Hearing screening of high-risk newborns with brainstem auditory evoked potentials: a follow-up study

Numerous techniques have been used in attempts to find a reliable and efficient screening method for determining auditory function in the newborn. The brainstem auditory evoked potential (BAEP) is the latest method advocated for that purpose. The BAEP was evaluated as a hearing screening test in 168 high-risk newborns between 35 and 45 weeks of conceptual age. Follow-up data were obtained after 1 year (mean 17.3 months) on 134 of the infants (80%). Normal hearing was defined as a reproducible response in both ears to a 25 dB normal hearing level (nHL) click stimulus; 21 infants (12.5%) failed the initial screening test. Follow-up on 19/21 infants revealed 18 infants with normal hearing and one infant with an 80 dB nHL bilateral hearing loss substantiated. One infant with an abnormal screening test died before retesting, and the other infant was lost to follow-up but had only a unilaterally abnormal BAEP. None of the infants with a normal BAEP screening study had evidence of hearing loss on retesting. Sensitivity of the BAEP was 100%, specificity was 86%, predictive value of a positive test was 5.26%, and the predictive value of a negative test was 100%. The incidence of significant hearing loss in our population was between 0.75% (1/134 infants) confirmed, and 2.24% (3/134 infants) including infants who failed screening but were lost to follow-up. The BAEP is a sensitive procedure for the early identification of hearing-impaired newborns. However, the yield of significant hearing abnormalities was less than predicted in other studies using BAEP for newborn hearing screening.     

Utility of brainstem auditory evoked potentials in children with spastic cerebral palsy

In a retrospective study of 75 children with spastic cerebral palsy (CP), brainstem auditory evoked potentials (BAEP) were recorded and subsequently correlated with birthweight, gestational age, aetiology and type of CP, neuroradiological findings, additional impairments and disabilities (including the inability to walk independently). Seventeen patients (22.7%) had abnormal BAEP recordings. Thirteen of these 17 patients (76.5%) had spastic tetraplegia, 16 patients (94.1%) were full-term infants, 12 patients (70.6%) had myoskeletal problems, 9 (52.9%) had epilepsy, 16 (94.1%) had visual impairment, 13 patients (76.5%) were unable to walk independently, while all 17 patients (100%) had speech impairment and mental retardation. The aetiology of CP was prenatal in 2 of these 17 patients (11.8%) and perinatal in 15 patients (88.2%). Thirteen patients (76.5%) had cortical atrophy determined by either computed tomography or magnetic resonance imaging, two patients (11.8%) had an infarct picture and two patients (11.8%) had maldevelopment of the central nervous system. There was a definite statistically significant association between abnormal BAEP recordings and full-term delivery, perinatal aetiology of CP, spastic tetraplegia, speech, visual and myoskeletal impairments, epilepsy, mental retardation, inability to walk independently and cortical atrophy on neuroimaging (p < 0.001). We conclude that abnormal BAEP recordings in children with spastic CP are indicative of poor prognosis and associated with a "multihandicap state". BAEP testing should be incorporated into the diagnostic plan of all children with spastic CP newly referred to neurodevelopmental centres.     

Indications for studying evoked potentials in childhood. Methods--indications--value

Evoked potentials (EP) represent a valuable addition to currently applied diagnostic methods in neuropediatrics. Profound knowledge of the neurophysiological conditions producing EP-alterations allows basic conclusions, that cannot be gained or replaced by other investigations. EP-investigation demonstrate the existence but not the nature of a lesion in the CNS. Further diagnostic work-up usually will be necessary. Proved EP-alterations produce reproducible diagnostic results and give clues regarding its localization. Evoked potentials can be used as a screening-method for neuropediatric diseases. By follow-up examinations it is possible to show, wether there is progression or not. Testing for evoked potentials is indicated in suspected cerebral palsy in infants, in all cases of psychomotor retardation of unknown origin, impairment of vision or hearing, in cases of brain trauma or in suspected brainstem process, lesions of N. opticus or visual projective systems, neurometabolic or degenerative CNS disease, phacomatosis, progressive myoclonic epilepsy, ceroidlipofuscinosis Jansky-Bielschowski, benign partial epilepsy with extreme somatosensory evoked potentials, Ramsey-Hunt-Syndrome and aplasia of the corpus callosum.     

