Benitez J T, Bouchard K R, Lane-Szopo D
Am J Otol. 1980 Jan;1(3):163-7.
A Caucasian male suffered a skull fracture at age twenty-two. There was bilateral deafness with partial recovery of hearing in the left ear. He was able successfully to wear a hearing aid in this ear for only thirteen years. His balance remained impaired until death at age fifty of unrelated causes. Temporal bone histologic studies revealed bilateral transverse fractures extending through the ampullated end of the three semicircular canals and vestibula. The cochleae were not involved. In the right ear, the organ of Corti was missing in the basal 14 mm and shrunken in the remainder of the cochlea. In the left ear, the organ of Corti was missing in the basal 14 mm and present with partial hair cell loss in the remainder of the cochlea. Spiral ganglion neuron loss correlated in severity with the organ of Corti lesions bilaterally. The membranous vestibular labyrinth showed distension and ruptures with total loss of sensory epithelium of the cristae and maculae bilaterally. Progressive deterioration of left ear residual hearing cannot be explained on the basis of sensorineural deficit; it probably was the result of biochemical alterations. Total loss of vestibular sensory epithelium bilaterally explained the persistent disequilibrium.
一名白种男性在22岁时颅骨骨折。双耳失聪，左耳听力部分恢复。他仅能成功佩戴该耳的助听器13年。直至50岁因无关病因去世，其平衡功能一直受损。颞骨组织学研究显示双侧横行骨折，骨折线延伸至三个半规管和前庭的壶腹端。耳蜗未受累。右耳，柯蒂器在蜗底14毫米处缺失，耳蜗其余部分萎缩。左耳，柯蒂器在蜗底14毫米处缺失，耳蜗其余部分存在部分毛细胞丢失。螺旋神经节神经元损失的严重程度与双侧柯蒂器病变相关。膜性前庭迷路显示扩张和破裂，双侧嵴和斑的感觉上皮完全丧失。左耳残余听力的进行性恶化不能基于感音神经性缺陷来解释；这可能是生化改变的结果。双侧前庭感觉上皮完全丧失解释了持续的平衡失调。
