Wang K, Luo L, He Z Z
Medical School of Ningbo University, Ningbo 315211, China.
Department of Biomedical Sciences, Creighton University, Omaha, Nebraska 68178, USA.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2019 Feb 7;54(2):149-156. doi: 10.3760/cma.j.issn.1673-0860.2019.02.013.
Auditory 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.
听觉神经病（AN）是一种听力障碍，其耳蜗内毛细胞和/或听觉神经功能受到破坏，而外毛细胞功能正常。它可影响从婴儿到成人的所有年龄段人群。患有听觉神经病的人可能听力阈值正常，或有轻度至重度的听力损失；他们的言语感知能力通常较差。这是一种异质性疾病，可能有先天性或后天性病因。听觉神经病可能是由于耳蜗内毛细胞的特异性丧失、内毛细胞带状突触神经递质释放紊乱、伴随听觉神经纤维丧失的传入神经阻滞、神经纤维脱髓鞘导致的神经不同步或传导阻滞以及听觉神经发育不全。尽管听觉神经病的定义包括中枢部分，但其发病率较低，病因和病理尚不清楚。本综述旨在概述与听觉神经病相关的遗传状况，并突出听觉神经病的神经和突触机制。还讨论了治疗听觉神经病的可能策略。
