Tuomas Klockars1, Erna Kentala. 1. Department of Otorhinolaryngology, Helsinki University Central Hospital, Helsinki, Finland. tuomas.klockars@fimnet.fi
Abstract
OBJECTIVE: To study the inheritance of Ménière's disease in the Finnish population. DESIGN: A detailed questionnaire was sent to patients with symptoms resembling Ménière's disease previously examined at the Department of Otorhinolaryngology, Helsinki University Central Hospital, Finland. PATIENTS: The study population comprised 118 patients with symptoms resembling Ménière's disease. The patients were divided into groups based on the diagnostic criteria by the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology-Head and Neck Surgery. MAIN OUTCOME MEASURES: Relatives with Ménière's disease, geographic distribution of birthplaces of grandparents, symptoms, vestibular findings, and audiologic and otoneurologic tests. RESULTS: Approximately 15% of the patients with definite Ménière's disease were found to represent familial disease. The majority of these patients were female, and they had more severe and intense attacks compared with patients with sporadic Ménière's disease. The mode of inheritance is autosomal dominant with incomplete penetrance. CONCLUSIONS: A significant part of Ménière's disease is inherited. The use of genetic isolates in which genetic homogeneity can be assumed might lead to the identification of gene defects leading to Ménière's disease.
OBJECTIVE: To study the inheritance of Ménière's disease in the Finnish population. DESIGN: A detailed questionnaire was sent to patients with symptoms resembling Ménière's disease previously examined at the Department of Otorhinolaryngology, Helsinki University Central Hospital, Finland. PATIENTS: The study population comprised 118 patients with symptoms resembling Ménière's disease. The patients were divided into groups based on the diagnostic criteria by the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology-Head and Neck Surgery. MAIN OUTCOME MEASURES: Relatives with Ménière's disease, geographic distribution of birthplaces of grandparents, symptoms, vestibular findings, and audiologic and otoneurologic tests. RESULTS: Approximately 15% of the patients with definite Ménière's disease were found to represent familial disease. The majority of these patients were female, and they had more severe and intense attacks compared with patients with sporadic Ménière's disease. The mode of inheritance is autosomal dominant with incomplete penetrance. CONCLUSIONS: A significant part of Ménière's disease is inherited. The use of genetic isolates in which genetic homogeneity can be assumed might lead to the identification of gene defects leading to Ménière's disease.
Authors: Wilhelm H Flatz; Annika Henneberger; Maximilian F Reiser; Robert Gürkov; Birgit Ertl-Wagner Journal: J Vis Exp Date: 2018-02-21 Impact factor: 1.355
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