Agrup Charlotte, Luxon Linda M
Department of Neuro-Otology, National Hospital for Neurology and Neurosurgery, London, UK.
Curr Opin Neurol. 2006 Feb;19(1):26-32. doi: 10.1097/01.wco.0000194143.02171.46.
Immune-mediated inner-ear disorders may present to different medical disciplines and new research findings emerge rapidly. The purpose of this review is to draw the different strands together to produce an overview describing the clinical presentation of immune-mediated inner-ear disorders and to discuss useful diagnostic criteria with a focus on tissue-specific and tissue-non-specific antibodies.
The importance of diagnosing an immune-mediated inner-ear disorder is highlighted in the context of it being one of few forms of treatable inner-ear disorder with a good response to immunosuppressive therapy. Due to a lack of reliable tests, the criteria upon which the diagnosis of immune-mediated inner-ear disease is based are often arbitrary. Previous and current research focuses mainly on the investigation of the aetiology of immune-mediated disorders, studying the presence of autoantibodies and the antigens responsible for their production. The prognostic and therapeutic values of inner-ear-specific antibodies are still unclear. Various antigens have been suggested. However, most antigens identified have been ubiquitous proteins not specific to the inner ear and therefore lack logical association with localized inner-ear pathology.
Early diagnosis of immune-mediated inner-ear disorders with prompt treatment may prevent irreversible damage to inner-ear structures. Accordingly, it is important to include immune-mediated inner-ear disorders in the differential diagnosis of patients presenting with 'idiopathic' audiovestibular dysfunction. To enable early diagnosis, we recommend that the current available routine immunological laboratory tests (antinuclear, antineutrophil cytoplasmic, antiendothelial cell, antiphospholipid/anticardiolipin and antithyroid antibodies) are used when assessing a patient with suspected immune-mediated inner-ear disorder.
免疫介导的内耳疾病可能涉及不同医学学科，且新的研究发现迅速涌现。本综述旨在将不同线索整合起来，对免疫介导的内耳疾病的临床表现进行概述，并讨论有用的诊断标准，重点关注组织特异性和非特异性抗体。
免疫介导的内耳疾病作为少数几种对免疫抑制治疗反应良好的可治疗内耳疾病之一，其诊断的重要性得到凸显。由于缺乏可靠的检测方法，免疫介导的内耳疾病的诊断标准往往具有随意性。既往和当前的研究主要集中在免疫介导疾病的病因学研究，即研究自身抗体的存在及其产生的抗原。内耳特异性抗体的预后和治疗价值仍不明确。已提出多种抗原。然而，大多数已鉴定的抗原都是普遍存在的蛋白质，并非内耳特异性，因此与局限性内耳病理缺乏逻辑关联。
免疫介导的内耳疾病的早期诊断并及时治疗可预防内耳结构的不可逆损伤。因此，在对出现“特发性”视听前庭功能障碍的患者进行鉴别诊断时，纳入免疫介导的内耳疾病很重要。为实现早期诊断，我们建议在评估疑似免疫介导内耳疾病的患者时，使用现有的常规免疫实验室检测（抗核抗体、抗中性粒细胞胞浆抗体、抗内皮细胞抗体、抗磷脂/抗心磷脂抗体和抗甲状腺抗体）。
