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摘要: 目的 分析儿童期发病的听神经病(AN)患儿的听力学特征，总结其听力学特点和转归情况。方法 对58例(108耳)AN患儿进行复诊随访，随访1~10年。检测项目包括行为测听、鼓室图、畸变产物耳声发射(DPOAE)、听性脑干反应(ABR)及耳蜗微音电位(CM)、听觉稳态反应(ASSR)。结果 ① 所有频率的行为测听听阈均未见明显变化(P>0.05)；②首诊复诊中鼓室图多为A或As型；③首诊和复诊DPOAE除750、1000 Hz引出率较低外，其余频率引出率较高，复诊全频引出率降低；④首诊中有7耳引出ABR及CM，复诊中有3耳引出ABR及CM；⑤复诊与首诊相比，除500 Hz外其余频率ASSR阈值差异均有统计学意义(P < 0.01)；⑥4000 Hz处行为测听听阈较ASSR阈值高，其余频率两者间无明显相关性(P>0.05)。结论 儿童期发病的AN患儿听力有变差趋势，DPOAE未引出且ABR未引出或严重异常者，需进行CM测试，避免误诊。应持续监测AN患儿听觉状态与言语交流能力，家长应在平时的生活中注意患儿的行为能力变化，做到定期复诊。Abstract: Objective To analyse the audiological characteristics of patients of children with auditory neuropathy(AN) for gaining a better understanding of the audiological characteristics prognosis of patients with AN.Methods 58 patients(108 ears) of children with AN were enrolled, all of whom had received further consultation within 10 years after the first consultation. Behavioral audiometry test, tympanogram test, distortion product otoacoustic emission(DPOAE), auditory brainstem response(ABR), cochlear microphonics(CM), auditory steady-state response(ASSR) were performed on these patients.Results ① There were no significant changes in behavioral audiometry threshold between first and further consultation(P>0.05);②Tympanograms were mostly of type A or As; ③The patients had worse DPOAE results in the further consultation, while the elicitation rate of other frequencies were higher except for the lower elicitation rate of 750 Hz and 1000 Hz; ④There were 7 ears that had present ABR and CM in the first consultation, while three ears had present ABR and CM in the further consultation; ⑤Except for 500 Hz, other frequency thresholds of ASSR in the further consultation were statistically significant compared with those in the first consultation(P < 0.01);⑥The threshold of behavioral audiometry at 4000 Hz was higher than that of ASSR, and there was no obvious correlation between the other frequencies(P>0.05).Conclusion There is a tendency of hearing deterioration in patients of children with AN. Patients with no DPOAE elicitation and no ABR elicitation or serious abnormalities need CM test to avoid misdiagnosis. The hearing status and speech communication ability of patients should be continuously monitored. Parents should pay attention to the changes in the behavioral ability of the children in daily life and make regular subsequent visits.
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Key words:
- child /
- auditory neuropathy /
- audiological characteristics
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表 1 首诊复诊行为测听比较
dB HL，x±s 频率/Hz 首诊 复诊 P值 PTA500~4000 78.64±21.22 82.23±24.09 0.309 PTA250~1000 82.42±20.37 83.59±23.90 0.744 250 87.55±24.81 83.64±24.35 0.396 500 81.27±20.78 83.11±23.89 0.584 1000 78.45±23.51 84.04±26.84 0.156 2000 78.27±26.00 80.33±27.41 0.615 4000 76.55±26.09 81.44±27.43 0.258 8000 85.73±33.17 81.82±33.28 0.423 
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表 2 DPOAE首诊与复诊各频率引出率比较
% 时间 频率/Hz 750 1000 1500 2000 3000 4000 6000 8000 首诊 43.4 51.8 62.7 73.5 75.9 80.7 77.1 72.3 复诊 20.5 26.5 42.2 44.6 54.2 63.9 60.2 62.7
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表 3 首诊复诊各频率ASSR阈值比较
dB HL，x±s 频率/Hz 首诊 复诊 P值 500 83.79±12.87 87.43±14.43 0.104 1000 84.32±12.32 89.45±12.41 0.008 2000 79.13±13.03 84.75±12.52 0.009 4000 67.54±13.77 78.66±16.35 < 0.001
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表 4 行为测听与ASSR阈值比较
频率/Hz 行为测听/dB HL ASSR/dB nHL r P值 500 85.48±15.93 82.18±13.69 0.180 0.163 1000 85.56±18.31 86.69±14.44 0.176 0.170 2000 87.26±20.48 80.68±15.64 0.185 0.150 4000 85.16±24.58 72.55±15.56 0.367 0.003
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