Vinceneux P, Couloigner V, Pouchot J, Bouccara D, Sterkers O
Service de Médecine Interne, Hôpital Louis Mourier, Colombes.
Presse Med. 1999 Nov 6;28(34):1904-10.
A REAL ENTITY: Deafness resulting from an autoimmune mechanism is suggested by a growing number of clinical and experimental arguments. The audio-vestibular system is known to be involved in a certain number of systemic diseases, particularly Cogan's disease. In other cases, the inner ear alone is involved; deafness may be the first manifestation of a systemic disease or result from a possible immunological mechanism.
Autoimmune deafness is very invalidating. Bilateral perception deafness is observed in 80% of the cases and vestibular involvement is found in 70%
No one simple reliable test is known which can establish the diagnosis of autoimmune deafness. Other causes must be ruled out by appropriate clinical and complementary explorations. For humoral immunity, the western blot method has given promising results suggesting a possible role of the heat shock protein in the underlying immunological mechanism.
Immediate care is needed but no standard treatment has been defined. High-dose corticosteroids can provide symptom relief, particularly in case of abnormal immunological tests. The role of immunosuppressive therapy, sometimes proposed in case of corticosteroid resistance, remains to be defined.
一种真实的疾病实体：越来越多的临床和实验证据表明，自身免疫机制可导致耳聋。已知听觉前庭系统与一些全身性疾病有关，特别是科根氏病。在其他情况下，仅内耳受累；耳聋可能是全身性疾病的首发表现，或由可能的免疫机制引起。
自身免疫性耳聋使人极度虚弱。80%的病例表现为双侧感音神经性耳聋，70%的病例有前庭受累。
目前尚无一种简单可靠的检测方法可确诊自身免疫性耳聋。必须通过适当的临床和辅助检查排除其他病因。对于体液免疫，蛋白质印迹法已取得了有前景的结果，提示热休克蛋白在潜在免疫机制中可能发挥作用。
需要立即治疗，但尚未确定标准治疗方法。大剂量皮质类固醇可缓解症状，尤其是在免疫检查异常的情况下。对于皮质类固醇耐药时有时采用的免疫抑制疗法的作用仍有待确定。
