听觉脑干反应和耳声发射在高危儿听力筛查中的应用

目的：耳声发射(OAE)和听觉脑干反应(ABR) 是新生儿听力筛查的常用方法。该研究旨在探讨畸变产物耳声发射(DPOAE) 和ABR应用于重症监护病房(NICU)高危新生儿听力筛查的差异和意义。方法：分别应用Smart-EP型听觉脑干诱发电位仪和Smart-OAE畸变产物耳声发射检查仪对600例(1 200耳)不同病因所致的高危儿同时进行DPOAE和ABR检查,将两种方法取得的检测结果进行比较。结果：在600例(1 200耳)高危新生儿中,ABR的异常率(78.6%,943/1 200耳)远高于DPOAE的未通过率(22.3%,268/1 200耳);二种检查的共同阴/阳性率分别为20.8%(241/1 200耳)和21%(252/1 200耳)。1 200耳中有493耳DPOAE和ABR的测试结果一致,占41.1%;707耳的测试结果不一致,占58.9%。DPOAE测试的假阳性率为6.0%(16/268耳),假阴性率为74.1%(691/932耳)。结论： DPOAE仅反映耳蜗功能,单独用于高危新生儿听功能筛查的价值有限。ABR检查果相对可靠,NICU高危新生儿听力筛查应先做ABR检查,ABR异常者再做OAE检查。ABR和OAE二种检测方法相互结合,方能提高高危新生儿听力筛查的准确性。     

Auditory brainstem response and otoacoustic emissions in hearing screening of full term infants with hyperbilirubinemia

目的 探讨听性脑干反应(ABR)和畸变产物耳声发射(DPOAE)应用于高胆红素血症新生儿听力筛查中的差异和意义.方法 对197例诊断为高胆红素血症的新生儿同时进行ABR和DPOAE检查,异常者于生后3-4个月进行随访.结果 197例患儿临床上无明显胆红素脑病表现,ABR和DPOAE的总异常率分别为76.65%和14.47%;血清胆红素水平越高,ABR异常率越高(P<0.01),而DPOAE异常率与黄疸程度无明显相关性;DPOAE异常者多并存潜在的围生期其他导致听力损伤的高危因素.重度ABR异常者在生后3～4个月复查时仍有40.7%无改善.结论 临床上无明显胆红素脑病表现的高胆红素血症新生儿ABR异常率高,对此类患儿进行听力检查应先ABR后DPOAE或者两者同时进行以避免漏诊.     

2367例新生儿听力筛查初步报告

目的　筛查新生儿听力障碍。方法　采用诱发畸变耳声发射（ＤＰＯＡＥ）对２ ３６７ 例新生儿进行听力筛查，连续监测异常者，最后由脑干听觉诱发电位（ＡＢＲ） 确诊。结果　５ 例听觉障碍，其中单耳聋２ 例，双耳聋３ 例。中度听觉障碍２例，重度听觉障碍３ 例。结论　ＤＰＯＡＥ测试是适合于新生儿听力筛查可靠、有效、快速、简便的好方法。     

东莞地区新生儿听力筛查的多中心研究

目的：探索适合于新生儿听力筛查的临床策略,通过多中心合作获得新生儿听力损失发病的基本资料。方法：应用畸变产物耳声发射技术(DPOAE)进行听力普遍筛查。对复筛未通过者,于生后3个月左右采用听性脑干反应(ABR)和40Hz听性相关电位(40HzAERP)技术诊断。结果：该研究实际筛查17360例,筛查率为89.1%。初筛通过15624例(90.0%)。需复筛的1736例,实际复筛1597例,复筛率92%,复筛通过1491例,复筛通过率93.4%。41例复查ABR其中确诊新生儿先天性听力损失39例,减去2例假阳性,该组新生儿先天性听力损失的发生率为2.25‰。结论：新生儿听力普查十分必要。DPOAE是一种快速行之有效的新生儿听力初筛方法,两步筛查法可及早发现听力损失,听力筛查、诊断、康复统筹运作是使聋儿得以康复的重要保证。     

听性脑干反应和耳声发射在足月新生儿高胆红素血症听力筛查中的应用

目的探讨听性脑干反应(ABR)和畸变产物耳声发射(DPOAE)应用于高胆红素血症新生儿听力筛查中的差异和意义。方法对197例诊断为高胆红素血症的新生儿同时进行ABR和DPOAE检查,异常者于生后3～4个月进行随访。结果 197例患儿临床上无明显胆红素脑病表现,ABR和DPOAE的总异常率分别为76.65%和14.47%;血清胆红素水平越高,ABR异常率越高(P<0.01),而DPOAE异常率与黄疸程度无明显相关性;DPOAE异常者多并存潜在的围生期其他导致听力损伤的高危因素。重度ABR异常者在生后3～4个月复查时仍有40.7%无改善。结论临床上无明显胆红素脑病表现的高胆红素血症新生儿ABR异常率高,对此类患儿进行听力检查应先ABR后DPOAE或者两者同时进行以避免漏诊。     