Brain stem evoked response audiometry in neonates

Brain stem evoked response audiometry (BERA) was performed in 50 normal full term newborn infants (25 male, 25 female) to generate normative data base for this age group. The stimuli of 30 dB, 40 dB and 80 dB nHL (normal hearing level) intensities were delivered to each subjects. 30 dB stimulus failed to produce clear wave patterns, whereas upto six vertex positive waves were recognised among which waves I, III and V were commonly present. The absolute latencies of waves I and V at 40 dB nJL were 2.54 +/- 0.21 msec and 7.56 +/- 0.26 msec and at 80 dB nHL these were 2.06 +/- 0.15 msec and 7.09 +/- 0.17 msec respectively. I-V interpeak latencies (IPL) were 5.02 +/- 0.13 msec at 40 dB and 5.03 +/- 0.13 at 80 dB nHL. The latencies of waves I and V were decreased with the increase of intensity of stimuli with the rate of 0.012 msec/dB, bit I-V IPL, i.e., central conduction time remained almost constant. There were no statistically significant differences in the brain stem auditory evoked responses between male and female subjects. Brain stem evoked response audiometry is objective and reliable for hearing screening in neonates.     

新生儿重度高胆红素血症伴听力损伤预后相关因素研究

目的探讨影响重度高胆红素血症新生儿听力损伤预后情况的相关因素。方法选择2008年11月至2009年10月本院新生儿科收治的重度高胆红素血症患儿,进行脑干听觉诱发电位（BAEP）的检测,对异常者分别于生后1个月、3个月、6个月、1岁时复查BAEP,直至BAEP恢复正常或年龄至1岁。按照随访时BAEP能否恢复正常分为随访正常组和随访异常组。对随访异常的影响因素进行单因素分析,并对单因素分析有统计学意义的因素纳入Logistic回归模型进行多因素分析。结果 967例重度高胆红素血症患儿中BAEP异常168例,其中150例按时进行随访的患儿纳入本研究。至随访结束,94.2%的轻度听力损伤者、85.5%的中度听力损伤者和19.2%的重度听力损伤者恢复正常。单因素分析结果显示,酸中毒、B/A比值、黄疸持续时间、BAEP异常程度、胆红素脑病临床表现是随访异常的影响因素。多因素Logistic回归分析结果显示,BAEP重度异常（OR=9.291）和胆红素脑病临床表现（OR=9.176）是听力损伤的重度高胆红素血症患儿随访异常的危险因素。结论新生儿重度高胆红素血症伴轻中度BAEP异常者1年内大多可恢复正常,但当存在BAEP重度异常和胆红素脑病临床表现时,1年内听力持续异常的可能性大,需要给予更加积极的神经康复治疗。     

巨细胞病毒感染引起听力损害的相关因素分析

目的 探讨引起巨细胞病毒(CMV)感染患儿听力损害的相关因素分析。方法 依据脑干听觉诱发电位(BAEP)检测结果将158 例CMV 感染患儿分为听力正常组(n=117,BAEP ≤ 35)和听力异常组(n=41,BAEP>35)。收集两组患儿一般资料、血常规、肝功能、尿液及乳汁病毒拷贝数等进行回顾性分析;绘制受试者工作特征曲线(ROC)预测导致BAEP 异常的CMV-DNA 病毒载量;采用Spearman 秩相关分析对尿CMV-DNA拷贝数与听力损害程度、血小板计数间进行相关性分析。结果 听力异常组血小板异常率、肝功能异常率和尿CMV-DNA 拷贝数均高于听力正常组(P<0.01)。ROC 分析结果显示,当尿CMV-DNA 拷贝数为1.415×10⁶/mL时,预测病毒载量导致听力损伤的敏感性为46.3%,特异性为93.2%。相关性分析结果显示,听力损害的程度随着CMV-DNA 拷贝数的增加而增高(r=0.382,P<0.01);血小板计数和尿CMV-DNA 拷贝数之间存在负相关(r=-0.233,P=0.003)。结论 尿CMV-DNA 病毒载量可能与CMV 感染导致神经性听力障碍相关;当CMVDNA载量达到1.415×10⁶/mL 时,更易出现听力损害。存在血小板减少的CMV 感染患儿,应加强听力监测。     

