Zheng Dandan, Liu Xiao
Department of Otorhinolaryngology, 2nd Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.
Front Neurosci. 2020 May 21;14:447. doi: 10.3389/fnins.2020.00447. eCollection 2020.
Auditory neuropathy is a special type of hearing loss caused by dysfunction of the synapse of the inner hair cells, the auditory nerve, and/or the auditory nerve itself. For patients with auditory neuropathy who have severe to profound hearing loss or failed auditory skills development with hearing-aids, cochlear implantation (CI) serves as the only possible effective treatment. It is accepted that the exact sites of lesion causing auditory neuropathy determine the CI performance. Mutations in the gene were the first identified and the most common cause of congenital auditory neuropathy. The site of lesion in patients with auditory neuropathy caused by biallelic mutations (-related auditory neuropathy) is presumed to be presynaptic, leaving auditory nerve function intact. Thus, -related auditory neuropathy is expected to have good CI performances. In this review, we describe the CI outcomes in patients with mutations. We will focus on whether biallelic mutations are ideal indications for CI in patients with auditory neuropathy. Also, the factors that may still influence the CI outcomes in patients with mutations are discussed.
听神经病是一种由内毛细胞、听神经和/或听神经本身的突触功能障碍引起的特殊类型的听力损失。对于患有重度至极重度听力损失或使用助听器后听觉技能发育不良的听神经病患者，人工耳蜗植入（CI）是唯一可能有效的治疗方法。人们认为，导致听神经病的病变确切部位决定了人工耳蜗植入的效果。该基因的突变是首次被发现且是先天性听神经病最常见的原因。由双等位基因突变引起的听神经病患者（与相关的听神经病）的病变部位被推测为突触前，听神经功能完好。因此，与相关的听神经病预计人工耳蜗植入效果良好。在本综述中，我们描述了携带突变患者的人工耳蜗植入结果。我们将重点关注双等位基因突变是否是听神经病患者人工耳蜗植入的理想适应症。此外，还讨论了可能仍会影响携带突变患者人工耳蜗植入结果的因素。
