Department of Otorhinolaryngology-Head and Neck Surgery, Xinhua Hospital, Shanghai Jiaotong University School of Medicine.
Shanghai Jiaotong University School of Medicine Ear Institute.
Curr Opin Otolaryngol Head Neck Surg. 2022 Oct 1;30(5):332-338. doi: 10.1097/MOO.0000000000000829. Epub 2022 Jul 18.
Auditory neuropathy is a disorder of auditory dysfunction characterized by the normal function of the outer hair cells and malfunction of the inner hair cells, synapses, postsynapses and/or auditory afferent nervous system. This review summarizes the process of discovery and naming of auditory neuropathy and describes the acquired, associated genetic disorders and management available.
In the last 40 years, auditory neuropathy has undergone a process of discovery, naming and progressive elucidation of its complex pathological mechanisms. Recent studies have revealed numerous acquired and inherited causative factors associated with auditory neuropathy. Studies have analyzed the pathogenic mechanisms of various genes and the outcomes of cochlear implantation. New therapeutic approaches, such as stem cell therapy and gene therapy are the future trends in the treatment of auditory neuropathy.
A comprehensive understanding of the pathogenic mechanisms is crucial in illustrating auditory neuropathy and assist in developing future management strategies.
听觉神经病是一种听觉功能障碍，其特征为外毛细胞功能正常，而内毛细胞、突触、突触后和/或听觉传入神经系统功能异常。本综述总结了听觉神经病的发现和命名过程，并描述了获得性相关遗传疾病和现有治疗方法。
在过去的 40 年中，听觉神经病的发现、命名和其复杂病理机制的逐步阐明经历了一个过程。最近的研究揭示了许多与听觉神经病相关的获得性和遗传性致病因素。研究分析了各种基因的致病机制和人工耳蜗植入的结果。干细胞治疗和基因治疗等新的治疗方法是听觉神经病治疗的未来趋势。
全面了解致病机制对于阐明听觉神经病并协助制定未来的管理策略至关重要。
