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Peripheral· Onset: Episodic; episodes last 20 min–12 h · Duration: Lifelong; fluctuating with progressive hearing loss
Ménière's disease
Overview
Summary
Idiopathic syndrome of recurrent episodic vertigo with fluctuating sensorineural hearing loss, aural fullness, and tinnitus, attributed to endolymphatic hydrops.
Diagnostic criteria
See the Bárány Society / clinical practice guideline papers[8,9] listed on the references page for the consensus diagnostic framework used in this profile.
Epidemiology
Prevalence ~200/100,000. Onset 40–60s. Bilateral in 15–30% over time.
Pathophysiology
Endolymphatic hydrops — distension of the endolymphatic compartment — with episodic rupture of the membranous labyrinth (potassium shower theory) or pressure-mediated mechanical disruption.
Prognosis
Vertigo 'burns out' over decades in many; hearing loss progresses. Disability is variable.
Key examination findings
- ◆During an attack: spontaneous horizontal nystagmus, often initially toward affected ear (irritative phase), later away (paretic phase)
- ◆Between attacks: examination may be normal early; later, signs of unilateral hypofunction
- ◆Tuning-fork tests confirm low-frequency SNHL (Rinne AC>BC, Weber to better ear)
Investigations
- ▸Audiogram: fluctuating low-frequency SNHL (early); flat or peaked pattern later
- ▸Caloric: progressive unilateral weakness over years
- ▸ECochG: enlarged SP/AP ratio (>0.4) supports hydrops
- ▸Gadolinium-enhanced MRI inner ear (cisternographic sequences): direct hydrops visualization
Management
- ●Low-sodium diet (<2 g/day) and diuretic (HCTZ 25 mg) — first line
- ●Betahistine widely used outside the US (evidence mixed)
- ●Intratympanic steroids for breakthrough vertigo
- ●Intratympanic gentamicin (chemical labyrinthectomy) for refractory unilateral disease
- ●Endolymphatic sac surgery or labyrinthectomy/vestibular nerve section in failures
Clinical pearls
- ★AAO-HNS criteria (2015): definite Ménière's requires ≥2 vertigo episodes 20 min–12 h + documented low-to-medium frequency SNHL on affected side + fluctuating aural symptoms.
- ★Vestibular migraine is the most common Ménière's mimic.