Outcomes of neonatal screening for hearing loss by otoacoustic emission.

AIM: To assess universal neonatal screening for bilateral hearing impairments averaging 40 dBHL or worse in the better ear, using transient evoked otoacoustic emission screening (TEOAE) testing. METHODS: A three year cohort (14,353 infants born from January 1992 to 1995) was screened and subsequently followed up by hearing surveillance methods, including a distraction test screen from 7 months of age. The entire cohort was used to evaluate the outcome of the screen. A subcohort of 8172 district residents was used to evaluate the continuing worth of the distraction test programme. RESULTS: Nineteen infants (1.3/1000) with a targeted hearing impairment failed the neonatal TEOAE test. Six profoundly deaf infants identified by the TEOAE screen, were fitted with hearing aids at a median age of 16 weeks. One remained without an aid. Of 12 infants with a moderate impairment, only seven accepted hearing aid fitting and the median age of being fitted with an aid increased to 42 weeks. By the time of the analysis 22 children with a targeted hearing impairment (1.5/1000) had been identified from the cohort. Of the three missed neonatally, one was cared for elsewhere, another had a progressive loss, and the third had central deafness. Twenty children were ascertained with a congenital peripheral deafness. Of these, eight (40%) had risk factors identifiable neonatally. Only the child with central deafness was missed by TEOAE screening and subsequently identified by behavioural tests in infancy. The TEOAE screen outperformed the distraction test in terms of processes and yield and was 25% less expensive. CONCLUSIONS: The analysis confirmed the worth within the district of the TEOAE hearing screen. It will thus be continued as a universal neonatal screen with the distraction test being retained as a selective screen in the latter half of infancy.     

先天性巨细胞病毒感染对婴幼儿听力及智力影响的研究

目的　探讨先天性巨细胞病毒 (CMV)感染对婴幼儿生长发育、听力及智力的影响。方法　对 65例先天性CMV感染儿及 82例非感染儿于新生儿期进行生长发育及听力的检测 ,并于生后 6个月至 4岁进行随访。随访内容包括 :体格检查、发育商测试及听力测试。结果　两组生长发育状况无显著性差异。感染组发育商落后8例 ,对照组仅 1例 ,二者有显著性差异。感染组新生儿期、随访时分别有 7例 9耳、9例 11耳未通过TEOAE听力测试 ,对照组均为 1例 1耳未通过 ,未通过率感染组明显高于对照组。新生儿期、随访时双耳反应能量感染组明显低于对照组。 14例进行了ABR检测 (感染组 12例 ,对照组 2例 ) ,其中感染组 9例 17耳听力异常 ,对照组 1例 1耳听力异常。结论　先天性CMV感染对婴幼儿生长发育可能无明显影响 ,但对听力、智力的损害却不容忽视     

Newborn oto-acoustic emission hearing screening tests: preliminary evidence for a marker of susceptibility to SIDS

OBJECTIVE: To evaluate the newborn transient evoked otoacoustic emission (TEOAE) hearing screening tests of infants later diagnosed with the sudden infant death syndrome (SIDS). STUDY DESIGN: In a case-controlled study, the newborn TEOAE hearing screens of 31 infants who subsequently died of SIDS were retrospectively compared to those of 31 newborn infants that survived the first year of life. SIDS cases were individually matched to surviving controls based on gender, term versus preterm age and NICU versus well baby nursery. RESULTS: The TEOAE screens of SIDS infants demonstrated significantly decreased signal to noise ratios at 2000, 3000, and 4000 Hz (p<0.05) on the right side compared to healthy control infants. CONCLUSION: Newborns at risk for SIDS are currently indistinguishable from other newborns and are only identified following a later fatal event. A unilateral difference in cochlear function is a unique finding that may offer the opportunity to identify infants at risk of SIDS during the early postnatal period with a simple non invasive hearing screen test. The ability to implement preventative measures well in advance of a potential critical incident would be an important breakthrough.     

