Cogan’s syndrome is a rare, chronic inflammatory disorder that typically targets the eyes and vestibuloauditory apparatus, but it may also involve other organs. Three pediatric cases of Cogan’s syndrome (ages 5, 13, and 18 years) are reported with long-term follow-up and complete and regular cochleovestibular functional evaluation and ophthalmologic and neurologic examinations. One case was a typical form (characterized by an interstitial keratitis and cochleovestibular impairment), whereas the other 2 cases were atypical forms with uveitis and polyarthritis. In all 3 cases, the first clinical sign was nonspecific eye redness misdiagnosed as a banal conjunctivitis, initially or secondarily associated with bilateral endocochlear sensorineural hearing loss and complete bilateral peripheral vestibular deficit. During the acute phase, early steroid treatment (prednisone, 1 mg/kg/day) was effective in treating the ocular lesions (3 of 3 cases) and improving hearing (2 of 3 cases) but less effective for the vestibular loss (2 of 3 cases). Adverse effects and dependence on the steroid occurred in 2 cases, and immunosuppressive drugs were necessary to avoid recurrences in 1 case. Over the long-term, the disease was controlled in 2 cases but continued to progress in the other. Cogan’s syndrome in childhood should be suspected in cases of conjunctivitis associated with inner-ear symptoms; a prompt steroid treatment can avoid progressive impairment of multiple sensorineural functions (vision, balance, hearing). Long-term management involves limiting disease recurrences by adaptive therapies, screening for complications (aortitis in particular), and planning rehabilitation for the sensorial deficits.
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机译：科根综合症是一种罕见的慢性炎症性疾病，通常以眼睛和前庭听觉器官为目标，但也可能涉及其他器官。据报道，有3例Cogan综合征的儿科病例（分别为5、13和18岁），并进行了长期随访，完整而定期的耳蜗前庭功能评估以及眼科和神经科检查。 1例是典型形式（以间质性角膜炎和耳蜗前庭损害为特征），而其他2例是非典型形式的葡萄膜炎和多关节炎。在所有3例病例中，第一个临床体征是非特异性眼红，被误诊为陈旧性结膜炎，最初或次要与双侧耳蜗内感觉神经听力丧失和双侧外周前庭完全性缺失相关。在急性期，早期类固醇治疗（泼尼松1 mg / kg /天）可有效治疗眼部病变（3例，3例）和改善听力（3例，2例），但对前庭丢失的疗效较差（2例，3例）。 3例）。不良反应和对类固醇的依赖发生2例，为避免复发，必须使用免疫抑制剂治疗1例。从长期来看，该病控制在2例中，但另一例仍在继续发展。如果伴有内耳症状的结膜炎，应怀疑儿童时期的科根综合症。及时的激素治疗可以避免多种感觉神经功能（视觉，平衡，听力）的逐步损害。长期管理涉及通过适应性疗法来限制疾病的复发，筛查并发症（尤其是主动脉炎）以及规划感觉缺陷的康复。
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