中文摘要：

目的 分析新生儿听力筛查中检出的听神经病谱系障碍（auditory neuropathy spectrum disorder,ANSD）的流行病学特征,剖析新生儿ANSD的临床转归及其相关因素。方法 回顾性分析2003年12月-2013年11月在解放军总医院出生的5134例DPOAE及AABR均完善的新生儿听力筛查结果,统计分析新生儿人群中ANSD的患病率及其相关影响因素,并对不同因素与ANSD的相关性进行分析。结果 5134例新生儿中共有8例ANSD患儿,患病率为0.156%。其中,1）单双侧比例为3：1,男女比例为3：1;顺产新生儿中患病率为0.135%,剖宫产为0.171%,单双侧别间,不同性别间和不同分娩方式间患病率无显著性差异;2）DPOAE初筛通过的新生儿中ANSD的患病率为0.087%,未通过者为0.765%,二者间差异有统计学意义。AABR复筛未通过者的ANSD的患病率为10.127%,通过者为0.160%,二者间有非常显著性差异,DPOAE初筛未通过（r=-0.052,P〈0.01）和AABR复筛未通过（r=-0.316,P〈0.01）是新生儿ANSD的危险因素;3）ANSD在具有耳聋高危因素新生儿中患病率为0.255%,在早产儿中的患病率为0.429%,在NICU住院史的新生儿中患病率为0.207%,早产（r=0.015,P〉0.05）和NICU住院史（r=0.004,=0.764〉0.05）与ANSD无明显相关性。8例新生儿ANSD经诊断性听力学随访仅1例确诊为ANSD。结论 1）DPOAE初筛未通过、AABR未通过是新生儿ANSD的危险因素;2）本研究数据尚不能证明NICU住院史、早产及高胆红素血症是ANSD发病高危因素;3）新生儿ANSD具有暂时性和难以预测性,需密切随访。

英文摘要：

Objective To analyze the prevalence of auditory neuropathy spectrum disorder （ANSD） in Chinese new- borns hearing screening and the related factors, and to analyze the clinical outcomes of ANSD in newborns. Methods Be- tween December 2003 to November 2013, 5134 newborns in Chinese PLA general hospital were recruited. DPOAE was screened in the first step, and the DPOAE plus AABR was screened in the second step screening. The prevalence of ANSD and the related factors were analyzed, as well as the correlation analysis between various factors and ANSD. Results： Among the ascertained newborns, 8 had normal DPOAE and abnormal or deficient ABR results, which were consistent with ANSD. The prevalence of ANSD in newborns was 0.156%. 1） The ratio of unilateral and bilateral ANSD was 3：1, the ratio of male to female was 3：1, and the prevalence of ANSD among eutocia newborns and cesarean section newborns were 0.135% and 0.171%, respectively. There were no significant difference between different gender and different childbirth way （P 〉 0.05）, 2） The prevalence of ANSD among the DPOAE pass and refer cases in the first screening were 0.160% and 10.127%, respectively, the difference of which had statistical significance. The prevalence of ANSD among the AABR pass and refer cases in the second screening step were 0.087% and 0.765%, respectively, which had significant difference; DPOAE refer in the first step and AABR refer in the second step were risk factors for neonatal ANSD; 3） Only one of the 8 babies had risk factors, which had no significant correlation with ANSD. Only one of the eight newborn ANSD was diagnosed as ANSD after diagnostic audiological detection. Conclusion 1） DPOAE refer in the first step and AABR refer in the second step were risk factors for neonatal ANSD; 2） In this study, the data can not prove that the history of NICU hospitalization, premature birth and hyperbilimbinemia are the risk factors for neonatal ANSD; 3） Newborn ANSD has the temporary charac- ter, which is hard to