Grover N, Whiteside O J H, Ramsden J D
Department of Otolaryngology Head and Neck Surgery, John Radcliffe Hospital, Oxford, UK.
J Laryngol Otol. 2011 Aug;125(8):856-8. doi: 10.1017/S0022215111000600. Epub 2011 Apr 12.
Susac syndrome comprises a triad of vestibulocochlear dysfunction, retinopathy and multifocal encephalopathy, which is characterised pathophysiologically by microangiopathy of the ear, retina and brain. Diagnosis is confirmed by magnetic resonance imaging of the brain and ophthalmological examination, which reveals branch retinal artery occlusion. Hearing loss persists in 90 per cent of patients. We present a case of successful hearing rehabilitation by cochlear implantation in a young woman with this syndrome.
A 36-year-old woman presented with neurological symptoms suggestive of encephalitis. She subsequently developed vestibulocochlear symptoms. The diagnosis was confirmed upon magnetic resonance imaging and fluorescein angiography, which showed multiple peripheral retinal arterial occlusions. Hearing loss was fluctuant but gradually progressive over nine months, to bilateral profound sensorineural hearing loss.
A left cochlear implant was placed, with a good outcome.
In this Susac syndrome patient, the outcome of cochlear implantation was encouraging, notwithstanding the possible involvement of retrocochlear pathways.
Susac综合征由前庭蜗神经功能障碍、视网膜病变和多灶性脑病组成，其病理生理特征为耳、视网膜和脑的微血管病变。通过脑部磁共振成像和眼科检查确诊，后者显示视网膜分支动脉阻塞。90%的患者听力损失持续存在。我们报告一例年轻女性Susac综合征患者通过人工耳蜗植入成功实现听力康复的病例。
一名36岁女性出现提示脑炎的神经症状。随后出现前庭蜗神经症状。磁共振成像和荧光素血管造影确诊，显示多处周边视网膜动脉阻塞。听力损失呈波动性，但在9个月内逐渐进展为双侧极重度感音神经性听力损失。
植入左侧人工耳蜗，效果良好。
在该Susac综合征患者中，尽管蜗后通路可能受累，但人工耳蜗植入的效果令人鼓舞。
