未通过听力筛查婴幼儿的听力诊断及随访

目的 探讨未通过听力筛查的新生儿和婴幼儿的听力变化及其特点,为听力评估及临床诊断和随访工作提供参考,为早期诊断,早期干预提供较为科学的依据.方法 2003年9月至2009年11月由我省各地州市接受了出生后初筛或复筛2次或2次以上听力筛查双耳或单耳未通过,转诊到我听力中心进行听力诊断性检查的婴幼儿143例,最终以听性脑干反应(auditory brainstem response,ABR)、40 Hz听觉相关电位(40 Hz auditory event related potentials,40 Hz-AERP)、畸变产物耳声发射(distortion product otoacoustic emission,DPOAE)、声导抗结果进行评估和确认.对确诊为双耳重度和极重度听力损失的婴幼儿填写聋人调查表,进行跟踪随访,提出早期干预方案.结果 143例婴幼儿中确认听力损失者110例,占76.92%.其中单耳72例,占65.45%,双耳38例,占34.55%.听力损失中轻度31例,占28.18%;中度27例,占24.55%;重度27例,占24.55%;极重度25例,占22.72%.110例听力异常者有14例复查,占12.73%.复查者中双耳听力损失6例,单耳听力损失8例.14例双耳重度、极重度耳聋患儿,有10例配戴助听器,这些患儿正在接受语言康复训练.结论 耳声发射和听性脑干反应联合应用于新生儿及婴幼儿听力筛查,能提高其听力筛查的精确性和可靠性,对未通过听力筛查的婴幼儿,有必要在出生后3个月左右接受听性脑干反应和40 Hz听觉相关电位检查,以确保较为科学而可行的早期诊断、早期干预.本组听力异常患儿复查率低和失访率高仍是目前亟待解决的问题.     

听力筛查未通过婴幼儿听力评估及转归

目的探讨烟台地区听力筛查未通过婴幼儿的听力学特点及其转归。方法回顾性分析烟台地区2011年12月至2013年12月听力筛查未通过进行听力学诊断的264例婴幼儿病例资料,男150例,女114例,初诊年龄为3个月~3岁,平均为4.4月龄,所有未通过听筛婴幼儿均进行了听性脑干反应、听性稳态反应、畸变产物耳声发射及鼓室导抗图测试等检查。结果 264例听力筛查不通过婴幼儿中102例(38.64%)双耳听力正常,听力损失总耳数为257耳(48.67%257/528),其中单耳听力损失67例(25.38%,67/264),双耳听力损失95例(35.98%,95/264);感音神经性听力损失137耳(25.95%,137/528)传导性听力损失或混合性听力损失120耳(22.73%,120/528);264例婴幼儿中ABR波V反应阈正常271耳(51.33%,271/528);轻度听力损失120耳(22.73%,120/528);中度听力损失75耳(14.21%,75/528);重度听力损失19耳(3.60%,19/528);极重度听力损失43耳(8.15%,43/528)。诊断小耳畸形4例(4耳,1.51%,4/528),诊断大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)患儿6例(12耳,2.27%,12/528),其中5例发现短潜伏期负反应波。结论本组听力筛查转诊婴幼儿中听力损失检出率为61.36%,6个月以下轻中度听力损失的婴幼儿,由于中耳因素及听觉系统发育不断完善,随着月龄增加听力损失有好转趋势;因此对婴幼儿的听力评估需要结合多种检测方法并进行必要的跟踪随访。     

