2368例新生儿听力筛查的临床分析

目的通过新生儿听力筛查,了解新生儿听力障碍的发病状况,以利早期诊断和干预。方法应用美国B io—logic公司筛查型耳声发射仪对2368例新生儿进行听力筛查,经过初筛和复筛,对复筛未通过者建议进行脑干听觉诱发电位（ABR）检查。结果初筛异常率为24.24%,复筛异常率为4.37%,确诊听力障碍者3例,新生儿听力障碍发病率1.27‰。结论新生儿听力筛查可以及早发现新生儿听力障碍,对早期治疗及干预听力残疾有较大意义。     

吕梁地区新生儿听力筛查现状的研究

目的分析吕梁市开展新生儿听力筛查的现状,为制定合适的听力筛查、随访和干预策略提供依据。方法应用瞬态诱发耳声发射对吕梁市2011年3月至2011年6月出生的1147例中在我院生产的100例正常新生儿进行听力筛查,初筛未通过者分别于42天复查,复筛未通过使用听性脑干反应,40Hz听觉相关电（40Hz-aerp）和声导抗进行听力损失诊断。结果可接受听力筛查新生儿1147例,实际筛查100例,初筛率8.71%（100/1147）,初筛通过率92%（92/100）;应复筛8例,实际接受复筛7例,复率为87.5%（7/8）;复筛通过4例（57.1%,4/7）,未通过3例（1%,3/100）,应转诊1例（1%,1/100）,实际转诊1例;8例复筛新生儿中1例未按时接受检查,实际接受诊断性检查3例,其中双耳听力正常2例,确诊先天性听力损失1例。结论在吕梁市实施新生儿普遍听力筛查是可行的,而且是必要的,能为早期发现先天性听力障碍患儿并给予早期干预提供科学依据。     

浙江嘉善地区新生儿听力筛查结果分析

目的了解嘉善县新生儿听力筛查开展现状,探讨进一步提高和改进新生儿听力筛查工作方法。方法根据浙江省听力筛查实施方案和管理流程,采用瞬态声诱发耳声发射（TEOAE）进行新生儿听力初筛,各助产单位专职筛查人员在新生儿出生后48-72h进行。初筛未通过者,新生儿满月时进行复筛。复筛仍未通过者,转诊到指定听力诊断中心进行诊断型听性脑干诱发电位（ABR）检测,要求初次诊断性听力评估于出生后3个月内完戍。听力损失分度参考我国耳聋分级标准。结果2009-2013年嘉善县各助产医院活产数总计24639例,初次OAE筛查率为98.68％,初筛未通过新生儿数为2671例,初筛未通过率10.99％,复查率87.27％。复查未通过儿童数533例,转诊率87.24％。确诊听力障碍儿童数为46例,听力障碍发生率1.89‰。结论嘉善县新生儿听力筛查工作已达到较高水平,进一步提高需多方协作。     

无锡地区8757例新生儿听力筛查临床分析

目的通过新生儿听力筛查,早期发现听力障碍患儿,做到早发现、早诊断、早干预。方法采用美国产Bio-logic耳声发射仪进行筛查,可疑阳性病例采用丹麦MADSEN公司生产的脑干诱发电位仪BRA2进行诊断性测试。结果对2010年无锡市妇幼保健院出生的8757例新生儿进行听力筛查,其中正常出生新生儿8184人,NICU住院高危儿573人,确诊听力障碍16例,占总筛查人数的1.83‰,其中高危儿重度聋6例,占NICU住院患儿的1.57%,遗传性耳聋4例占听力障碍总人数的25.00%。结论全面普及开展新生儿听力筛查、宣教、随访工作,尤其是对高危儿采用听力筛查技术的同时联合遗传性耳聋相关基因检测十分必要。     

