Mittal Rahul, Nguyen Desiree, Patel Amit P, Debs Luca H, Mittal Jeenu, Yan Denise, Eshraghi Adrien A, Van De Water Thomas R, Liu Xue Z
Department of Otolaryngology, University of Miami Miller School of MedicineMiami, FL, United States.
Department of Otolaryngology, Xiangya Hospital, Central South UniversityChangsha, China.
Front Mol Neurosci. 2017 Jul 31;10:236. doi: 10.3389/fnmol.2017.00236. eCollection 2017.
Neurosensory responses of hearing and balance are mediated by receptors in specialized neuroepithelial sensory cells. Any disruption of the biochemical and molecular pathways that facilitate these responses can result in severe deficits, including hearing loss and vestibular dysfunction. Hearing is affected by both environmental and genetic factors, with impairment of auditory function being the most common neurosensory disorder affecting 1 in 500 newborns, as well as having an impact on the majority of elderly population. Damage to auditory sensory cells is not reversible, and if sufficient damage and cell death have taken place, the resultant deficit may lead to permanent deafness. Cochlear implants are considered to be one of the most successful and consistent treatments for deaf patients, but only offer limited recovery at the expense of loss of residual hearing. Recently there has been an increased interest in the auditory research community to explore the regeneration of mammalian auditory hair cells and restoration of their function. In this review article, we examine a variety of recent therapies, including genetic, stem cell and molecular therapies as well as discussing progress being made in genome editing strategies as applied to the restoration of hearing function.
听觉和平衡的神经感觉反应由特殊神经上皮感觉细胞中的受体介导。促进这些反应的生化和分子途径的任何破坏都可能导致严重缺陷，包括听力丧失和前庭功能障碍。听力受环境和遗传因素影响，听觉功能受损是最常见的神经感觉障碍，每500名新生儿中就有1人受影响，并且对大多数老年人群体也有影响。听觉感觉细胞的损伤是不可逆的，如果发生了足够的损伤和细胞死亡，由此产生的缺陷可能导致永久性耳聋。人工耳蜗被认为是聋人患者最成功且一致的治疗方法之一，但只能以丧失残余听力为代价提供有限的恢复。最近，听觉研究界对探索哺乳动物听觉毛细胞的再生及其功能恢复的兴趣有所增加。在这篇综述文章中，我们研究了各种最新疗法，包括基因疗法、干细胞疗法和分子疗法，并讨论了应用于听力功能恢复的基因组编辑策略所取得的进展。
