Ménière Disease

Basics

  • An inner ear (labyrinthine) disorder characterized by recurrent attacks of hearing loss, tinnitus, vertigo, and sensations of aural fullness
  • The International Diagnostic Criteria define Ménière disease as a condition with at least two spontaneous episodes of vertigo lasting longer than 20 minutes but <12 hours, an audiogram showing evidence of low- to medium-frequency sensorineural hearing loss in one ear at any point in time, and fluctuating aural symptoms such as hearing loss, tinnitus, or aural fullness (1).
  • Clinically, it involves the triad of:
    • Vertigo lasting 20 minutes to 12 hours
    • Audiometrically documented sensorineural hearing loss (predominantly low frequency)
    • Fluctuating aural symptoms (tinnitus or aural fullness)

Description

  • Often unilateral initially; nearly half become bilateral over time.
  • Severity and frequency of vertigo may diminish with time, but hearing loss is often progressive and/or fluctuating.
  • Usually idiopathic (Ménière disease) but may be secondary to another condition causing endolymphatic hydrops (Ménière syndrome)
  • There are 5 clinical subtypes of both unilateral and bilateral disease (1).
    • Type 1: refers to classic unilateral MD and metachronic bilateral MD (symptom onset in one ear followed by the other)
    • Type 2: refers to delayed unilateral MD (hearing loss onset preceding vertigo onset by months or years) or synchronic bilateral MD (simultaneous symptom onset in both ears)
    • Type 3: familial MD (most families have bilateral hearing loss, but unilateral patient may coexist in the same family)
    • Type 4: sporadic MD with migraine
    • Type 5: sporadic MD with an autoimmune disease
  • System(s) affected: nervous
  • Synonym(s): Ménière syndrome; endolymphatic hydrops

Epidemiology

  • Predominant age of onset: 40 to 60 years
  • Predominant gender: female > male (2:1)
  • Race/ethnicity: white, Northern European > blacks

Incidence
Estimates 1 to 150/100,000 per year

Etiology and Pathophysiology

  • Ménière syndrome may be secondary to injury or other disorders (e.g., reduced middle ear pressure, allergy, endocrine disease, lipid disorders, vascular, viral, syphilis, autoimmune). Any disorder that could cause endolymphatic hydrops could be implicated in Ménière syndrome.
  • Theories include increased pressure of the endolymph fluid due to increased fluid production or decreased resorption. This may be caused by endolymphatic sac pathology, abnormal development of the vestibular aqueduct, or inflammation caused by circulating immune complexes. Increased endolymph pressure may cause rupture of membranes and changes in endolymphatic ionic gradient.
  • Others include vascular compromise, cochlear trauma, and viral infection or reactivation

Genetics
Family history and genetic predisposition is found in 10% of cases with an autosomal dominant inheritance pattern (2).

Risk Factors

May include

  • Stress
  • Allergy
  • Increased salt intake
  • Caffeine, alcohol, or nicotine
  • Chronic exposure to loud noise
  • Certain vascular abnormalities (including migraines)
  • Certain viral exposures (especially herpes simplex virus [HSV])

General Prevention

Reduce known risk factors.

Commonly Associated Conditions

  • Anxiety (secondary to the disabling symptoms)
  • Migraines
  • Hyperprolactinemia
  • Hypothyroidism

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