Ménière Disease



  • Disease of the inner ear
  • Classically unilateral ear involvement (may be bilateral in up to 40% of cases)
  • Characterized by recurrent spontaneous and episodic vertigo, sensorineural hearing loss, “roaring” tinnitus, and aural fullness
  • Estimated incidence about 15/100,000 in the U.S.
  • Slight female > male (1.3:1)
  • Positive family history up to 20%
  • May develop at any age
    • Peak incidence is age 20–40 yr
  • Affects more Whites of Northern European descent than Africans or Blacks
  • A benign disease without cure
  • Can be associated with significant morbidity


  • Idiopathic
  • Endolymphatic hydrops: Most often-cited theory describes blockage of the endolymphatic sac and duct leading to endolymphatic outflow obstruction and increased hydraulic pressure within the endolymphatic system:
    • Increased pressure causes a break in the membrane that separates the perilymph (potassium-poor extracellular fluid) and the endolymph (potassium-rich intracellular fluid)
    • The resultant chemical mixture bathes the vestibular nerve receptors, leading to a depolarization blockade and transient loss of function
  • May be associated with structural abnormalities such as atrophy of the sac, hypoplasia of the vestibular aqueduct, narrowing of the endolymphatic duct, forwardly located lateral sinus causing compression, and obstruction of the endolymphatic sac
  • Autoimmune processes suggested as an etiology, with immune complex deposition in endolymphatic sac and autoantibodies directed against endolymphatic sac
  • Thought to have a genetic predisposition, autosomal dominant inheritance pattern, leading to earlier age of onset and more severe symptoms
  • Other proposed mechanisms are subclinical viral infection causing hydrops many decades later, and ischemia of the endolymphatic sac and the inner ear
  • Need to differentiate Ménière syndrome from other disease processes that interfere with normal production or resorption of endolymph (e.g., thyroid disease, inner ear inflammation due to syphilis, medication)

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