Sanz J J, Martínez P, Escobar J J, Menéndez L M
Servicio de Otorrinolaringología, Hospital Clínic, Barcelona.
Acta Otorrinolaringol Esp. 2002 Feb;53(2):121-5. doi: 10.1016/s0001-6519(02)78290-3.
Cogan's syndrome is a rare autoimmune disease with systemic involvement. It appears in young people and has two presentations: the typical form with keratitis, sudden deafness with or without vestibular syndrome, and the atypical form with different non keratitic ocular diseases and a great variety of systemic symptoms in relation with the autoimmune etiology of the process. Cogan's syndrome has a bad prognosis and deafness appears in 25% of the cases with the right treatment and in 60% of patients without treatment. The best treatment is systemic and ocular corticotherapy. The second treatment of choice is cyclophosphamide or cyclosporine A. We present two cases of atypical Cogan's syndrome with unilateral deafness in both.
科根综合征是一种罕见的累及全身的自身免疫性疾病。多见于年轻人,有两种表现形式:典型形式伴有角膜炎、突发耳聋(伴或不伴前庭综合征),非典型形式伴有不同的非角膜性眼部疾病以及与该疾病自身免疫病因相关的多种全身症状。科根综合征预后较差,接受正确治疗的病例中25%会出现耳聋,未接受治疗的患者中60%会出现耳聋。最佳治疗方法是全身及眼部皮质激素治疗。二线治疗选择是环磷酰胺或环孢素A。我们报告两例非典型科根综合征病例,均为单侧耳聋。