Sekhar H K, Sachs M
Laryngoscope. 1976 Jan;86(1):117-25. doi: 10.1288/00005537-197601000-00023.
A case of bilaterally symmetrical genetic aplasia conforming to Mondini type of congenital deformity in a 12-day-old child is presented with the help of temporal bone sections. Cochlear changes include a stunted modiolus, deficient interscalar septum between the middle and upper coils forming a scala communis cochleae, a degenerated organ of Corti and reduced spiral ganglion cells and dendrites. The vestibule is malformed, with membranous labyrinth being deficient. The utricle and semicircular canals are absent. There is no oval window or stapedial footplate, and the facial nerve is hypoplastic. An interesting feature is the unusual association of bilateral bony choanal atresia, atrial septal defect, cleft lip, absence of olfactory bulbs in the brain, and congenital ophthalmic anomalies.
借助颞骨切片展示了一例12日龄儿童双侧对称性遗传性发育不全病例，符合蒙迪尼型先天性畸形。耳蜗变化包括蜗轴发育不良、中上部蜗圈之间的蜗管间隔缺失形成共通蜗管、柯蒂氏器退化以及螺旋神经节细胞和树突减少。前庭畸形，膜迷路缺失。椭圆囊和半规管缺如。没有卵圆窗或镫骨底板，面神经发育不全。一个有趣的特征是双侧骨性后鼻孔闭锁、房间隔缺损、唇裂、脑内嗅球缺如和先天性眼部异常的异常关联。
