免疫介导性内耳病的诊断和治疗研究进展  

Advances in diagnosis and therapies of immune-mediated inner ear diseases

作　　者：王浩然 王大勇[1] 王秋菊[1] WANG Haoran;WANG Dayong;WANG Qiuju

机构地区：[1]解放军总医院耳鼻咽喉头颈外科解放军耳鼻咽喉研究所,北京100853

出　　处：《临床耳鼻咽喉头颈外科杂志》2020年第3期276-281,共6页Journal of Clinical Otorhinolaryngology Head And Neck Surgery

基　　金：国家自然科学基金重点项目(No:81830028,No:81530032);青年项目(No:81900951,No:81900950);军队医学科技青年培育计划孵化项目(No:19QNP058)。

摘　　要：免疫介导性内耳病(immune-mediated inner ear diseases,IMIED)是由异常的免疫反应所引起的一种感音神经性听力损失,是少数可治疗的感音神经性聋(SNHL)之一。1958年,Lehnhardt首次报道了双侧进展性听力损失与自身免疫反应相关;1979年,McCabe研究18例双侧快速进展性SNHL患者时,发现类固醇激素+环磷酰胺治疗有效,首次提出并定义这一特殊的疾病实体为自身免疫感音神经性聋(autoimmune sensorineural hearing loss,ASNHL),因不仅有耳蜗功能损害,亦有HU庭功能受损•后称为自身免疫性内耳病(autoimmune inner ear diseases,AIED);1984年,Hughes等考虑到AIED患者中约有30%与系统性自身免疫病相关.遂根据发生部位将免疫反应仅发生于内耳的SNHL称为原发性AIED,将继发于系统性自身免疫性疾病的SNHL称为继发性AIED;2010年,Aftab等[1]将病理局限于内耳的自身免疫性SNHL称为AIED,将与系统性自身免疫病相关的听力损失及前庭功能障碍称为IMIED或继发AIED。Immune-Mediated Inner Ear Diseases(IMIED) is one of the curable sensorineural hearing loss caused by abnormal immune response, which may be primary inner ear diseases or associated with systemic autoimmune diseases. Since the complex differential diagnosis and no reliable diagnostic tests, the early identification of IMIED was extremely tricky. The treatment still mainly depends on glucocorticoid, but as researchs move along, directing at different pathogenesis, more and more therapies have been proposed. This article reviews the diagnosis and different treatment methods of IMIED.

关 键 词：免疫介导性内耳病 自身免疫性内耳病 感音神经性听力损失 诊断 治疗 

分 类 号：R764.43[医药卫生—耳鼻咽喉科]