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【自身免疫性感音神经性聋】1979年Mc-Cabe描述了18例他认为是继发于自身免疫现象的感音神经性聋患者。这一提议的根据是皮质激素和环磷酰胺改善了听力。自身免疫疾病通过两种方式致病:耳内免疫复合物的沉积可损害其功能,或耳内抗原可被身体认为是外来的,从而发生免疫应答反应。Kornbl ut注意到在Wegener氏肉芽肿病的患者中36%有感音神经性聋,此病由抗体引起。Veldm an描述一例感音神经性聋患者,其听力随着皮质激素治疗而改善,活检证实其他脏器有血管炎。Keone(原文为Leone,误)等提供了内耳免疫复合物沉积的直接证据。他们复查了1例死于Wegener氏肉芽肿病并发症患者的颞骨组织切片,发现内淋巴囊血管而非耳蜗血管存在血管炎。这提示免疫复合物沉积于内淋巴囊,妨碍内淋巴过滤,继而引起听力障碍。
Autoimmune Sensorineural Deafness Mc-Cabe described 18 patients with sensorineural deafness that he thought were secondary to autoimmune phenomena in 1979. The rationale behind this proposal is that corticosteroids and cyclophosphamide improve hearing. Autoimmune diseases are pathogenic in two ways: the deposition of immune complexes in the ear can impair their function, or the intra-ear antigens can be considered foreign by the body and an immune response occurs. Kornblaut noted that 36% of patients with Wegener’s granulomatosis had sensorineural deafness caused by antibodies. Veldm an describes a patient with sensorineural deafness whose hearing improves with corticosteroid therapy and biopsy confirmed vasculitis in other organs. Keone (formerly Leone) provides direct evidence of deposition of immune complexes in the inner ear. They reviewed the temporal bone tissue section of a patient who died of a complication of Wegener’s granulomatosis and found that vasculitis was present in the endolymphatic sac rather than the cochlear. This suggests that the immune complex deposits on the endolymphatic sac, hindering endolymphatic filtration, which in turn causes hearing impairment.