蜗神经发育不良患者的影像及听力学表现分析  

Imaging and audiological characteristics of patients with cochlear nerve dysplasia

作　　者：鲁兆毅[1] 潘滔[1] 王宇[1] LU Zhao-yi;PAN Tao;WANG Yu(Department of Otorhinolaryngology Head and Neck Surgery,Peking University Third Hospital,Beijing 100191,China)

机构地区：[1]北京大学第三医院耳鼻咽喉头颈外科,北京100191

出　　处：《解剖学报》2020年第5期693-698,共6页Acta Anatomica Sinica

基　　金：北京大学深圳研究院科研项目(A68489-02);北京大学第三医院临床重点项目(青年项目,BYSY2018045)。

摘　　要：目的探讨蜗神经发育不良(CND)患者的影像学表现和听力学表现以及两者的相关性。方法对北京大学第三医院耳鼻咽喉科拟行单侧人工耳蜗植入,合并蜗神经发育异常的患者共7例(12耳)进行回顾性分析,对患者进行听力学检查,以及颞骨高分辨率CT及内耳道MRI等影像学检查,并在颞骨CT中测量内耳道(IAC)及蜗神经管(BCNC)直径,在内耳道MRI中测量内耳道内神经束数目、蜗神经有无狭窄或缺失等指标,并观察有无内耳畸形。分析不同影像学表现下听力情况的差异。结果7例患者影像学表现均提示蜗神经发育不良。颞骨CT中,3例(5耳)提示内耳道异常,6例(9耳)提示蜗神经管狭窄或缺失,内耳道MRI所见中;7例(12耳)提示蜗神经狭窄或缺失,其内耳道内神经束数目在0~3根不等。此外,影像学检查发现,4例(8耳)合并不同类型的内耳畸形。7例患者听力学表现均为双耳重度或极重度感音神经性听力下降。CND的不同影像学表现(包括IAC、BCNC、内耳道神经束数目、有无内耳畸形等)下,各组间平均听阈差异不显著(P>0.05)。结论蜗神经发育异常的影像学特点包括颞骨CT中内耳道及蜗神经管的异常,内耳道MRI中神经束数目及蜗神经的异常等,听力多表现为重度或极重度感音神经性聋,影像学及听力学表现之间未发现明显关联。对该类患者的人工耳蜗植入存在较大的挑战。Objective To investigate the imaging and audiological characteristics of patients with cochlear nerve dysplasia(CND),and the relationship between them.Methods Seven patients undergoing unilateral cochlear implantation with abnormal cochlear nerve development were studied retrospectively.The patients were examined by audiology,high-resolution CT of temporal bone and MRI of internal auditory canal(IAC).The diameter of IAC and bony cochlear nerve canal(BCNC)were measured in temporal bone CT.The number of nerve bundles in IAC and the stenosis or absence of cochlear nerve were measured in the MRI of IAC,and inner ear was checked whether there is deformity.The differences of hearing performance in different imaging manifestations were analyzed.Results In the imaging findings,7 cases all indicated cochlear nerve dysplasia.In temporal bone CT,3 cases(5 ears)showed abnormal IAC,6 cases(9 ears)showed stenosis or absence of BCNC.In the MRI findings,7 cases(12 ears)showed stenosis or absence of the cochlear nerve.The number of nerve bundles in IAC varied from 0 to 3.In addition,4 cases(8 ears)with different types of inner ear malformations were found by imaging examination.The audiological performance of 7 patients were severe or profound sensorineural hearing loss.There were no significant differences in the pure tone average threshold in different imaging manifestations.(including IAC,BCNC,number of nerve bundles,and whether combined with inner ear malformations)(P>0.05).Conclusion The imaging features of CND include the abnormalities of the IAC and BCNC in temporal bone CT,the number of nerve bundles in IAC and abnormalities of the cochlear nerve in MRI.The audiogram usually shows severe or profound sensorineural hearing loss.There is no significant correlation between the imaging and audiological manifestations.There is a big challenge for cochlear implantation in such cases.

关 键 词：蜗神经发育不良 内耳道 蜗神经管 蜗神经 高分辨率CT 磁共振成像 人 

分 类 号：R764.73[医药卫生—耳鼻咽喉科]