ASPECTS OF INNER EAR IMMUNOLOGY

大量的证据表明，某些形式的 耳聋可能有免疫学基础。这可能会作为 正常的宿主防御病原体(即病毒、细菌)或作为 一种自身免疫状况的后果，这种状况发生并具有内在的 耳朵作为其目标之一。这是一个需要理解的重要概念 由于免疫事件引起的耳聋可能是潜在的 通过适当的治疗或操纵宿主的免疫来逆转 回应。由于麦凯布对这一新的临床实体的原始描述 (自身免疫性内耳疾病)自1979年以来，许多患者都被 推定为这种疾病的确诊和治疗，这两种情况都可能 有严重的潜在副作用。通常是他们诊断的基础 是快速进行性耳聋的存在和几项测试 要么是系统性风湿病共存，要么是 可能是耳朵特有的自身免疫性疾病。和其他风湿病一样 自身免疫性内耳疾病似乎困扰着女性 优先考虑(63%)。尽管人们对这种疾病和 在治疗方面的持续努力，迫切需要 开发一种针对这种情况的特定诊断测试，并 更好地了解免疫紊乱的作用机制 影响内耳的。实现这一目标的一个主要缺点是 缺乏抗原靶向(S)的鉴定。我们的 实验室一直在全面参与提供科学基础 对于内耳可以成为 免疫反应和确定抗原靶点的研究 对于这种紊乱，希望能发展出一种敏感和具体的 诊断受苦患者的手段。具体目标是 确定与AED相关的内耳抗原，以确定 AEDs自身免疫功能靶点的研究 内耳炎的免疫学机制是 AIED的病因。我们认为，已经取得的进展 在这一领域的重大突破是在两个诊断 和治疗在不久的将来是很容易实现的。

Abundant evidence has accumulated indicating that certain forms of deafness may have an immunologic basis. This can occur as a part of the normal host defense against pathogens (i.e. viruses, bacteria) or as a consequence of an autoimmune condition which develops and has the inner ear as one of its targets. This is an important concept to understand and recognize since deafness due to immunologic events can be potentially reversed with appropriate therapy or manipulation of a host's immune response. Since McCabe's original descriptio of this new clinical entity (Autoimmune Inner Ear Disease) in 1979, many patients have been putatively identified and treated for this condition, both of which may have serious potential side effects. Often the basis of their diagnosis is the presence of rapidly progressive deafness and several tests which either point to the coexistence of systemic rheumatologic illnesses or possibly an ear-specific autoimmune condition. As in other rheumatologic conditions, autoimmune inner ear disease appears to afflict women preferentially (63%). Despite considerable interest in the disease and continued efforts in treatment, there is a compelling need for the development of a specific diagnostic test for this condition and to better understand the mechanisms at play in immunological disorders affecting the inner ear. A major drawback to accomplishing this has been lack of identification of the antigenic target(s) of AIED. Our laboratory has been integrally involved in providing the scientific basis for the original concept that the inner ear can be the target of the immunological responses and in research to determine the antigenic target of this disorder, in the hopes of developing a sensitive and specific means of diagnosing afflicted patients. The specific aims are to identify inner ear antigens associated with AIED, to determine the functional targets of autoimmunity in AIED and to investigate the immunological mechanisms of inner ear inflammation that are etiopathogenic in AIED. We believe that the progress that has been made in this field is such that significant breakthroughs in both diagnosis and treatment are readily achievable in the near future.