Miyamoto R T, Robbins A J, Myres W A, Pope M L
Am J Otol. 1986 Jul;7(4):258-61.
We report the case of a profoundly deaf 4-year-old boy with congenital deafness as a result of Mondini's dysplasia. The Mondini inner ear malformation is the result of arrested labyrinthine development during embryogenesis and is characterized by both bony and membranous anomalies of the inner ear. The dysplastic cochlear anatomy does not preclude successful cochlear implantation, and electrical threshold measurements are similar to those recorded in pediatric subjects deafened as a result of other causes.
我们报告了一例因Mondini发育异常导致先天性耳聋的4岁重度失聪男孩的病例。Mondini内耳畸形是胚胎发育过程中迷路发育停滞的结果，其特征是内耳存在骨质和膜性异常。发育异常的耳蜗解剖结构并不妨碍成功进行人工耳蜗植入，并且电阈值测量结果与因其他原因致聋的儿科受试者所记录的结果相似。