广东省珠江三角洲地区听力筛查阳性患儿追踪观察研究

目的探索广东省新生儿听力筛查的临床方法，了解广东省新生儿听力损失的发病状况，以便制定适合广东省新生儿听力筛查的实施方案，提出听力损失的婴幼儿的早期干预措施。方法应用畸变产物诱发耳声发射技术(简称DPOAE)对27322例出生后2～4天的新生儿进行听力筛查(初筛)，未通过者于生后6周复筛，对复筛未通过者，于生后3个月左右采用多频稳态听觉诱发反应(ASSR)测听技术进行听力学诊断。对有听损失高危因素者于6个月～3岁之间每半年监测一次听力变化。结果自2001年7月至2003年11月，四家医院可供听力筛查的新生儿数为33706例，实际接受初筛查27322例，新生儿初筛率为81．05％；初筛通过23332例(85．45％)，需复筛的共3990例，实际复筛人数3391例，通过3003例，门诊复筛率为84％。复筛未通过例数388例，复筛后阳性率为1．4％。确诊新生儿先天性听力损失28例。由本组资料显示新生儿先天性听力损失在筛查儿中的发病率为1．02‰(单耳和双耳)。单耳听力损失在筛查儿中的发病率为0．29‰，双耳听力损失在筛查儿中的发病率为0．73‰。初筛的阳性率为14．55％。假阳性率为14．51％，两步筛查后的阳性率为1．40％，假阳性率为1．33％。本组未发现假阴性病例。结论通过应用畸变产物诱发耳声发射技术(DPOAE)，ASSR对新生儿进行听力筛查以及听力学诊断，可早期发现有听力损失的新生儿，及早提出医学意见，并早期进行干预治疗。     

听力筛查未通过婴幼儿的听力评估及随访北大核心CSCD

目的分析广东省部分听力筛查未通过婴幼儿的听力评估特点和随访情况。方法回顾性分析广东省2014年12月至2016年11月听力筛查未通过转诊至广东省妇幼保健院的1 052例婴幼儿的临床资料,所有婴幼儿均进行了听性脑干反应(ABR)、听性稳态反应(ASSR)及鼓室导抗图测试,每3个月随访一次,随访至3岁;分析不同程度、不同性质听力损失患儿的听力特点及随访结果。结果 1 052例听力复筛未通过婴幼儿中双耳听力正常411例(39.07%),听力损失患儿641例(60.93%,641/1 052)1 043耳(49.57%,1 043/2 104),其中单耳听力损失239例(22.72%),双耳听力损失402例(38.22%);轻度听力损失315例(29.94%),中度听力损失151例(14.35%),重度听力损失67例(6.37%),极重度听力损失108例(10.27%);传导性或混合性听力损失506耳(20.05%),感音神经性听力损失537耳(25.53%)。分泌性中耳炎242例392耳(18.64%,392/2 104),先天性小耳畸形76例84耳(3.99%,84/2 104),大前庭水管综合征16例21耳(1.0%,21/2 104),听神经病42例82耳(3.90%,82/2 104)。随访至3岁,首次诊断轻度听力损失者听力好转180例,中度听力损失者好转76例,重度、极重度听力损失者好转37例。结论本组听力筛查未通过转诊婴幼儿中听力损失检出率为60.93%;初诊为轻中度听力损失婴幼儿随着月龄增加和发育完善,听力损失有明显的好转趋势。     

6072例新生儿听力筛查结果分析

目的分析6072例新生儿听力筛查结果,了解本组新生儿听力损失的发病情况。方法应用畸变产物耳声发射(DPOAE)对福建医科大学附属第一医院2017年1月~2019年9月出生的6072例活产新生儿进行听力筛查,初筛于出生后48 h至出院前完成,初筛未通过者于出生后42天复筛,复筛未通过者于出生后3个月行DPOAE、声导抗、听性脑干反应(ABR)、听性稳态反应(ASSR)等检查,必要时行影像学检查。分析初筛率、复筛率、听力损失检出率,并比较新生儿不同性别、耳别的听力筛查通过率。结果活产新生儿6072例,实际初筛5843例(包括新生儿重症监护病房),初筛率96.23%(5843/6072);初筛通过5284例(90.43%,5284/5843)左耳通过率92.71%(5417/5843)低于右耳通过率(93.80%,5481/5843)(χ^2=5.574,P<0.05);男婴通过率89.38%(2853/3192)低于女婴(91.70%,2431/2651)(χ^2=9.022,P<0.01)。初筛未通过559例,其中474例接受复筛(84.79%,474/559);复筛通过385例(81.22%,385/474);复筛未通过89例,其中77例进行听力学诊断(86.52%,77/89);共确诊听力损失患儿14例,听力损失检出率2.40‰(14/5843),其中8例轻度听力损失(2例双耳传导性听力损失,1例双耳感音神经性听力损失,1例单耳传导性听力损失,4例单耳感音神经性听力损失),2例双耳中度感音神经性听力损失,1例双耳重度感音神经性听力损失,3例单耳极重度感音神经性听力损失。结论本组新生儿听力损失检出率为2.40‰,女婴听力筛查通过率高于男婴,右耳通过率高于左耳。     