开化地区14116例新生儿听力筛查结果分析

目的通过对新生儿听力筛查情况进行分析,以便早期发现新生儿听力损失,从而进行早期诊断和干预。方法对2008年1月～2012年9月间出生的15014例新生儿进行听力筛查,初筛、复筛均采用DPOAE,复筛未通过新生儿行ABR检查进行听力学评估和医学诊断。对筛查结果异常新生儿定期电话随访。结果2008年1月～2012年9月,共出生新生儿15014例,14116例新生儿接受听力筛查,初筛率94．02％（14116／15014）,初筛未通过2209例,初筛未通过率15．65％（2209／14116）;应复筛2209例,实际复筛1452例,复筛率65．73％（1452／2209）,复筛未通过138例,复筛未通过率9．50％（138／1452）;106例复筛未通过新生儿接受听力学诊断,最终确诊39例先天性听力损失,听力损失检出率为0．28％。结论开化县新生儿听力筛查复筛率较低,失访率较高,应加强宣教和随访。     

1280例新生儿听力与耳聋基因联合筛查的临床研究

目的探讨新生儿听力和耳聋基因联合筛查在诊断和防治新生儿听力障碍的临床价值。方法对1280例新生儿进行听力和耳聋基因联合筛查,出生后3~5天采用耳声发射法(OAE)进行听力初筛,未通过者于出生后30~42天采用OAE和快速脑干诱发电位(AABR)联合复筛,复筛未通过者于3个月内进行听力学诊断;耳聋基因筛查4个耳聋基因(GJB2、GJB3、SLC26A4和线粒体12Sr RNA)。结果 11280例新生儿初筛95例(7.42%)未通过,复筛14例未通过(14.74%),3个月听力学诊断为听力障碍4例,听力障碍率为0.313%(4/1280)。2耳聋基因筛查结果显示:GJB2基因异常45例(3.52%),SLC26A4基因异常20例(1.56%),GJB3基因异常7例(0.55%),线粒体12Sr RNA基因异常2例(0.16%);耳聋基因筛查阳性74例(5.78%),致病突变11例(0.859%)。3听力与耳聋基因联合筛查出致病突变和听力障碍13例(1.02%),高于听力筛查的听力b障碍率0.313%(P0.05)。4高危新生儿组的听力障碍率、耳聋基因突变率和杂合携带率均高于正常新生儿组(P0.01)。结论对新生儿进行听力和耳聋基因联合筛查是可行的、有必要的,有助于综合分析以尽早发现迟发性或潜在性耳聋患儿,为患儿提供早期干预,值得在临床上推广。     

杭州市2018—2020年新生儿听力筛查结果分析

目的 通过分析杭州市新生儿听力筛查情况,探讨提高新生儿听力筛查成效措施。方法 选取杭州市2018年1月1日至2020年12月31日出生的全部活产新生儿为听力初筛对象,听力初筛相关资料由各筛查机构登记、录入信息系统。采用耳声发射（TEOAE）对新生儿进行听力初筛,未通过者42天采用自动听性脑干反应（AABR）进行复筛,复筛未通过者采用听性脑干反应（ABR）、声导抗等技术进行诊断。结果 2018年至2020年杭州市活产儿共345379例,新生儿听力筛查初筛342769例,初筛率为99.24%,初筛未通过21207例,初筛未通过率6.19%。复筛20436例,复筛未通过3310例,复筛未通过实际转诊3281例,最终确诊异常794例,听力障碍发生率2.32‰。结论 杭州市新生儿听力筛查初筛率稳步增长,但需要加强听力复筛召回管理,进一步完善确诊患儿的干预随访机制,减少儿童听力和言语残疾,提高生命质量。     

12532例新生儿听力筛查结果分析

目的早期发现婴儿听力损失,以便进行早期诊断和干预,促进其正常的言语发育,提高出生人口素质。方法采用耳声发射技术对新生儿进行初筛,未通过者42天进行复筛,复筛仍未通过者采用脑干听觉诱发电位技术进行诊断性检查。结果初筛率维持在99.64%~99.94%之间,未通过初筛者召回率为100%,通过率90.60%;复筛未通过者召回率已达100%;确诊听力障碍12例,发病率9.58/万。结论开展新生儿听力筛查工作十分必要,建立完善的新生儿听力筛查网络和管理模式,对早期发现和干预听力障碍有重要的意义。     

