Arweiler D J, Jahnke K, Grosse-Wilde H
Universitäts-Hals-Nasen-Ohren-Klinik Essen.
Laryngorhinootologie. 1995 Aug;74(8):512-5. doi: 10.1055/s-2007-997791.
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.
在一年时间内（1993年），我们在48例新诊断的梅尼埃病患者中发现5例有阳性家族史，频率为10.4%。我们在五个家族中分别发现2至7例患者。该疾病在两到四代人中遵循常染色体显性遗传模式。听力测定和前庭功能测定检查确诊了该疾病。临床重点关注触发因素，如感染、身体和心理压力、自主神经系统以及代谢和内分泌紊乱。没有证据表明任何单一触发因素的发病率显著升高。这项研究还包括人类白细胞抗原（HLA）分型，其最重要的结果是，与欧洲平均人群中仅28.9%的比例相比，梅尼埃病阳性家族史患者中90%携带HLA A2，散发性梅尼埃病患者中75%携带HLA A2。在家族性梅尼埃病中HLA B44的频率为70%，在散发性梅尼埃病中为37.5%。在欧洲平均人群中的频率为12.3%。更引人注目的是HLA A2 B44的组合，其出现频率分别为60%、37%和5%。这些结果表明梅尼埃病是多因素病因并伴有遗传易感性，这可能是由6号染色体短臂上的突变引起的。
