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Genetic aspects and clinical characteristics of familial Meniere's disease in a South Korean population

 Jeon Mi Lee  ;  Mi Joo Kim  ;  Jinsei Jung  ;  Hyun Ji Kim  ;  Young Joon Seo  ;  Sung Huhn Kim 
 LARYNGOSCOPE, Vol.125(9) : 2175-2180, 2015 
Journal Title
Issue Date
Adult ; Age of Onset ; Family* ; Female ; Genetic Predisposition to Disease* ; Humans ; Male ; Meniere Disease/epidemiology ; Meniere Disease/genetics* ; Middle Aged ; Pedigree ; Prevalence ; Republic of Korea/epidemiology ; Surveys and Questionnaires
Meniere's disease ; autosomal dominant ; familial ; inheritance ; migraine
OBJECTIVES/HYPOTHESIS: This study was undertaken to investigate the prevalence, inheritance patterns, and clinical characteristics of familial Meniere's disease (MD) in a South Korean population. STUDY DESIGN: Direct interviews, telephone interviews, and reviews of the medical records of definite Meniere's disease patients and their families. METHODS: Direct and telephone interviews were performed for 286 definite MD patients and their family members who were suspected of having MD. The diagnosis of MD in family members was made by obtaining a detailed history, performing basic neurotological examinations and reviewing hearing test results. The clinical characteristics as well as the prevalence and inheritance patterns of familial MD were analyzed. RESULTS: The prevalence of familial Meniere-like syndrome (at least one family member with definite MD and other members with probable MD) and definite familial MD (two or more family members with definite Meniere's disease) were 9.8% and 6.3%, respectively, and the most common inheritance pattern was autosomal dominant with incomplete penetrance. The significant clinical characteristics of familial cases were an early disease onset and a higher prevalence of migraines. CONCLUSIONS: This is the first report describing the genetic aspects of MD in a single large Asian population. The prevalence of definite familial MD was 6.3% with an incomplete autosomal dominant inheritance pattern in most cases. Early-onset age and a high prevalence of migraines were significant clinical features of familial MD in this South Korean population. These data could provide a basis for the analysis of the genetic mechanism of familial MD in Asian populations.
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1. College of Medicine (의과대학) > Dept. of Otorhinolaryngology (이비인후과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Mi Joo(김미주)
Kim, Sung Huhn(김성헌)
Kim, Hyun Ji(김현지)
Lee, Jeon Mi(이전미)
Jung, Jinsei(정진세) ORCID logo https://orcid.org/0000-0003-1906-6969
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