Inheritance of Ménière’s Disease in the Finnish Population

Abstract

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.

Familial Ménière's disease: clinical and genetic aspects

“…A recent paper from Finland gives a comparable figure of approx. 15 per cent 24 . In our second paper, also in 1987 25 , we reported 35 first degree relatives from a series of 671 confirmed MD patients (5.4 per cent) rising to an overall frequency of familial cases of 7.7 per cent if second and third degree relatives were included: these figures however were extracted from the family history in clinical records without actual diagnostic confirmation in many of the relatives.…”

Finnish familial Meniere disease is not linked to chromosome 12p12.3, and anticipation and cosegregation with migraine are not common findings

“…There have been numerous reports on familial MD (FMD), [3][4][5][6] the estimated proportion of which in Finland is approximately 15%. 7 In addition to familial clustering, differences in race and population incidences 8 and geographical distribution 2 support the role of genetic factors in the development of MD. A link has been demonstrated between migraine and MD, 9,10 and it has even been suggested that MD develops as a complication of migraine-associated symptoms.…”

Epidemiologia da Doença de Ménière: Revisão da Literatura

“…48 Klockars e Kentala encontram uma prevalência de 15% de casos de doença de Ménière familiar (DMF) na Finlândia, sendo a grande maioria do sexo feminino. 49 Morrison reporta pela primeira vez antecipação na DMF, ou seja, um aparecimento mais precoce na DMF tendo em conta a idade, assim como uma tendência no aumento da severidade e intensidade dos ataques nas gerações sucessivas de DMF. 50 Estes achados foram suportados mais tarde por estudos efetuados por Fung et al, Oliveira et al e Klockars e Kentala.…”

“…50 Estes achados foram suportados mais tarde por estudos efetuados por Fung et al, Oliveira et al e Klockars e Kentala. 49,51,52 Contudo, em algumas famílias esta antecipação não foi observada. 51 Morrison et al documentam uma antecipação, da quinta para a quarta década de vida, no aparecimento da DMF quando comparada com a DM esporádica.…”