目的:报告一例先天性耳聋患者的Susac综合征致死病例,患者植入人工耳蜗,有偏头痛史,强调有既往疾病的患者的诊断挑战。
方法:一名33岁男性先天性耳聋,人工耳蜗,和慢性偏头痛,表现为轻度亚急性听觉障碍和头痛,后来发展为严重脑病。
方法:进行非磁共振成像(MRI)兼容的人工耳蜗植入,然后进行MRI,眼底镜检查,和免疫抑制药物的管理。
方法:诊断通过特征性MRI表现和视网膜半动脉阻塞的存在得到证实。
结果:经过数周的免疫抑制治疗,患者死于不明原因的全脑缺血事件.
结论:对于先前存在感音神经性听力损失和人工耳蜗植入的患者,Susac综合征提出了诊断挑战。在没有人工耳蜗失败的情况下,听觉障碍应提示进一步评估视觉障碍和脑病。应进行MRI和眼底镜检查以检测疾病的其他特征。
OBJECTIVE: To report a fatal case of Susac syndrome in a congenitally deaf patient with a cochlear implant and a history of migraines, emphasizing the diagnostic challenges in patients with preexisting conditions.
METHODS: A 33-year-old male with congenital hearing loss, a cochlear implant, and chronic migraines who presented with mild subacute auditory disturbance and headaches that later progressed to severe encephalopathy.
METHODS: Explantation of a non-magnetic resonance imaging (MRI) compatible cochlear implant followed by MRI, fundoscopy, and the administration of immunosuppressive medications.
METHODS: Diagnosis was confirmed by characteristic MRI appearance and the presence of a hemi-retinal artery occlusion.
RESULTS: After weeks of immunosuppressive treatment, the patient died of a global cerebral ischemic event of unknown origin.
CONCLUSIONS: For patients with preexisting sensorineural hearing loss and cochlear implants, Susac syndrome poses a diagnostic challenge. Auditory disturbances in the absence of cochlear implant failure should prompt further evaluation for visual disturbances and encephalopathy. MRI and fundoscopy should be performed to detect other features of the disease.