Waardenburg综合征患者螺旋神经节细胞数量分布特征的分析  

Distribution of Spiral Ganglion Neuron in Waardenburg Syndrome

作　　者：倪晓琛 罗意 李佳楠[1,2,3,4] 陈伟 杨仕明[1,2,3,4] NI Xiaochen;LUO Yi;LI Jianan;CHEN Wei;YANG Shiming(Department of Otolaryngology-Head&Neck Surgery,the Sixth Medical Center of PLA General Hospital,National Clinical Research Center for Otolaryngologic Diseases,State Key Lab of Hearing Science,Ministry of Education,Beijing Key Lab of Hearing Impairment Prevention and Treatment,Beijing 100853,China)

机构地区：[1]解放军总医院第六医学中心耳鼻咽喉头颈外科医学部,北京100853 [2]国家耳鼻咽喉疾病临床医学研究中心,北京100853 [3]聋病教育部重点实验室,北京100853 [4]聋病防治北京市重点实验室,北京100853

出　　处：《中华耳科学杂志》2023年第4期447-452,共6页Chinese Journal of Otology

基　　金：国家自然科学基金面上项目(819708901011106)。

摘　　要：目的本研究旨在研究Waardenburg综合征(Waardenburg Syndrome,WS)患者的耳蜗中螺旋神经节细胞(spiral ganglion neuron,SGN)的数量与非综合征性感音神经性聋(Nonsyndromic sensorineural hearing loss,NS)患者相比是否具有差异,以及耳蜗不同位置之间的数量分布差异。方法本研究采用横断面研究,收集2000年至2020年间就诊于中国人民解放军总医院行人工耳蜗植入的WS患者,收集患者相关信息,包括植入年龄、植入侧别、植入电极型号以及人工耳蜗植入术中的电刺激复合动作电位(Electrical compound action potential,ECAP)测量数据以及其幅值增长曲线(amplitude growth function,AGF)。为控制植入年龄,植入电极类型等混杂因素带来的偏倚,制定对照组患者纳入标准,通过采用窗函数的计算方式,应用Matlab(R2016a)计算两组中不同刺激电极的slope值。依据刺激电极在耳蜗中的位置,将电极分为近蜗顶组,中间组以及近蜗底组,应用采用t检验比较两组患者中不同位置之间的slope值。结果依据纳入以及排除标准,最终纳入16名WS患者以及16名NS患者,经两独立样本t检验证实两组之间具有可比性。WS患者与NS患者的总体slope具有显著差异(P=0.007<0.05;Cohen's d=1.022>1),在不同位置中,以中间组以及近蜗底组的slope值具有显著差异(P=0.001<0.05;P=0.026<0.05,Co⁃hen's d=1.302>1;Cohen's d=0.828>0.8)。结论WS患者SGN的数量明显少于NS患者,并且在耳蜗的不同位置数量改变不同,尤其以底回以及中回的数量改变尤为显著。Objective To report the distribution pattern of spiral ganglion neurons in the cochlea in patients with Waardenburg Syndrome(WS)in comparison to patients with nonsyndromic sensorineural hearing loss(NS).Methods This was a cross-sectional study on patients with WS who underwent cochlear implantation at the Chinese People's Liberation Army General Hospital between 2000 and 2020.Information collected included age at implantation,implantation side and electrically evoked compound action potentials(ECAPs)measured during implantation as well as amplitude growth function(AGF).A control group was included for comparison purposes using matching inclusion criteria.The AGF slope value of each stimulation electrode was calculated for both WS and control(NS)groups using window functions in Matlab(R2016a).According to their positions in the cochlea,stimulation electrodes were categorized as in the apical,medial and basal regions.The two-sample t test was used to compare the AGF slope for all electrode positions between the two groups.Results Sixteen WS patients and 16 NS patients meeting inclusion/exclusion criteria were included,and the comparability between the two groups was confirmed by the two-sample independent t test.The overall slope was significantly different between WS and NS patients(P=0.007<0.05,Cohen's d=1.022>1).Slopes were also different between the two groups for medial and basal region electrode positions(P=0.001<0.05,Cohen's d=1.302>1 and P=0.026<0.05,Cohen's d=0.828>0.8,respectively).Conclusion The number of SGNs in WS patients appears to be significantly less than that in NS patients,especially in the basal and middle turns.

关 键 词：WAARDENBURG综合征 螺旋神经节细胞 ECAP slope值 

分 类 号：R764[医药卫生—耳鼻咽喉科]