BASICS

Ménière disease is an inner ear (labyrinthine) disorder characterized by recurrent attacks of: - hearing loss - tinnitus - vertigo - aural fullness

Diagnostic criteria include: - ≥2 spontaneous episodes of vertigo lasting >20 minutes but <12 hours
- Audiogram showing low to medium frequency sensorineural hearing loss in one ear at any time
- Fluctuating aural symptoms: hearing loss, tinnitus, or aural fullness【1】.

Clinical triad:

Vertigo (20 minutes to 12 hours)
Audiometrically documented low-frequency sensorineural hearing loss
Fluctuating aural symptoms (tinnitus or aural fullness)

DESCRIPTION

Often unilateral initially; ~50% become bilateral over time.
Vertigo severity/frequency may diminish but hearing loss is progressive/fluctuating.
Usually idiopathic (Ménière disease), but may be secondary to other causes of endolymphatic hydrops (Ménière syndrome).

Five clinical subtypes (unilateral and bilateral)【1】:

Type 1: classic unilateral MD and meta-chronic bilateral MD (one ear then the other)
Type 2: delayed unilateral MD or synchronic bilateral MD (simultaneous onset)
Type 3: familial MD (often bilateral)
Type 4: sporadic MD with migraine
Type 5: sporadic MD with autoimmune disease

System affected: nervous
Synonyms: Ménière syndrome; endolymphatic hydrops

EPIDEMIOLOGY

Age onset: 40 to 60 years
Gender: female > male, but fairly equal overall
Race: white, Northern European > blacks
Incidence: up to 150/100,000 person-years

ETIOLOGY AND PATHOPHYSIOLOGY

May be secondary to injury or conditions such as:
reduced middle ear pressure
allergy, endocrine disease
lipid disorders, vascular, viral, syphilis, autoimmune
Theories:
Increased endolymphatic fluid pressure due to increased production or decreased resorption → endolymphatic sac pathology, abnormal vestibular aqueduct, immune complexes
Membrane rupture → altered ionic gradients
Other factors: vascular compromise, cochlear trauma, viral infection/reactivation

Genetics

Family history in 10% with autosomal dominant inheritance【2】.

RISK FACTORS

Stress
Allergy
Increased intake of salt, caffeine, alcohol, nicotine
Chronic loud noise exposure
Vascular abnormalities (migraines)
Viral exposures (herpes simplex virus)

GENERAL PREVENTION

Reduce known risk factors.

COMMONLY ASSOCIATED CONDITIONS

Anxiety (secondary)
Migraines
Hyperprolactinemia
Hypothyroidism

DIAGNOSIS

HISTORY

Spontaneous symptomatic episodes often preceded by aura: ear fullness, tinnitus
May occur in clusters with symptom-free intervals
American Academy of Otolaryngology-Head and Neck Surgery diagnostic criteria【3】:
≥2 vertigo episodes >20 min to 12 hr (rotatory or rocking)
Tinnitus or aural fullness (fluctuating)
Audiometric low- to mid-frequency sensorineural hearing loss on at least one occasion correlated with vertigo
Severe attacks may include pallor, sweating, nausea, vomiting, falling, prostration.

PHYSICAL EXAM

No pathognomonic findings; rules out other diagnoses
Horizontal nystagmus during attacks
Otoscopy usually normal
Dix-Hallpike test triggers BPPV vertigo (shorter duration, head-movement triggered), not Ménière disease

DIFFERENTIAL DIAGNOSIS

Acoustic neuroma / CNS tumor
Multiple sclerosis
Autoimmune inner ear disease
Temporal bone fractures
Syphilis
Viral labyrinthitis
TIA, migraine
Diabetes or thyroid dysfunction
Medication side effects

DIAGNOSTIC TESTS & INTERPRETATION

Initial Tests

Serologic tests for Treponema pallidum if at risk
Thyroid, fasting blood sugar, lipid profile
MRI to rule out acoustic neuroma/CNS pathology

Diagnostic Procedures

Audiometry: low-frequency sensorineural hearing loss, impaired speech discrimination
Tuning fork tests, ABR, MRI for acoustic neuroma exclusion
Electrocochleography: may confirm diagnosis
Caloric testing: reduced activity consistent but not diagnostic
Head-impulse testing【4】
Cytochemical analysis: altered AQP4/AQP6, cochlin, mitochondrial proteins【5】
Familial MD associated with DTNA and FAM136A genes【6】

