自身免疫性内耳病3例报告并文献复习  

Three Case Studies of Autoimmune Inner Ear Disease

作　　者：王萌萌[1] 韩维举[2] Wang Mengmeng;Han Weiju(Tianjin First Central Hospital,Department of Rheumatism and Immunology,Tianjin,300192,China)

机构地区：[1]天津市第一中心医院风湿免疫科,天津300192 [2]解放军总医院第一医学中心耳鼻咽喉头颈外科

出　　处：《听力学及言语疾病杂志》2019年第5期507-510,共4页Journal of Audiology and Speech Pathology

摘　　要：目的探讨自身免疫性内耳病患者的临床表现及听力特点。方法回顾3例自身免疫性内耳病患者的临床资料,分析其临床表现、免疫学指标、听力特征及其应用激素和免疫抑制剂治疗的疗效。结果3例患者均有全身性免疫性疾病,分别被确诊为类风湿关节炎、复发性多软骨炎、免疫球蛋白G4(immunoglobulin G4,IgG4)相关疾病,其中病例1抗核抗体及抗环瓜氨酸肽抗体阳性,病例3抗核抗体阳性;3例均表现为双耳听力下降,纯音听阈图显示为感音神经性听力损失,以高频下降为主;给予激素及免疫抑制剂治疗后听力有不同程度的恢复。结论自身免疫性内耳病多表现为感音神经性听力下降,可累及双耳,患者可伴有全身免疫性疾病,血清中存在非特异性自身抗体及抗内耳组织特异性抗体;激素及免疫抑制剂治疗有效,病程短、听力损失轻的患者听力恢复较好。Objective To investigate the clinical and audiological characteristics of patients with autoimmune inner ear disease.Methods We analyzed the clinical characteristics,immunological index,audiological characteristics and responses to steroid and immunosuppressive agents of 3 cases with autoimmune inner ear disease.Results The three cases were diagnosed as rheumatoid arthritis,recurrent polychondritis and IgG4-related diseases.Antinuclear antibody and anti-cyclic citrullinated peptide antibody were positive in case 1.Antinuclear antibody was positive in case 3.The audiological characteristics of the three patients showed essentially sensorineural deafness of high frequency dominance.The hearing levels of the three cases were improved using steroid and immunosuppressive agents.Conclusion Audiological characteristics of autoimmune inner ear disease are mostly sensorineural hearing loss.Some patients are accompanied by systemic immune diseases.Non-specific autoantibodies and anti-inner ear tissue specific antibodies can be detected in the serum of patients.Timely treatment using steroid and immunosuppressive agents can be effective.Cases with a shorter duration and less hearing loss can get more benefits.

关 键 词：自身免疫性疾病 自身免疫性内耳病 听力损失 

分 类 号：R764.3[医药卫生—耳鼻咽喉科]