Sensorineural hearing loss (SNHL) becomes an inevitable worldwide public health issue, and deafness treatment is urgently imperative; yet their current curative therapy is limited. Auditory neuropathies (AN) were proved to play a substantial role in SNHL recently, and spiral ganglion neuron (SGN) dysfunction is a dominant pathogenesis of AN. Auditory pathway is a high energy consumption system, and SGNs required sufficient mitochondria. Mitochondria are known treatment target of SNHL, but mitochondrion mechanism and pathology in SGNs are not valued. Mitochondrial dysfunction and pharmacological therapy were studied in neurodegeneration, providing new insights in mitochondrion-targeted treatment of AN. In this review, we summarized mitochondrial biological functions related to SGNs and discussed interaction between mitochondrial dysfunction and AN, as well as existing mitochondrion treatment for SNHL. Pharmaceutical exploration to protect mitochondrion dysfunction is a feasible and effective therapeutics for AN.

中文翻译：

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听神经病中的线粒体功能障碍和治疗靶点。

感音神经性听力损失（SNHL）成为不可避免的世界性公共卫生问题，耳聋治疗刻不容缓；然而他们目前的治疗方法是有限的。最近证明听神经病 (AN) 在 SNHL 中起重要作用，螺旋神经节神经元 (SGN) 功能障碍是 AN 的主要发病机制。听觉通路是一个高能量消耗系统，SGNs 需要足够的线粒体。线粒体是 SNHL 的已知治疗靶点，但 SGN 中的线粒体机制和病理并未受到重视。在神经退行性疾病中研究了线粒体功能障碍和药物治疗，为 AN 的线粒体靶向治疗提供了新的见解。在这篇综述中，我们总结了与 SGNs 相关的线粒体生物学功能，并讨论了线粒体功能障碍与 AN 之间的相互作用，以及现有的 SNHL 线粒体治疗。保护线粒体功能障碍的药物探索是一种可行且有效的治疗 AN 的方法。