开展新生儿听力筛查的几点体会

目的通过对新生儿的听力筛查,早期发现新生儿听力损伤,以便进行早期治疗,促进早日康复,有效减少听力语音障碍的发生率。方法用畸变产物耳声发射(DPOAE)和脑干听觉诱发电位(ABR)对626例正常新生儿及31例高危儿进行听力筛查,未通过者42天后接受听力复查,42天后复查仍未通过者,转诊到上级医院听力中心进行听力诊断性检查。结果正常新生儿听力筛查626例,初筛通过率95.9%,复筛通过率100%,0例新生儿诊断听力障碍。高危新生儿听力筛查31例,OAE初筛通过率90.3%,复筛通过率100%,ABR初筛通过率87.1%,复筛通过率75%,1例新生儿诊断听力障碍。结论新生儿听力筛查可以早期发现听力障碍儿童,有效减少听力语音障碍的发生。     

太仓市6803例新生儿听力筛查结果分析

目的：通过对新生儿进行听力筛查,及早发现听力障碍的儿童,了解本地区新生儿听力障碍的发病状况,探讨新生儿听力筛查的运作模式。方法采用畸变产物耳声发射仪(DPOAE)或自动听性脑干诱发电位仪(AABR)对2013年本市出生的6803例新生儿进行听力初筛,未通过者42d时采用(DPOAE+AABR)复筛,复筛未通过者3月龄时进行中耳声导抗测试和听性脑干反应(ABR)诊断检查。结果2013年活产数6894例,筛查6803例,经初筛、复筛有10例未通过需做诊断检查,最终确诊8例听力障碍,男5例,女3例,(其中1例男童后经诊断患听神经瘤),听力障碍发病率1.18译;其中双耳听力障碍3例,单耳听力障碍5例,双耳听力障碍发病率0.44译。结论新生儿听力筛查是早期发现听力障碍的有效模式,而DPOAE联合AABR是适合新生儿听力筛查的理想的检测技术。太仓市2010年开始把新生儿听力筛查纳入市母婴阳光工程的免费项目,提高了听力筛查率和听力障碍的检出率,使更多的听力障碍儿童得到了早期干预治疗。     

富阳地区2008年～2012年新生儿听力筛查结果分析

目的分析富阳市2008～2012年间新生儿听力筛查情况，以便旱期诊断、治疗、干预。方法采用耳声发射仪（OAE）对新生儿进行初筛，未通过者42天进行复筛，复筛仍未通过者采用脑干听觉诱发电位（AABR）进行确诊。结果初筛率AK2008年的97．54％上升至2012年100．00％，通过率88．05％；未通过初筛者召回率为99．88％，通过率89．74％，复筛召回率平均99．88％，确诊听力障碍54例，发病率1．52‰。结论新生儿听力筛查是早期诊断新生儿听力障碍的可行性方法，提高筛查率、复筛率，做到早期诊断与治疗，减少残疾儿童的发生。     

2623例新生儿听力筛查和GJB2基因检测结果分析

目的探讨常规听力筛查的同时进行GJB2基因检测的可行性。方法采取知情同意、自愿选择的原则,对2623例新生儿出生后2-3天采集足跟血,利用飞行时间质谱技术对GJB2耳聋基因进行检测,包括5个热点突变位点235delC、299-300delAT、35delG、l76-191dell6、167delT突变,并采用GSI耳声发射仪（DPOAE）进行新生儿听力筛查。结果2623例新生儿中GJB2基因检测阳性率3．20％,其中听力初筛通过婴儿中基因阳性率2．50％,听力初筛未通过婴儿中基因阳性率5．21％,听力初筛未通过GJB2耳聋基因阳性率高于听力初筛通过婴儿,差异性显著（P〈0．01）。l例GJB2235del纯合突变经ABR检查确诊为双耳中重度听力损失。结论 将JB2基因筛查和常规听力筛查联合对早期发现新生儿语前听力损失或迟发的听力损失,及婚育指导具有重要意义。     

