耳蜗神经发育不良患儿人工耳蜗植入术前影像和电生理评估

黄玉宇; 程岚; 杨军; 黄琦; 沈敏; 陈建勇

doi:10.13201/j.issn.1001-1781.2019.08.012

耳蜗神经发育不良患儿人工耳蜗植入术前影像和电生理评估

- 上海交通大学医学院附属新华医院耳鼻咽喉头颈外科上海交通大学医学院耳科学研究所上海市耳鼻疾病转化医学重点实验室(上海, 200092)
基金项目:
上海市科技人才计划项目 (No:16XD1402200)

详细信息

通讯作者: 程岚,E-mail:chenglan@xinhuamed.com.cn; 杨军,E-mail:yangjun@xinhuamed.com.cn

中图分类号: R764.93

收稿日期: 2019-02-20

Preoperative imaging and electrophysiological evaluation of cochlear implantation in children with cochlear nerve dysplasia

- Department of Respiratory Medicine, Affiliated Hospital of North China University of Technology, Tangshan, 063000, China

More Information

Corresponding authors: CHENG Lan ; YANG Jun

Received Date: 20 February 2019

摘要

摘要: 目的:探讨耳蜗神经发育不良(CND)患儿在人工耳蜗植入(CI)术前应用CT、MRI、功能性磁共振(fMRI)及电诱发听觉脑干反应(EABR)评价听觉通路、听皮层功能和耳蜗神经功能是否正常。方法:6例双侧极重度感音神经性聋患儿,其中4例CT显示双侧内听道狭窄,1例单侧内听道狭窄,余1例内听道无狭窄;3例内听道斜矢状位MRI重建双侧仅见面神经和前庭神经。6例患儿影像学诊断为CND。其中3例行fMRI,3例行EABR检查。全部患儿接受单侧CI,术后听力及言语康复随访至少1年。结果:3例CND并接受fMRI检查的患儿,其中1例左耳给声后,fMRI影像示右侧听皮层被激活,右耳给声,左侧听皮层未见激活;1例左耳、右耳及双耳2000 Hz给声,双侧颞横回均激活;另1例双侧均无激活。2例患儿EABR检测引出V波。6例患儿于CI术后1、6、12个月接受听力学及言语评估,其中4例患儿术后6个月内听力改善,声场听阈为(48.15±6.60)dBHL,有意义听觉整合量表和CAP评分改善,3例患儿SIR评分改善有限。1例患儿的听力提高,但因年龄偏大,言语康复不佳。结论:术前综合应用影像学、电生理评估CND患儿,可更准确地评估其听觉通路的完整性、了解耳蜗神经功能,对于是否实施手术有重要的参考意义。CND患儿CI术后的听觉言语康复差异较大。
- 耳蜗植入术 /
- 耳蜗神经发育不良 /
- 功能性磁共振 /
- 电诱发听觉脑干反应
Abstract: Objective: The aim of this study is to investigate the use of CT, magnetic resonance imaging (MRI), functional magnetic resonance (fMRI) and electrical evoked auditory brainstem response in children with cochlear nerve deficiency (CND) before cochlear implantation (CI) (electrically evoked auditory brain stem response, EABR) evaluated auditory pathway, auditory cortex function, and cochlear nerve function. Method: Of 6 children with bilateral profound sensorineural hearing loss, bilateral internal auditory meatus stenosis was diagnosed by CT as in 4 cases and unilateral internal auditory meatus stenosis in 1 case. In 3 cases, oblique sagittal MRI reconstruction of the internal auditory meatus showed only facial nerve and vestibular nerve existence. 6 cases were diagnosed as CND by imaging, among which underwent fMRI and EABR in 3 cases, respectively. All the children received unilateral CI and were followed up for at least 1 year after hearing and speech rehabilitation. Result: fMRI was examed in 3 cases with CND. It showed that the right auditory cortex was activated in 1 case after sound given to the left ear but no activation was found in the left cortex when sound given to the right ear. 1 case showed activation in bilateral transverse temporal gyrus while sound was given to the left, right and both ears at 2000Hz. Another case had no activation in both auditory cortex. EABR detection elicited V wave in 2 of 3 cases. 6 children received audiological and speech evaluation 1 month, 6 and 12 months after CI. Among them, the hearing level was improved within 6 months after surgery in 4 cases, and the threshold of sound field was (48.15±6.60) dB HL, MAIS and CAP scores were improved. However, SIR score improvement is limited in 3 cases. The hearing level was improved in 1 case, but speech rehabilitation was poor mainly due to the older age. Conclusion: Preoperative comprehensive application of imaging and electrophysiological evaluation of children with CND can more accurately assess the integrity of the auditory pathway and understand the function of the cochlear nerve. It has important reference significance for whether or not to perform surgery. There was a significant difference in auditory speech rehabilitation after CND in children with CND.
- cochlear implantation /
- cochlear nerve deficiency /
- functional magnetic resonance imaging /
- electrically evoked auditory brainstem response

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