不同种属实验动物耳蜗毛细胞年龄相关性缺失的不同模式  

Different patterns of age-related damage to cochlear hair cells in different species of experimental animals

作　　者：丁大连[1,2,3,4] 李鹏 亓卫东[5] 张建辉 蒋海燕 宣伟军[4] DING Dalian;LI Peng;QI Weidong;ZHANG Jianhui;JIANG Haiyan;XUAN Weijun(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,Chengdu 610014,China;Department of Otorhinolaryngology Head and Neck Surgery,the First Clinical Medical College of Guangxi University of Chinese Medicine,Nanning 530023,China;Department of Otorhinolaryngology Head and Neck Surgery,Huashan Hospital,Fudan University,Shanghai 200040,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]广西中医药大学第一临床医学院耳鼻咽喉头颈外科,广西南宁530023 [5]复旦大学附属华山医院耳鼻咽喉头颈外科,上海200040

出　　处：《中国耳鼻咽喉颅底外科杂志》2023年第4期7-13,共7页Chinese Journal of Otorhinolaryngology-skull Base Surgery

基　　金：国家自然科学基金面上项目(82171147,82071050,81973913);四川省医学科研课题计划(S20006)。

摘　　要：目的展示自然衰老和耳聋相关基因遗传缺陷之间耳蜗毛细胞缺失的不同模式。方法用不同龄的长尾猴、南美栗鼠、豚鼠、Sprague-Dawley大鼠、CBA/CaJ小鼠、C57BL/6J小鼠、A/J小鼠、DBA/2J小鼠和侏儒灰色突变纯合子(dwg/dwg)小鼠作为受试对象。所有测试动物的耳蜗基底膜都被制作成平坦的耳蜗基底膜铺片。沿着耳蜗基底膜的全长,基底膜上所有的内外毛细胞都被完整计数,毛细胞的计数结果被输入到耳蜗图软件并自动生成每组实验条件的平均耳蜗图。结果在天然衰老的动物中,耳蜗毛细胞的缺失总是发生在老年阶段。与此不同的是,在耳聋相关基因缺陷的动物中,耳蜗毛细胞的缺失却是发生在青年阶段甚至幼年阶段。发生在天然老化动物的耳蜗毛细胞缺失总是呈均匀分布或从耳蜗的顶回向底回扩展。但是,发生在具有耳聋相关基因遗传缺陷动物的耳蜗毛细胞缺失却通常表现为从耳蜗的底回向顶回扩展。结论本实验观察结果表明,发生在天然衰老的不具备耳聋相关基因缺陷动物身上的年龄相关性耳蜗毛细胞缺失反映的是真正由衰老引起的耳蜗退化性病变,而发生在伴有耳聋相关基因遗传缺陷的年幼动物身上的年龄相关性耳蜗毛细胞缺失可能与耳聋相关基因的遗传缺陷有关。Objective To demonstrate distinct patterns of cochlear hair cell loss between aging and genetic defects of deafness-associated genes.Methods Vervet monkeys,chinchillas,guinea pigs,Sprague-Dawley rats,CBA/CaJ mice,C57BL/6 mice,DBA/2J mice,and dwarf gray mutant homozygous(dwg/dwg)mice with different ages were used as experimental subjects.The cochlear basilar membranes of all tested animals were prepared as flat surface preparations.All inner and outer hair cells on the cochlear basilar membrane were counted along entire length of the basilar membrane.The hair cell counts were then input into the cochleogram software to generate the averaged cochleogram for each group.Results In natural aging animals,cochlear hair cell loss occurred in the elderly stage.In contrast,in the deaf-related genetic defective animals,cochlear hair cell loss occurred in the younger stage or even in the childhood.The cochlear hair cell loss in natural aging animals was always evenly distributed or expanded from the cochlear apical turn to the basal turn.However,the cochlear hair cell loss in the animals with genetic defects of deafness-associated genes often manifested an extension from the cochlear basal turn to the apical turn.Conclusion The age-related cochlear hair cell loss on elderly animals without deafness-related genetic defects reflects the degenerative lesions caused by real aging,while the age-related cochlear hair cell loss on young animals with deafness-related genetic defects is likely related to the genetic defects of deafness-related genes.

关 键 词：耳聋 耳蜗毛细胞图 毛细胞缺失 年龄相关性耳蜗毛细胞缺失 耳聋相关基因缺陷 

分 类 号：R764.3[医药卫生—耳鼻咽喉科]