Key Facts

Ménière disease is an idiopathic inner ear disorder characterised by episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural fullness. The underlying pathology is endolymphatic hydrops — distension of the endolymphatic compartment of the inner ear.

Epidemiology

• Prevalence: 50-200 per 100,000
• Peak onset: 40-60 years
• Slight female predominance (1.3:1)
• Bilateral involvement: 25-40% over 10-20 years
• Rare in children

Aetiology

• Exact cause unknown; likely multifactorial
• Endolymphatic hydrops is the pathological hallmark
• Proposed mechanisms: impaired endolymph reabsorption by endolymphatic sac, abnormal endolymph production, altered ion homeostasis
• Possible contributing factors: viral infection, autoimmune mechanisms, genetic predisposition (familial in 5-15%), allergy, vascular insufficiency

Pathophysiology

• Excess endolymph accumulates in the scala media (cochlear duct) and vestibular labyrinth
• Distension of the membranous labyrinth (Reissner membrane bulges)
• Membrane rupture may cause mixing of endolymph (high K+) with perilymph (high Na+)
• Potassium-rich endolymph exposure to hair cells and vestibular nerve causes depolarisation block
• This results in acute vertigo, hearing loss, and tinnitus during attacks
• Membrane heals, symptoms resolve, but repeated attacks cause progressive hair cell damage
• Over time, hearing loss becomes permanent and vertigo attacks may diminish ('burn out')

Typical Attack

• Episodic rotational vertigo: lasting 20 minutes to 12 hours (usually 2-4 hours)
• Associated nausea, vomiting, pallor, sweating
• Fluctuating hearing loss: low-frequency initially; recovers between attacks initially but becomes progressive
• Tinnitus: usually low-pitched roaring, worsens during attacks
• Aural fullness: pressure sensation in affected ear
• Attacks are unpredictable; may cluster or be separated by months

Between Attacks

• May be asymptomatic initially
• Progressive hearing loss between attacks over years
• Persistent tinnitus
• Imbalance/unsteadiness

Late Disease ('Burnt Out')

• Vertigo attacks reduce in frequency and severity
• Permanent SNHL (often moderate-severe)
• Persistent tinnitus
• Chronic imbalance

Red Flags

• Persistent vertigo >24 hours (consider vestibular neuritis, stroke)
• Acute onset without fluctuation (consider labyrinthitis, stroke)
• Progressive unilateral SNHL without vertigo (exclude vestibular schwannoma)
• Neurological symptoms (diplopia, dysarthria, dysphagia) — exclude posterior circulation stroke
• Drop attacks (Tumarkin's otolithic crisis) — sudden falls without loss of consciousness

Bedside

• Otoscopy: usually normal
• Nystagmus assessment: horizontal nystagmus during acute attack (towards affected ear initially, then away)
• Head impulse test: may be positive (abnormal) on affected side
• Romberg test: positive, falling towards affected side
• Dix-Hallpike: negative (rules out BPPV)

Bloods

• FBC, TFTs, glucose, syphilis serology: to exclude other causes of SNHL
• Autoimmune screen if bilateral or atypical

Imaging

• MRI IAM with gadolinium: to exclude vestibular schwannoma (essential for unilateral SNHL)
• MRI inner ear with intratympanic gadolinium: can demonstrate endolymphatic hydrops (research/specialist centres)

Special Tests

• Pure tone audiometry: low-frequency SNHL (250Hz, 500Hz, 1kHz) during or shortly after attack; may be normal between attacks early in disease
• Caloric testing: reduced or absent vestibular response on affected side (canal paresis)
• Electrocochleography (ECoG): elevated SP/AP ratio (>0.4) supports diagnosis
• Glycerol dehydration test: temporary improvement in hearing after oral glycerol (rarely used)
• Serial audiometry: documents fluctuating hearing loss

Non-pharmacological

• Low-salt diet: <1.5g sodium/day (reduces endolymph volume; widely recommended)
• Avoid caffeine, alcohol, and tobacco (may trigger attacks)
• Vestibular rehabilitation: for chronic imbalance between attacks
• Stress management
• Driving: must inform DVLA; must not drive during attacks; Group 1 licence may continue if attacks controlled

Pharmacological

• Acute attack:
  • Prochlorperazine 5-10mg PO/IM TDS or buccal 3-6mg BD (vestibular sedative)
  • Cyclizine 50mg PO/IM TDS
  • Ondansetron 4-8mg for severe nausea/vomiting
• Prophylaxis:
  • Betahistine 16mg TDS (H1 agonist/H3 antagonist; improves inner ear microcirculation)
  • Titrate up to 48mg TDS if needed
  • BEMED trial showed no significant difference vs placebo, but widely used in UK practice

Surgical/Interventional

• Intratympanic steroid injection (dexamethasone 4mg/ml): for acute episodes or refractory disease (hearing-sparing)
• Intratympanic gentamicin: chemical labyrinthectomy; controls vertigo in >85% but risk of further hearing loss (vestibulotoxic)
• Endolymphatic sac decompression/shunt: controversial; modest evidence of benefit
• Labyrinthectomy: definitive vertigo control for unilateral Ménière with no useful hearing; destroys residual hearing
• Vestibular neurectomy: selective section of vestibular nerve; preserves hearing; major neurosurgical procedure

Referral Criteria

• ENT referral: all suspected Ménière disease for diagnosis confirmation and management
• Audiology: serial audiometry monitoring
• Urgent ENT: sudden SNHL, suspected vestibular schwannoma, disabling vertigo
• Driving advice: DVLA notification required

• 60-70% of patients achieve adequate symptom control with conservative measures
• Vertigo attacks typically burn out over 5-15 years as vestibular function declines
• 50% develop moderate-severe hearing loss in the affected ear over 10 years
• Bilateral involvement: 25-40% within 10-20 years (significant impact on quality of life)
• Tumarkin's otolithic crisis (drop attacks): occurs in 5-10%, risk of serious injury
• Quality of life significantly impacted during active disease
• With appropriate management, most patients maintain functional independence

AAO-HNS 2020 Diagnostic Criteria for Definite Ménière Disease

Management Escalation Ladder

Ménière Disease vs Vestibular Migraine