高胆红素血症新生儿听力损伤的研究

目的 通过对高胆红素血症新生儿进行畸变产物耳声发射(DPOAE)及听性脑干反应(ABR)检测,了解其听力损伤的特点,为早期干预提供依据.方法 对105例足月高胆红素血症新生儿进行DPOAE检查,其中有17例急性胆红素脑病患儿同时进行ABR检测.105例新生儿根据胆红素水平分为轻、中、重度3组,中、重度黄疸新生儿再分为溶血组与非溶血组,对其检测结果进行统计学分析.结果 高胆红素血症患儿DPOAE总异常率为33.7%.DPOAE异常率随着胆红素浓度增高逐渐增高,42 d后逐渐恢复;溶血组DPOAE异常率(48%)较非溶血组异常率(35.23%)高;胆红素脑病组ABR的异常率(61.76%)与DPOAE异常率比较(47.06%)无统计学意义.结论 高胆红素血症可引起新生儿耳蜗功能异常,但可逐渐恢复.新生儿溶血是引起胆红素听力损伤的高危因素之一.ABR与DPOAE联合应用对早期评估高胆红素血症新生儿听力损伤比单独应用更准确.     

高胆红素血症对新生儿听力的影响

目的研究高胆红素患儿听力障碍的发生率,探讨与听力障碍发生有关的高危因素。方法采用畸变产物耳声发射(DPOAE)对高胆红素血症患儿于病情稳定、出院前行听力筛查初筛,未通过者于生后42 d左右进行复筛,复筛未通过者行听觉脑干反应(ABR)确诊检查;选取产科出生正常新生儿作为对照。结果检测高胆红素血症患儿235例,初筛异常58例,占24.7%;复筛58例,异常11例,占18.9%;复筛未通过者3个月龄时行ABR检查,确诊听力障碍5例,高胆红素血症患儿听力障碍发生率为2.13%(5/235)。检测对照组正常新生儿182例,初筛异常18例,占9.9%;18例复筛均通过。结论高胆红素血症患儿是听力障碍发生高危人群。先天性巨细胞病毒感染、新生儿败血症及溶血病是导致发生听力障碍的危险因素。对高危新生儿应进行听力随访。     

新生儿听力筛查的临床应用

新生儿听觉障碍分为先天性听觉障碍和与分娩诸多高危因素相关的获得性听力损伤,其确切发病率尚鲜有报道。为了解我国新生儿听力障碍的发病率及相关因素,配合我科对小婴儿神经发育早期干预的研究,我们应用畸变产物耳声发射测定仪(DPOAE)对近两年我院新生儿的听力进行检测,现将结果报告如下。     

111 例未通过听力筛查新生儿常见耳聋基因突变位点分析

目的了解未通过听力筛查新生儿耳聋基因突变情况。方法随机选取听力筛查未通过、经听觉脑干诱发电位(ABR)测试为感音神经性耳聋患儿111例,收集足跟血血片,提取基因组DNA后,检测GJB2、SLC26A4和线粒体12 Sr RNA基因中的11个热点的突变,分析听力损失程度与突变的关联。结果 111例新生儿中,共检出携带耳聋基因突变24例(21.6%)。其中,GJB 2基因突变14例(12.6%),包括235 del C单杂合突变5例,235 del C和299_300 del AT复合杂合突变5例,以及235del C纯合突变、299_300del AT单杂合突变、176_191del16和235del C复合杂合突变、299_300del AT和508_511 dup AACG复合杂合突变各1例;SLC 26 A 4基因突变10例(9.0%),包括IVS 7-2 AG单杂合突变2例,1226 GA单杂合突变3例,2168 AG单杂合突变2例,IVS7-2AG和2168 AG复合杂合突变3例。本组耳聋患儿中未检出线粒体基因突变。结论未通过听力筛查新生儿中,超过1/5检测到聋基因突变,并以GJB2基因突变最常见,实施热点致聋基因检测可以提高耳聋的病因诊断率。     

不同日龄新生儿诱发性耳声发射测试

对20例正常新生儿在出生后第1～5天逐日行瞬态诱发性耳声发射(TEOAE)和畸变产物耳声发射(DPOAE)的测试.了解不同日龄新生儿畸变产物耳声发射(DPOAE)和瞬态诱发性耳声发射(TEOAE)的特征,探讨应用OAE作新生儿听力筛查的最佳时机.随新生儿日龄的增加,其TEOAE和DPOAE检出率和反应幅值逐步提高,新生儿生后第1～2天的检出率和反应幅值显著低于第3～5天,至生后第3～5天,TEOAE和DPOAE检出率接近或达到100%,反应幅值也趋于稳定.结论应用TEOAE和DPOAE进行新生儿听力筛查时,其日龄至少应在3天或3天以上,测试TEOAE和DPOAE快速、方便,不失为普遍性新生儿听力筛查首选方法.     

