Azami Ahad, Maleki Nasrollah, Kalantar Hormozi Mohammadreza, Tavosi Zahra
Department of Internal Medicine, Imam Khomeini Hospital, Ardabil University of Medical Sciences, Ardabil, Iran.
Department of Endocrine and Metabolic Diseases, The Persian Gulf Tropical Medicine Research Center, Bushehr University of Medical Sciences, Bushehr 7514763448, Iran.
Case Rep Med. 2014;2014:830831. doi: 10.1155/2014/830831. Epub 2014 Mar 4.
Cogan's syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan's syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement.
科根综合征（CS）是一种病因不明的慢性炎症性疾病，最常影响年轻人。临床特征为双侧间质性角膜炎和前庭听觉功能障碍。CS与系统性血管炎以及主动脉炎之间也存在关联。CS的诊断基于特征性炎性眼病和前庭听觉功能障碍的存在。我们描述了一名来自阿尔达比勒的47岁女性的典型科根综合征。该患者因头痛、眩晕、恶心、呕吐、右腿间歇性跛行、肌肉骨骼疼痛、双侧听力丧失和失明入院，病程长达两个月。眼科检查显示双侧视力均为0.1。裂隙灯检查发现结膜充血、双侧白内障和间质性角膜炎。纯音听力图（PTA）和听觉脑干反应（ABR）显示双侧感音神经性听力损失。对CS的其他鉴别诊断进行了研究并排除。静脉注射甲基泼尼松龙和环磷酰胺，随后口服泼尼松龙和环磷酰胺。临床随访显示病情有部分改善。
