Here is a thorough overview of Meniere's disease based on authoritative otolaryngology and medicine textbooks:
Meniere's Disease
What Is It?
Meniere's disease (MD) is a disorder of the inner ear caused by endolymphatic hydrops - an abnormal buildup of fluid (endolymph) in the membranous labyrinth. It produces a characteristic set of episodic symptoms affecting both hearing and balance.
Classic Symptoms (The "Tetrad")
- Fluctuating sensorineural hearing loss - typically affects low frequencies early on
- Tinnitus - ringing or roaring in the affected ear, which often worsens before an attack
- Aural fullness - a sensation of pressure or blockage in the ear
- Episodic vertigo - sudden attacks of spinning/dizziness lasting 30 minutes to 2 hours
The tinnitus, fullness, and hearing loss typically intensify as a warning before a vertigo attack strikes. - K.J. Lee's Essential Otolaryngology
Important Variants
- Cochlear hydrops: fluctuating hearing loss and tinnitus without vertigo
- Vestibular hydrops: episodic vertigo and aural fullness without hearing symptoms
- Lermoyez syndrome: tinnitus and hearing loss worsen progressively, then are suddenly relieved by a vertigo attack
- Crisis of Tumarkin (drop attacks): sudden loss of extensor power causing the patient to fall, without loss of consciousness - occurs late in the disease
Epidemiology
- In 85% of patients, only one ear is affected (unilateral)
- If the second ear becomes involved, it usually happens within 36 months
- Spontaneous remission occurs in about 60% of patients eventually
Diagnosis
- Audiometry: shows fluctuating low-tone sensorineural hearing loss with little tone decay
- ENG (electronystagmography): often normal between attacks; during attacks may show spontaneous nystagmus
- Glycerol test: oral glycerol (1.2 mL/kg) may temporarily improve hearing, tinnitus, and fullness within 1-3 hours - used as a diagnostic aid
Pathophysiology
The underlying cause is thought to involve immune dysfunction and/or abnormalities in endolymph production/reabsorption. The stria vascularis (a structure in the inner ear responsible for endolymph production) has been increasingly implicated - a 2025 systematic review (PMID: 40987969) specifically examined its role in disease pathogenesis.
A 2025 meta-analysis (PMID: 40134376) also found an association between Meniere's disease and thyroid disease, suggesting metabolic/autoimmune links.
Treatment
Medical (Conservative)
- Vestibular suppressants (e.g., meclizine) - to reduce vertigo during attacks
- Diuretics - to reduce endolymph volume
- Vasodilators - to improve inner ear circulation
- Low-sodium diet - helps reduce endolymph fluid buildup
- Intratympanic (IT) steroids (e.g., dexamethasone) - injected through the eardrum; can achieve vertigo control in 80% of patients and reduce tinnitus in ~74%, and can help many patients avoid ablative surgery
Surgical (when medical therapy fails)
Hearing-destructive procedures (when no useful hearing remains):
- Labyrinthectomy
- Translabyrinthine eighth nerve section
Hearing-preserving procedures:
- Selective vestibular nerve section (via middle cranial fossa, retrolabyrinthine, or retrosigmoid approach)
- Intratympanic gentamicin (chemical ablation of vestibular function)
- Endolymphatic sac decompression or mastoid shunt
- Cochleosacculotomy (for elderly patients with disabling vertigo and poor hearing)
Key Points to Remember
| Feature | Detail |
|---|
| Cause | Endolymphatic hydrops (fluid excess in inner ear) |
| Vertigo duration | 30 min - 2 hours per attack |
| Hearing loss type | Sensorineural, low-frequency, fluctuating |
| Laterality | Unilateral in 85% |
| Spontaneous remission | ~60% of cases |
| First-line treatment | Diet, diuretics, vestibular suppressants |
Sources: K.J. Lee's Essential Otolaryngology; Cummings Otolaryngology Head and Neck Surgery