Wendt Wendi-Jo, Hashikawa Andrew
From the University of Michigan, Ann Arbor, MI.
Pediatr Emerg Care. 2019 Jul;35(7):e135-e137. doi: 10.1097/PEC.0000000000001043.
Enlarged vestibular aqueduct syndrome (EVAS) is the most common congenital ear anomaly that causes sensorineural hearing loss in children and may predispose a child to sudden hearing loss from sudden pressure changes or minor head trauma. We report a case of a 4-year-old boy with a history of a ventriculoperitoneal shunt and migraines who presented to the emergency department with parental and child care provider reports of acute hearing loss, without a history of trauma, infection, or hardware malfunction, who was diagnosed with bilateral EVAS. Diagnosis of EVAS occurs with specific temporal bone imaging with either high-resolution, thin-cut computed tomography or magnetic resonance imaging scans. Enlarged vestibular aqueduct syndrome is typically refractory to medical treatment and often results in hearing loss that is too severe to benefit from amplification, requiring cochlear implantation.
扩大的前庭导水管综合征（EVAS）是导致儿童感音神经性听力损失最常见的先天性耳部异常，可能使儿童因突然的压力变化或轻微头部外伤而容易出现突发性听力损失。我们报告一例4岁男孩，有脑室腹腔分流术和偏头痛病史，因家长和儿童护理人员报告其急性听力损失而就诊于急诊科，无外伤、感染或硬件故障史，被诊断为双侧EVAS。EVAS的诊断通过特定的颞骨成像进行，采用高分辨率薄层计算机断层扫描或磁共振成像扫描。扩大的前庭导水管综合征通常对药物治疗无效，常导致听力损失严重到无法从放大装置中获益，需要进行人工耳蜗植入。
