新生儿听力筛查方法影响听神经病谱系障碍的诊断时机。

Newborn hearing screening methodology impacts the timing of diagnosis for auditory neuropathy spectrum disorder.

机构信息

Department of Otolaryngology - Head and Neck Surgery, Naval Medical Center, San Diego, United States of America.

Children's Hospital Colorado, University of Colorado School of Medicine, United States of America.

出版信息

Am J Otolaryngol. 2023 Jul-Aug;44(4):103920. doi: 10.1016/j.amjoto.2023.103920. Epub 2023 May 5.

DOI:10.1016/j.amjoto.2023.103920

PMID:37207575

Abstract

INTRODUCTION

Auditory Neuropathy Spectrum Disorder (ANSD) accounts for 10 % to 15 % of pediatric hearing loss. In most cases, otoacoustic emissions (OAE) are present as the outer hair cell function is normal, and the auditory brainstem response (ABR) is abnormal. Newborn hearing screen (NBHS) is completed using OAE or ABR depending on the institution. Because OAEs are often present in ANSD, NBHS done solely with OAE can miss and delay diagnosis of patients with ANSD.

OBJECTIVES

To assess whether NBHS methodology impacts the age of diagnosis of ANSD.

METHODS

This is a retrospective study of patients, 0-18 years of age, diagnosed with ANSD at two tertiary pediatric hospitals from 1/01/2010 to 12/31/2018 after referral from NBHS performed in the community. Data recorded included patient demographics, method of NBHS, NICU stay, and age at ANSD diagnosis.

RESULTS

264 patients were diagnosed with ANSD. Of those, 123 (46.6 %) were female, and 141 (53.4 %) were male. Ninety-seven (36.8 %) were admitted to NICU and the mean stay was 6.98 weeks (STD = 10.7; CI = 4.8-9.1). The majority (244, 92.4 %) of patients had NBHS with ABR, and 20 (7.5 %) had NBHS with OAE. Patients screened with ABR were diagnosed with ANSD earlier than those who screened with OAE, with a mean age of 14.1 versus 27.3 weeks (p = 0.0397, CI = 15.2-39.3). Among those screened with ABR, median age at diagnosis was 4 months for NICU infants and 2.5 months for infants with no history of NICU stay over 5 days. In comparison, median diagnosis age was 8 months for non-NICU infants screened with OAEs.

CONCLUSION

Patients with ANSD who had NBHS with ABR were diagnosed earlier than those with OAE. Our data suggest that universal screening with ABR may facilitate earlier diagnosis of ANSD and earlier evaluation for aural rehabilitation, especially in high-risk cohorts such as NICU patients. Further research is needed into factors that contribute to earlier diagnosis among patients screened with ABR.

摘要

介绍

听觉神经病变谱系障碍（ANSD）占儿童听力损失的 10%至 15%。在大多数情况下，由于外毛细胞功能正常，耳声发射（OAE）存在，而听脑干反应（ABR）异常。新生儿听力筛查（NBHS）可根据机构使用 OAE 或 ABR 完成。由于 ANSD 中经常存在 OAE，因此仅使用 OAE 进行 NBHS 可能会错过并延迟 ANSD 患者的诊断。

目的

评估 NBHS 方法是否会影响 ANSD 的诊断年龄。

方法

这是一项对 2010 年 1 月 1 日至 2018 年 12 月 31 日期间因社区进行的 NBHS 转诊而在两家三级儿科医院诊断为 ANSD 的 0-18 岁患者的回顾性研究。记录的数据包括患者人口统计学资料、NBHS 方法、新生儿重症监护病房（NICU）住院时间和 ANSD 诊断年龄。

结果

共诊断出 264 例 ANSD 患者。其中，女性 123 例（46.6%），男性 141 例（53.4%）。97 例（36.8%）入住 NICU，平均住院时间为 6.98 周（标准差=10.7；置信区间=4.8-9.1）。大多数（244 例，92.4%）患者接受了 ABR 联合 NBHS，20 例（7.5%）接受了 OAE 联合 NBHS。接受 ABR 筛查的患者比接受 OAE 筛查的患者更早被诊断为 ANSD，平均年龄为 14.1 周和 27.3 周（p=0.0397，置信区间=15.2-39.3）。在接受 ABR 筛查的患者中，NICU 婴儿的中位诊断年龄为 4 个月，无 NICU 住院 5 天以上的婴儿为 2.5 个月。相比之下，接受 OAE 筛查的非 NICU 婴儿的中位诊断年龄为 8 个月。