Ménière's disease
Exam Tips
- Use Bárány-style criteria: definite disease needs >=2 spontaneous vertigo episodes (20 min-12 h), documented low/mid-frequency SNHL in affected ear, fluctuating aural symptoms, and no better alternative diagnosis.
- In OSCEs, always screen central red flags in acute vertigo: focal neurology, new severe headache, normal head impulse in acute vestibular syndrome, or new unilateral hearing loss needing urgent assessment.
- Classic symptom triad is vertigo + fluctuating SNHL + tinnitus; aural fullness is a common fourth feature and can precede attacks.
- State that there is no single diagnostic blood test; audiometry supports diagnosis and MRI is mainly to exclude retrocochlear/central pathology.
- Mention safety counselling marks: driving/DVLA advice, falls risk, and medication sedation warnings.
Definition
Ménière's disease is an idiopathic inner-ear disorder causing recurrent spontaneous vertigo with fluctuating unilateral sensorineural hearing loss, tinnitus, and aural fullness. It is diagnosed clinically (supported by audiometry) after excluding alternative vestibular or central causes; if an underlying cause is identified, the term Ménière's syndrome is preferred.
Pathophysiology
The leading mechanism is endolymphatic hydrops (excess endolymph within the membranous labyrinth), probably from disordered endolymph production/absorption. Episodic pressure changes may distort or transiently disrupt labyrinthine membranes (classically Reissner's membrane), producing ionic disturbance and dysfunction of cochlear and vestibular hair-cell/neural signalling, which explains fluctuating hearing symptoms and episodic vertigo; with time, cumulative injury contributes to persistent hearing loss and tinnitus. See Figure: membranous labyrinth/endolymphatic hydrops diagrams in standard ENT texts (vertigo chapter).
Risk Factors
- Age 30-60 years (typical onset in mid-life)
- Female sex (slight predominance)
- Migraine association (possible vascular overlap)
- Autoimmune disease (often bilateral presentations)
- Recent viral illness or viral inner-ear injury
- Head/acoustic trauma
- Genetic susceptibility/family history
- Metabolic/endocrine contributors (for example hypothyroidism, inner-ear electrolyte dysregulation)
- Infective mimics/associations to exclude (for example syphilis, Lyme disease)
- Allergic predisposition (including possible food-triggered symptoms)
Clinical Features
Symptoms
- Recurrent spontaneous vertigo (spinning/rocking), usually lasting 20 minutes to hours (typically <24 hours)
- Nausea and vomiting during attacks
- Fluctuating unilateral hearing loss (initially low-frequency sensorineural)
- Tinnitus (often described as roaring), later more persistent
- Aural fullness/pressure, sometimes prodromal before vertigo
- Post-attack disequilibrium/unsteadiness lasting hours to days
- Clustered attacks with remissions (weeks to years)
- Drop attacks (Tumarkin otolithic crises) without loss of consciousness in a minority
Signs
- Often normal otological/head-neck examination between attacks
- Horizontal or rotatory nystagmus during acute episodes (may be suppressed by visual fixation)
- Impaired balance testing during attacks (positive Romberg/tandem gait difficulty)
- Unterberger/Fukuda stepping tendency to rotate toward affected side
- No focal neurological deficit in typical peripheral disease (neurological signs suggest central pathology)
Investigations
Management
Lifestyle Modifications
- Explain episodic course; safety-net that most acute attacks settle within 24 hours, and review urgently if not improving by 5-7 days or if deteriorating
- Advise immediate risk reduction during attacks: stop driving, avoid heights, machinery, and swimming alone
- Follow DVLA guidance for liability to sudden disabling dizziness (stop driving and notify DVLA when required)
- Keep rescue anti-vertigo/antiemetic medication accessible
- Early multidisciplinary input (ENT, audiology, vestibular physiotherapy, hearing therapy, psychological support)
- Consider individual trigger management (for example migraine triggers, caffeine/alcohol/salt moderation) where patient-specific benefit is seen
Pharmacological Treatment
Acute vestibular suppressants/antiemetics (short-term only)
- Prochlorperazine 5-10 mg oral up to three times daily PRN (or buccal 3 mg twice daily)
- Cyclizine 50 mg oral up to three times daily PRN
- Promethazine teoclate 25 mg at night, increasing to 25 mg twice daily if needed
Use for brief attack control; prolonged regular use can impair central vestibular compensation. Phenothiazines can cause sedation, dystonia/parkinsonism, QT-risk and anticholinergic effects; avoid/caution in Parkinson's disease, severe CNS depression, and older adults at falls risk.
Attack-frequency reduction (specialist-guided long-term therapy)
- Betahistine 16 mg three times daily initially; maintenance commonly 24-48 mg/day in divided doses
Evidence is mixed but widely used in UK practice for symptom reduction. Contraindicated in pheochromocytoma; use caution in asthma and active/previous peptic ulcer disease. Review benefit periodically and stop if ineffective.
Rescue treatment for refractory severe episodes (secondary care)
- Ondansetron 4-8 mg oral/IV for severe vomiting
- Prochlorperazine 12.5 mg IM for severe acute vertigo with vomiting
Use where oral therapy fails. Check QT-prolongation risk, hydration status, and extrapyramidal adverse effects.
Surgical / Interventional
- Intratympanic corticosteroid (for persistent vertigo despite conservative treatment)
- Intratympanic gentamicin (chemical ablation for refractory disabling vertigo; risk of further hearing loss)
- Endolymphatic sac decompression/shunt in selected patients
- Vestibular nerve section for intractable unilateral disease with serviceable hearing
- Labyrinthectomy for refractory unilateral disease when hearing is already non-serviceable
Complications
- Falls and injury from imbalance or drop attacks
- Progressive hearing impairment; occasional bilateral severe-profound loss
- Persistent tinnitus and chronic disequilibrium
- Psychological morbidity (anxiety, depression, agoraphobia)
- Social/occupational impact including driving restrictions and reduced independence
Prognosis
The condition is chronic and often fluctuates early, with episodes separated by remission. Over years, hearing loss tends to progress and tinnitus may become persistent, while vertigo attacks often reduce in frequency and may eventually 'burn out' after roughly 5-15 years, leaving residual imbalance and unilateral auditory symptoms in many patients.
Sources & References
🏥BMJ Best Practice(2)
💊BNF Drug References(1)
- Betahistine dihydrochloride[management.pharmacological]
✅NICE Guidelines(1)
- Ménière's disease[overview]