The Milan Project: a newborn hearing screening programme

Aim: Since 1997 a newborn hearing screening programme has been implemented by the U.O. Neurologia-Neurofisiopatologia and Dipartimento di Neonatologia of the Istituti Clinici di Perfezionamento ICP in Milan for both babies with no risk and those at risk of hearing impairment. This programme was named the Milan Project. Methods: The protocol for no-risk babies consisted of three stages: in the first two stages, newborns were tested with transient click-evoked otoacoustic emissions (TEOAE), in the third one with conventional auditory brainstem responses (ABR). The first TEOAE test was performed by 36 h of age, before discharge, the second one after 15-30 d in case of referral, and the third one, by ABR, for those babies who failed the second TEOAE stage. Newborns at audiological risk were submitted to conventional ABR before the third month of corrected age. Some of this latter population was also submitted to the TEOAE test. The entire tested population (no-risk babies and newborns at audiological risk) consisted of 19 777 babies: 19 290 without risk (“no risk”) and 487 at risk (“at risk”). Results: During the course of the Milan Project, hearing impairment (ABR threshold equal to or greater than 40 dB nHL) was identified in 63 newborns (19 from the no-risk and 44 from the at-risk population), with a prevalence of 0.32%. Bilateral hearing impairment (BHI) was found in 33 newborns (10 from the no-risk and 23 from the at-risk population), corresponding to 0.17%. Among infants with bilateral hearing impairment, 30.3% had no risk factors. The prevalence of hearing impairment was determined on days 15-30 after birth.

Conclusions: The results show that the implementation of a hospital-based, universal neonatal hearing screening programme for babies with and without audiological risk is feasible and effective. The effectiveness of the programme has increased as a function of the years since its inception, with a strong decrease in the referral rate. Further improvement is obtained if the TEOAE measurements are repeated in cases of referral scoring before discharge.     

Preauricular tags and pits in the newborn: the role of hearing tests

The aim of this study was to determine the role of audiometry in a group of newborn infants with preauricular tags or pits. During the 2 years of prospective study, 26 infants were born with preauricular tags or pits (5.7 per 1000 livebirths) and were assessed for hearing impairment by performing behavioural audiometry on day 3 of life and brainstem-evoked response audiometry at 4 months of age. Five infants had associated congenital anomalies (19%). The behavioural audiometry was abnormal in seven infants (27%). The evoked response audiometry was abnormal in 4/23 (17%) newborn infants with isolated tags or pits, and revealed both conductive and/or sensorineural hearing impairment. The incidence of hearing impairment with isolated preauricular tag/pit was significantly higher ( p < 0.001) than the incidence reported in preschool children. We conclude that hearing assessment is recommended in the routine evaluation of the newborn with isolated preauricular tags or pits. This policy may facilitate early diagnosis and treatment of hearing impairment in this population.     

Prediction of permanent hearing loss in high-risk preterm infants at term age

The aim of this series was to assess hearing screenings; auditory brainstem responses (ABR), transient evoked otoacoustic emissions (TEOAE) and free field auditory responses (FF) for the prediction of permanent bilateral hearing loss in high-risk preterm infants at term post-conceptional age. A total of 51 preterm infants (gestational age <34 weeks, birth weight <1500 g) underwent examinations at term and hearing, speech and neurological development were followed up until a corrected age of 18 months. Significant hearing defects were verified by broader ABR examinations under sedation and by clinical ward observation including responsiveness to sounds and enhancement of hearing using an amplification device. Seven bilateral fails in ABR were found, together with nine bilateral fails in TEOAE and four fails in FF screening at term age. Six preterm infants were later confirmed to have a significant permanent bilateral hearing loss, four of whom had also cerebral palsy. Bilateral failure in ABR screening predicted hearing loss with a sensitivity of 100% and a specificity of 98%, TEOAE with a sensitivity of 50% and a specificity of 84% and in the FF examination at the levels of 50% and 98%, respectively. Conclusion Transient evoked otoacoustic emissions alone seem not to be so applicable to the neonatal screening of hearing in high-risk preterm infants as shown earlier in full-term infants, possibly because a hearing defect may be due to retrocochlear damage. Consequently, auditory brainstem response screening seems to be more suitable for very low birth weight preterm infants. Received: 21 September 1999 / Accepted: 5 January 2000     

United Kingdom school-entry hearing screening: current practice.