DPOAE＋AABR联合筛查在婴儿听力筛查中的应用

目的：探讨畸变产物耳声发射（DPOAE）和自动听性脑干反应（AABR）联合筛查在听力筛查中的应用价值。方法2014年1月～2014年12月对6060例婴儿运用DPOAE＋AABR进行联合听力筛查,未通过者两周后进行复筛,复筛未通过者应用ABR和声导抗进行听力诊断。结果6060例接受DPOAE＋AABR 联合听力筛查,5427例通过筛查,633例未通过;603例接受复筛,复筛未通过97例;经听力学诊断,听力异常58例,其中传导性听力损失7例,感音神经性听力损失51例,听力损失检出率为9．57‰（58／6060）。DPOAE＋AABR均未通过的44例中,7例听力正常,19例单耳听力异常,18例为双耳听力异常;DPOAE通过AABR未通过的21例中,17例听力异常,单耳11例,双耳6例,其中1例被诊断为听神经病;DPOAE未通过AABR通过的16例中,12例听力正常,4例单耳听力异常。结论 DPOAE＋AABR联合筛查可提高听力筛查的准确性、可靠性,有利于听力损失患儿的早期发现、早期诊断、早期干预。     

新生儿听力复筛未通过的婴幼儿听力随访及干预情况

目的 探讨新生儿听力复筛未通过婴幼儿的听力随访及干预情况.方法 对200例(400耳)新生儿听力复筛未通过的婴幼儿使用耳声发射(OAE)、声导抗、听性脑干反应(ABR)测试进行听力学初诊及复诊,观察患儿的听力变化,了解其干预情况.结果 200例(400耳)婴幼儿,初诊听力正常61例(30.50%,61/200),139例(69.50%,139/200)有单耳或双耳听力损失,其中,轻度87例(62.59%,87/139),中度22例(15.83%,22/139),重度9例(6.47%,9/139),极重度21例(15.11%,21/139).139例听力损失患儿接受复诊91例(65.47%,91/139),失访48例(34.53%,48/139),复诊听力正常57例,听力损失34例,其中,轻度5例,中度4例,重度6例,极重度19例,极重度听力损失者中有6例合并脑部发育异常(脑瘫3例、脑损伤综合症3例).34例听力损失患儿中接受干预24例,其中轻度2例(40%,2/5)、中度2例(50%,2/4)、重度3例(50.0%.3/6)、极重度17例(89.47%,17/19).合并有脑部发育异常的患儿同时进行综合性康复.结论 本组新生儿听力复筛未通过婴幼儿中,中度以上听力障碍的息儿随访及干预接受率较高,中度以下的听力障碍患儿随访及干预接受率较低;重度以上感音神经性听力障碍息儿尤其是伴有听力障碍高危因素的婴幼儿的听力无明显变化.     

