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Meniere's Disease (Ménière's Disease)
Definition
Meniere's disease (MD) is a disorder of the inner ear characterized by the classic tetrad of:
- Episodic vertigo - attacks lasting 30 minutes to 2 hours
- Fluctuating sensorineural hearing loss (SNHL) - typically low-frequency, low-tone
- Tinnitus - fluctuating, usually unilateral
- Aural fullness - sensation of pressure or fullness in the affected ear
The underlying pathology is endolymphatic hydrops - excess fluid accumulation in the membranous labyrinth of the inner ear.
Epidemiology & Natural History
- Affects one ear in 85% of patients
- If the second ear becomes involved, it typically does so within 36 months
- Spontaneous remission occurs in approximately 60% of patients, but cannot be predicted
- Onset is usually in the 4th-5th decade; no strong sex predilection
Pathophysiology
The core defect is endolymphatic hydrops: abnormal distension of the endolymphatic compartment due to impaired reabsorption or overproduction of endolymph. Contributing factors include:
- Immune dysfunction (autoimmune mechanisms are increasingly implicated)
- Viral/inflammatory injury to the endolymphatic sac
- Anatomical obstruction of endolymphatic drainage
- Possible genetic predisposition
During an attack, rupture of Reissner's membrane allows potassium-rich endolymph to flood the perilymphatic space, causing hair cell dysfunction.
Clinical Variants
| Variant | Features |
|---|---|
| Cochlear hydrops | Fluctuating SNHL + tinnitus, no vertigo |
| Vestibular hydrops | Episodic vertigo + aural fullness, no hearing loss |
| Lermoyez syndrome | Worsening tinnitus/hearing loss relieved AFTER a vertigo attack |
| Tumarkin crisis (drop attack) | Sudden loss of extensor muscle tone, fall to ground, no loss of consciousness - occurs late in disease; no warning |
Diagnosis
Diagnosis is clinical, based on history and audiometry. There is no single confirmatory test.
Diagnostic criteria require:
- Two or more definitive spontaneous vertigo episodes (20 min - 12 hours)
- Audiometrically documented low-to-mid frequency SNHL in the affected ear on at least one occasion
- Fluctuating aural symptoms (fullness, tinnitus, or hearing change) in the affected ear
- Not better accounted for by another diagnosis
Investigations:
- Audiometry: Fluctuating low-tone SNHL; little or no tone decay
- ENG/VNG: Often normal between attacks; spontaneous nystagmus during attacks (may show direction-changing components even during caloric testing)
- Glycerol test: Oral glycerol (1.2 mL/kg) - transient improvement in hearing, tinnitus, and fullness within 1-3 hours suggests endolymphatic hydrops; diagnostic utility
- MRI: To exclude acoustic neuroma, other retrocochlear pathology
- Electrocochleography (ECochG): Elevated SP/AP ratio suggests hydrops
Treatment
There is no cure. Treatment aims to reduce frequency and severity of attacks and preserve hearing.
Conservative / Medical (First-Line)
| Approach | Details |
|---|---|
| Low-salt diet | <2 g sodium/day; reduces endolymphatic pressure |
| Diuretics | Hydrochlorothiazide, acetazolamide - reduce endolymph production |
| Vestibular suppressants | Meclizine, promethazine, benzodiazepines - for acute attacks |
| Vasodilators | Betahistine (widely used; improves cochlear microcirculation) |
| Lifestyle | Avoid caffeine, alcohol, tobacco; stress reduction |
Intratympanic Therapy (Second-Line)
Intratympanic dexamethasone (IT steroids)
- Supported by immune dysfunction hypothesis
- Protocol: Dexamethasone 4-24 mg/mL, daily or weekly injections
- ~80% vertigo relief in some studies; avoidance of ablative surgery in 81-91% of patients when used as needed
- Minimal hearing risk
- A 2026 systematic review of RCTs (Martínez et al., Eur Arch Otorhinolaryngol) confirmed efficacy for vertigo control
- A 2025 meta-analysis on IT gentamicin (Devantier et al., Acta Otolaryngol) also evaluated ablative IT therapy outcomes
Intratympanic gentamicin (IT gentamicin)
- Aminoglycoside - selectively ablates vestibular hair cells (chemical labyrinthectomy)
- Highly effective for vertigo control (>85%)
- Risk: hearing loss (dose-dependent)
- Delivered via injection, Silverstein MicroWick, or round window microcatheter
Noninvasive Procedures
- Meniett device: Low-pressure micropulse generator via tympanostomy tube; delivers intermittent positive pressure to middle ear; second-line when medications fail; modest evidence
Surgical Options (Refractory Cases)
| Surgery | Indication | Notes |
|---|---|---|
| Endolymphatic sac decompression/shunt | Useful hearing present | Hearing-preserving; decompresses the sac |
| Selective vestibular neurectomy | Useful hearing, failed conservative Rx | Retrolabyrinthine or retrosigmoid approach |
| Labyrinthectomy | No useful hearing, disabling vertigo | Definitive; abolishes vestibular function |
| Cochleosacculotomy | Elderly, disabling vertigo, poor hearing | Performed under local anesthesia |
Prognosis & Progression
- A 2025 meta-analysis (Hudson et al., Otol Neurotol) found a significant long-term risk of progression from unilateral to bilateral disease - important for counseling
- Hearing loss tends to be progressive and eventually permanent in the affected frequency range despite treatment
- Vertigo attacks often become less frequent over time (burnout), but hearing loss and tinnitus may persist
Emerging Therapy
SPI-1005 (ebselen) - An investigational drug targeting neuroinflammation, granted FDA Breakthrough Therapy designation in December 2025. Phase 3 data showed positive results for hearing loss, tinnitus, and vertigo in Meniere's disease. This represents the most promising pipeline drug to date.
Sources: Cummings Otolaryngology Head and Neck Surgery; K.J. Lee's Essential Otolaryngology; Bradley and Daroff's Neurology in Clinical Practice; Devantier et al. [PMID 40421807]; Hudson et al. [PMID 40210232]; Martínez et al. [PMID 41241712]; Hearing Health Foundation
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