镰状细胞病患儿的感音神经性听力损失

Sensorineural hearing loss in children with sickle cell disease.

作者信息

Farrell Annie N, Landry April M, Yee Marianne E, Leu Roberta M, Goudy Steven L

机构信息

Emory University School of Medicine, 1648 Pierce Drive NE, Atlanta, GA, 30307, USA.

Emory University School of Medicine, 1648 Pierce Drive NE, Atlanta, GA, 30307, USA; Children's Healthcare of Atlanta, Department of Otolaryngology, Division of Pediatric Otolaryngology, 2015 Uppergate Drive, Atlanta, GA, 30322, USA.

出版信息

Int J Pediatr Otorhinolaryngol. 2019 Mar;118:110-114. doi: 10.1016/j.ijporl.2018.12.002. Epub 2018 Dec 5.

DOI:10.1016/j.ijporl.2018.12.002

PMID:30599285

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6443092/

Abstract

INTRODUCTION

Sensorineural hearing loss (SNHL) has been reported to occur at increased frequency in the pediatric sickle cell disease (SCD) population, likely secondary to ototoxic medication regimens and repeat sickling events that lead to end organ damage. Risk and protective factors of SNHL in this population are not fully characterized. The objective of this study was to describe audiology results in children with SCD and the prevalence and sequelae of SNHL.

METHODS

A comprehensive clinical database of 2600 pediatric SCD patients treated at 1 institution from 2010-16 was retrospectively reviewed to identify all patients who were referred for audiologic testing. Audiologic test results, patient characteristics, and SCD treatments were reviewed.

RESULTS

181 SCD children (97 male, 153 HbSS) underwent audiologic testing, with 276 total audiology encounters, ranging 1-9 per patient. Mean age at first audiogram was 8.9 ± 5.2 years. 29.8% had prior cerebrovascular infarct and an additional 25.4% had prior abnormal transcranial Doppler screens documented at time of first audiogram. Overall, 13.3% had documented hearing loss, with 6.6% SNHL. Mean pure tone average (PTA) among patients with SNHL ranged from mild to profound hearing loss (Right: 43.3 ± 28.9, Left: 40.8 ± 29.7), sloping to more severe hearing loss at higher frequencies.

CONCLUSIONS

Hearing loss was identified in a significant subset of children with SCD and the hearing loss ranged from normal to profound. Though the overall prevalence of SNHL in SCD patients was low, baseline audiology screening should be considered.

摘要

引言

据报道，小儿镰状细胞病（SCD）患者中感音神经性听力损失（SNHL）的发生率增加，这可能继发于耳毒性药物治疗方案以及导致终末器官损伤的反复镰变事件。该人群中SNHL的风险和保护因素尚未完全明确。本研究的目的是描述SCD患儿的听力学结果以及SNHL的患病率和后遗症。

方法

回顾性分析了2010年至2016年在1家机构接受治疗的2600例小儿SCD患者的综合临床数据库，以确定所有接受听力学检查的患者。对听力学检查结果、患者特征和SCD治疗情况进行了回顾。

结果

181例SCD患儿（97例男性，153例HbSS）接受了听力学检查，共进行了276次听力学检查，每位患者1至9次不等。首次听力图检查时的平均年龄为8.9±5.2岁。29.8%的患儿曾有过脑血管梗死，另有25.4%的患儿在首次听力图检查时记录有既往异常经颅多普勒检查结果。总体而言，13.3%的患儿有听力损失记录，其中6.6%为SNHL。SNHL患者的平均纯音平均听阈（PTA）范围从轻度到重度听力损失（右侧：43.3±28.9，左侧：40.8±29.7），在高频时听力损失更严重。