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自身免疫性耳聋,不论是单独为一临床表现,还是作为综合征的部分症状,日趋常见。是可用药物治疗的为数甚少的感觉神经性耳聋中的一种。作者介绍一例硬皮病伴有耳聋的病理组织学所见。据认为其是全身硬皮病累及颞骨的首例。作者发现此例硬皮病患者中耳及内耳的脉管损害与其他自身免疫性疾病的脉管损害毫无一致,尤其是符合Mc Cabe氏发现的自身免疫性感觉神经性耳聋的病理所见。在内耳自身免疫病变尚未确诊之前,给予免疫抑制剂治疗后,其听力有所改善。
Autoimmune deafness, more or less common as a clinical manifestation alone or as part of a syndrome, is becoming more common. Is one of the very few sensory nerve deafness available for drug treatment. The author describes a case of scleroderma with deafness seen in the histopathology. It is thought to be the first case of systemic scleroderma involving the temporal bone. The authors found that this case of scleroderma in the middle ear and inner ear vascular damage and other autoimmune diseases vascular inconsistency, especially in line with Mc Cabe’s pathology found in autoimmune sensory neuropathy. Before the autoimmune lesions of the inner ear have not been diagnosed, their hearing is improved after immunosuppressive therapy is given.