Meniere Disease
An inner ear disorder causing sudden attacks of spinning dizziness, ringing in the ear, and progressive hearing loss due to fluid buildup.
Emergency Management: Severe, intractable vomiting leading to severe dehydration and acute kidney injury during an attack requires IV fluids, IV antiemetics, and hospital admission.
Meniere Disease is a chronic disorder of the inner ear defined by the classic triad of episodic spontaneous vertigo, fluctuating sensorineural hearing loss, and tinnitus, often accompanied by a sensation of aural fullness.
Detailed Overview
The underlying pathological mechanism is endolymphatic hydrops, an accumulation of endolymph fluid leading to distension of the membranous labyrinth. The attacks are episodic, severely debilitating, and last from 20 minutes to several hours. Over time, the fluctuating hearing loss becomes a permanent, progressive decline in low-frequency hearing. Management ranges from conservative dietary restrictions and diuretics to destructive surgical procedures for refractory vertigo.
Epidemiology & Demographics
Prevalence is approximately 190 per 100,000 in the US. Typically presents between the ages of 40 and 60. Slightly more common in females.
Etiological Mechanism
Primarily idiopathic. It is thought to result from impaired resorption of endolymphatic fluid in the endolymphatic sac, leading to increased pressure.
Primary Causes
Unknown (idiopathic). Secondary causes of endolymphatic hydrops (Meniere's syndrome) include viral infections (HSV, VZV), head trauma, and autoimmune inner ear disease.
Defective absorption of endolymph in the endolymphatic sac causes the endolymphatic volume and pressure to increase (Endolymphatic Hydrops). This ballooning distorts the structures of the inner ear. The acute vertigo attacks are hypothesized to occur when Reissner's membrane ruptures, mixing potassium-rich endolymph with sodium-rich perilymph. This sudden chemical imbalance depolarizes the vestibular nerve, firing an intense false signal of movement (vertigo) to the brain. Healing of the membrane halts the attack, but repeated ruptures eventually destroy the delicate cochlear hair cells, causing permanent sensorineural hearing loss.
Diagnostic Criteria & Guidelines
Clinical diagnosis defined by the Barany Society: 1) Two or more spontaneous episodes of vertigo lasting 20 mins to 12 hours. 2) Audiometrically documented low- to medium-frequency sensorineural hearing loss in one ear. 3) Fluctuating aural symptoms (tinnitus, fullness) in the affected ear. 4) Not better accounted for by another diagnosis.
Conservative: Low sodium diet (<1500 mg/day), restrict caffeine/alcohol. Pharmacotherapy for prevention: Diuretics (Hydrochlorothiazide 25mg PO daily or Acetazolamide) or Betahistine 16mg PO TID. For acute attacks: Vestibular suppressants (Meclizine 25mg PO Q6H, Diazepam, or Promethazine).
Second-Line & Adjunctive Therapy
Intratympanic Corticosteroid Injections (Dexamethasone) for refractory vertigo with preserved hearing. Meniett device (delivers pressure pulses to the ear).
Surgical & Procedural Management
For severe refractory cases: Endolymphatic sac decompression (hearing-sparing). If hearing is already poor and vertigo is disabling: Intratympanic Gentamicin ablation, Vestibular Nerve Section, or complete Labyrinthectomy (destructive procedures).
Patient Counseling & Advice
Warn the patient that the disease causes permanent hearing loss over time. Assure them that while the vertigo attacks are terrifying, they are not life-threatening. Discuss the risks of 'drop attacks' (falling).
Follow-Up & Monitoring Schedule
Annual audiograms to track the progression of hearing loss. Frequent evaluations to assess the efficacy of dietary and diuretic therapies in controlling vertigo.
Preventive Strategies
Attacks can be minimized by strict dietary control, but the underlying progression of the disease cannot be entirely prevented.
Highly variable. About 50-70% respond to conservative medical management. Ultimately, vertigo 'burns out' after many years, leaving the patient with significant unilateral hearing loss and potential chronic imbalance.