Auditory brainstem response in neonates with hypoxic-ischemic-encephalopathy following perinatal asphyxia.

The technique of auditory brainstem evoked responses testing (ABR) was applied to twenty four new born infants with asphyxia complicated by hypoxic-ischemic-encephalopathy (HIE) in an attempt to study potential influence of HIE on hearing impairment. Twenty normal term neonates with no apparent neurological disorder, were also examined for comparison. Twenty two per cent (n = 5) of the patients with HIE showed some abnormality in the ABR pattern, the major one being a transient elevation in threshold of wave V (n = 4; 16.6%). ABR abnormalities, however, were found with greater frequency in neonates with Stage II HIE (75% vs 10%, p less than 0.001). Further ABR abnormalities were found in Stage II HIE only when duration of neurological abnormalities was greater than 5 days. There was no difference, however, between the ABR latencies of the asphyxiated and non-asphyxiated newborn infants (p greater than 0.05). One neonate (4%) with severe HIE, however, had persistent ABR abnormality in the form of bilateral absence of all waves in the later part of the ABR with preservation of wave I. This implied only cochlear functions and absence of any brainstem conduction. These results indicate that birth asphyxia complicated by HIE is a significant high risk factor for hearing impairment in the affected neonates. This justifies ABR testing of neonates with HIE (particularly Stage III), at the time of their discharge, as a screening procedure for early detection of permanent hearing loss.     

Confirmation of deafness in infancy.

AIM: To assess delay in confirming hearing impairment in infants identified by universal neonatal screening and to investigate the causes. PATIENTS: Infants identified from 25 199 babies screened from January 1992 to December 1997. METHODS: A two stage transient evoked oto-acoustic emission test (TEOAE), with a threshold auditory brainstem response (ABR) recording undertaken on those who failed. The screen identified infants with a permanent congenital hearing impairment (PCHI) averaging 40 dBnHL or worse in the best ear. Those with less impairment were also ascertained. The positive predictive value (PPV) of the ABR test and measures of delay between identification and eventual diagnosis were analysed. RESULTS: A targeted PCHI was found in 1.18/1000 neonates. The PPV of the ABR for confirming a targeted PCHI was 100% when the ABR threshold was >/= 80 dBnHL. Nine of 11 infants with this threshold had severe or profound permanent deafness. The delay from ABR to audiological certainty was about 1 month-diagnosis was confirmed around 3 months. There was uncertainty when the ABR was 40-80 dBnHL. The PPV was 60% and 8% when the ABR thresholds were 70 dBnHL and 50 dBnHL, respectively. 85 of 111 infants with ABR thresholds in this range had a temporary conductive impairment. Their early diagnosis depended upon the type and degree of hearing impairment and diagnosis was delayed to about 8 months in these infants. CONCLUSIONS: Hearing impairments identified by universal screening are delayed in all but those with severe or profound bilateral PCHI. This delay can be reduced by applying in early infancy a battery of audiological tests and requires further exploration.     

西北地区751例新生儿耳聋基因突变筛查

目的初步了解西北地区新生儿常见耳聋基因突变类型和携带率,探讨耳聋基因突变筛查对于辅助诊断和防治新生儿遗传性耳聋的临床价值。方法采集西北地区751例新生儿的足跟血,采用15项遗传性耳聋基因检测试剂盒(微阵列芯片法)对中国人群常见4种耳聋基因15个突变位点进行筛查。结果在751例新生儿中,检测到39例新生儿携带耳聋基因突变,总突变携带率为5.18%。其中GJB2基因突变19例,突变携带率为2.53%;SLC26A4基因突变16例,突变携带率为2.12%;线粒体12SrRNA 1555A>G均质突变4例,突变携带率为0.53%。新疆出生的新生儿279例中,检测到耳聋基因突变11例,突变携带率3.95%;甘肃省出生的新生儿277例中,检测到耳聋基因突变12例,突变携带率4.32%;陕西省出生的新生儿178例中,检测到耳聋基因突变12例,突变携带率6.74%;青海省出生的新生儿17例中,检测到耳聋基因突变4例,突变携带率23.52%。结论西北地区新生儿耳聋基因突变携带率偏高,但是GJB2基因235 del C位点的突变携带率偏低,可能与西北地区的地域特征和人口遗传学特点有关。新生儿耳聋基因筛查对于听力筛查具有很好的互补作用,可以从基因水平发现可能出现迟发性耳聋和药物敏感性耳聋的高危新生儿。     

Auditory screening of special care neonates using the auditory response cradle.

