Wang Yujun, Jin Yuan, Zhang Qiong, Xiong Ying, Gu Xiang, Zeng Shan, Chen Wei
Department of Intensive Care Unit, The Central Hospital of Wuhan, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Department of Otorhinolaryngology-Head and Neck Surgery, The Central Hospital of Wuhan, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Front Cell Neurosci. 2023 Jun 2;17:1208406. doi: 10.3389/fncel.2023.1208406. eCollection 2023.
Hearing loss is the most common congenital sensory impairment. Mutations or deficiencies of the gene are the most common genetic cause of congenital non-syndromic deafness. Pathological changes such as decreased potential in the cochlea, active cochlear amplification disorders, cochlear developmental disorders and macrophage activation have been observed in various transgenic mouse models. In the past, researchers generally believed that the pathological mechanisms underlying -related hearing loss comprised a K circulation defect and abnormal ATP-Ca signals. However, recent studies have shown that K circulation is rarely associated with the pathological process of -related hearing loss, while cochlear developmental disorders and oxidative stress play an important, even critical, role in the occurrence of -related hearing loss. Nevertheless, these research has not been systematically summarized. In this review, we summarize the pathological mechanisms of -related hearing loss, including aspects of K circulation, developmental disorders of the organ of Corti, nutrition delivery, oxidative stress and ATP-Ca signals. Clarifying the pathological mechanism of -related hearing loss can help develop new prevention and treatment strategies.
听力损失是最常见的先天性感觉障碍。该基因的突变或缺陷是先天性非综合征性耳聋最常见的遗传原因。在各种转基因小鼠模型中已观察到诸如耳蜗电位降低、耳蜗主动放大障碍、耳蜗发育障碍和巨噬细胞激活等病理变化。过去，研究人员普遍认为，与该基因相关的听力损失的病理机制包括钾循环缺陷和异常的ATP - 钙信号。然而，最近的研究表明，钾循环很少与该基因相关的听力损失的病理过程相关，而耳蜗发育障碍和氧化应激在该基因相关的听力损失的发生中起重要甚至关键的作用。尽管如此，这些研究尚未得到系统总结。在本综述中，我们总结了该基因相关听力损失的病理机制，包括钾循环、柯蒂氏器发育障碍、营养供应、氧化应激和ATP - 钙信号等方面。阐明该基因相关听力损失的病理机制有助于制定新的预防和治疗策略。
