耳蜗发育不全解剖学特点及人工耳蜗植入术后效果研究  

Anatomical characteristics of cochlear hypoplasia and postoperative outcomes of cochlear implantation

作　　者：薛书锦 魏兴梅 高振橙 孔颖[1] 陆思萌 陈彪[1] 石颖[1] 崔丹默[1] 李永新[1] Xue Shujin;Wei Xingmei;Gao Zhencheng;Kong Ying;Lu Simeng;Chen Biao;Shi Ying;Cui Danmo;Li Yongxin(Department of Otorhinolaryngology Head and Neck Surgery,Beijing Tongren Hospital,Capital Medical University,Beijing 100730 China)

机构地区：[1]首都医科大学附属北京同仁医院耳鼻咽喉头颈外科,北京100730

出　　处：《首都医科大学学报》2024年第6期956-965,共10页Journal of Capital Medical University

基　　金：国家重点研发计划项目(2022YFC2402705);北京市自然科学基金项目(7244308);北京市医院管理中心青年人才培养“青苗”计划项目(QML20230204)。

摘　　要：目的评估不同亚型耳蜗发育不全(cochlear hypoplasia,CH)患者耳蜗骨性结构形态学特点,初步探究CH患者人工耳蜗植入(cochlear implantation,CI)术后听觉言语康复效果及其与畸形种类和解剖学特点的关系。方法对在首都医科大学附属北京同仁医院诊断为CH的CI患者临床资料进行收集并随访术后效果。利用颞骨计算机断层扫描(computed tomography,CT)三维重建技术分割重建不同亚型患者耳蜗三维图像并在其中测量和分析耳蜗长径、耳蜗宽径、耳蜗高度、耳蜗体积、底转长度及耳蜗管长度。使用效果问卷评估术后两年的听觉及言语康复情况,探究不同亚型及蜗神经发育情况患者术后效果特点,初步探究耳蜗解剖参数与术后效果的关系。结果53例患者中共102耳为CH畸形,其中9耳耳蜗形态特殊无法区分具体亚型。不同亚型CH耳蜗各解剖参数差异有统计学意义(P<0.05)。54.90%CH患者合并蜗神经发育不良(cochlear nerve deficiency,CND)。对其中38例患者进行术后效果随访,不同CH亚型间各项术后效果差异无统计学意义,是否合并CND在听觉行为分级标准(Categories of Auditory Performance,CAP)、言语可懂度分级标准(the Speech Intelligibility Rating,SIR)、有意义言语使用量表(Meaningful Use of Speech Scale,MUSS)得分差异有统计学意义(P<0.01)。蜗神经发育正常的CH患者各项术后效果得分与多项耳蜗解剖参数间正相关。结论CH患者耳蜗形态变异复杂,合并CND概率高,术后效果个体间差异大。术前颞骨CT三维重建能够辅助评估CH患者耳蜗形态,进行不同亚型鉴别诊断以及识别特殊形态耳蜗。合并CND患者效果差于蜗神经发育正常患者,蜗神经发育正常时耳蜗发育越好术后效果越好。术前应对CH患者耳蜗形态及蜗神经发育情况进行鉴别诊断及全面评估,以对手术方式及术后效果做出合理预估。Objective To assess the morphological characteristics of the bony structure of the cochlea in patients with different subtypes of cochlear hypoplasia(CH)and to explore the relationship between the auditory and speech rehabilitation outcomes following cochlear implantation(CI)in patients with CH and the subtype of malformation as well as anatomical characteristics.Methods Clinical data of CI patients diagnosed with CH at Beijing Tongren Hospital,Capital Medical University were collected and their postoperative outcomes were followed up.Temporal bone computed tomography(CT)3D reconstruction technology was used to reconstruct 3D images of the cochlea in patients with different subtypes.Measurements and analyses were conducted on the cochlear length,cochlear width,cochlear height,cochlear volume,basal turn length,and cochlear duct length.A questionnaire was used to evaluate auditory and speech rehabilitation two years postoperatively.The study investigated the effects of different subtypes and cochlear nerve development on postoperative outcomes and explored the relationship between cochlear anatomical parameters and postoperative results.Results Among 53 patients,a total of 102 ears were identified with CH malformations,with 9 ears having unique cochlear morphology that could not be classified into specific subtypes.Significant differences were found in the anatomical parameters among different CH subtypes(P<0.05).54.90%of CH patients had concomitant cochlear nerve deficiency(CND).Postoperative follow-up was conducted on 38 of the patients.There were no significant differences in postoperative outcomes among different CH subtypes,but the presence of CND showed significant differences in Categories of Auditory Performance(CAP),the Speech Intelligibility Rating(SIR)and Meaningful Use of Speech Scale(MUSS)scores(P<0.01).In CH patients with normal cochlear nerve development,there was a positive correlation between postoperative outcome scores and several cochlear anatomical parameters.Conclusions CH patients exhibit com

关 键 词：内耳畸形 人工耳蜗植入 颞骨计算机断层扫描 

分 类 号：R764[医药卫生—耳鼻咽喉科]