Ménière's Disease

Ménière's Basics - Dizzying Definitions

• Disorder of the inner ear; linked to endolymphatic hydrops (excess fluid).
• Classic Triad:
  • Episodic vertigo: spinning, lasts 20 mins to 12 hrs.
  • Sensorineural Hearing Loss (SNHL): fluctuating, often low-frequency.
  • Tinnitus (ringing) &/or aural fullness.
  • 📌 Mnemonic: "Can't Hear, Can't Stand, Ringing Sound".
• Epidemiology:
  • Onset typically 30-60 years.
  • Slight female predilection.

⭐ The classic triad of Ménière's disease is episodic vertigo, sensorineural hearing loss (typically low-frequency and fluctuating), and tinnitus or aural fullness.

Pathophysiology - Pressure Problems

• Core pathology: Endolymphatic hydrops - significant distension of the membranous labyrinth due to an ↑ in endolymph volume.
• Primary mechanism: Believed to be impaired resorption of endolymph, primarily within the endolymphatic sac and duct.
• Etiological theories:
  • Idiopathic (most common)
  • Viral (e.g., HSV)
  • Autoimmune
  • Genetic factors
  • Vascular insufficiency
  • Trauma

⭐ The fundamental pathophysiological basis of Ménière's disease is endolymphatic hydrops.

Clinical Picture & Dx - Cracking the Case

• Key Symptoms (Classic Tetrad):

  • Episodic vertigo: Rotatory, lasts 20 mins - 12 hrs.
  • Sensorineural Hearing Loss (SNHL): Fluctuating, progressive; initially ↓low-frequency.
  • Tinnitus: Typically low-pitched, roaring or hissing.
  • Aural fullness/pressure in the affected ear.

• Associated Features: Nausea, vomiting common during vertigo. Tumarkin's otolithic crisis (sudden drop attacks without loss of consciousness) is rare but characteristic.

• Diagnostic Criteria (AAO-HNS 2015 - Simplified):

  • Definite Ménière's Disease:
    • Two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours.
    • Audiometrically documented low- to mid-frequency SNHL in the affected ear on at least one occasion before, during, or after one of the vertigo episodes.
    • Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the affected ear.
  • Probable Ménière's Disease:
    • Two or more episodes of vertigo or dizziness, each lasting 20 minutes to 24 hours.
    • Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the affected ear.
    • (Other causes excluded by investigation).

• Investigations:

  • Audiometry: Confirms SNHL (typically ↓low-frequency in early stages), may show recruitment, diplacusis.

    ⭐ Audiometry in early Ménière's disease typically reveals a fluctuating, low-frequency sensorineural hearing loss.

  • Electrocochleography (ECochG): May show an ↑Summating Potential (SP) / Action Potential (AP) ratio (e.g., >0.4); supports diagnosis of endolymphatic hydrops.
  • Vestibular Evoked Myogenic Potentials (VEMP): Can assess saccular and utricular function; may be abnormal.
  • MRI Brain (with gadolinium): Essential to rule out retrocochlear pathology (e.g., vestibular schwannoma, MS).

• Diagnostic Flow (Definite MD):

Management Maze - Treatment Tactics

Goals: Reduce vertigo frequency/severity, preserve hearing, alleviate tinnitus/fullness.

• Acute Attack Management:
  • Vestibular suppressants: Diazepam, Dimenhydrinate
  • Antiemetics: Promethazine, Prochlorperazine
• Chronic/Prophylactic Management:
  • Lifestyle: Low salt diet (<2-3 g/day), avoid caffeine/alcohol/nicotine, stress reduction.

    ⭐ A low-salt diet (e.g., <2-3 grams/day) is a cornerstone of conservative management for Ménière's disease.

  • Medical: Diuretics (HCTZ + triamterene), Betahistine (16-48 mg TID).
• Intratympanic Therapy (For refractory cases):
  • Steroids (e.g., Dexamethasone)
  • Gentamicin (chemical labyrinthectomy - for severe, intractable vertigo in unilateral disease).
• Surgical Options (Last resort for refractory cases):
  • Endolymphatic sac decompression/shunt
  • Vestibular neurectomy
  • Labyrinthectomy (sacrifices hearing).

High‑Yield Points - ⚡ Biggest Takeaways

• Classic triad: Episodic vertigo, fluctuating low-frequency SNHL, and tinnitus; often with aural fullness.
• Pathophysiology: Endolymphatic hydrops (distension of membranous labyrinth).
• Electrocochleography (ECoG) is diagnostic: ↑ SP/AP ratio (> 0.4).
• Acute management: Vestibular sedatives (e.g., prochlorperazine, diazepam), antiemetics.
• Chronic management: Salt restriction, diuretics (acetazolamide, HCTZ), betahistine.
• Refractory cases: Intratympanic gentamicin, endolymphatic sac surgery, or labyrinthectomy.
• Tumarkin's otolithic crisis (sudden drop attacks without loss of consciousness) is a severe manifestation.