Familial Ménière's disease: clinical and genetic aspects
Autor: | G A J Morrison, A W Morrison, Mark E.S. Bailey |
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Jazyk: | angličtina |
Rok vydání: | 2009 |
Předmět: |
Adult
Male medicine.medical_specialty Pediatrics Adolescent Pedigree chart Disease White People Tinnitus Young Adult Epidemiology medicine Humans Genetic Predisposition to Disease Age of Onset Child Hearing Loss Meniere Disease Aged Aged 80 and over business.industry Incidence (epidemiology) General Medicine Middle Aged medicine.disease Penetrance United Kingdom Pedigree Surgery Otorhinolaryngology Child Preschool Anticipation (genetics) Vertigo Female Age of onset business Meniere's disease |
ISSN: | 0022-2151 |
Popis: | 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. |
Databáze: | OpenAIRE |
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