Paniagua Lauren Medeiros, Dorfman Maria Elza Kazumi Yamaguti, Lavinsky Luiz, Sleifer Pricila
Doctor of Science in Children's and Teenager's Health, Federal University of Rio Grande do Sul-UFRGS, Porto Alegre/RS, Brazil ; Professor of Speech-Language Pathology, Fatima's University (RS), Rio de Janeiro/RJ, Brazil.
Speech-Language Pathologist, Voice Specialist, Hospital de Clinicas de Porto Alegre, Porto Alegre/RS, Brazil.
Int Arch Otorhinolaryngol. 2013 Oct;17(4):403-6. doi: 10.1055/s-0033-1351669.
Background Harboyan syndrome, defined as congenital corneal dystrophy associated with progressive sensorineural hearing loss, was first described by Harboyan in 1971. It is a hereditary disease manifested by eye lesions consistent with corneal endothelial dystrophy and progressive sensorineural hearing loss. There is bilateral symmetric progressive hearing loss, which may be either dominant or recessive. Objective To report a case of a patient with a diagnosis of Harboyan syndrome. Case Report A 25-year-old woman with profound bilateral sensorineural hearing loss, showing poor hearing performance while using a personal sound amplification device, underwent hearing rehabilitation with a cochlear implant. Conclusion Rehabilitation was imperative in this case. The cochlear implant has proven to be the best therapeutic option, providing the patient with a better quality of life.
哈博扬综合征定义为与进行性感音神经性听力损失相关的先天性角膜营养不良,由哈博扬于1971年首次描述。它是一种遗传性疾病,表现为与角膜内皮营养不良一致的眼部病变和进行性感音神经性听力损失。存在双侧对称性进行性听力损失,可能为显性或隐性。目的:报告一例诊断为哈博扬综合征的患者。病例报告:一名25岁双侧重度感音神经性听力损失的女性,使用个人扩音设备时听力表现不佳,接受了人工耳蜗植入听力康复治疗。结论:该病例必须进行康复治疗。人工耳蜗已被证明是最佳治疗选择,为患者提供了更好的生活质量。