OBJECTIVE: To determine if the school-entry hearing screening (SEHS) programme continues to make a useful contribution to the identification of childhood hearing impairment in the light of the recent implementation of universal newborn hearing screening, and thereby to inform future policy development. DESIGN: Postal questionnaire survey to determine current implementation and effectiveness of SEHS SETTING: 244 school health services managed within primary care and acute trusts throughout the UK. PARTICIPANTS: 229 SEHS service leads approached; 195 responded. MAIN OUTCOME MEASURE: Details of implementation; positive predictive value of the screening test and its referral criteria. RESULTS: Implementation of the SEHS is variable, and there is no national approach to data collection, audit and quality assurance. Less than 10% of services had available robust data. The yield from screening ranges from 0.05% to 0.59% for permanent sensorineural hearing impairment and from 0.07% to 0.44% for permanent conductive hearing impairment. The positive predictive values from screen referral vary from 0.62% to 12.16% for permanent sensorineural hearing impairment and 1.24% to 17.56% for permanent conductive hearing impairment. CONCLUSION: This comprehensive survey provides a previously unavailable national examination of the SEHS. The few available data on yield indicate that the SEHS may have a small but important role to play in identification of childhood hearing impairment, but the overwhelming conclusion is the urgent need for national guidelines on implementation of this screening programme to determine its value since the implementation nationally of universal newborn hearing screening.     

Outcomes of very‐low‐birthweight infants at 3 years of age born in 2003–2004 in Japan

Background: The aim of this study was to describe and compare neurodevelopmental outcomes with birthweight (BW) groups at 250‐g intervals of very‐low‐birthweight (VLBW) infants at 3 years of age in a multicenter cohort in Japan. Methods: A total of 3104 VLBW infants born in 2003 and 2004 registered in a NICU‐network database were followed in the study. Neurodevelopmental impairment (NDI) was defined as any of the following impairments: cerebral palsy, unilateral or bilateral blindness, severe hearing impairment, or developmental delay; a developmental quotient (DQ) <70 measured using the Kyoto Scale of Psychological Development test or judged by physicians in infants without the test. Results: A total of 257 infants died and follow‐up data were obtained from 1826 infants. Of the 1826 infants, 155 (8.5%) had cerebral palsy, 25 (1.4%) had visual impairment, and 12 (0.7%) had hearing impairment. Of the 1197 infants in whom DQ was measured, 184 (15.4%) had DQ < 70. The proportion of NDI in the evaluated infants was 19.2% (n= 350), ranging from 11.9% (BW 1251–1500 g) to 42.0% (BW ≤ 500 g). Odds ratios (95%CI) of NDI or death against the group BW 1251–1500 g were 20.62 (13.29–31.97) in BW ≤ 500 g, 7.25 (5.45–9.64) in BW 501–750 g, 2.85 (2.12–3.82) in BW 751–1000 g and 1.18 (0.85–1.64) in BW 1001–1250 g. Conclusion: The increasing proportion of NDI or death, an indicator of adverse outcome, was associated with decrement in the BW of the groups. Although we have to consider a bias due to loss of follow‐up data, the incidence of NDI was similar to previous overseas cohort studies despite the higher survival proportion in our study.     

Feasibility of neonatal hearing screening program with two-stage transient otoacoustic emissions in Turkey

BACKGROUND: The objective of this study was to investigate the incidence of hearing loss in neonates and evaluate the feasibility of a two-stage Transient Evoked Otoacoustic Emission (TEOAE) screening test. Maternal concerns about hearing screening were also studied. METHODS: Neonatal intensive care patients and well babies were screened using a two-stage TEOAE test, which was followed by an Auditory Brainstem Response (ABR) test for those babies who failed the first test twice. RESULTS: In total, 711 neonates were screened. At the end of the two TEOAE tests, the cumulative pass rate was 99.3% and false-positive rate was 0.3%. Five neonates (0.7%) were referred for the ABR test. Sensorineural hearing loss was found in three of them (0.4%). Of these three neonates, one was from the well baby nursery and two were from the NICU population. Families generally welcomed the screening program, with no refusals. Positive test results have not caused important maternal concerns. CONCLUSIONS: Congenital hearing impairment is a prevalent disease in Turkey. The two-stage TEOAE program is suitable for the neonatal hearing screening program. In general, hearing screening tests do not cause notable maternal concerns.     

