摘要听神经病(auditory neuropathy)是指由听觉编码异常所造成的听觉障碍.这种听觉障碍除了影响听力外,还影响了言语理解能力.听神经病可以由内毛细胞、内毛细胞带状突触或耳蜗螺旋神经节细胞受损引起.目前遗传学、生理学及动物模型研究显示,破坏内毛细胞带状突触功能(通过改变基因表达从而影响突触前突触囊泡谷氨酸装载、钙离子内流或突触囊泡胞吐作用)可导致类似“听觉突触病变”的听力受损.此外,动物研究已证明过度声刺激会造成内毛细胞带状突触兴奋性毒性损伤,这可能是噪声暴露或年龄相关性听力损失造成听力障碍的机制.虽然听神经病的定义包括了中枢部分,但由于其发病少、病因和病理机制均不清楚,而大部分听神经病发生在外周听觉系统,所以本文主要集中阐述听神经病外周(突触和听神经)病理机制及相应临床发现,讨论听神经病患者听力康复的现行策略,并对未来恢复听力的治疗方式进行展望.

abstractsAuditory neuropathy (AN) is a hearing disorder where cochlear inner hair cell and/or the auditory nerve function is disrupted while outer hair cell function is normal.It can affect people of all ages,from infancy to adulthood.People with auditory neuropathy may have normal hearing threshold,or hearing loss ranging from mild to severe;they always have poor speech-perception abilities.It is a heterogeneous disorder which can have either congenital or acquired causes.AN may result from specific loss of cochlear inner hair cells,disordered release of neurotransmitters by inner hair cell ribbon synapses,deafferentation accompanying loss of auditory nerve fibers,neural dys-synchrony or conduction block as a result of demyelination of nerve fibers and auditory nerve hypoplasia.Although the definition of AN includes the central part,its incidence is low,and the etiology and pathology are not clear.The present review aimed to provide an overview of the genetic conditions associated with AN and highlight the neural and synaptic mechanism of AN.Possible strategy for treatments of AN was also discussed.