Bachor E, Karmody C S
Department of Otorhinolaryngology, Universität Ulm, Germany.
J Laryngol Otol. 2001 Mar;115(3):243-6. doi: 10.1258/0022215011907118.
The temporal bones of a 26-month-old white female with a paralytic syndrome clinically and pathologically identical to poliomyelitis were examined. The aetiological agent was unknown although a non-poliomyelitis enterovirus infection seemed likely. There was a complete absence of the cochlear neurons and substantially reduced peripheral and central axons with loss of some inner hair cells but preservation of outer hair cells. Scarpa's ganglion, and the geniculate ganglion were partially atrophied. The saccule and utricle were mildly dilated and Reissner's membrane of the apical turn was bulging. In two previous audiological studies a 10--20 dB bilateral sensorineural hearing loss was found in poliomyelitis patients and a neuronal lesion was postulated which is now supported by our findings. This is a rare example of an almost pure neural hearing loss.
对一名26个月大的白人女性的颞骨进行了检查,该女性临床上和病理上患有与小儿麻痹症相同的麻痹综合征。尽管非脊髓灰质炎肠道病毒感染似乎很有可能,但病因不明。耳蜗神经元完全缺失,外周和中枢轴突显著减少,一些内毛细胞丢失,但外毛细胞保留。斯卡帕神经节和膝状神经节部分萎缩。球囊和椭圆囊轻度扩张,顶转的瑞氏膜膨出。在之前的两项听力学研究中,小儿麻痹症患者发现双侧感音神经性听力损失10 - 20分贝,并推测存在神经元病变,我们的研究结果现在支持了这一点。这是一个几乎纯神经性听力损失的罕见例子。