单侧听神经病儿童的听力学特征分析

Clinical Features in Children with Unilateral Auditory Neuropathy

目的 分析儿童单侧听神经病(Unilateral Auditory Neuropathy,UAN)听力学特点,为该类儿童诊断提供可靠依据。方法 回顾分析12例UAN儿童(男8人,女4人),平均年龄3.92岁(1-7岁)。对比38例(76耳)双侧(Auditory Neuropathy,AN)病例,分析游戏测听(Play audiometry,PA)、听性脑干反应(Auditory brainstem response,ABR)、稳态听觉诱发电位(Steady-state auditory evoked potential,ASSR)、耳蜗微音器电位(Cochlear microphonic potential,CM)以及耳声发射(Otoacoustic emission,OAE)的临床听力学特点。结果 (1)UAN组儿童PA阈值比双侧AN组儿童更高,极重度听力损失占33.33%,完全听力损失占66.67%;(2)UAN组儿童PA阈值显著高于双侧AN组,UAN组儿童ASSR阈值与双侧AN组无显著性差异;(3)UAN组儿童ABR阈值引出率为50%,高于双侧AN组的25%。结论(1)UAN更具隐匿性,较之双侧AN,其临床特征并不显著,UAN行为测听表现出的听力损失更加严重,多表现为极重度、完全听力损失;(2)在儿童听力学诊断当中遵循交叉验证原则极其重要,单侧ABR严重异常或纯音测听/PA显示单侧听力损失的儿童都应进行AN的核心评估组合,如CM、OAE与ABR测试。

Objective To report audiologic characteristics of unilateral auditory neuropathy(UAN) in children to provide a solid basis for improvement of its diagnosis.Methods Data from 12 children with UAN(4 males and 2 females) with an average age of 5.17 years(3-6 years) were reviewed,including play audiometry,auditory brainstem responses(ABRs),auditory steady-state response(ASSR),cochlear microphonics(CM) and otoacoustic emissions(OAEs),and compared with 38 cases(76 ears) of bilateral auditory neuropathy(BAN).Results Behavioral audiometric thresholds in UAN children were higher than in BAN children,indicating profound(33.33%) and total(66.67%) hearing loss.ABR thresholds in the UAN group were also significantly higher than those in the BAN group,with no significant difference in ASSR thresholds between the two groups.The rate of ABR detection in the UAN group was 50%,compared to 25% in the BAN group.Conclusion UAN is more insidious,with no distinct clinical manifestations as compared with BAN.Hearing loss in UAN is more severe,mostly at profound to total loss levels.It is extremely important to follow the principle of cross-validation in pediatric audiology diagnosis.Children with severe unilateral ABR abnormality or with pure tone audiometry/pediatric behavioral audiometry showing unilateral hearing loss should receive the core AN assessment with a combination CM,OAE and ABR tests.