脑干听觉诱发电位在儿科临床应用的价值

本文对１２４０例患儿ＢＡＥＰ资料进行了分析,正常５１８例,异常７２２例。其中新生儿,乳幼儿４８６例;轻－重度异常占７４．５％双侧极重度异常占２５．５％。ＢＡＥＰ异常最高的疾患为听力障碍,听力障碍危险因素,语言障碍,脑干肿瘤智力障碍和脑性瘫痪。     

Hearing loss at the age of 5 years of children born preterm–a matter of definition

The effect of three common hearing impairment criteria on the prevalence of hearing loss was evaluated in 58 prospectively followed-up 5-year-old children born preterm at 32 weeks of gestation. Audiological assessment was done as part of an extensive neurodevelopmental evaluation at the age of 5 years. With the criterion based on the classification of the World Health Organization (average threshold hearing level >25dB at frequencies of 0.5, 1 and 2 kHz, classified according to the less impaired side) there were two preterm children with mild hearing impairment. With Clark's criterion (unilateral average threshold hearing level > 15dB at frequencies of 0.5, 1 and 2 kHz) eight children had slight hearing impairment; seven of these had conductive hearing problems. With the criterion of a single frequency-specific deficit > 15 dB for 0.25-4 kHz the number of hearing-impaired children was 28 out of 54 (51.9%), most of whom had conductive or unspecified hearing deficits. Moreover, of the four multiply handicapped, retarded children whose pure tone thresholds were not assessed mon-aurally, three would belong to the hearing-impaired group according to Clark's criterion and four according to the frequency-specific criterion, Hearing impairment, hearing impairment classification, low birthweight, preterm     

Clinical features of 21 patients with lissencephaly type I (agyria-pachygyria)

Lissencephaly (agyria-pachygyria) is the most severe neuronal migration disorder, characterized by total or partial absence of gyri. In this study, 21 patients with lissencephaly type I (9 girls, 12 boys) with a mean age of 19 +/- 21 months (2 weeks-8 years) were evaluated clinically and graded according to neuroradiological findings (19 patients by magnetic resonance imaging MRI and 2 by computed tomography CT). Three patients were classified as lissencephaly grade 2 and 18 patients as grade 3 or 4. Clinically, 12 patients (57%) had microcephaly, and eight (38%) had facial dysmorphism. All the patients had prominent psychomotor retardation, moderate to severe; the most frequent neurological findings were spastic guadriplegia (36.4%) and hypotonia with exaggerated tendon reflexes (27.3%). Seventy-eight percent of the patients had epileptic seizures resistant to conventional treatment. Lissencephaly is a cerebral cortical malformation that should be considered in children with developmental delay with or without microcephaly and facial dysmorphism. In addition, it should be investigated in the etiology of early-onset childhood epilepsy.     