TREATMENT

Primarily symptomatic relief of vertigo and nausea.
- Bed rest with eyes closed during attacks (usually <4 hours)

GENERAL MEASURES

Salt restriction diet (inconclusive evidence)【7】

MEDICATION

First Line: Acute attack
- Benzodiazepines (e.g., diazepam): reduce vertigo/anxiety
- Antihistamines (meclizine/dimenhydrinate): reduce vertigo/nausea
- Anticholinergics (transdermal scopolamine): reduce nausea/emesis
- Antidopaminergics (metoclopramide, promethazine): reduce nausea, anxiety
- Rehydration and electrolyte replacement
- Steroid taper for acute hearing loss

Maintenance: prevent/reduce attacks
- Lifestyle changes (e.g., low-salt diet)
- Diuretics (hydrochlorothiazide, hydrochlorothiazide/triamterene, acetazolamide) may reduce attacks but evidence insufficient

Precautions:
- Atropine: cardiac disease, arrhythmias, prostatic enlargement
- Scopolamine: children, elderly, prostatic enlargement
- Diuretics: electrolyte abnormalities, renal disease
- Sedating drugs caution in elderly; avoid driving/machinery

Second Line:
- Steroids (intratympanic/systemic) for hearing loss【3】
- Famciclovir (Famvir) lacks strong evidence but may help hearing more than balance

ISSUES FOR REFERRAL

ENT and neurology
Formal audiometry recommended

ADDITIONAL THERAPIES

Meniett device: intermittent pressure via myringotomy; shown to relieve vertigo【8】
Vestibular rehabilitation: beneficial between attacks; safe for unilateral vestibular dysfunction【9】

SURGERY/OTHER PROCEDURES

Hearing-preserving:
- Endolymphatic sac surgery: controls vertigo in ~75% of refractory patients
- Vestibular nerve section: invasive, reduces vertigo while preserving hearing
- Tympanostomy tubes: may decrease symptoms by lowering middle ear pressure

Non-hearing-preserving:
- Labyrinthectomy: controls vertigo but causes deafness
- Vestibular neurectomy
- Endoscopic vestibular nerve section
- Cochlear implantation

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Monitor hearing for progression and for serious causes (e.g., acoustic neuroma)
Salt restriction if food triggers identified

PROGNOSIS

Variable course: alternating attacks and remissions
Between attacks, patient often limited by fear/lingering symptoms
50% resolve spontaneously in 2-3 years; some last >20 years
Severity/frequency of vertigo diminish, but hearing loss often progressive
90% successfully treated medically; 5-10% require surgery for incapacitating vertigo

COMPLICATIONS

Hearing loss
Injury during attacks
Work disability

REFERENCES

Borowiec E, Crossley J, Hoa M. Understanding fluctuating hearing loss. Hear J. 2020;73(6):12-13.
Perez-Carpena P, Lopez-Escamez JA. Current understanding and clinical management of Meniere's disease: a systematic review. Semin Neurol. 2020;40(1):138-150.
Basura GJ, Adams ME, Monfared A, et al. Clinical practice guideline: Ménière's disease executive summary. Otolaryngol Head Neck Surg. 2020;162(4):415-434.
Lee SU, Kim HJ, Koo JW, et al. Comparison of caloric and head-impulse tests during the attacks of Meniere's disease. Laryngoscope. 2017;127(3):702-708.
Ishiyama G, Lopez IA, Sepahdari AR, et al. Meniere's disease: histopathology, cytochemistry, and imaging. Ann N Y Acad Sci. 2015;1343:49-57.
Frejo L, Giegling I, Teggi R, et al. Genetics of vestibular disorders: pathophysiological insights. J Neurol. 2016;263(Suppl 1):S45-S53.
Shim T, Strum DP, Mudry A, et al. Hold the salt: history of salt restriction as a first-line therapy for Menière's disease. Otol Neurotol. 2020;41(6):855-859.
Ahsan SF, Standring R, Wang Y. Systematic review and meta-analysis of Meniett therapy for Meniere's disease. Laryngoscope. 2015;125(1):203-208.
Dunlap PM, Holmberg JM, Whitney SL. Vestibular rehabilitation: advances in peripheral and central vestibular disorders. Curr Opin Neurol. 2019;32(1):137-144.

Clinical Pearls

Ménière disease is characterized by vertigo (20 min to 12 hr), hearing loss, tinnitus ± aural fullness.
Differential diagnosis is broad; full investigation of symptoms is required.
Multiple medical, surgical, and rehabilitative treatments can reduce attack severity and frequency.