先天性听力损失的确诊和随访及病因分析

目的了解先天性听力损失在母婴同室(well-baby nursery ,WBN)与新生儿重症监护室(newborn intensive care unit,NICU)中的发病率以及可能导致听力损失的高危因素,探讨较为科学而可行的早期诊断、跟踪随访及早期干预模式.方法采用瞬态诱发耳声发射(transient evoked otoacoustic emission,TEOAE)进行新生儿听力普遍筛查(2阶段筛查);对复筛未"通过"者,于生后3个月采用听性脑干反应(auditory brainstem response,ABR)和40 Hz听觉相关电位(40 Hz auditory event related potentials, 40 Hz-AERP)技术予以诊断.ABR异常婴幼儿及听力损失高危儿于生后6个月～3岁前进行跟踪随访和定期的听力学评估,对诊断为永久性听力损失的婴幼儿于生后6个月提出早期干预方案.结果新生儿及婴幼儿先天性听力损失患儿占同期接受新生儿普遍听力筛查总数的5.73‰(91/15 885);WBN中听力损失患儿占同期WBN中接受新生儿普遍听力筛查总数的3.67‰(51/13 887),NICU中听力损失患儿占同期NICU中接受新生儿普遍听力筛查总数的20.02‰(40/1998).结论对于NICU新生儿,无论其是否"通过"新生儿听力普遍筛查,都有必要在生后3个月左右接受ABR检查;3个月后的随访人群包括ABR异常婴幼儿和听力损失高危儿;追踪随访与早期干预应体现个体化.     

甘肃省河西走廊19323例新生儿听力筛查结果分析

目的了解甘肃省河西走廊先天性听力损失发病情况,并探寻适合偏远、经济欠发达、多民族聚集地区新生儿听力普遍筛查的模式。方法采用畸变产物耳声发射(DPOAE)对2009年7月～2010年9月出生于甘肃省河西走廊地区的19 323例新生儿及婴儿在其出生后两月龄内实施普遍听力筛查,初筛未通过者间隔一月进行DPOAE复筛,复筛未通过者在3月龄(第一次诊断)和6月龄时(第一次诊断听力异常者,进行第二次诊断)进行DPOAE、ABR、声导抗等听力学检查及跟踪随访。结果接受听力筛查的19 323例新生儿及婴儿中,初筛通过16 843例(87.17%),未通过2 480例(12.83%)。18 417例正常儿中初筛通过16 078例(87.30%),未通过2 339例(12.70%);906例高危儿中初筛通过765例(84.44%),未通过141例(15.56%)。初筛未通过需进行复筛2 480例,实际复筛916例,复筛率36.94%,复筛通过853例(93.12%),未通过63例(6.88%)。接受复筛的875例正常儿中复筛通过824例(94.17%),未通过51例(5.83%)。接受复筛的41例高危儿中复筛通过29例(70.73%),未通过12例(29.27%)。初筛复筛未通过由当地筛查机构转诊接受进一步听力学诊断检查的受试儿应有63例,实际进行诊断的仅19例,11例最终确诊为听力损失的患儿中8例为传导性听力损失(6例单耳,1例双耳,1例右耳先天性外耳道闭锁),3例为感音神经性听力损失(1例双耳、1例单耳极重度听力损失,1例前庭水管扩大)。结论应用畸变产物耳声发射对农村及城乡结合地区广泛开展新生儿听力筛查是可行的,但目标人群复筛率、转诊率低,漏筛率、失访率高仍是亟待解决的问题。     

新生儿听力筛查11894例结果分析

目的 了解新生儿听力筛查的通过率、听力损失的发病率,分析可能导致新生儿听力损失的原因。方法 瞬态诱发性耳声发射(TEOAE)按两阶段方案进行听力筛查, 11894例新生儿中未通过者3月龄后行畸变产物耳声发射(DPOAE)、1000Hz探测音鼓室导抗、听性脑干反应(ABR)测试,结果用统计学软件SPSS15.0分析。结果 初筛通过率为82.64%,复筛通过率为78.71%。产科病区新生儿初筛通过率为84.63%,新生儿重症监护病房(NICU)新生儿初筛通过率为78.00%,经比较两者差异有统计学意义(ANOVA,P<0.05)。确诊有听力损失患者68例(106耳),发病率约为0.57%,其中传导性听力损失31例,感音神经性听力损失16例,混合性听力下降21例。听力筛查双耳未通过组和单耳未通过组听力损失的患病率分别为12.92%(38/294)、5.00%(30/600),中度及中度以上听力损失的比例分别为3.33%(1/30)、18.42%(14/76),经比较差异有统计学意义(ANOVA,P<0.05)。引起听力损失的可能原因有：黄疸(24.56%,14/57),感染(24.56%,14/57),窒息(19.30%,11/57),低体质量(17.54%,10/57),其他因素(14.04%,8/57)。结论 听力筛查是早期发现新生儿听力损失的基础性工程;双耳听力筛查未通过比单耳听力筛查未通过患儿存在听力损失的可能性更大,听力损失的程度也可能更重;黄疸、感染、窒息和新生儿低体质量是导致听力损失的常见原因。     

