Karelle S, Demanez L, Zangerle P F, Blaise P, Moonen G, Poirrier A L
ENT Department, CHR-Citadelle, Liege, Belgium.
B-ENT. 2012;8(2):135-9.
Sudden sensorineural hearing loss is a perplexing entity in otology. Susac's syndrome (also called retinocochleocerebral vasculopathy) is a rare disorder that consists of microangiopathy of the brain, retina, and inner ear, and usually affects women in young adulthood. We describe the clinical aspects, radiographic findings, and management of one such case.
A 30-year-old woman was admitted to the hospital because of sudden onset of bilateral deafness and headache. During her hospitalization, she developed discrete right hemiparesis and hypoesthesia.
Magnetic resonance imaging revealed multiple signal hyperintensities and atrophy of the corpus callosum. The differential diagnosis was a myelinating condition, such as multiple sclerosis or acute demyelinating encephalomyelitis.
Retinal fluorescein angiography helped the diagnosis of Susac's syndrome.
突发性感音神经性听力损失是耳科学中一个令人困惑的病症。苏萨克综合征（也称为视网膜耳蜗脑微血管病）是一种罕见的疾病，由脑、视网膜和内耳的微血管病变组成，通常影响年轻成年女性。我们描述了这样一例病例的临床情况、影像学表现及治疗。
一名30岁女性因突发双侧耳聋和头痛入院。住院期间，她出现了右侧轻度偏瘫和感觉减退。
磁共振成像显示胼胝体有多个信号增强和萎缩。鉴别诊断为脱髓鞘疾病，如多发性硬化或急性脱髓鞘性脑脊髓炎。
视网膜荧光血管造影有助于苏萨克综合征的诊断。
