1例以听力下降为主要表现的Chiari畸形Ⅰ型及其人工耳蜗植入效果分析

杨腾裕; 平陆; 牛晓敏; 高儒真; 高俊; 陈晓巍

doi:10.13201/j.issn.1001-1781.2019.09.010

1例以听力下降为主要表现的Chiari畸形Ⅰ型及其人工耳蜗植入效果分析

- 1 中国医学科学院北京协和医学院北京协和医院耳鼻咽喉科(北京, 100730);
- 2 中国医学科学院北京协和医学院北京协和医院神经外科[JP]
基金项目:
国家重点研发计划资助项目“罕见病临床队列研究”(No:2016YFC0901500)

详细信息

通讯作者: 陈晓巍,E-mail:chenxw_pumch@163.com

中图分类号: R764.43

收稿日期: 2019-05-07

Outcome of cochlear implantation in a patient with Chiari malformation typeⅠ who presented with hearing loss

More Information

Corresponding author: CHEN Xiaowei, E-mail: chenxw_pumch@163.com

Received Date: 07 May 2019

摘要

- Chiari畸形 /
- 听力下降 /
- 听力康复 /
- 耳蜗植入术
Abstract: Chiari malformation type Ⅰ(CMI) is a disorder characterized by tonsilla cerebelli herniating into an underdeveloped posterior cranial fossa, hearing loss is often covered by more striking neurological symptoms. Hearing loss in this syndrome is not specific in terms of gender side, degree, age of onset, and progression. The hearing improvement after posterior fossa decompression is controversial on the basis of literature, while satisfactory result was obtained after cochlear implantation in the patient reported here, who was diagnosed as CMI with hearing loss as the main symptom. Therefore, after ensuring the integrity of the auditory pathway, cochlear implantation may be considered in CMI patients with bilateral severe or profound without other severe neurological symptoms.
- Chiari malformation /
- hearing loss /
- hearing rehabilitation /
- cochlear implantation

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