摘要
目的:探讨小儿听神经病的临床表现。方法:对一组年龄为4～68个月,听力学检查表现为听神经病患儿的临床资料进行回顾性研究。受试者的入选标准为短声听觉脑干反应(ABR)严重异常而畸变产物耳声发射(DPOAE)和(或)耳蜗微音电位(CM)正常。分析其短声ABR、DPOAE、行为听力测试、声导纳以及内耳MRI等结果。结果:①40例听力学表现为双侧听神经病,8例为单侧;②68.2%耳(60/88)在短声ABR的最大输出强度100dBnHL无反应,而有些耳仅在很高的测试强度有波形分化较差的Ⅴ波;③所有耳均可记录到CM,而只有41.5%能记录到DPOAE;④该组患儿的行为听力结果各种各样,为从轻度到极重度的听力损失;⑤4例经内耳MRI诊断为蜗神经发育不全。结论:在听力学表现为听神经病的儿童:①CM的诊断敏感度较DPOAE高;②各种客观听力测试无法预估行为听力;③需作内耳MRI检查,以除外蜗神经发育不全。
Objective：To present the clinical data of a group of children with audiological profile of auditory neuropathy. Method：Fourty eight infants and young children who had severely abnormal click ABRs along with present CMs and/or DPOAEs were included in this retrospective study. Click ABRs, CMs, DPOAEs, tympano-grams, behavioral thresholds and inner ear MRI were analysed. Result：Fourty children present with bilateral audio-logical profile of AN, 8 were unilateral. Most of the ears （68.2%） were of absent click ABRs at the maximum presentation level of 100 dB nHL. However, some of them had repeatable Wave V at very high presentation levels. All ears showed present CMs while only 41.7% showed preserved DPOAEs. Four children were diagnosed as CND by further investigation of inner ear MRI. Conclusion： It is recommended that CM testing be carried out routinely in children with severely abnormal click ABRs so as not to miss out AN; Behavioral hearing can not be predicted by auditory evoked responses; if audiological profile of AN is detected, further MRI exploration of well-developed cochlear nerve is necessary.
出处
《临床耳鼻咽喉头颈外科杂志》
CAS
CSCD
北大核心
2009年第13期580-583,587,共5页
Journal of Clinical Otorhinolaryngology Head And Neck Surgery
关键词
听神经病
蜗神经发育不全
auditory neuropathy
cochlear nerve deficiency