未通过听力筛查的婴幼儿听力追踪分析

目的探讨未通过听力筛查的婴幼儿听力变化及其特点。方法2001年1月至2003年10月期间在北京市海淀区妇幼保健院接受瞬态诱发耳声发射（transient evoked otoacoustic emission，TEOAE）进行听力筛查的新生儿14785名，未通过者106例转诊至北京同仁医院北京市耳鼻咽喉科研究所进行听力诊断性检查和跟踪随访1年，最终以听性脑干反应、畸变产物耳声发射（distortion product otoacoustic emission，DPOAE）、声导抗和行为测听的结果进行综合评估和确认。结果106例婴幼儿最后确认双耳听力正常65例，占61．3％，听力损失39例，占36．8％，2例未接受最后听力确认，患儿家长接受了电话随访，占1．9％。39例中传导性听力损失15例（14．2％），感音性听力损失24例（22．6％）；39例中轻度13例（12．3％），中度14例（13．2％）、重度6例（5．7％），极重度6例（5．7％）。本组资料提示，各种听力损失（包括单耳和双耳）的患病率为0．264％（39／14785）。从初诊到最后确认，重度和极重度以上听力损失者的听力基本无改变，而轻-中度听力损失者的听力有改善和正常化的趋势。结论重度以上听力损失者，3个月内的早期诊断和6个月内的早期干预是可行的；轻-中度听力损失者，应进行至少2次以上听力检测和至少6个月以上的跟踪随访，综合分析各项检测结果后才可得出正确诊断。     

听力复筛不通过的转诊婴儿听力学评估

目的评估两次听力筛查未通过转诊婴儿的临床听力学。方法对210耳两次听力筛查未通过转诊的婴儿进行听性脑干反应（ABR）、听性稳态反应（ASSR）、40 Hz听觉相关电位(40 Hz AERP)、畸变产物耳声发射（DPOAE）、声导抗（AI）等检查,并分析其结果。结果两次未通过听力筛查的210耳中，有听力损失143耳，听力损失的检出率为68.10％(143/210)。其中传导性听力损失62耳，占听力损失耳43.36%(62/143)；感音神经性听力损失81耳，占听力损失耳56.64%(81/143)。确诊大前庭导水管扩大综合征（LVAS）16耳，发病率为7.62%（16/210）。诊断为类听神经病6耳，发病率为2.85%（6/210）。结论两次听力筛查未通过的听力损失检出率较高，对于听力高危因素婴幼儿的听力动态随访有待更加关注，应结合婴儿生理特点更加精准的进行听力学诊断,及其本身的生理发育特点全面评估。     

听力筛查未通过婴幼儿的听力学特点及原因

目的探讨听力筛查未通过婴幼儿的听力学特点及原因。方法应用听性脑干反应（auditory brainstem response,ABR）、40Hz听觉相关电位（40 Hz auditory event related potential,40 Hz AERP）、畸变产物耳声发射（distortion product otoacoustic emissions,DPOAE）、鼓室声导抗及声反射方法,对126例听力筛查未通过的婴幼儿进行听力学诊断性检查,确认听力损失的程度及性质,并对听力损失婴幼儿的原因进行分析。结果126例（252耳）婴幼儿中,感音神经性聋61例（48.41%）,传导性聋48例（38.09%）,ABR反应阈值正常范围17例（13.50%）。此组患儿既往病史有母孕期感染病史21例、先兆流产9例、高龄6例、延期妊娠7例、伴全身系统疾病10例;新生儿期黄疸13例、缺氧窒息18例、早产儿及低体重8例、伴其他新生儿疾病8例、有听力损失家族史5例,颅面畸形3例,中枢神经系统疾病6例,第2胎9例。结论听力筛查未通过的婴幼儿听力评估后有不同的表现,本组感音神经性聋较传导性聋所占比例高,多数听力损失患儿伴有高危因素。     

