MENIERES-DISEASE AS AUTOSOMAL-DOMINANT IN HERITED TRAIT

Within one year (1993) we found a positive family history in five out of forty-eight new patients with Meniere's disease, corresponding to a frequency of 10.4%. We found between two and seven cases in each of f ive families. The disease followed a dominant autosomal hereditary pat tern over two to four generations. Audiometric and vestibulometric exa mination confirmed the diagnosis. Clinical emphasis was placed on trig ger 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 trigg er factor. The most important result of this study, which also include d human leucocyte antigen (HLA) typing, is the fact that HLA AZ was re presented in 90% of patients with positive family history of Meniere's disease and in 75% of patients with solitary Meniere's disease in con trast to only 28.9% in the average European population. The frequency of HLA B44 was 70% in family-linked Meniere's disease and 37.5% in sin gle Meniere's disease. The frequency in the average European populatio n is 12.3%. What is even more striking is the combination HLA AZ B44, occurring with respective frequencies of 60%, 37%, and 5%. These resul ts suggest a multi-factor etiology of Meniere's disease combined with a genetic predisposition, which might be caused by mutations on the sh ort arm of chromosome 6.