Arnold-Chiari畸形1型的人工耳蜗植入：拓展小儿神经耳科学的边界

文献信息

中文摘要

Arnold-Chiari畸形（ACM）是一种罕见的先天性后脑异常，根据后颅窝内容物疝出的程度分为1-4型。1型ACM的发病率最高，大多数病例无症状。病例常被偶然发现；然而，有些可能表现为头痛、咳嗽、感觉异常，以及极少数情况下下组颅神经受压的症状，如感音神经性听力损失。由于相关的骨骼畸形和颅内压升高，预计手术具有挑战性。本文报告了最年轻的病例——一名3岁女孩，有言语和语言发育里程碑延迟，被偶然诊断为1型ACM。听力学检查显示双耳重度感音神经性听力损失。经多学科评估许可后，她接受了右侧人工耳蜗植入。术中所面临的挑战包括扩张的导静脉、前置的乙状窦和低位硬脑膜。然而，实现了完全电极插入，并通过神经反应遥测确认。术后过程顺利。文献回顾表明，这是1型ACM中人工耳蜗植入的最年轻报告病例。虽然本例中的听力损失可能与ACM无关，但通过细致的术前计划和术中警惕，成功的人工耳蜗植入是可行的。经过全面的解剖评估、多学科术前评估、术中警惕和神经麻醉师支持，1型ACM患儿可以安全地进行人工耳蜗植入。

英文摘要

Arnold-Chiari malformation (ACM) is a rare congenital hindbrain anomaly, with types 1-4 depending of degree of herniation of posterior fossa contents. The incidence of type 1 ACM is highest, with most cases being asymptomatic. Cases are incidentally picked up; however, some may present with headache, cough, paresthesia, and very rarely, symptoms of compression of lower cranial nerves such as sensorineural hearing loss. Surgical challenges are anticipated due to associated skeletal malformations and raised intracranial pressure. The youngest reported case-a 3-year-old girl with delayed speech and language milestones-is presented, in whom ACM type 1 was incidentally diagnosed. Audiological workup revealed profound sensorineural hearing loss in both ears. After multidisciplinary clearance, she underwent right-sided cochlear implantation. Intraoperative challenges faced were dilated emissary veins, an anteriorly placed sigmoid sinus, and a low-lying dura. However, a full electrode insertion was achieved and confirmed on neural response telemetry. The postoperative course was uneventful. Literature review suggests this to be the youngest reported case of cochlear implantation in ACM type 1. While hearing loss in this case may have been unrelated to ACM, nonetheless, successful cochlear implantation is feasible with meticulous preoperative planning and intraoperative vigilance. After a thorough anatomical assessment, multidisciplinary preoperative workup, intraoperative vigilance, and neuro-anesthetist backup, cochlear implantation can be safely performed in children with ACM type 1.