The Linco-Bennett auditory response cradle is a microprocessor controlled device for screening the hearing of neonates. A total of 396 neonates admitted to a special care unit were tested on the cradle and later followed up in a comprehensive test programme between the ages of 3 months and 8 months. Altogether 374 (94%) were available for follow up. The use of the cradle resulted in the detection of six neonates with appreciable deafness. One neonate who passed the cradle test has severe bilateral hearing impairment. The false alarm rate for neonates failing two tests on the cradle but having normal hearing at follow up was 4.3%. The auditory response cradle was designed for use in mass screening programmes but testing the hearing of all newborns would require many staff. It is argued that this is unrealistic when resources are scarce, but that neonates in high risk groups should have their hearing screened at birth by an objective test such as this. The cradle has considerable potential but its method of use and the ''decision making'' programme could be improved.     

AUDITORY BRAINSTEM RESPONSES (ABR) IN HIGH-RISK NEONATES

ABSTRACT. In the present study, auditory brainstem responses (ABR) were recorded in 60 highrisk neonates in the intensive care unit selected by the following criteria: Birth-weight <2000 g, hyperbilirubinemia requiring phototherapy or exchange transfusion, idiopathic respiratory distress syndrome, artificial ventilation, asphyxia, sepsis or meningitis, intracranial haemorrhage, neurological symptoms and potential ototoxic medication (aminoglycoides, furosemide). The infants tested ranged in gestational age from 27-44 weeks. The ABR testing was performed in a sound-proof room using the Madsen (ERA-74) equipment. Four infants did not reveal responses to 70 dB HL ("nonresponders"), and the total of 10 neonates (16.6%) had abnormal ABR-tests, when the physiological changes related to gestational age and conceptional age (gestational age plus the age after birth) were taken into account. The 10 neonates with abnormal tests were reexamined after discharge, and in six there were no improvement of threshold sensitivity. Three of the "nonresponders" were retested several times within the two years after birth (one died at age 18 months of pertussis), and none of them revealed ABR at stimulus intensity of 70 dB HL. They all attend an audiological training program started at age of six months as a consequence of the early diagnosis of impaired auditory function. It is our opinion that a routine ABR-evaluation should be performed on high risk neonates (criteria mentioned above) in the newborn intensive care unit. Retesting of infants with abnormal responses within three months, and several times within the next two years if abnormal responses persist, is important. Transient impairment of auditory functions is not uncommon in these infants. However, the children with persisting hearing impairment should be discovered early to attend an early audiological training program.     

Confirmation of deafness in infancy

AIM—To assess delay in confirming hearing impairment in infants identified by universal neonatal screening and to investigate the causes.
PATIENTS—Infants identified from 25 199 babies screened from January 1992 to December 1997.
METHODS—A two stage transient evoked oto-acoustic emission test (TEOAE), with a threshold auditory brainstem response (ABR) recording undertaken on those who failed. The screen identified infants with a permanent congenital hearing impairment (PCHI) averaging 40 dBnHL or worse in the best ear. Those with less impairment were also ascertained. The positive predictive value (PPV) of the ABR test and measures of delay between identification and eventual diagnosis were analysed.
RESULTS—A targeted PCHI was found in 1.18/1000 neonates. The PPV of the ABR for confirming a targeted PCHI was 100% when the ABR threshold was ⩾ 80 dBnHL. Nine of 11 infants with this threshold had severe or profound permanent deafness. The delay from ABR to audiological certainty was about 1 month—diagnosis was confirmed around 3 months. There was uncertainty when the ABR was 40-80 dBnHL. The PPV was 60% and 8% when the ABR thresholds were 70 dBnHL and 50 dBnHL, respectively. 85 of 111 infants with ABR thresholds in this range had a temporary conductive impairment. Their early diagnosis depended upon the type and degree of hearing impairment and diagnosis was delayed to about 8 months in these infants.
CONCLUSIONS—Hearing impairments identified by universal screening are delayed in all but those with severe or profound bilateral PCHI. This delay can be reduced by applying in early infancy a battery of audiological tests and requires further exploration.