儿童感音神经性聋听觉稳态诱发反应阈与听性脑干反应阈比较

目的研究听觉稳态诱发反应(ASSR)和听性脑干反应(ABR)阈与纯音听阈的差别和相关性。方法选择74例儿童感音神经性聋患者(118耳)分别进行ASSR、ABR和电测听检查,比较ASSR、ABR反应阈及纯音听阈,同时就ASSR、ABR反应阈与纯音听阈进行相关性分析。结果 ASSR和ABR反应阈与纯音听阈均有良好的相关性。ABR的反应阈与纯音听阈阈值接近,而ASSR反应阈与纯音听阈间差值较大。ASSR反应阈与纯音听阈间的相关性要优于ABR反应阈与纯音听阈间的相关性。结论 ASSR和ABR均为较好的评估行为听阈的客观测听方法。     

Newborn hearing screening in Kuwait

OBJECTIVES: The purpose of the study was to detect permanent hearing loss in a universal newborn hearing screening (UNHS) program that included 200 normal ("well baby") newborn children and 15 children at high risk for hearing loss at Ahmadi Hospital in Kuwait. PATIENTS AND METHODS: Distortion product oto-acoustic emissions (DPOAEs) were conducted in the ward on newborns at the age of 2 days. Newborns who did not pass the DPOAE the second time (at the age of 1 month) were evaluated by a brainstem evoked response audiometry (BERA) test within the age of 3 months. If the infant failed the BERA test, the test was repeated at the age of 6 months to confirm any permanent hearing loss. RESULTS: The peak latencies and interpeak intervals of BERA were established for newborns (5-90 days) at our clinic (ms), I: 1.58 + 0.11, III: 4.16 + 0.22, V: 6.70 + 0.24, I-III: 2.57 + 0.19, III-V: 2.59 + 0.24, I-V: 5.12 + 0.26. The incidence of permanent hearing loss was different across nursery levels. In the "well baby" group, 1% had profound sensorineural hearing loss, 1% had severe (70 dBnHL) sensorineural hearing loss, and 98% had normal hearing level. In the "high-risk" group, 26.67% had profound sensorineural hearing loss, 20% had moderate (60 dBnHL) sensorineural hearing loss, and 53.33% had normal hearing level. CONCLUSIONS: The newborn hearing screening program revealed that hearing loss in the "well baby" group is 2%, while in the "high risk" group it is 46.67%. The results warrant the establishment of UNHS programs in Kuwait and other countries in the Middle East to detect permanent hearing loss very early in life and provide appropriate medical treatment.     

脑干听觉诱发电位(BAEP)在新生儿及婴儿听力检测中的应用价值

目的:探讨BAEP在新生儿及婴儿听力检测中的应用价值。方法:对60例出生后利用耳声发射仪做听力筛查"未通过"的新生儿及婴儿进行BAEP检测。结果:BAEP异常52例(占86.7%),作BAEP听阈测定有耳聋的48例(占80%),其中轻度耳聋27只(占60例的22.5%),中度耳聋20只(占60例的16.7%),重度耳聋33只(占60例的27.5%)。结论:对耳声发射仪做听力筛查"未通过"的新生儿及婴儿做BAEP检查,能够了解听力障碍的程度以及类型,为临床的早期诊断和早期干预提供可靠的依据。     

听觉脑干诱发电位及畸变产物耳声发射在婴幼儿？…

目的 对婴幼儿听力进行正确的定量定位评估。为婴幼儿早期听力语言康复治疗提供依据。方法 以听觉脑干诱发电位（ＡＢＲ）、畸变产物耳声发射（ＤＰＯＡＥ）,对疑听力障碍的１００例婴幼儿进行听力检测,记录Ⅴ波反应域,ＤＰＯＡＥ幅值、ＤＰ－ＮＦ幅值差。结果 以ＡＢＲ Ｖ波反应阈４０ｄＢ为正常判定标准。２００例中ＡＢＲ异常１３５耳（６７．５％）,ＤＰＯＡＥ以超过本底噪音１０ｄＢ为有效,与正常组比较,ＤＰＯＡＥ异     

