MéNièRe Disease

Basics

Description

  • Vertigo secondary to disease(s) of 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
  • Relatively uncommon (190/100,000 in the US) and dx of exclusion
  • Slight female > male (1.3:1)
  • Positive family history up to 20%
  • May develop at any age:
    • Peak incidence is age 40–60 yr
  • Affects more Caucasians of Northern European descent than individuals of African descent
  • No cure
  • Can be associated with significant morbidity

Etiology

  • Idiopathic
  • Endolymphatic hydrops (most cited): Blockage of the endolymphatic sac/duct > duct distension > Reissner membrane rupture > toxic bathing of basal surface hair cells and CNVIII with endolymph (potassium-rich intracellular fluid) > auditory and vestibular impact
  • Structural abnormalities: 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: Immune complex deposition in endolymphatic sac + autoantibodies directed against endolymphatic sac
  • Genetic: Autosomal dominant inheritance pattern, leading to earlier age of onset and more severe symptoms
  • Other proposed mechanisms: Hormonal (ADH, histamine, thyroid supplementation), subclinical viral infection causing hydrops many decades later (HSV secondary to recurrent activation), inhalant and food allergies (wheat), 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 (eg, thyroid disease, inner ear inflammation due to syphilis, medication)

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