Author: Joseph S. Kass, MD, JD, FAAN
DefinitionMénière disease is a syndrome characterized by recurrent vertigo with fluctuating hearing loss, tinnitus, and fullness in the ear.
Synonyms
- Endolymphatic hydrops
- Lermoyez syndrome
- Idiopathic endolymphatic hydrops
| ICD-10CM CODES | | H81.01 | Ménière disease, right ear | | H81.02 | Ménière disease, left ear | | H81.03 | Ménière disease, bilateral | | H81.09 | Ménière disease, unspecified ear |
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Epidemiology & DemographicsIncidence (In U.S.):Approximately 190/100,000 persons
Predominant Sex:Female:male ratio of 1.3:1
Peak Incidence:Fourth to sixth decade of life
Physical Findings & Clinical Presentation
- Hearing may be unilaterally decreased.
- Pallor, sweating, and nausea may occur during a severe attack.
- Usually the patient develops a sensation of fullness and pressure along with decreased hearing and tinnitus in a single ear.
- The patient typically experiences severe vertigo, which peaks within minutes, then slowly subsides over hours.
- May see spontaneous nystagmus on examination.
- Persistent sense of disequilibrium for days is typical after an acute episode.
- May have vestibulopathy demonstrable with a positive head thrust test.
Etiology
- Unknown; viral, autoimmune, and genetic causes have been suggested.
- Endolymphatic hydrops is the postmortem histologic hallmark. Endolymphatic hydrops may create cytochemical changes that disturb endolymphatic fluid homeostasis, leading to spiral ganglion cell death.
Proposed guidelines by the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) for diagnosis and severity of Ménière disease (Box E1).
BOX E1 American Academy of Otolaryngology-Head and Neck Surgery Criteria for Ménière Disease Severity1,2
In 1996, the Committee on Hearing and Equilibrium reaffirmed and clarified the 1985 guidelines, adding initial staging and reporting guidelines. Vertigo
- Any treatment should be evaluated no sooner than 24 mo.
- Formula to obtain numeric value for vertigo: ratio of average number of definitive spells per mo after therapy divided by definitive spells per mo before therapy (averaged over a 24-mo period) ×100 = numeric value
- Numeric value scale
- 0: Class A: Complete control of definitive spells
- 41-80: Class B: Limited control of definitive spells
- 81-120: Class C: Insignificant control of definitive spells
- >120: Class D
- Class E: Secondary treatment initiated
- Disability
- No disability
- Mild disability: intermittent or continuous dizziness/unsteadiness that precludes working in a hazardous environment
- Moderate disability: intermittent or continuous dizziness that results in a sedentary occupation
- Severe disability: symptoms so severe as to exclude gainful employment
Hearing
- Hearing is measured by a four-frequency pure tone average (PTA) of 500 Hz and 1, 2, and 3 kHz
- Pretreatment hearing level: worst hearing level during 6 mo prior to surgery
- Posttreatment hearing level: poorest hearing level measured 18-24 mo after institution of therapy
- Hearing classification:
- Unchanged ≤10-dB PTA improvement or worsening or ≤15% speech discrimination improvement or worsening
- Improved >10-dB PTA improvement or >15% speech discrimination improvement
- Worse >10-dB PTA worsening or >15% speech discrimination worsening
- In 1996, the Committee on Hearing and Equilibrium reaffirmed and clarified the guidelines, adding initial staging and reporting guidelines.
Initial Hearing LevelFour-Tone Average (Db)
- Stage 1: ≤25
- Stage 2: 26-40
- Stage 3: 41-70
- Stage 4: >70
Functional Level ScaleRegarding my current state of overall function, not just during attacks: - My dizziness has no effect on my activities at all.
- When I am dizzy, I have to stop for a while, but it soon passes, and I can resume my activities. I continue to work, drive, and engage in any activity I choose without restriction. I have not changed any plans or activities to accommodate my dizziness.
- When I am dizzy, I have to stop what I am doing for a while, but it does pass, and I can resume activities. I continue to work, drive, and engage in most activities I choose, but I have had to change some plans and make some allowance for my dizziness.
- I am able to work, drive, travel, and take care of a family or engage in most activities, but I must exert a great deal of effort to do so. I must constantly make adjustments in my activities and budget my energies. I am barely making it.
- I am unable to work, drive, or take care of a family. I am unable to do most of the active things that I used to do. Even essential activities must be limited. I am disabled.
- I have been disabled for 1 year or longer and/or I receive compensation because of my dizziness or balance problem.
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From Flint PW et al: Cummings otolaryngology, head and neck surgery, ed 7, Philadelphia, 2021, Elsevier.
Differential Diagnosis
- Acoustic neuroma
- Migrainous vertigo
- Multiple sclerosis
- Autoimmune inner ear syndrome
- Otitis media
- Vertebrobasilar disease
- Labyrinthitis
Workup
- Diagnosis is primarily made by history, although further diagnostic tests may help support the diagnosis. Guidelines to define Ménière disease are described in Table 1.
- Audiogram may show sensorineural hearing loss with lower frequencies primarily affected. Hearing loss may recover either partially or completely after an attack. Recurrent attacks may lead to a persistent and progressive sensorineural hearing loss.
- Electronystagmography may show peripheral vestibular deficit.
- Both vestibular-evoked myogenic potential (VEMP) studies and electrocochleography (ECoG) have low sensitivity and specificity for Ménière disease and are not clinically useful.
TABLE 1 Guidelines to Define Ménière Disease
| Definition | Symptoms |
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| - Histopathologic confirmation
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| - ≥2 definitive spontaneous episodes of vertigo 20 min to 12 h
- Audiometrically documented low- to medium-frequency sensorineural hearing loss in one ear, defining the affected ear on at least one occasion before, during, or after one of the episodes of vertigo
- Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the affected ear
- Not better accounted for by another vestibular diagnosis
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| - One definite episode of vertigo
- Audiometrically documented hearing loss on at least one occasion
- Tinnitus or aural fullness in the treated ear
- Other causes excluded
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| - Episodic vertigo without documented hearing loss, or sensorineural hearing loss (SNHL) fluctuating or fixed, with disequilibrium but nonepisodic
- Other causes excluded
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Laboratory TestsNo laboratory serologic test is specific for Ménière disease. A thyroid panel, glucose, hemoglobin A1C, antinuclear antibodies, urinalysis, chemistry panel, RPR, Lyme disease antibodies, and allergy testing can be ordered to screen for other disorders such as thyroid or autoimmune diseases, diabetes, otorenal syndrome, syphilis, Lyme disease, and allergy-mediated Ménière disease.
Imaging Studies
- MRI to rule out acoustic neuroma or other retrocochlear lesion, especially if cerebellar or central nervous system dysfunction is present.
- Recent efforts have shown a role for MRI with intratympanic gadolinium.