Infant hearing loss and connexin testing in a diverse population

PurposePrevious studies of connexin-related hearing loss have typically reported on mixed age groups or adults. To further address epidemiology and natural history of connexin-related hearing loss, we conducted a longitudinal study in an ethnically diverse cohort of infants and toddlers under 3 years of age. Our study compares infants with and without connexin-related hearing loss to examine differences in the prevalence of connexin and non-connexin-related hearing loss by ethnic origin, detection by newborn hearing screening, phenotype, neonatal risk factors, and family history. This is the first study to differentiate infants with and without connexin-related hearing loss.MethodsWe enrolled 95 infants with hearing loss from whom both exons of Cx26 were sequenced and the Cx30 deletion was assayed. Demographic, family history, newborn hearing screening data, perinatal, and audiologic records were analyzed.ResultsGenetic testing identified biallelic Cx26/30 hearing loss-associated variants in 24.7% of infants with a significantly lower prevalence in Hispanic infants (9.1%). Eighty-two infants underwent newborn hearing screening; 12 infants passed, 3 had connexin-related hearing loss. No differences in newborn hearing screening pass rate, neonatal complications, or hearing loss severity were detected between infants with and without connexin-related hearing loss. Family history correlates with connexin-related hearing loss.ConclusionsConnexin-related hearing loss occurs in one quarter of infants in an ethnically diverse hearing loss population but with a lower prevalence in Hispanic infants. Not all infants with connexin-related hearing loss fail newborn hearing screening. Family history correlates significantly with connexin-related hearing loss. Genetic testing should not be deferred because of newborn complications. These results will have an impact on genetic testing for infant hearing loss.     

Second stage of Universal Neonatal Hearing Screening – A way for diagnosis and beginning of proper treatment for infants with hearing loss

PurposeTo analyze retrospectively the results of hearing testing in infants at the second stage of the Polish Universal Neonatal Hearing Screening Program carried out in the Department of Otolaryngology at the Medical University of Warsaw.Material/methodsA total of 351 infants referred to our Department for the second stage of UNHS were included in the study. There were 39.60% infants referred due to positive result of hearing screening at the first stage of the Program performed in neonatal units, 55.27% with negative screening but risk factors present, and 5.13% without any tests due to equipment failure in the maternity unit.ResultsRisk factors were identified in 86.61% of the infants. The most frequent ones were hyperbilirubinemia (71.51%), premature birth (63.25%), and ototoxic medication (62.11%). Otoacoustic emission test showed fail results in 17.66% of the infants, and auditory brainstem responses confirmed hearing loss in 16.81%. Correlation between risk factors and confirmed hearing loss was found for hyperbilirubinemia, low birth weight, intensive therapy for at least 7 days, low Apgar scores, and craniofacial abnormalities.ConclusionsThe early identification of infants with hearing loss is essential for early intervention. Not only infants who fail the initial screening but also the ones with risk factors of hearing impairment should be referred to the centers that are capable of providing the necessary diagnostic services required for the second stage of the UNHSP. Those two steps are needed to both minimize the risk of overlooking a child with hearing loss and properly diagnose hearing impairment.     

BAEP和EMG在后颅凹手术监护中的应用

目的：评价脑干听觉诱发电位（BAEP）和面肌肌电图（EMG）对后颅凹手术监护的价值。方法：对44例后颅凹病变的患者进行术中BAEP和面肌EMG的监护,观察其对脑干、面神经功能和有效听力的保护结果。结果：术中BAEP报警10例（23％）,均发生在微血管减压组、听神经瘤组和脑干肿瘤组。脑干功能异常3例（7％）,死亡1例（2％）,各组间差异无显著意义。1年后随访,听神经瘤组面神经功能保留18例（82％）,2例术前具有有效听力的患者术后听力均得以保留,1例重度听力障碍的患者术后恢复有效听力。结论：BAEP对微血管减压、听神经瘤和脑干肿瘤手术的脑干功能监护价值较大,对听力保护意义较小;EMG显著提高面神经功能保留率,但需要加强手术医生与监护者之间的协作。