Audiological evaluation of very low birthweight infants

A population of consecutively surviving very low birthweight (VLBW) infants comprising 41 infants (24 female) birthweight less than 1000 g and 108 infants (63 female) birthweight 1000-1500 g received detailed audiological evaluation. The audiological test battery comprised auditory brainstem evoked response (ABR) prior to hospital discharge, behavioural audiometry and tympanometry at 8-12 months and monitoring as necessary. The ABR results were interpreted with reference to a normative group of 36 full-term infants (birthweight 2.4-4.5 kg). Of the 142 VLBW infants completing audiological evaluation, one (0.7%) had evidence of moderate-severe high frequency sensorineural hearing loss, 83 (58.5%) evidence of conductive dysfunction (18 severe, 42 moderate and 23 mild) and only 58 (40.8%) normal hearing. The 19 infants with severe auditory impairment were more likely to have suffered moderate-severe apnoea, greater than or equal to two courses of mechanical ventilation, prolonged oxygen therapy and recurrent upper respiratory tract infections in the first year of life than infants without severe impairment (P less than 0.05). Because of the incidence of conductive pathology, difficulties occurred when attempting to compare ABR status at 36-42 weeks postmenstrual age with peripheral hearing status at 8-12 months as assessed by visual reinforcement orientation audiometry (VROA) and impedance audiometry. The most useful ABR test parameters as screening measures of peripheral auditory status were Wave I-III-V morphology, wave V threshold levels and wave V absolute latency values when used in combination as a test battery.(ABSTRACT TRUNCATED AT 250 WORDS)     

NICU‐only versus universal screening for newborn hearing loss: Population audit

Aim: Targeted newborn hearing screening for infants in neonatal intensive care units (NICUs) may be considered when resources preclude universal newborn hearing screening (UNHS). However, process outcomes have not been compared between stand‐alone NICU hearing screening programs and NICU screening within a full UNHS program. Methods: Comparison of two consecutive hearing screening programs delivered under similar conditions in the four NICUs in Victoria, Australia. All NICU infants were eligible for pre‐discharge automated auditory brainstem response (AABR) hearing screening. Capture, referral and diagnostic data were collected for all NICU infants during the NICU‐only (April 2003–February 2005) and subsequent UNHS (April 2005–June 2006) programs. Results: 4704 eligible infants were admitted during the 23‐month NICU‐only period, and 3160 during the 15‐month UNHS period. Double AABR using ALGO 3i equipment was planned for both programs but, due to clinician concern about this high‐risk clinical population, the NICU‐only protocol was amended to single AABR using AccuScreen equipment. Capture rates were 71.1% (NICU‐only) vs. 95.4% (UNHS) (P < 0.001), successful follow‐up rates were 85.8% vs. 96% (P= 0.004), and mean corrected age at the first audiology appointment was 51.5 vs. 40.2 days (P= 0.05). Conclusions: NICU screening offered within a larger UNHS program outperformed the stand‐alone NICU hearing screening program on all measured parameters. Greater resourcing might address shortcomings of the stand‐alone program but would also reduce its potential savings. The high loss to follow‐up also argues against the often‐advocated approach of referring all NICU infants for diagnostic audiologic testing, bypassing hearing screening altogether.     

Screening of hearing impairment in the newborn using the auditory response cradle.

The Auditory Response Cradle (ARC) is a fully automated microprocessor controlled machine that was designed for the hearing screening of full term neonates. In order to evaluate the ARC, 6000 babies were screened at a district maternity hospital over a period of three years. Every infant subsequently entered a three year follow up programme. One hundred and two babies (1.7%) failed the ARC screen (that is, they failed two ARC tests) and 20 of these were found to have some hearing impairment: in 10 it was severe (80-90 dBHL), in seven moderate (45-60 dBHL), and in three it was mild to moderate (less than 45 dBHL). In addition, of the 20 babies who failed a first test and were discharged before a second could be performed, two were confirmed to have a severe hearing loss; 79 infants failing the screen were cleared on further testing, giving the ARC a false positive rate of 1.3%. On following up all 6000 infants for three years, seven children who passed the neonatal screen were subsequently found to have a hearing loss. For two babies the aetiology was unknown but for five the hearing impairment was either due to a hereditary progressive loss or definite postnatal factors. Progressive and acquired hearing losses cannot be detected at a neonatal screen and this emphasises the need for follow up screens at other stages in the child's life. In this long term study the ARC has been found to have a high detection rate for severe hearing loss and confirms the practical possibility of using a behavioural technique for the universal screening of hearing in neonates.     