Further examination of infants referred from newborn hearing screening

OBJECTIVES: Universal newborn hearing screening (UNHS) is considered beneficial and is accepted worldwide. However, some problems remain, and administrative systematization has yet to be established in many countries. This study assessed the hearing screening of referred newborn infants and discusses the problems that remain. MATERIALS AND METHODS: Over the two years from July 2001 to June 2003, 98 ears of 49 infants were judged as a "referral" from a newborn hearing screening program, and were subsequently referred to our hospital for further examination using conventional ABR and other audiological tests. The methodology used for hearing screening varied between practitioners and hospitals that utilized both different recording apparatus for AABR and/or automated DPOAEs and independent protocols. RESULTS: Conventional ABR identified 21 infants with bilateral normal hearing, 12 with unilateral hearing loss, and 16 with bilateral hearing loss, and a total correspondence rate of 40.8% (20 out of 49 infants). In a comparative analysis, 26 ears out of 98 (26.5%) were determined as false-positive, seven out of 98 as false-negative (7.1%), and there was a total correspondence rate of 66.3% (65 out of 98 ears). Five of the seven false-negative cases who were referred with unilateral hearing loss exhibited moderate to profound bilateral hearing loss (moderate; one infant, severe to profound; four infants). Of the 16 infants with bilateral hearing loss, nine with more than moderate loss had hearing aids fitted at our hospital or related educational institution before most were six months old. CONCLUSIONS: Our results suggest the accuracy of newborn hearing screening remains an issue, but may be improved by an experienced examiner and better protocols including a two-stage process and altered timing of screening. Other ongoing health care programs need to monitor for signs of hearing loss even in the "passed" infants because of possible false-negatives and delayed-onset hearing loss. Improvement is needed in both the intervention systems and diagnostic follow-up of hospitals. Early public support is also required for infants with either severe to profound or moderate hearing loss. From the viewpoint of test conditions and puerperal parental psychological problems, it is considered that the timing of screening needs further discussion. Here it is suggested that screening should be performed within the first three months of infant's life but not be limited to before hospital discharge, and incorporated into the routine health care program for one-month-old infants without reducing efficiency.     

新生儿听视同步筛查的临床模式

目的探索新生儿听力、眼病同步筛查的可行性和临床模式；了解新生儿听力下降及眼病的发病情况。方法在母婴同室应用瞬态诱发耳声发射，新生儿重症监护病房采用两阶段筛查技术进行新生儿听力筛查；同步进行新生儿眼病筛查。听力复筛未通过者，生后3个月进入诊断程序并进行跟踪随访和定期的听力学评估；眼病筛查疑似病例及时转诊到专业眼科进一步确诊。结果自2002年10月1日至2005年4月30日在济南市妇幼保健院共出生16800例新生儿，对其中的15398例（91．7％）新生儿进行了听力、眼病同步筛查。新生儿听力损失在同步筛查儿中的检出率：双耳0．312％（48／15398），单耳0．227％（35／15398）；有4例单耳或双耳先天性感音神经性聋伴发眼病或眼部阳性体征：1例极重度感音神经性聋（双）、听神经病伴双眼先天性完全性白内障，1例轻度感音神经性聋（双）伴发左眼永存瞳孔膜，1例轻度感音神经性聋（双）伴双眼底静脉扩张，1例右耳轻度感音神经性聋伴双眼永存玻璃体动脉。在15398例统计取样的新生儿中，共检出先天性眼病或眼部阳性体征13种2211例。结论新生儿期听力损失和眼病或眼部阳性体征在临床中并不少见。新生儿听视同步筛查在听力下降以及眼病监测和防治中是可行的，亦是有效的。     

