摘要
听神经病(auditory neuropathy，AN)是1996年Starr首次提出的，定义为第Ⅷ脑神经的听神经支受损而引起的一种临床表现特殊的感音神经性聋。其特点包括：听性脑干反应(ABR)缺失或严重异常，诱发性耳声发射(EOAE)正常，言语分辨率不成比例的明显差于纯音听阈，听力图多以低频听阈升高为主，镫骨肌声反射消失或阈值升高和EOAE对侧抑制消失等。这是一组不同于一般感音神经性聋、具有特征的听功能障碍的症侯群，近年来已引起越来越多的广泛关注。目前对其命名、病变部位意见不一，病因及发病机制尚不清楚，治疗亦无十分有效措施。这一课题已成为目前国内外耳科领域研究新的热点之一。
目的
本课题旨在总结听神经病(AN)的临床表现特点和听力学特征，探讨听力学检测在AN诊断中的意义；观察AN患者伴发前庭上、下神经损害的特点，分析其与听神经病的关系；调查AN并发其他神经系统疾病的情况；分析单侧AN的临床表现和听力学特点；以提高对AN的诊断和进一步加深认识。同时根据听神经髓鞘组成特点，通过合成具有抗原特异性的PLP_(139-151)和PO_(180-199)多肽，分别诱导大鼠产生实验性自身免疫性脑脊髓炎(EAE)和
Auditory neuropathy (AN) first named by Starr in 1996, which has the specific clinical characteristics, is one kind of sensorineural hearing loss caused by the injury of acoustic nerve branch of the eighth cranial nerve. The most important audiological characteristic of AN patients is that auditory brainstem responses (ABR) are absent or abnormal severely, but evoked otoacoustic emissions (EOAE) are normally recorded, and the speech hearing is worse obviously than the pure tone hearing, and the audiogram is bilateral ascending curve mostly, and pure tone auditory threshold is higher obviously at low frequencies, and acoustic stapedius reflex is absent and the contralateral suppression effect of distortion product otoacoustic emissions(DPOAE) is disappeared.AN is different from other common sensorineural deafness, which has the specific hearing impairment. AN has attracted more and more attention recently. The name of AN and site of AN lesion are still need discuss.The pathogeny and pathogenesis of AN are unknown. The effective treatment of AN is still absent. The present subject has been the one of the hotspots in Otorhinolaryngology.
引文
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