Ten-year quality assurance of the nationwide hearing screening programme in Dutch neonatal intensive care units

Aim: To evaluate 10‐year quality assurance of newborn hearing screening (NHS) in Dutch neonatal intensive care units (NICU). Methods: Results of the two‐stage automated auditory brainstem response (AABR) screening and diagnostic examination in NICU graduates were centrally registered between October 1998 and December 2008. This registration facilitates screening, tracking and follow‐up after abnormal screening results. Outcome measures are referral rates, prevalence rate of hearing loss and (trends of) coverage rates and timeliness of follow‐up. Results: Thirty‐two thousand one hundred and two infants have been screened. Referral rates were 9.2% at the first and 26.3% at the second stage. Hearing loss was diagnosed in 728 infants (2.2%). Coverage rates were 98.7% at the first, 92.1% at the second stage, 92.3% for the diagnostic examination and 97.9% for the complete programme. After correction for gestational age, 95.8% of the infants had their first AABR <1 month, 81.8% of the referred infants had their second AABR <6 weeks and 67.1% were diagnosed <3 months. There was a positive trend in referred infants that had their second AABR <6 weeks (p = 0.004) as well as in infants diagnosed <3 months (p < 0.001). Conclusion: The NHS in Dutch NICUs is effective. Timely identification of hearing loss is improving over time.     

Comparison of costs and referral rates of 3 universal newborn hearing screening protocols

OBJECTIVE: To investigate the costs and referral rates of 3 universal newborn hearing screening programs: transient evoked otoacoustic emissions (TEOAE), automated auditory brainstem response (AABR), and a combination, two-step protocol in which TEOAE and AABR are used. STUDY DESIGN: Clinical outcomes (referral rates) from 12,081 newborns at 5 sites were obtained by retrospective analysis. Prospective activity-based costing techniques (n = 1056) in conjunction with cost assumptions were used to analyze the costs based on an assumed annual birth rate of 1500 births. RESULTS: Referral rates differed significantly among the 3 screening protocols (AABR, 3.21%; two-step, 4.67%; TEOAE, 6.49%; P <.01), with AABR achieving the best referral rate at discharge. Although AABR had the lowest referral rate at discharge and the highest pre-discharge costs, the total pre- and post-discharge costs per infant screened (AABR, $32.81; two-step, $33.05; TEOAE, $28.69) and costs per identified child (AABR, $16,405; two-step, $16,527; TEOAE, $14,347) were similar among programs. CONCLUSION: Although AABR incurs higher costs during pre-discharge screening, it has lower referral rates than either the TEOAE or two-step program. As a result, the total costs of newborn hearing screening and diagnosis are similar among the 3 methods studied.     

Parental suspicion and identification of hearing impairment.

The contribution of parental suspicion in the original identification of a 16 year cohort of 171 children with varying degrees of hearing impairment who were screened and identified in childhood was studied. Only a quarter of the children with permanent hearing loss were identified as a result of parental concern. The presence of parental suspicion preceding the audiological diagnosis was also measured. Of the children with severe or profound deafness, the parents only suspected the presence of hearing loss in 44%. Parental suspicion was even lower for those with a mild or moderate permanent hearing loss, and for those with an otherwise symptomless conductive hearing loss caused by otitis media with effusion. Some parents did identify hearing impairment in their children, and parental suspicion should never be professionally disregarded. Most parents, however, experienced initial difficulty in recognising their children''s hearing loss, even when the children were comparatively old. The study confirms the need to continue to identify deafness early by both parental vigilance and sensitive hearing screening programmes.     

State programs for universal newborn hearing screening

Published reports of several statewide and hospital-based systems for universal newborn hearing screening demonstrate that successful large-scale programs that appropriately identify infants with hearing loss in the earliest months of life can be developed. These programs are characterized by nursery-based screening rates of 95% or higher, referral rates of 6% or less, and reasonable per-infant costs. Less data are available regarding the outcome of these screening programs in ensuring confirmation of hearing loss by 3 months of age and initiation of intervention by 6 months of age. The results of the MDNC survey provide important information on the status of newborn hearing screening, audiologic assessment, and intervention services in 16 states. The survey reveals that hospitals have initiated universal newborn hearing screening programs using appropriate technology but that confirmation of hearing loss, fitting of amplification, and enrollment in early intervention are often delayed beyond the JCIH recommendations. Several factors might contribute to late confirmation of hearing loss and delayed amplification and intervention. First, as shown in the Colorado report, lack of a mandatory statewide system for tracking and reporting may delay transition of infants and families from screening to diagnosis, and diagnosis to intervention. In addition, many states lack a centralized system for reporting confirmed hearing loss. Successful statewide programs for universal newborn hearing screening, audiologic diagnosis, and early intervention depend on data-reporting strategies that facilitate transition of infants and families through a system of care. Second, lack of understanding about the urgent need for intervention in the earliest months of life may hinder referral to early intervention programs. Recent data from Colorado's universal newborn hearing screening program reveals that infants who are deaf or who have hearing losses achieve significantly better language development outcomes if intervention begins before age 6 months than infants whose intervention begins after 6 months of age. Hopefully, as these data become more widely available, the compelling need for early intervention will facilitate transition into these services. Although universal newborn hearing screening programs are increasing rapidly, states have not yet developed the coordinated systems for linking universal newborn hearing screening programs to audiologic diagnostic services and audiologic diagnostic services to early intervention programs. Key issues impeding development of these systems may be lack of tracking and reporting systems, lack of standardized guidelines for screening, diagnostic audiologic assessment, hearing aid fitting for very young infants, and lack of understanding about the compelling need for intervention in the earliest months of life. Development of complete systems of care must become a priority for universal newborn hearing screening to provide its ultimate benefit.     