脑干听觉诱发电位与畸变产物耳声发射在听觉通路损伤患儿中的应用研究

目的　采用客观听力检测技术 ,分析听觉通路病损患儿的临床听力学特点 ,评价感音神经性聋患儿的蜗性及蜗后病变特征。方法　对感音神经性聋患儿共 310例 (5 0 0耳 ,年龄 1个月至 6岁 ,平均 2 4 2个月 )进行临床分析 ,其中中枢神经系统 (CNS)病变伴听力障碍 10 5例 (2 0 5耳 ) ,将其分为核黄疸 脑瘫组、额叶发育不良组和其他中枢性病变 3组。同年龄段对照组 6 0例 (10 4耳 )。同时检测脑干听觉诱发电位 (BAEP)和畸变产物耳声发射(DPOAE) ,对比不同组别间BAEP波V阈值及DPOAE各自的特点、同一组间不同BAEP波V阈值耳DPOAE的变化特征。结果　 (1)全组感音神经性聋患儿中有CNS病变者比例较高 (10 5例 2 0 5耳 /310例 5 0 0耳 )。 (2 )CNS病变患儿中核黄疸 脑瘫组可见严重的蜗后性听力损失 ,耳蜗功能也轻度受累 ;额叶发育不良组仅出现轻度蜗后听力损失 ;其他中枢性病变一般不累及耳蜗功能。 (3)蜗性听力损失者 ,BAEP波V阈值达 6 0nHLdB时 ,DPOAE幅值明显下降 ;达 70nHLdB以上者 ,DPOAE幅值严重下降或消失。结论　CNS病变伴感音神经性聋患儿听力障碍表现为多样性 ,临床上需联合听力专科采用不同客观听力检测技术进行综合评估 ,才能作出正确诊断。     

Chronic suppurative otitis media in children of Luanda, Angola

Aim: Evaluation of clinical characteristics, bacteriology and hearing in paediatric patients with and without chronic suppurative otitis media (CSOM) in Luanda, Angola. Methods: Interview, clinical examination, ear‐discharge culture, open air pure‐tone audiometry and brainstem auditory‐evoked potentials of 23 outpatients with CSOM and 23 controls in a paediatric hospital. Results: Of the CSOM vs. control children, 35% vs. 26% had running water, 70% vs. 70% electricity, 64% vs. 0% HIV (p < 0.0001) and 36% vs. 0% tuberculosis in history (p = 0.002). Ten (43%) children had bilateral CSOM. The major ear‐discharge pathogens were Proteus spp. (44%) and Pseudomonas (22%). Hearing impairment of >25 dB was present in 52% of CSOM‐affected ears and bilateral hearing loss in 7 (30%) CSOM children vs. zero control child (p = 0.009). Only one hearing‐impaired child’s family had previously detected the handicap. Conclusion: CSOM occurred in children with high co‐morbidity. Persistent otorrhoea was usually caused by Proteus spp. or Pseudomonas, and often suggestive of either HIV or hearing impairment. In the developing countries, prompt diagnosis and treatment of CSOM would enhance the children’s linguistic and academic development.     

Intrauterine growth retardation and brainstem auditory-evoked response in preterm infants

Intrauterine growth retardation is frequently associated with intrauterine undernutrition, and can deleteriously affect brain function. Twenty-eight premature small for gestational age infants were compared with 28 premature appropriate for gestational age infants to determine whether intrauterine growth retardation was associated with abnormalities in the auditory pathway in the early neonatal period. The auditory pathway was studied between 4-18 wk of life by analysis of brainstem auditory-evoked potentials elicited by a 10/s 75 decibel above normal adult hearing level (dB nHL) click stimulus presented at the infants' ears. Peak latencies of components I, III and V, and interpeak latencies I-III, III-V and I-V, yielded no statistically significant differences between groups. The present study indicates that intrauterine growth-retarded premature infants may not have abnormalities of brainstem auditory-evoked response in the early neonatal period.     

Hearing Impairment in Connection with Preauricular Tags

ABSTRACT. Between 1977 and 1984, 230 newborns (5.4/1000 livebirths) were registered at the two maternity hospitals of Goteborg as having preauricular tags. Of these 188 were available for hearing assessment. In 10 children (5%) the tag was associated with other malformations of the ear/face region. All these children had hearing impairment (HI), 8 conductive, 1 sensorineural and 1 mixed. In 178 neonates the tag was the only defect. Of these, 23 (13%) were found to have HI, all sensorineural and of mild to moderate degree. In the total group of children a positive family history for HI was found in 29% and for malformation in 24%. In the children where HI was found (33 cases in total) the figures for heredity rose to 67% (HI) and 30% (malformation). In the 23 cases with ear tag and HI, a hereditary tendency for HI was found to be 78%. Accordingly there is a clearly elevated risk for HI in connection with ear tags and we therefore recommend routine hearing assessment in all children with preauricular tags.     

