Goodall A F, Siddiq M A
Department of Otolaryngology, St Helens & Knowsley Hospitals NHS Trust, St Helens, UK.
Clin Otolaryngol. 2015 Oct;40(5):412-9. doi: 10.1111/coa.12432.
Autoimmune inner ear disease (AIED) is a poorly understood form of sensorineural hearing loss that causes bilateral, asymmetric, progressive hearing loss, sometimes with vestibular symptoms, often associated with a systemic autoimmune disease, which is noteworthy as the only sensorineural loss responsive to medical therapy. Despite much research interest of the past 25 years, its aetiopathogenesis is still unproven.
To succinctly consolidate research and opinion regarding the pathogenesis of autoimmune inner ear disease, in ongoing efforts to elucidate the molecular and intracellular pathways that lead to inner ear damage, which may identify new targets for pharmacotherapy.
Systematic review
PubMed/MEDLINE search using key terms to identify articles published between January 1980 and Apr 2014. Additionally, any landmark works discussed in this body of literature were obtained and relevant information extracted as necessary.
Inclusion criterion was any information from animal or human studies with information relevant to possible aetiopathogenesis of AIED. Studies that focused on diagnosis, ameliorating symptoms or treatment, without specific information relevant to mechanisms of immune-mediated injury were excluded from this work. Articles meeting the inclusion criteria were digested and summarised.
A proposed pathogenic mechanism of AIED involves inflammation and immune-mediated attack of specific inner ear structures, leading to an excessive Th1 immune response with vascular changes and tissue damage in the cochlea. Studies have identified self-reactive T cells and immunoglobulins, and have variously implicated immune-complex deposition, microthrombosis and electrochemical disturbances causing impaired neurosignalling in the pathogenesis of AIED. Research has also demonstrated abnormalities in the cytokine milieu in subjects with AIED, which may prove a target for therapy in the future.
Ongoing research is needed to further elucidate the aetiopathogenesis of AIED and discern between various mechanisms of tissue injury. Large-cohort clinical studies employing IL-1 receptor blockade are warranted to determine its potential for future therapy.
自身免疫性内耳疾病（AIED）是一种人们了解较少的感音神经性听力损失形式，可导致双侧、不对称、进行性听力损失，有时伴有前庭症状，常与全身性自身免疫性疾病相关，这作为唯一对药物治疗有反应的感音神经性听力损失值得关注。尽管在过去25年中有很多研究兴趣，但其病因发病机制仍未得到证实。
简要整合关于自身免疫性内耳疾病发病机制的研究和观点，持续努力阐明导致内耳损伤的分子和细胞内途径，这可能确定药物治疗的新靶点。
系统综述
使用关键词在PubMed/MEDLINE中检索，以识别1980年1月至2014年4月发表的文章。此外，获取该文献中讨论的任何标志性著作，并在必要时提取相关信息。
纳入标准是来自动物或人体研究的任何与AIED可能的病因发病机制相关的信息。专注于诊断、改善症状或治疗且无与免疫介导损伤机制相关的具体信息的研究被排除在本研究之外。对符合纳入标准的文章进行消化和总结。
AIED的一种推测致病机制涉及炎症和对特定内耳结构的免疫介导攻击，导致过度的Th1免疫反应，伴有耳蜗血管变化和组织损伤。研究已鉴定出自身反应性T细胞和免疫球蛋白，并以各种方式暗示免疫复合物沉积、微血栓形成和电化学紊乱在AIED发病机制中导致神经信号传导受损。研究还表明AIED患者细胞因子环境存在异常，这可能在未来成为治疗靶点。
需要持续研究以进一步阐明AIED的病因发病机制，并区分各种组织损伤机制。有必要进行采用白细胞介素-1受体阻断剂的大样本队列临床研究，以确定其未来治疗潜力。