Neonatal hearing screening with transient evoked otoacoustic emissions in Western Saudi Arabia

OBJECTIVE: To study the incidence of congenital hearing impairment in the Saudi population and to evaluate the need of establishing a Saudi universal neonatal hearing screening program based on transient evoked otoacoustic emission. METHODS: A total of 11986 well non-high-risk neonates were screened by transient evoked otoacoustic emission over period of 8 years from September 1996 to February 2004. The universal hearing screening was consequently done in a daily base before discharge from nursery. Those who failed the initial screening were followed up diagnostically until hearing loss was confirmed or excluded. RESULTS: From the total number of 11,986 neonates (41.4% male and 58.6% females) examined in this study 10943 (91.3%) neonates passed the first screening step while 1043(8.7%) neonates failed. From the 1043 neonates examined in the second screening step in the 5th day of life, 300 (29%) neonates failed. At the age of 5 months, all the 300 infants that failed the second screening step underwent a comprehensive audiological assessment to confirm the existence of hearing loss. The 278 infants that passed the assessment were considered as normal; while 22 failed and were confirmed to have congenital hearing loss. Of these 22 infants, 2 had unilateral sensorineural hearing loss, and the remaining 20 had bilateral sensorineural hearing loss. The incidence of sensorineural hearing loss was estimated to be 0.18% while the incidence of bilateral sensorineural hearing loss was 0.17%. No significant difference between males and females was found. The average age at confirmation of congenital hearing loss was 5.5 months. CONCLUSION: The incidence of congenital hearing loss in the western region of Saudi Arabia is relatively high compared with international figures. Hearing screening for all neonates using transient evoked otoacoustic emission should be part of the standard medical care in Saudi Arabia.     

Auditory neuropathy/auditory dys-synchrony detected by universal newborn hearing screening

The implementation of neonatal hearing screening has enabled early detection and intervention in hearing loss. The use of otoacoustic emissions (OAE) and auditory brainstem response testing in universal screening has led to the recognition of this recently described disorder called auditory neuropathy/auditory dys-synchrony (AN/AD). This diagnosis indicates that the infant has significant hearing loss despite having normal outer hair cells in the cochlea. We reviewed the characteristics and natural history of nine infants detected to have AN/AD from universal newborn hearing screening in a national pediatric hospital. Fifty-two cases of hearing loss were detected from 14,807 consecutively screened cases. Of the 52 cases, 9 had electrophysiological test results consistent with AN/AD. They include both premature infants who had major neonatal complications and term infants with no perinatal complications. Six cases had bilateral and three cases had unilateral findings. We suggest that AN/AD can occur in low-risk infants and hence screening of high-risk cases alone is insufficient. Our findings are discussed with reference to the current literature.     

The New York State universal newborn hearing screening demonstration project: ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention

OBJECTIVE: To determine the ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention through a multi-center, state-wide universal newborn hearing screening project. DESIGN: Universal newborn hearing screening was conducted at eight hospitals across New York State. All infants who did not bilaterally pass hearing screening before discharge were recalled for outpatient retesting. Inpatient screening and outpatient rescreening were done with transient evoked otoacoustic emissions and/or auditory brain stem response testing. Diagnostic testing was performed with age appropriate tests, auditory brain stem response and/or visual reinforcement audiometry. Infants diagnosed with permanent hearing loss were considered for hearing aids and early intervention. Ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention were investigated regarding nursery type, risk status, unilateral versus bilateral hearing loss, loss type, loss severity, and state regions. RESULTS: The prevalence of infants diagnosed with permanent hearing loss was 2.0/1000 (85 of 43,311). Of the 85 infants with hearing loss, 61% were from neonatal intensive care units (NICUs) and 67% were at risk for hearing loss. Of the 36 infants fitted with hearing aids, 58% were from NICUs and 78% were at risk for hearing loss. The median age at identification and enrollment in early intervention was 3 mo. Median age at hearing aid fitting was 7.5 mo. Median ages at identification were less for infants from the well-baby nurseries (WBNs) than for the NICU infants and for infants with severe/profound than for infants with mild/moderate hearing loss, but were similar for not-at-risk and at-risk infants. Median ages at hearing aid fitting were less for well babies than for NICU infants, for not-at-risk infants than for at-risk infants, and for infants with severe/ profound hearing loss than for infants with mild/ moderate hearing loss. However, median ages at early intervention enrollment were similar for nursery types, risk status, and severity of hearing loss. CONCLUSIONS: Early ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention can be achieved for infants from NICUs and WBNs and for infants at risk and not at risk for hearing loss in a large multi-center universal newborn hearing screening program.     

