Waardenburg综合征(Waardenburg Syndrome,WS)是一类以感音神经性听力损失,虹膜、皮肤及毛发着色异常为特征的疾病。后来的研究发现,前庭障碍也是WS的另一重要临床表现,包括前庭器官畸形及前庭功能障碍。本文探讨WS中黑色素缺乏对前庭障碍的影响,分析WS患者缺乏前庭功能障碍症状的原因,并提醒临床医生对需行人工耳蜗植入的WS患者要密切关注有无内耳畸形。
Waardenburg syndrome (WS) is characterized by pigmentary anomalies of irides, skin and hair and sensorineural hearing loss. Recent studies have found that vestibular disorders are important clinical manifestations of WS, which include vestibular organs malformation and vestibular dysfunction. In this article, the authors discuss the effects of melanin deficiency on the vestibular disorders in WS, analyze the reasons for the lack of vestibular dysfunction in patients with WS and remind clinicians of paying close attention to the inner ear malformations in WS patients who need cochlear implantation.