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蜗神经发育不良儿童的临床特征分析

Clinical Characteristics in Children with Cochlear Nerve Dysplasia

钟梅芳;洪晓纯;李楚凌;

1:东莞市妇幼保健院

2:东莞市妇女儿童医院耳鼻咽喉头颈外科

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摘要

目的探讨蜗神经发育不良(cochlear nerve deficiency,CND)儿童的临床特征。方法以43例(60耳)CND患儿(4个月～10岁,平均2.6±2.8岁)为研究对象,总结分析其是否存在听力损失高危因素、影像学检查及听性脑干反应(ABR)、畸变产物耳声发射(DPOAE)、Chirp声诱发听性稳态反应(Chirp-ASSR)检测等结果。结果 43例患儿中,26例(60.5%,26/43)为单侧病变,17例(39.5%,17/43)为双侧病变;仅7例患儿有听力损失高危因素。50耳(83.3%,50/60)为蜗神经缺如,10耳(16.7%,10/60)为蜗神经细小;16耳(26.7%,16/60)伴面神经细小,8耳(13.3%,8/60)伴前庭神经异常。4耳(6.7%,4/60)仅伴前庭畸形为第一组,21耳(35%,21/60)合并耳蜗畸形或同时合并前庭畸形为第二组,35耳(58.3%,35/60)不伴内耳畸形为第三组。26耳(43.3%,26/60)ABR仅见波Ⅲ以前波形分化,随后波形消失;23耳(38.3%,23/60)ABR无波形分化;11耳(18.3%,11/60)可见分化不良的波V,其反应阈值为75～100 dB nHL。24耳(40%,24/60)DPOAE或/和CM引出,且第三组CND患儿DPOAE的信噪比(SNR)值和引出率均明显高于CND合并内耳畸形的第一、二组患儿。49耳ABR最大声输出时未引出波V的CND患儿中,41耳(83.7%,41/49)Chirp-ASSR可在不同频率不同程度引出,500、1 000、2 000和4 000 Hz Chirp-ASSR平均反应阈分别为87.14±21.33、89.27±16.09、89.37±15.85和91.10±15.77 dB corHL。结论本组CND患儿多无明确听力损失高危因素或病因,多表现为重度或极重度感音神经性聋,高发于先天性单侧感音神经性聋婴幼儿,可表现出听神经病的特征,ABR仅见波III以前波形分化,随后波形消失,Chirp-ASSR测得残余听力明显优于ABR检测结果。

关键词

蜗神经发育不良;单侧聋;儿童;听神经病

基金项目(Foundation):

作者

钟梅芳;洪晓纯;李楚凌;

参考文献

1 沙炎,罗德红,李恒国,主编.头颈部影像学——耳鼻咽喉头颈外科卷[M].北京:人民卫生出版社,2014.42～42.

2 Chinnadurai V,Sreedhar CM,Khushu S.Assessment of cochlear nerve deficiency and its effect on normal maturation of auditory tract by diffusion kurtosis imaging and diffusion tensor imaging:A correlational approach[J].Magn Reson Imaging,2016,34:1305.

3 卫生部关于印发《新生儿疾病筛查技术规范(2010年版)》的通知[R].中华人民共和国国家卫生和计划生育委员会公报,2011(1):12～22.

4 黄治物.婴幼儿听力损失的早期诊断评估及干预(5)[J].听力学及言语疾病杂志,2013,21:669

5 银力,高珊仙,屠文河,等.单侧聋患者人工耳蜗植入的进展[J].听力学及言语疾病杂志,2017,25:210.

6 王秋菊,Starr A.听神经病:从发现到渐入精准[J].中华耳鼻咽喉头颈外科杂志,2018,53:161.

7 Govaerts PJ,Casselman J,Daemers K,et al.Cochlear implants in aplasia and hypoplasia of the cochleovestibular nerve[J].Otol Neurotol,2003,24:887.

8 钟梅芳,李楚凌.东莞市1419例婴幼儿听力缺失诊断结果分析[J].听力学及言语疾病杂志,2019,27:604

9 Wilkins A,Prabhu SP,Huang L,et al.Frequent association of cochlear nerve canal stenosis with pediatric sensorineural hearing loss[J].Arch Otolaryngol Head Neck Surg,2012,138:383.

10 Clemmens CS,Guidi J,Caroff A,et al.Unilateral cochlear nerve deficiency in children[J].Otolaryngol Head Neck Surg,2013,149:318.

11 Sunwoo W,Lee WW,Choi BY.Extremely common radiographic finding of cochlear nerve deficiency among infants with prelingual single-sided deafness and its clinical implications[J].Int J Pediatr Otorhinolaryngol,2018,112:176.

12 Nakano A,Arimoto Y,Matsunaga T.Cochlear nerve deficiency and associated clinical features in patients with bilateral and unilateral hearing loss[J].Otol Neurotol,2013,34:554.

13 黄丽辉.婴幼儿听神经病谱系障碍诊断及处理指南——婴幼儿听神经病诊断及处理指南研讨会制订(2008年,意大利,科莫)[J].听力学及言语疾病杂志,2012,20:64.

14 Han JJ,Suh MW,Park MK,et al.A predictive model for cochlear implant outcome in children with cochlear nerve deficiency[J].Sci Rep,2019,9:1154.

15 Huang BY,Roche JP,Buchman CA,et al.Brain stem and inner ear abnormalities in children with auditory neuropathy spectrum disorder and cochlear nerve deficiency[J].Am J Neuroradiol,2010,31:1972.

16 Buchman CA,Roush PA,Teagle HF,et al.Auditory neuropathy characteristics in children with cochlear nerve deficiency[J].Ear Hear,2006,27:399.

17 史伟,王秋菊,刘志成.小儿听神经病患者ASSR检测结果分析[J].听力学及言语疾病杂志,2014,22:117.

18 李楚凌,谭淑娟,王远明,等.Chirp-ASSR对婴幼儿听神经病谱系障碍诊断的应用研究[J].听力学及言语疾病杂志,2018,26:243.

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钟梅芳洪晓纯李楚凌