Ostri B, Johnsen T, Bergmann I
Department of Audiology, Bispebjerg Hospital, Roskilde, Denmark.
Clin Otolaryngol Allied Sci. 1991 Apr;16(2):163-7. doi: 10.1111/j.1365-2273.1991.tb01969.x.
A family group with confirmed branchio-oto-renal (BOR) syndrome was investigated in this study. Computerized tomography of the temporal bones has demonstrated that the malformations of the inner ear consist of hypoplastic structural changes within the cochlea with reduced vertical diameters, and absent or hypoplastic semicircular canals and normal endolymphatic ducts. It is concluded that in the present cases, the Mondini malformation of the cochlea is not associated with the BOR syndrome.
本研究对一个确诊为鳃耳肾（BOR）综合征的家族群体进行了调查。颞骨计算机断层扫描显示，内耳畸形包括耳蜗内结构发育不全，垂直径减小，半规管缺如或发育不全，以及内淋巴管正常。得出的结论是，在目前的病例中，耳蜗的Mondini畸形与BOR综合征无关。
