Gluth Michael B, Baratz Keith H, Matteson Eric L, Driscoll Colin L W
Department of Otorhinolaryngology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
Mayo Clin Proc. 2006 Apr;81(4):483-8. doi: 10.4065/81.4.483.
To evaluate the disease manifestations and clinical course of patients affected by Cogan syndrome (a syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms) at a single institution during roughly a half century.
Medical records of all patients diagnosed as having Cogan syndrome at the Mayo Clinic in Rochester, Minn, and who were followed up from 1940 to 2002 were comprehensively reviewed. Otolaryngologic, ophthalmologic, and systemic manifestations of disease were analyzed. Analysis included patient demographics, presenting manifestations, delayed manifestations, laboratory testing, physical examination features, therapeutic interventions, disease course, and hearing and vision outcomes.
Sixty patients were identified as having Cogan syndrome, with follow-up from 1940 to 2002. Most patients presented initially with vestibuloauditory symptoms, most commonly sudden hearing loss (50%). The most common inflammatory ophthalmologic condition noted was bilateral interstitial keratitis. Headache (40%), fever (27%), and arthralgia (35%) were the most frequently encountered systemic manifestations. Evidence of aortitis was found in 12% of patients. Complete hearing loss was eventually noted in 52% of affected patients, whereas permanent loss of any degree of vision was uncommon. Cochlear implantation outcomes were uniformly good. Death directly or indirectly attributed to the effects of Cogan syndrome was noted in 4 patients.
The major disease-related morbidities were due to vestibuloauditory disease and only infrequently due to systemic manifestations such as vasculitis, with or without aortitis. Cochlear implantation has been of major benefit in modern hearing rehabilitation for this patient population. We advise caution before institution of protracted courses of high-dose corticosteroids and/or chemotherapy for patients without pronounced systemic disease or severe eye disease unmanageable by topical or periocular corticosteroids alone.
评估在一家机构中约半个世纪里受科根综合征（一种非梅毒性间质性角膜炎和前庭听觉症状综合征）影响患者的疾病表现和临床病程。
全面回顾了明尼苏达州罗切斯特市梅奥诊所诊断为科根综合征且在1940年至2002年期间接受随访的所有患者的病历。分析了疾病的耳鼻喉科、眼科和全身表现。分析内容包括患者人口统计学资料、初始表现、延迟表现、实验室检查、体格检查特征、治疗干预、疾病病程以及听力和视力结果。
确定60例患者患有科根综合征，随访时间为1940年至2002年。大多数患者最初表现为前庭听觉症状，最常见的是突发听力丧失（50%）。最常见的炎症性眼科疾病是双侧间质性角膜炎。头痛（40%）、发热（27%）和关节痛（35%）是最常遇到的全身表现。12%的患者有主动脉炎证据。最终52%的受累患者出现完全听力丧失，而任何程度的永久性视力丧失并不常见。人工耳蜗植入效果均良好。4例患者的死亡直接或间接归因于科根综合征的影响。
主要的疾病相关发病原因是前庭听觉疾病，仅偶尔由血管炎等全身表现导致，无论有无主动脉炎。人工耳蜗植入对该患者群体的现代听力康复有很大益处。对于没有明显全身疾病或仅局部或眼周使用糖皮质激素无法控制的严重眼部疾病的患者，在开始长期大剂量糖皮质激素和/或化疗疗程之前，我们建议谨慎行事。
