Suga F, Naunton R F, Maitland S K, Hedberg K E
J Laryngol Otol. 1976 Jul;90(7):667-85. doi: 10.1017/s0022215100082566.
Progressive sensorineural hearing losses found in seven members of three families are presented. Genetic transmission patterns in the study appeared to be autosomal dominant in two families and recessive in one family. The common audiological features of these cases include bilaterally symmetrical audiometric configuration and fairly good speech discrimination. The hearing losses of most of the cases appeared to begin at high frequencies progressing later to involve lower frequencies. The main histopathological changes in the temporal bones of one of the cases were degeneration of the organ of Corti and of the spiral ganglion in the lower cochlear coils and cystic degeneration of the stria vascularis in the upper coils.
本文报告了在三个家族的七名成员中发现的进行性感音神经性听力损失。研究中的遗传传递模式在两个家族中似乎是常染色体显性遗传，在一个家族中是隐性遗传。这些病例的常见听力学特征包括双侧对称的听力图形态和相当好的言语辨别力。大多数病例的听力损失似乎始于高频，随后发展至累及低频。其中一例颞骨的主要组织病理学变化是耳蜗下部螺旋器和螺旋神经节的退变以及耳蜗上部血管纹的囊性退变。
