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[科根综合征I型。进行性感音神经性听力损失中一种罕见的鉴别诊断]

[Cogan-I-syndrome. A rare differential diagnosis in progressive sensorineural hearing loss].

作者信息

Massinger C, Keilmann A

机构信息

Klinik für Kommunikationsstörungen, Klinikum der Johannes-Gutenberg-Universität Mainz.

出版信息

HNO. 2003 Jan;51(1):66-70. doi: 10.1007/s00106-002-0707-8.

DOI:10.1007/s00106-002-0707-8
PMID:12557101
Abstract

We report on two patients affected by Cogan's syndrome since infancy.Cogan's syndrome is described as the association of abrupt or intermittent worsening of vestibulo-auditory function and non-syphilic interstitial keratitis or, in patients with "atypical" Cogan's syndrome, various severe, inflammatory eye diseases. The first patient was affected with different inflammatory eye diseases from her fifth year and became blind in one eye when she was 7 years old. At that time, a deterioration of her hearing ability was also diagnosed. The hearing loss progressed to a severe hearing disorder. The second patient suffered from different inflammatory eye diseases from his second year. When he was 3 years old,he was diagnosed with a severe hearing disorder that progressed.Cogan's syndrome was diagnosed when he was 13 years old. The recognition of Cogan's syndrome is particularly complex in young patients. The difficulty of the diagnosis requires a search for ophthalmic diseases in young children with acute and progressive vestibulo-auditory symptoms.

摘要

我们报告了两名自婴儿期起就患有科根综合征的患者。科根综合征被描述为前庭听觉功能突然或间歇性恶化与非梅毒性间质性角膜炎相关联,或者在“非典型”科根综合征患者中,与各种严重的炎症性眼病相关联。第一名患者从5岁起就患有不同的炎症性眼病,7岁时一只眼睛失明。当时,她的听力也被诊断出恶化。听力损失发展为严重的听力障碍。第二名患者从2岁起就患有不同的炎症性眼病。3岁时,他被诊断出患有严重的听力障碍且病情不断发展。他13岁时被诊断出患有科根综合征。在年轻患者中,科根综合征的识别尤为复杂。诊断的困难在于需要对有急性和进行性前庭听觉症状的幼儿进行眼科疾病排查。

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1
[Cogan-I-syndrome. A rare differential diagnosis in progressive sensorineural hearing loss].[科根综合征I型。进行性感音神经性听力损失中一种罕见的鉴别诊断]
HNO. 2003 Jan;51(1):66-70. doi: 10.1007/s00106-002-0707-8.
2
[Cogan-I-syndrome.A rare differential diagnosis in progressive sensorineural hearing loss].[科根综合征I型。进行性感音神经性听力损失的一种罕见鉴别诊断]
HNO. 2003 Jan;51(1):66-70. doi: 10.1007/s00106-002-0707-8.
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引用本文的文献

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Paediatric Cogan´s syndrome - review of literature, case report and practical approach to diagnosis and management.小儿科 Cogan 综合征——文献综述、病例报告及诊断与管理的实用方法。
Pediatr Rheumatol Online J. 2023 Jun 8;21(1):54. doi: 10.1186/s12969-023-00830-x.
2
[Atypical Cogan syndrome as a differential diagnosis of sudden sensorineural hearing loss].[非典型科根综合征作为突发性感音神经性听力损失的鉴别诊断]
HNO. 2022 May;70(5):405-414. doi: 10.1007/s00106-022-01168-8. Epub 2022 Apr 14.
3
[Cogan's disease. Report of two cases].[科根氏病。两例报告]
HNO. 2010 Jul;58(7):698-703. doi: 10.1007/s00106-009-1980-6.
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[Cogan's syndrome: a diagnostic challenge].[科根综合征:一项诊断挑战]
HNO. 2006 Aug;54(8):619-23. doi: 10.1007/s00106-005-1316-0.