Ménière disease
Description
- Endolymphatic hydrops - distension of the endolymphatic system of the inner ear -> recurrent attacks of vertigo, hearing loss, tinnitus, aural fullness
- Classic tetrad: episodic vertigo + fluctuating sensorineural hearing loss + tinnitus + aural fullness - all four needed for definite diagnosis
Epidemiology
- Peak onset 40-60 years
- Unilateral at onset in most; bilateral in up to ~30-50% over time
- Relatively uncommon compared to BPPV/vestibular neuritis but important because of progressive hearing loss
Aetiopathogenesis
- Endolymphatic hydrops - excess endolymph volume/pressure in the membranous labyrinth, mechanism incompletely understood
- Proposed contributors: impaired endolymphatic sac resorption, autoimmune, viral, genetic predisposition, migraine overlap
- Idiopathic in most; secondary hydrops can follow trauma, otosclerosis, or labyrinthitis
Diagnosis
Clinical (diagnosis is clinical, per AAO-HNS criteria)
- >=2 spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours
- Fluctuating sensorineural hearing loss documented audiometrically on at least one occasion, associated with the affected ear
- Fluctuating aural symptoms (tinnitus, aural fullness) in the affected ear
- Not better accounted for by another vestibular diagnosis
Investigations
- Audiometry - low-frequency sensorineural hearing loss characteristic, fluctuates with attacks, becomes fixed/progressive over time
- MRI brain/IAM with gadolinium - to exclude vestibular schwannoma and other structural causes, especially if asymmetric/unilateral features
- Electrocochleography - adjunctive, not routinely required for diagnosis
Management
A. Acute attack
- Vestibular suppressants (prochlorperazine, benzodiazepine) and antiemetics for the duration of the attack only
B. Prevention of attacks - lifestyle first
- Low-sodium diet and adequate hydration - first-line, most widely recommended
- Reduce caffeine, alcohol; stress management
- Betahistine - widely used for prophylaxis; evidence is modest but it remains standard first-line pharmacological prophylaxis in most guidelines
C. Refractory disease (recurrent disabling attacks despite lifestyle + betahistine)
- Intratympanic corticosteroid injection - preferred escalation, hearing-preserving
- Intratympanic gentamicin (chemical labyrinthectomy) - effective for vertigo control but risk of further hearing loss - reserved for those willing to trade residual hearing for vertigo control
- Diuretics (e.g. betahistine plus a thiazide) sometimes trialled, evidence limited
- Endolymphatic sac surgery or vestibular nerve section/labyrinthectomy - last-line surgical options for truly refractory unilateral disease
D. Hearing and disability support
- Hearing aid referral as sensorineural loss progresses
- Vestibular rehabilitation for interictal imbalance
- Driving/occupational safety counselling given unpredictable attacks
Associations
- Migraine (significant clinical overlap and shared triggers)
- Autoimmune inner ear disease
- Family history in a subset (genetic predisposition)
Natural history & complications
- Relapsing-remitting course - unpredictable attack frequency, can remit for long periods
- Progressive, eventually fixed, sensorineural hearing loss in the affected ear(s) over years
- Bilateral involvement develops in a substantial minority over the disease course
- "Tumarkin drop attacks" (otolithic crisis) - sudden falls without loss of consciousness, can occur in advanced disease
- Significant quality-of-life impact from unpredictability of attacks
🔒
6 more sections, plus exam facts
Premium unlocks every note across every specialty, and the full exam fact library behind it.
Get premium access