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Familial Ménière's disease: clinical and genetic aspects

Published online by Cambridge University Press:  11 July 2008

A W Morrison ,
M E S Bailey  and
G A J Morrison
A W Morrison
Affiliation:
Late Honorary Consultant, The Royal London Hospital, London, UK
M E S Bailey
Affiliation:
Division of Molecular Genetics, Institute of Biomedical and Life Sciences, University of Glasgow, London, UK
G A J Morrison*
Affiliation:
Department of Otolaryngology, Guy's and St Thomas' NHS Trust, London, UK
*
Address for Correspondence: Mr Gavin A J Morrison, Department of Otolaryngology, St Thomas' Hospital, London SE1 7EH, UK. E-mail: GAJM@GavinMorrison.com
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Abstract

Background and purpose:

Ménière's disease is not uncommon, with an incidence in Caucasians of about one in 2000. The incidence peaks in the fifth decade. Cases are usually isolated or sporadic, but in perhaps five per cent other family members are affected. We report here the clinical and genetic characteristics of a comprehensive set of familial Ménière's disease cases from the UK.

Methods:

Forty-six affected families were studied. All cases were diagnosed using the American Academy of Otolaryngology–Head and Neck Surgery committee on hearing and equilibrium 1995, or more stringent, criteria.

Outcomes and results:

Autosomal dominant inheritance with reduced penetrance was the most likely mode of inheritance overall. Apparent genetic anticipation was observed, but may also be a result of ascertainment bias given the collection strategy. There was also a slight tendency for cases to result from maternal transmission within the families in this set. The family pedigrees are presented, and the authors have also set up a website at which all the pedigrees may be viewed in greater detail.

Keywords

Ménière's DiseaseFamilialEpidemiologyGeneticsDominantAnticipation

Information

Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 1 , January 2009 , pp. 29 - 37
Copyright
Copyright © JLO (1984) Limited 2008

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References

Supplementary material

Supplementary Figures are available online at http://www.jlo.co.uk.

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