What is Meniere's disease and how is it managed?

Ménière's Disease: Definition and Management

Ménière's disease is an inner ear disorder characterized by spontaneous vertigo attacks lasting 20 minutes to 12 hours, with documented low- to mid-frequency sensorineural hearing loss and fluctuating aural symptoms including tinnitus and ear fullness. 1

Definition and Diagnostic Criteria

Definite Ménière's disease requires:

• Two or more spontaneous attacks of vertigo, each lasting 20 minutes to 12 hours 1
• Audiometrically documented fluctuating low- to mid-frequency sensorineural hearing loss in the affected ear on at least one occasion before, during, or after one of the episodes of vertigo 1
• Fluctuating aural symptoms (hearing loss, tinnitus, or fullness) in the affected ear 1

Pathophysiology

• Associated with increased inner ear fluid (endolymph) volume, resulting in episodic ear symptoms 1
• The exact etiology remains unclear, though endolymphatic hydrops is a key histopathological feature 2
• Multiple biological and environmental factors may contribute, including genetics, autoimmunity, infection, trauma, and allergies 2

Clinical Presentation

• Vertigo: Sensation of self-motion (rotary spinning) or environment movement when neither is occurring 1
• Fluctuating hearing loss: Abrupt changes in hearing that alternate between worsening and improving 1
• Tinnitus: Ringing, buzzing, or other noises in the ear without external sound source 1
• Aural fullness: Sensation of pressure or plugged feeling in the ear 1
• Drop attacks (Tumarkin's Otolithic Crisis): Sudden falls without warning or loss of consciousness (may occur in later stages) 1

Differential Diagnosis

Several conditions may mimic Ménière's disease, including:

• Vestibular migraine: Attacks may be shorter or longer than MD; hearing loss less likely 1
• Benign paroxysmal positional vertigo: Positional vertigo lasting seconds; no associated hearing loss or tinnitus 1
• Vestibular schwannoma: Typically presents with chronic imbalance rather than episodic vertigo; hearing loss doesn't fluctuate 1
• Labyrinthitis: Sudden severe vertigo with prolonged symptoms (>24 hours) and profound hearing loss 1
• Stroke/ischemia: May include other neurologic symptoms; usually no associated hearing loss 1

Management Approach

First-Line: Lifestyle and Dietary Modifications

• Low-sodium diet (1500-2300 mg daily) to reduce inner ear fluid accumulation 1, 3
• Limit alcohol consumption as it may trigger attacks 1, 3
• Limit caffeine intake which can be a trigger for some patients 1, 3
• Identify and manage allergies, which may contribute to symptoms in up to 30% of patients 3
• Implement stress management techniques including relaxation, regular exercise, and adequate sleep 3
• Maintain a symptom diary to identify personal triggers 1, 3

Pharmacotherapy for Acute Attacks

• Offer limited courses of vestibular suppressants only during acute vertigo attacks 3
• Options include antihistamines (dimenhidrinato, meclizine) 3
• Benzodiazepines may help with associated anxiety but use cautiously due to dependency risk 3

Maintenance Therapy

• Diuretics to alter electrolyte balance in the endolymph and reduce its volume 3
• Betahistine (histamine analogue) to increase vasodilation to the inner ear, though evidence for efficacy is questionable based on recent trials 3

Second-Line: Intratympanic Therapies

• Intratympanic steroids for patients with active disease not responsive to non-invasive treatments 3
  • 85-90% improvement in vertigo symptoms compared to 57-80% with conventional therapy 3
  • Risks include hearing loss, tympanic membrane perforation, and persistent imbalance 3
• Intratympanic gentamicin for patients with persistent vertigo 3
  • Complete vertigo control rate of approximately 73.6% across studies 3
  • Risk of hearing loss varies by administration method 3

Surgical Options for Refractory Cases

• Endolymphatic sac surgery (hearing-sparing) 1, 4
• Vestibular nerve section (hearing-sparing) 1, 4
• Labyrinthectomy (hearing-ablative) for patients with non-usable hearing 1, 3, 4
• Combined approach of demolitive surgery with cochlear implantation may preserve hearing function 4

Important Considerations

• Physical examination findings are often unremarkable in Ménière's disease 1
• Conventional imaging is typically normal 1
• No definitive cure exists; treatment focuses on symptom management 3, 2
• The American Academy of Otolaryngology-Head and Neck Surgery does not recommend positive pressure therapy (Meniett device) 3
• Vestibular rehabilitation is not recommended for acute attacks but may help with chronic imbalance between episodes 3
• The disease may affect the contralateral ear in some patients, which should be considered before ablative procedures 1

Disease Course

• Adult-onset disorder (most commonly between 40-70 years) 1
• Vertigo attacks and symptom fluctuations are sporadic and unpredictable 1
• Hearing may worsen, persist, or stabilize over time 1
• Residual or permanent inner ear balance loss may require long-term vestibular therapy 1