Moscicki R A
Massachusetts General Hospital, Cambridge.
Baillieres Clin Neurol. 1994 Nov;3(3):547-63.
Immune-mediated inner ear disease is characterized by sensorineural hearing loss which is most often rapidly progressive and bilateral, may be accompanied by vestibular symptoms, and for which no cause can otherwise be identified. The problem may occur alone or in combination with other systemic autoimmune disorders. Recognition of this entity is important as a substantial number of such patients experience improvement in hearing following treatment with corticosteroids with or without other immunosuppressive agents. Immune response to inner ear antigens can be helpful in establishing the diagnosis. The lymphocyte transformation assay has been used in identifying such responses but is limited by sensitivity and inhibited by immunosuppression. Western blot identification of serum antibodies to a 68-kD antigen of the inner ear appears to be a promising means of identifying patients with immune-mediated inner ear disease. The antibody is present in the majority of patients with clinical features consistent with the disorder; it correlates with disease activity and is predictive of response to treatment. Nevertheless, response to corticosteroid therapy remains the most convincing evidence of the disease.
免疫介导性内耳疾病的特征为感音神经性听力损失，这种听力损失通常进展迅速且为双侧性，可能伴有前庭症状，并且无法找出其他病因。该问题可能单独出现，也可能与其他全身性自身免疫性疾病并发。认识到这一疾病实体很重要，因为大量此类患者在使用或未使用其他免疫抑制剂的情况下接受皮质类固醇治疗后听力会有所改善。对内耳抗原的免疫反应有助于确诊。淋巴细胞转化试验已用于识别此类反应，但受敏感性限制且会被免疫抑制所抑制。通过蛋白质印迹法鉴定血清中针对内耳68-kD抗原的抗体似乎是识别免疫介导性内耳疾病患者的一种有前景的方法。大多数具有与该疾病相符临床特征的患者体内存在这种抗体；它与疾病活动相关，并且可预测对治疗的反应。然而，对皮质类固醇治疗的反应仍然是该疾病最有说服力的证据。
