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Familial Ménière's disease: clinical and genetic aspects

A W Morrison1, M E S Bailey, G A J Morrison

  • 1Late Honorary Consultant, The Royal London Hospital, London, UK.

The Journal of Laryngology and Otology
|July 12, 2008
PubMed
Abstract

Insights

Familial Ménière

Area of Science:

  • Genetics
  • Otolaryngology
  • Neurology

Background:

  • Ménière's disease affects approximately 1 in 2000 Caucasians, typically in their fifth decade.
  • While often sporadic, 5% of Ménière's disease cases have a familial component.
  • This study focuses on the clinical and genetic aspects of familial Ménière's disease in the UK.

Purpose of the Study:

  • To investigate the clinical and genetic characteristics of familial Ménière's disease.
  • To analyze inheritance patterns within affected families.

Main Methods:

  • Studied 46 families with Ménière's disease.
  • Diagnoses confirmed using the American Academy of Otolaryngology-Head and Neck Surgery criteria (1995) or stricter guidelines.

Main Results:

  • Autosomal dominant inheritance with reduced penetrance is the most probable inheritance pattern.
  • Potential genetic anticipation was noted, possibly due to ascertainment bias.
  • A slight trend towards maternal transmission was observed.

Conclusions:

  • Familial Ménière's disease exhibits autosomal dominant inheritance with reduced penetrance.
  • Further research into genetic anticipation and transmission patterns is warranted.
  • Detailed family pedigrees are available for review.

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