儿童听神经病(附14例分析)

目的:探讨儿童听神经病的临床和听力学特征。方法:对14例(28耳)儿童听神经病的临床和听力 学检查进行分析。结果:7例伴不同音调耳鸣,以低调为主。言语分辨力差。1例于初诊后1年出现外周性神经 病症状。纯音听阈(PTT)检查示上升型,匙型,峰型,倒S型,W型和缓降型PTT图。上升型和匙型占53.57% (15/28耳)。PTT呈轻、中度升高。病程0.5～1.0年与1.5～2.0年者相比PTT升高程度差异无统计学意义 (t=0.24,P>0.05)。镫骨肌声反射(AR)引不出或AR阈值升高。言语识别率为0%～46%,平均(14.64± 15.42)%。听性脑干反应引不出或严重异常。畸变产物耳声发射引出率为100%,反应幅值正常,且不被对侧白 噪声所抑制。自发性耳声发射引出率为82.14%(23/28耳)。结论:儿童听神经病的听力学特征显著。系统听力 学检查对认识、诊断以及研究该病具有重要的临床意义。

Auditory neuropathy in children(analysis of 14 cases)

OBJECTIVE: To study the clinic findings and the audiological characteristics of auditory neuropathy in children. METHOD: Clinical findings and systemic audiometry were analyzed in 14 cases(28 ears) of auditory neuropathy in children. RESULT: In 14 cases the primary onset age was 7 approximately to 13 year-old,4 male and 10 female. 7 cases had differential pitch tinnitus,mainly low pitch with poor speech discrimination. 1 case manifested peripheral neuropathy symptoms at 1 year after diagnosis. Audiograms of pure tone threshold (PTT) showed various curves including ascending slope, spoon, peak, lain letter S, letter W, gentle slope. Both ascending slope and spoon were 53.57% (15/28 ears). PTT revealed a elevated range from mild to moderate degrees. There was no significant difference between the cours for 0.5 approximately 1.0 year and 1.5 approximately 2.0 years ( t =0.24, P >0.05). Either the acoustic reflex (AR) was absent or the AR threshold was elevated. Speech discrimination scores (SDS) were 0% approximately 46%, average (14.64+/-15.42) %. Auditory brainstem response (ABR) was absent or severely abnormal. Prevalence of distortion product otoacoustic emissions (DPOAE) were 100%, amplitude of DPOAE was normal, which could not be suppressed by contralateral white noise. Prevalence of spontaneous otoacoustic emissions (SOAE) were 82.14% (23/28 ears). CONCLUSION: Audiological characteristics in pediatric auditory neuropathy is obviously. Systemic audiometry has important clinical signification in recognition, diagnosis and research of auditory neuropathy.