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。     

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.     

Congenital cytomegalovirus infection: association between virus burden in infancy and hearing loss

OBJECTIVE: To determine the relationship between the virus burden in infancy and hearing loss in congenital CMV infection. STUDY DESIGN: A cohort of 76 infants with congenital cytomegalovirus (CMV) infection identified by means of newborn virologic screening was monitored for outcome. The amount of infectious CMV was analyzed in urine specimens obtained during early infancy. Peripheral blood (PB) samples obtained during early infancy were available from 75 children and CMV DNA was quantitated with a real-time quantitative polymerase chain reaction. RESULTS: Infants with clinical abnormalities at birth (symptomatic congenital CMV infection) had higher amounts of CMV in urine (P = .005) and CMV DNA in PB (P = .001) than infants with no symptoms. Eight children with and 4 children without symptoms had hearing loss. Among children without symptoms, those with hearing loss had a significantly greater amount of CMV in urine (P = .03) and PB virus burden (P = .02) during infancy than those with normal hearing. Infants with < 5 x 10(3) pfu/mL of urine CMV and infants with < 1 x 10(4) copies/mL of viral DNA in PB were at a lower risk for hearing loss. CONCLUSION: In children with asymptomatic congenital CMV infection, hearing loss was associated with increased amounts of urine CMV and PB CMV DNA during early infancy.     

Factors influencing the efficacy of universal newborn hearing screening

The debate surrounding the issue of universal hearing screening is being carried out on several levels. Although little disagreement exists over the educational, vocational, and quality-of-life benefits that would result from early identification and timely intervention of congenital hearing loss, the pragmatic issues, such as the effectiveness and the cost benefits associated with universal screening, cannot be ignored. This means that sensitivity, specificity, prevalence, and predictive value remain important factors. Determining the number of infants born with hearing loss in the United States each year, the prevalence issue is key to calculating the predictive value of newborn hearing screening. Emerging from current studies is that estimates of prevalence in the universal newborn population vary from 0.9 in 1000 for permanent bilateral hearing loss of more than 35 dB, to 3.24 in 1000 for bilateral hearing loss, to 5.95 in 1000 when unilateral and moderate hearing loss infants are counted. By comparison, the incidence of hearing loss in the NICU or at-risk population is accepted as high, somewhere between 2% and 4% or 20 to 40 in 1000. Incidence in the NICU varies depending on admission policies and level of care. In general, however, by screening the NICU and targeted at-risk populations, estimated to make up 10% to 16% of the newborn population, half or more of all newborns with severe to profound educationally disabling hearing loss are identified. Data from several well-conducted clinical studies, dating back to the first studies on the use of ABR to screen in the NICU, provide ample justification for the recommendation that all infants admitted to an NICU for longer than 24 hours should be screened for hearing impairment regardless of whether they have any of the at-risk indicators for hearing loss. In the author's opinion, screening in the NICU should be modeled on the operator-controlled ABR protocol outlined by Galambos and colleagues, with the addition that every ABR fail be screened by OAE before discharge. Unlike the targeted NICU population, the question remains for well infants, is sufficient clinical data or evidence available to justify screening all well newborns, specifically those with none of the at-risk for hearing loss indicators cared for in the well-infant nursery and who are discharged home within 24 or 48 hours? With the steady increase in the number of hospital-based universal newborn hearing screening programs implemented since the NIH Consensus Statement, additional data should become available to help resolve several outstanding issues, including prevalence and the predictive value of the various test protocols currently in use or proposed.