CAJ | 학술논문

听神经病的临床听力学特征主要表现在具有正常的耳蜗感受器电位和异常的听性脑干反应波形以及对语言的理解障碍，其病理学改变部位被确定是在周边听觉系统，主要表现在内毛细胞缺损、听神经末梢破坏、听神经纤维脱髓鞘病变、螺旋神经节细胞缺损，或上述一个或几个部位同时发生病变。本文介绍了模拟听神经病的各种实验动物模型，其中包括模拟单纯内毛细胞缺损的Slc19a2基因缺陷小鼠和Ggt1基因缺陷小鼠实验模型；模拟单纯Ⅰ型传入神经末梢损害的谷氨酸盐实验模型；模拟单纯脱髓鞘病变的甘油实验模型；模拟Ⅰ型螺旋神经节及其神经纤维损害的乌苯酐实验模型和免疫性疾病损害模型；模拟单纯神经纤维脱髓鞘病变的胆红素实验模型和髓鞘缺陷仓鼠模型；模拟单纯听神经近端脱髓鞘病变的阿霉素模型和压力夹伤听神经实验模型；以及模拟整个周边听觉系统全程病变的卡铂南美栗鼠实验模型。这些实验模型可以分别用于模拟听神经病的一种或几种临床表现及病变特征的实验研究。
은신경병적림상은역학특정주요표현재구유정상적이와감수기전위화이상적은성뇌간반응파형이급대어언적리해장애，기병이학개변부위피학정시재주변은각계통，주요표현재내모세포결손、은신경말소파배、은신경섬유탈수초병변、라선신경절세포결손，혹상술일개혹궤개부위동시발생병변。본문개소료모의은신경병적각충실험동물모형，기중포괄모의단순내모세포결손적Slc19a2기인결함소서화Ggt1기인결함소서실험모형；모의단순Ⅰ형전입신경말소손해적곡안산염실험모형；모의단순탈수초병변적감유실험모형；모의Ⅰ형라선신경절급기신경섬유손해적오분항실험모형화면역성질병손해모형；모의단순신경섬유탈수초병변적담홍소실험모형화수초결함창서모형；모의단순은신경근단탈수초병변적아매소모형화압력협상은신경실험모형；이급모의정개주변은각계통전정병변적잡박남미률서실험모형。저사실험모형가이분별용우모의은신경병적일충혹궤충림상표현급병변특정적실험연구。
Clinically, auditory neuropathy is characterized by normal or near normal otoacoustic emissions, audiomet?ric thresholds and cochlear microphonic potentials, but abnormal or absent auditory brainstem response waveforms and cen?tral auditory processing deficits such as poor speech perception, difficulty hearing in noise and poor temporal processing. The pathological changes underlying auditory neuropathy are believed to originate from the inner hair cells-auditory nerve system and may involve missing inner hair cells, synaptic dysfunction between the inner hair cell and type I auditory neurons, and de?generation or demyelination of auditory nerve fibers that project to the cochlear nucleus. This paper describes various experi?mental animal models that have been developed to mimic certain aspects of auditory neuropathy. These include animal mod?els with missing inner hair cell induced by deletion of the Slc19a2 or Ggt1 genes, degeneration of type I afferent terminals by glutamate excitotoxicity, demyelination of auditory nerve fibers by adriamycin, hyperbilirubin and glycerin, surgical compres?sion of the auditory nerve, autoimmune diseases, and carboplatin-induced degeneration of inner hair cells and type I auditory nerve fibers in chinchillas. These different experimental models have provided unique insights of auditory function and simu?late different clinical manifestations and pathological features of auditory neuropathy.