority
have no vestibulitis symptoms, screening by nasal swabs and PCR-based assays has demonstrated a 30% rate of S aureus colonization in hospital patients and an 11% rate of MRSA colonization in ICU patients. Elimination of the carrier state is challenging, but studies of mupirocin 2% nasal ointment application with chlorhexidine facial wash­ing (40 mg/mL) twice daily for 5 days have demonstrated decolonization in 39% of patients.
Ontario Health (Quality). Pre-surgical nasal decolonization of
Staphylococcus aureus: a health technology assessment. Ont Health Technol Assess Ser. 2022;22:1. [PMID: 36160757]
4. Invasive Fungal Sinusitis
Invasive fungal sinusitis is rare and includes both rhino­cerebral mucormycosis (Mucor, Absidia, and Rhizopus
spp.) and other invasive fungal infections, such as Aspergil­lus. The fungus spreads rapidly through vascular channels
and may be lethal if not detected early. Patients with mucormycosis almost invariably have some degree of immunocompromise, such as diabetes mellitus, long-term corticosteroid therapy, neutropenia associated with che­motherapy for hematologic malignancy, or end-stage renal disease. Occasional cases of sinonasal infection with Asper- gillus spp. have been reported in patients with untreated HIV/AIDS. The initial symptoms may be similar to those of acute bacterial rhinosinusitis, although facial pain is often more severe. Nasal drainage is typically clear or straw-colored, rather than purulent, and visual symptoms may be noted at presentation in the absence of significant nasal findings. On examination, the classic finding of mucormycosis is a black eschar on the middle turbinate, but this finding is not universal and may not be apparent if the infection is deep or high within the nasal bones. Often the mucosa appears normal or simply pale and dry. This may be noted on the hard palate as well. Early diagnosis requires suspicion of the disease and nasal biopsy with sil­ver stains, revealing broad nonseptate hyphae within tis­sues and necrosis with vascular occlusion. Imaging, such as CT or MRI, may initially show only soft tissue changes. Consequently, biopsy and ultimate debridement should be based on the clinical setting rather than radiographic dem­onstration of bony destruction or intracranial changes.
Invasive fungal sinusitis represents a medical and
surgical emergency. Once recognized, voriconazole may
be started by intravenous infusion, and prompt wide surgi­cal debridement is indicated for patients with reversible immune deficiency (eg, poorly controlled hyperglycemia in diabetes). Other antifungals, including amphotericin or the less nephrotoxic lipid-based amphotericin B (Ambi­some) and caspofungin, are alternatives to voriconazole and may be added to voriconazole depending on the fun­gus. Surgical management, while necessary for any possi­bility of cure, often results in tremendous disfigurement and functional deficits (eg, often resulting in the loss of at least one eye). Even with early diagnosis and immediate appropriate intervention, the prognosis is guarded. In per­sons with diabetes, the mortality rate is about 20%. If kid­ney disease is present or develops, mortality is over 50%;