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Abstract: 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.
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表 1 TNF-α拮抗剂治疗难治性IMIED
TNF-α拮抗剂 年份 作者 例数 用法 效果 infliximab 2013 Heywood等〔31〕 1 静脉注射，每2周1次，100 mg/次，持续10个月 类固醇激素和其他免疫抑制剂不能有效控制复发，只能短暂改善听力; 依那西普治疗6周无反应; 应用infliximab后，低频听力及耳鸣明显改善，很少急性发作，然而46周停药后复发，再次给药，听力再次改善。推荐用此药长期维持治疗，但尚需要进行一项大型的随机对照试验，验证其作用 2014 Gazeau等〔29〕 1 静脉注射，每8周1次，3 mg/kg 类风湿关节炎相关听力损失：间歇期延长后出现双耳突聋，给予高压氧及激素治疗后，听力有好转; 后因阑尾炎手术停止单抗治疗，导致听力急剧恶化，重新使用后，听力迅速改善 2006 Van Wijk等〔32〕 9 鼓室注射，每周1次，0.3mL/次，共4周 使4/5激素依赖性AIED患者逐渐减少激素使用，同时维持听力; 仅适用鼓室注射infliximab的非激素依赖性患者有3/4的听力得到改善; 减少疾病复发; 7/9的患者获益 依那西普 2005 Matteson等〔33〕 安慰剂对照20 皮下注射，每周2次，25mg/次，持续8周 依那西普治疗AIED的效果并不比安慰剂好 阿达木单抗 2016 Marsili等〔30〕 1 皮下注射，每2周1次，24 mg/m2; 同时激素减量治疗 成功治愈1例15岁白塞病男性患儿的听觉前庭症状并控制了白塞病病情 戈利木单抗 2014 Derebery等〔14〕 10 鼓室注射，每1~2周1次，0.3mL/次; 同时类固醇激素减量治疗 激素减量过程中，戈利木单抗可以维持听力及SDS，同时没有不良反应，保证了安全性 
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| 引用本文: |
王浩然, 王大勇, 王秋菊. 免疫介导性内耳病的诊断和治疗研究进展[J]. 临床耳鼻咽喉头颈外科杂志, 2020, 34(3): 276-281. doi: 10.13201/j.issn.2096-7993.2020.03.023
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| Citation: |
WANG Haoran, WANG Dayong, WANG Qiuju. Advances in diagnosis and therapies of immune-mediated inner ear diseases[J]. J Clin Otorhinolaryngol Head Neck Surg, 2020, 34(3): 276-281. doi: 10.13201/j.issn.2096-7993.2020.03.023
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