Hearing impairment in connection with preauricular tags

Between 1977 and 1984, 230 newborns (5.4/1,000 livebirths) were registered at the two maternity hospitals of G?teborg as having preauricular tags. Of these 188 were available for hearing assessment. In 10 children (5%) the tag was associated with other malformations of the ear/face region. All these children had hearing impairment (HI), 8 conductive, 1 sensorineural and 1 mixed. In 178 neonates the tag was the only defect. Of these, 23 (13%) were found to have HI, all sensorineural and of mild to moderate degree. In the total group of children a positive family history for HI was found in 29% and for malformation in 24%. In the children where HI was found (33 cases in total) the figures for heredity rose to 67% (HI) and 30% (malformation). In the 23 cases with ear tag and HI, a hereditary tendency for HI was found to be 78%. Accordingly there is a clearly elevated risk for HI in connection with ear tags and we therefore recommend routine hearing assessment in all children with preauricular tags.     

Diffusion tensor imaging of the inferior colliculus and brainstem auditory-evoked potentials in preterm infants

Background  Preterm and low-birth-weight infants have an increased risk of sensorineural hearing loss. Brainstem auditory-evoked potentials (BAEP) are an effective method to detect subtle deficits in impulse conduction in the auditory pathway. Abnormalities on diffusion tensor imaging (DTI) have been shown to be associated with perinatal white-matter injury and reduced fractional anisotropy (FA) has been reported in patients with sensorineural hearing loss. Objectives  To evaluate the possibility of a correlation between BAEP and DTI of the inferior colliculus in preterm infants. Materials and methods  DTI at term age and BAEP measurements were performed on all very-low-birth-weight or very preterm study infants (n=56). FA and apparent diffusion coefficient (ADC) of the inferior colliculus were measured from the DTI. Results  Shorter BAEP wave I, III, and V latencies and I–III and I–V intervals and higher wave V amplitude correlated with higher FA of the inferior colliculus. Conclusion:  The association between the DTI findings of the inferior colliculus and BAEP responses suggests that DTI can be used to assess the integrity of the auditory pathway in preterm infants.     

Sensory evoked potentials in infants with Down syndrome

Aim: To investigate the sensory functions of the peripheral to central pathways in infants with Down syndrome (DS) by sensory evoked potentials. Methods: Fifty-five infants, 30 DS infants and 25 controls, were examined by multimodal evoked potentials, including brainstem auditory evoked potentials (BAEP), visual evoked potentials (VEP) and short-latency somatosensory evoked potentials (SSEP). Results: No obvious difference was found in the peak latencies between the two groups for BAEP. Nine children with DS showed abnormal BAEP; six had hearing loss and three had prolonged wave I latencies. For VEP, the peak latencies of P₂ and N₂ were significantly longer and the amplitudes were smaller in the DS group than in the control group. Of the 30 infants with DS, five had significantly prolonged P₂ latencies and two had lower amplitudes. In SSEP, the mean latencies of N₂₀ and the interpeak latencies of N₁₃-N₂₀ of the infants with DS showed apparent prolongation compared to the controls. Seven of 30 (23.3%) DS patients had prolonged N₂₀ latencies.

Conclusion: Our results indicate that various sensory deficits occur in patients with DS during the first year of life.     

Visual and brainstem auditory evoked potentials in children with headache

Background: Headache is a common problem in the pediatric population. The purpose of the present study was to evaluate visual evoked potentials (VEP) and brainstem auditory evoked potentials (BAEP) in children with headache. Methods: Thirty‐seven children fulfilling the International Headache Society Criteria for a diagnosis of migraine, 35 children with tension‐type headache and 40 healthy children (control group) were enrolled in the study. Results: The mean age of patients and controls was 10.4 years. P100 latency and amplitudes of migraine patients were significantly higher than children with tension‐type headache and control subjects. Children with tension‐type headache also had higher P100 latency and amplitude values than control subjects but there was no statistical difference. BAEP responses were similar between all groups. Conclusion: Measurement of VEP latency and amplitude is a valuable and reliable test for the diagnosis of migraine and can be used safely in childhood.     

