双侧内耳道重复畸形与双侧人工耳蜗植入结果及综述
Bilateral duplication of the internal auditory canals and bilateral cochlear implant outcomes and review.
作者信息
Thompson Murray R, Birman Catherine S
机构信息
Department of Otolaryngology, Royal Prince Alfred Hospital, Missenden Road, Camperdown, 2050, NSW, Australia.
Department of Otolaryngology, Royal Prince Alfred Hospital, Missenden Road, Camperdown, 2050, NSW, Australia; Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Sydney, Australia; Children's Hospital at Westmead, Hawkesbury Road, Westmead, 2145, NSW, Australia; The Sydney Cochlear Implant Centre, Royal Institute of Deaf and Blind Children, PO Box 188, Gladesville, 1675, NSW, Australia; Department of Linguistics, Facility of Human Sciences, Macquarie University, North Ryde, Australia.
出版信息
Int J Pediatr Otorhinolaryngol. 2019 Apr;119:41-46. doi: 10.1016/j.ijporl.2019.01.015. Epub 2019 Jan 12.
OBJECTIVES
Bilateral duplication of the internal auditory canal (IAC) is rare and is associated with profound sensorineural hearing loss. The present study aims to review our experience with bilateral cochlear implantation (CI) in children with a duplication of the IAC and to review the literature.
METHODS
The Sydney Cochlear Implant Centre database was searched for children with duplication of the internal auditory canal. Data was collected regarding clinical history, MRI and CT findings, auditory brainstem responses (ABR), tympanometry and otoacoustic emissions (OAE), visually reinforced orientation audiometry, auditory brainstem response, electrocochleography (ECochG), transtympanic electrical auditory brainstem response (ABR), aided cortical evoked potentials (CAEP) and intraoperative neural response telemetry (NRT) and CI evoked electrical auditory brainstem testing.
RESULTS
two children with bilateral duplication of the IAC were identified who successfully underwent bilateral cochlear implantation. Audiological development was monitored for 2 and 3 years respectively, both children could spontaneously verbalise and displayed Categories of Auditory Performance (CAP) score of 5 and 6 respectively.
CONCLUSION
Children with duplication of the IAC, with accompanying cochlear nerve dysplasia (CND) can benefit from CI surgery, and verbal receptive and expressive language is possible.
目的
双侧内耳道重复畸形罕见，且与重度感音神经性听力损失相关。本研究旨在回顾我们对双侧内耳道重复畸形儿童进行双侧人工耳蜗植入（CI）的经验并复习相关文献。
方法
在悉尼人工耳蜗植入中心数据库中搜索内耳道重复畸形的儿童。收集有关临床病史、MRI和CT检查结果、听性脑干反应（ABR）、鼓室图和耳声发射（OAE）、视觉强化定向听力测定、听性脑干反应、耳蜗电图（ECochG）、经鼓膜电刺激听性脑干反应（ABR）、助听听觉皮层诱发电位（CAEP）以及术中神经反应遥测（NRT）和CI诱发的电刺激听性脑干测试的数据。
结果
确定了2例双侧内耳道重复畸形的儿童，他们成功接受了双侧人工耳蜗植入。分别对其听力发育进行了2年和3年的监测，两个孩子都能自发说话，听觉表现类别（CAP）评分分别为5分和6分。
结论
伴有耳蜗神经发育异常（CND）的双侧内耳道重复畸形儿童可从CI手术中获益，并且有可能实现言语接受和表达能力。
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