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 OTOF 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 OTOF mutations (OTOF-related auditory neuropathy) is presumed to be presynaptic, leaving auditory nerve function intact. Thus, OTOF-related auditory neuropathy is expected to have good CI performances. In this review, we describe the CI outcomes in patients with OTOF mutations. We will focus on whether biallelic OTOF mutations are ideal indications for CI in patients with auditory neuropathy. Also, the factors that may still influence the CI outcomes in patients with OTOF mutations are discussed.

中文翻译：

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OTOF 突变患者的人工耳蜗植入结果

听神经病是一种特殊类型的听力损失，由内毛细胞、听神经和/或听神经本身的突触功能障碍引起。对于患有严重至极重度听力损失或助听器听觉技能发展失败的听神经病患者，人工耳蜗植入 (CI) 是唯一可能的有效治疗方法。公认的是，导致听神经病变的确切病变部位决定了 CI 性能。OTOF 基因突变是先天性听神经病的第一个发现也是最常见的原因。由双等位基因 OTOF 突变引起的听神经病（OTOF 相关听神经病）患者的病变部位被认为是突触前的，听神经功能完好无损。因此，预计 OTOF 相关的听神经病具有良好的 CI 表现。在这篇综述中，我们描述了 OTOF 突变患者的 CI 结果。我们将关注双等位基因 OTOF 突变是否是听神经病患者 CI 的理想指征。此外，还讨论了可能仍然影响 OTOF 突变患者 CI 结果的因素。