Parental suspicion of hearing loss in children with otitis media with effusion

Objective This study aimed to evaluate the parental suspicion of hearing loss in children with otitis media with effusion (OME). As part of a population-based survey in a screening programme among 6- to 7-year-old Chinese children in Hong Kong, OME cases and controls were studied for the value of parental observations in the prediction of OME and hearing test results.Subjects and methods Prior to the otoscopic and tympanometric examination of the children on school premises, a self-administered binary-choice question was sent to the parents asking whether there was any suspicion of hearing impairment. Positive screens and randomly selected negative screens were seen in a hospital clinic for the confirmation of case and control status 2 to 3 weeks after the school screening. Aural examination under microscopy, repeated tympanometry and stapedial reflex testing, and pure-tone audiometry (PTA) were conducted, and 117 cases and 159 controls were included in this study.Results The average PTA conductive threshold levels in the individual children with OME ranged from 3.8 dB to 40.0 dB with a group mean of 17.0 dB in the better-hearing ears. Parental suspicion of hearing deficit was significantly associated with OME (p<0.001) but not PTA findings (p=0.686). The sensitivity of parent-suspected hearing impairment to detect OME however was very low (19.7%).Discussion In other words, if we had relied on parental suspicion as the first screening, at least 80% of the OME cases would have been missed. We conclude that the parental suspicion of hearing loss is inadequate for the identification of mild hearing loss as caused by OME. Health education is recommended to improve parental awareness of the disease.     

Mild impairment of neuro-otological function in early treated congenital hypothyroidism.

Pure tone audiometry, tympanometry, acoustic stapedial reflex thresholds (ASRTs), and auditory evoked brain stem responses (AEBRs) were carried out in 38 children with early treated congenital hypothyroidism aged 10-12 years, together with tests of vestibular function (electronystagraphy, rotational, and caloric tests). Sensorineural hearing loss with thresholds of greater than 15 dB was detected in 18 children (10 at 8 kHz only); only two children had more than 40 dB hearing loss, each in one ear. Raised ASRTs were found in eight children and two children had abnormal AEBRs. Of the 29 children tested, 12 had an abnormality of vestibular function. Although not significant at the 5% level, there was a tendency for the abnormalities to be more prevalent and severe in the children with more severe hypothyroidism, as judged by pretreatment plasma thyroxine. It is concluded that (i) mild abnormality of hearing is still common in children with congenital hypothyroidism despite early treatment but this is much less severe than that found before neonatal screening and (ii) mild abnormalities of vestibular function may be common in early treated congenital hypothyroidism.     

Mild impairment of neuro-otological function in early treated congenital hypothyroidism

Pure tone audiometry, tympanometry, acoustic stapedial reflex thresholds (ASRTs), and auditory evoked brain stem responses (AEBRs) were carried out in 38 children with early treated congenital hypothyroidism aged 10-12 years, together with tests of vestibular function (electronystagraphy, rotational, and caloric tests). Sensorineural hearing loss with thresholds of greater than 15 dB was detected in 18 children (10 at 8 kHz only); only two children had more than 40 dB hearing loss, each in one ear. Raised ASRTs were found in eight children and two children had abnormal AEBRs. Of the 29 children tested, 12 had an abnormality of vestibular function. Although not significant at the 5% level, there was a tendency for the abnormalities to be more prevalent and severe in the children with more severe hypothyroidism, as judged by pretreatment plasma thyroxine. It is concluded that (i) mild abnormality of hearing is still common in children with congenital hypothyroidism despite early treatment but this is much less severe than that found before neonatal screening and (ii) mild abnormalities of vestibular function may be common in early treated congenital hypothyroidism.