耳蜗毛细胞死亡引发耳蜗内延迟性继发病变的研究  

Study on delayed secondary lesions in the cochlea triggered by cochlear hair cell death

作　　者：丁大连[1,2,3] 李鹏 亓卫东[4] 张建辉 于进涛 吴学文[6] 蒋海燕[1] 孙虹[6] DING Dalian;LI Peng;QI Weidong;ZHANG Jianhui;YU Jintao;WU Xuewen;JIANG Haiyan;SUN Hong(State University of New York at Buffalo,NY 14214,USA;Department of Otorhinolaryngology Head and Neck Surgery,the Third Affiliated Hospital of Sun Yat-sen University,Guangzhou 510230,China;Department of Otorhinolaryngology Head and Neck Surgery,Chengdu Third People’s Hospital,Changdu 610014,China;Department of Otorhinolaryngology Head and Neck Surgery,Huashan Hospital,Fudan University,Shanghai 200040,China;Department of Otorhinolaryngology Head and Neck Surgery,Union Hospital,Tongji Medical College,Huazhong University of Science&Technology,Wuhan 430022,China;Department of Otorhinolaryngology Head and Neck Surgery,Xiangya Hospital,Central South University,Changsha 410008,China)

机构地区：[1]纽约州立大学布法罗大学,纽约布法罗14214 [2]中山大学附属第三医院耳鼻咽喉头颈外科,广东广州510230 [3]成都市第三人民医院耳鼻咽喉头颈外科,四川成都610014 [4]复旦大学附属华山医院耳鼻咽喉头颈外科,上海200040 [5]华中科技大学同济医学院附属协和医院耳鼻咽喉头颈外科,湖北武汉430022 [6]中南大学湘雅医院耳鼻咽喉头颈外科,湖南长沙410008

出　　处：《中国耳鼻咽喉颅底外科杂志》2022年第6期1-10,共10页Chinese Journal of Otorhinolaryngology-skull Base Surgery

基　　金：四川省医学科研课题计划(S20006);国家自然科学基金面上项目(82071050);广州市科技计划项目(201803010093);中山大学重点发展项目(201812281965)。

摘　　要：本文讨论了由耳蜗毛细胞死亡引发的耳蜗内一系列延迟性继发病变。在外毛细胞遭到破坏的早期阶段,外指细胞即刻膨胀开来并堵塞了螺旋器表面的穿孔。外指细胞的膨胀有效阻止了含高钾浓度的内淋巴液进入到螺旋器的内部,从而使剩余的毛细胞和支持细胞避免了钾中毒损害。膨胀的外指细胞随后分化成高大柱形细胞并继续支撑起耳蜗螺旋器的整个外形结构。在发生散在性外毛细胞缺失的耳蜗损害模型,由外指细胞转化的高大柱形细胞在外毛细胞缺失的位置永久支撑起耳蜗螺旋器的结构,使周围剩余的存活外毛细胞继续发挥其放大和转换声学振动信号的功能以维持残余听力。在发生大面积外毛细胞死亡的耳蜗损害模型,转化成高大柱形细胞的前外指细胞在外毛细胞缺失后30 d左右死亡,随后导致整个耳蜗螺旋器的结构坍塌和内毛细胞及其他支持细胞的继发性死亡,最后在耳蜗基底膜上仅存一层扁平上皮。无论是在内外毛细胞被同时破坏的实验模型还是在外毛细胞大面积死亡后继发支持结构坍塌和内毛细胞死亡的实验模型,耳蜗传出神经和传入神经都会在丧失内外毛细胞后数周内发生继发性破坏。位于蜗轴螺旋管内的螺旋神经节随后也因丧失神经刺激信号和缺乏神经营养因子而引发延迟性螺旋神经节死亡,螺旋神经节的死亡使与耳蜗核相连的听觉神经中枢端轴突也发生不可逆的破坏,最终导致耳蜗周围系统与中枢听觉系统的神经连接永久中断。This article discusses a series of delayed secondary lesions triggered by cochlear hair cell death.In the early stages of outer hair cell destructions,the Deiters’cells are immediately expanded to plug the perforations on the surface of the organ of Corti.The expansion of Deiters’cells can effectively prevent the infiltration of high potassium endolymph into the internal space of the organ of Corti to protect remaining outer hair cells and support cells from potassium toxicity.The expanded Deiters’cells then differentiate into tall columnar cells to support the overall shape of the organ of Corti.In the location of sporadic missing of outer hair cells,the tall columnar cells converted from former Dieter’s cells permanently support the structure of the organ of Corti,which allows the remaining survival outer hair cells to continue function of amplifying and converting the acoustic vibration signals to maintain the residual hearing.In the cochlear injury model with extensive outer hair cell death,the former Deiters’cells transformed into tall columnar cells die 30 days after redifferentiation,which subsequently leads to the collapse of the entire structure of the organ of Corti and the death of inner hair cells and support cells.Finally,only a layer of flat epithelium remains on the cochlear basilar membrane.Whether in an experimental model of both inner and outer hair cells missing or in an experimental model of massive death of outer hair cells followed by collapse of supporting structures and death of inner hair cells,cochlear efferent nerves and afferent fibers on the cochlear basilar membrane are destroyed within few weeks after hair cell missing.The spiral ganglion neurons in the Rosenthal’s canal of modiolus subsequently initiate the delayed spiral ganglion death due to loss of nerve stimulation signals and lack of neurotrophic factors.The death of the spiral ganglion neurons causes irreversible destruction of the axons at the central end of the auditory nerve connecting the cochlear nucleus,and

关 键 词：耳蜗 毛细胞 支持细胞 螺旋神经节 继发性病变 

分 类 号：R764.3[医药卫生—耳鼻咽喉科]