先天性听力损失的确诊和随访及病因分析

目的了解先天性听力损失在母婴同室(well-baby nursery，WBN)与新生儿重症监护室(newborn intensive care unit，NICU)中的发病率以及可能导致听力损失的高危因素，探讨较为科学而可行的早期诊断、跟踪随访及早期干预模式。方法采用瞬态诱发耳声发射(transient evoked otoaconstic emission，TEOAE)进行新生儿听力普遍筛查(2阶段筛查)；对复筛未“通过”者，于生后3个月采用听性脑干反应(auditory brainstem response，ABR)和40Hz听觉相关电位(40Hz auditory event related potentials，40Hz．AERP)技术予以诊断。ABR异常婴幼儿及听力损失高危儿于生后6个月～3岁前进行跟踪随访和定期的听力学评估，对诊断为永久性听力损失的婴幼儿于生后6个月提出早期干预方案。结果新生儿及婴幼儿先天性听力损失患儿占同期接受新生儿普遍听力筛查总数的5．73％0(91／15 885)；WBN中听力损失患儿占同期WBN中接受新生儿普遍听力筛查总数的3．67‰(51／13 887)，NICU中听力损失患儿占同期NICU中接受新生儿普遍听力筛查总数的20．02‰(40／1998)。结论对于NICU新生儿，无论其是否“通过”新生儿听力普遍筛查，都有必要在生后3个月左右接受ABR检查；3个月后的随访人群包括ABR异常婴幼儿和听力损失高危儿；追踪随访与早期干预应体现个体化。     

大连地区217641例新生儿听力筛查结果回顾性分析

目的了解大连地区新生儿听力障碍的发病状况,发现听力筛查过程中存在的主要问题。方法对近5年大连地区出生的新生儿,在出生后3～5天内用畸变产物耳声发射(Disto rtio n pro duct o toacoustic emission,DPOAE)筛查仪进行初筛,初筛不通过者在42天进行复筛,复筛不通过者3个月内进行听觉脑干反应(auditory brainstem response,ABR)、多频听觉稳态反应(auditory steady state response,ASSR)、40Hz相关电位(40Hz-auditory evoked related potentials,40Hz–AERP)、声导抗测听等检查,对患儿进行综合听觉评估。结果 5年时间内大连地区应该筛查新生儿总数233856人,实际筛查217641人,初筛率为93.1%;初筛通过率为90.6%(197254/217641),复筛率为74.3%(漏检率为25.7%,5236/20387),复筛通过率90.5%(12194/15151),复筛不通过2957人;新生儿听力障碍诊治中心接诊2069人,就诊率为70%(2069/2957);确诊为听力障碍373例(607耳),听力障碍发病率为1.71‰;双耳听力障碍234例(轻、中、重、极重度听力障碍发病率分别为0.23‰、0.36‰、0.19‰、0.29‰),单耳听力障碍139例;有45例(45/106)重度-极重度听力障碍患儿6个月内没有确诊,其中9例为初筛不通过而没有进行复筛,36例初筛、复筛均不通过却没有及时就诊。结论大连地区新生儿听力障碍发病率与既往报道类似;完善听力障碍高危儿童的召回制度,提高复筛率和就诊率是减少听力障碍儿童漏诊的关键。