Vollertsen R S, McDonald T J, Younge B R, Banks P M, Stanson A W, Ilstrup D M
Mayo Clin Proc. 1986 May;61(5):344-61. doi: 10.1016/s0025-6196(12)61951-x.
In this article, we review 78 cases of Cogan's syndrome--18 from our institution and 60 from the English medical literature--and describe the clinical, laboratory, radiographic, and pathologic findings associated with this disorder. Patients with Cogan's syndrome should be examined by an ophthalmologist, otolaryngologist, and internist because, in addition to the eye and audiovestibular involvement, systemic manifestations are common. An elevated erythrocyte sedimentation rate, anemia, leukocytosis, and thrombocytosis are common but nonspecific laboratory abnormalities. Serious outcomes include deafness and, less frequently, vasculitis, aortic insufficiency, blindness, and death. Glucocorticoids seem to be effective therapy for active Cogan's syndrome. Aortic valve replacement and vascular bypass grafting are indicated in selected cases. The roles of cytotoxic-immunosuppressive agents and cochlear implantation remain to be clarified.
在本文中，我们回顾了78例科根综合征患者——其中18例来自我们机构，60例来自英文医学文献——并描述了与该疾病相关的临床、实验室、影像学和病理学表现。科根综合征患者应由眼科医生、耳鼻喉科医生和内科医生进行检查，因为除了眼部和视听前庭受累外，全身表现也很常见。红细胞沉降率升高、贫血、白细胞增多和血小板增多是常见但非特异性的实验室异常。严重后果包括耳聋，较少见的有血管炎、主动脉瓣关闭不全、失明和死亡。糖皮质激素似乎是活动性科根综合征的有效治疗方法。在特定病例中需进行主动脉瓣置换和血管搭桥术。细胞毒性免疫抑制剂和人工耳蜗植入的作用仍有待阐明。
