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。 这可以作为 正常宿主防御病原体（即病毒，细菌）或作为 一种自身免疫性疾病的后果， 耳朵是它的目标之一。 这是一个需要理解的重要概念 并认识到，由于免疫事件引起的耳聋可能 通过适当的治疗或操纵宿主的免疫系统来逆转 反应 由于麦凯布最初对这种新的临床实体的描述 （自身免疫性内耳疾病）在1979年，许多患者已经 确定并治疗这种情况的脓疱，两者都可能 有严重的潜在副作用 通常他们的诊断依据 是存在快速进行性耳聋和几项测试， 要么指出全身性风湿病的共存， 可能是耳朵特有的自身免疫性疾病 与其他风湿病一样， 自身免疫性内耳疾病似乎折磨妇女 优先（63%）。 尽管对这种疾病有相当大的兴趣， 继续努力治疗，迫切需要 开发针对这种情况的特定诊断测试， 更好地理解免疫紊乱的机制 影响内耳。 实现这一目标的一个主要缺点是， 缺乏对AIED抗原靶点的鉴定。 我们 实验室一直参与提供科学依据， 对于最初的概念，内耳可以是目标的， 免疫反应和研究，以确定抗原的目标 这种疾病，希望开发一个敏感和具体的 诊断患者的方法。 具体目标是 鉴定与AIED相关的内耳抗原，以确定 AIED中自身免疫的功能靶点， 内耳炎症的免疫机制， AIED的病因。 我们认为已经取得的进展 在这一领域是如此的重大突破，在这两个诊断 治疗在不久的将来是可以实现的。

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.