[Meniere disease as an autosome dominant hereditary disease]

Within one year (1993) we found a positive family history in five out of forty-eight new patients with Menière's disease, corresponding to a frequency of 10.4%. We found between two and seven cases in each of five families. The disease followed a dominant autosomal hereditary pattern over two to four generations. Audiometric and vestibulometric examination confirmed the diagnosis. Clinical emphasis was placed on trigger factors such as infections, physical and psychological stress, the autonomic system, and metabolic and endocrinological disorders. There was no evidence of a significantly high incidence of any single trigger factor. The most important result of this study, which also included human leucocyte antigen (HLA) typing, is the fact that HLA A2 was represented in 90% of patients with positive family history of Menière's disease and in 75% of patients with solitary Menière's disease in contrast to only 28.9% in the average European population. The frequency of HLA B44 was 70% in family-linked Menière's disease and 37.5% in single Menière's disease. The frequency in the average European population is 12.3%. What is even more striking is the combination HLA A2 B44, occurring with respective frequencies of 60%, 37%, and 5%. These results suggest a multi-factor etiology of Menière's disease combined with a genetic predisposition, which might be caused by mutations on the short arm of chromosome 6.