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* Abbreviation: DHH — : deaf or hard of hearing Parents and pediatricians agree about the importance of ensuring each child’s optimal physical, emotional, and social development. Largely because of its impact on communication, congenital hearing loss seriously threatens that development. In deciding how to communicate with their children, the parents of children who are deaf or hard of hearing (DHH), 92% of whom have normal hearing,1 have been torn for many years by the acrimonious debate between advocates of sign language versus advocates of aural and oral education. Fortunately, advances in technology and practice over the past 25 years (eg, newborn hearing screening, digital hearing aids, cochlear implants, and early educational intervention) have dramatically improved the opportunities for children who are DHH to learn communication skills critical to optimal development at levels comparable to their hearing peers. The majority of parents with infants who are profoundly deaf are now opting for cochlear implants, and research about the benefits of cochlear implants and early intervention has mitigated some of the historic debate between those advocating for sign language and those advocating for aural and oral education for children who are DHH.2–5 However, the role of sign language in helping children who … Address correspondence to Karl R. White, PhD, Psychology Department, Utah State University, 2615 Old Main Hill, Education Building # 430, Logan, UT 84322. E-mail: karl.white{at}usu.edu

BACKGROUND AND OBJECTIVES: To date, no studies have examined vocabulary outcomes of children meeting all 3 components of the Early Hearing Detection and Intervention (EHDI) guidelines (hearing screening by 1 month, diagnosis of hearing loss by 3 months, and intervention by 6 months of age). The primary purpose of the current study was to examine the impact of the current EHDI 1-3-6 policy on vocabulary outcomes across a wide geographic area. A secondary goal was to confirm the impact of other demographic variables previously reported to be related to language outcomes. METHODS: This was a cross-sectional study of 448 children with bilateral hearing loss between 8 and 39 months of age (mean = 25.3 months, SD = 7.5 months). The children lived in 12 different states and were participating in the National Early Childhood Assessment Project. RESULTS: The combination of 6 factors in a regression analysis accounted for 41% of the variance in vocabulary outcomes. Vocabulary quotients were significantly higher for children who met the EHDI guidelines, were younger, had no additional disabilities, had mild to moderate hearing loss, had parents who were deaf or hard of hearing, and had mothers with higher levels of education. CONCLUSIONS: Vocabulary learning may be enhanced with system improvements that increase the number of children meeting the current early identification and intervention guidelines. In addition, intervention efforts need to focus on preventing widening delays with chronological age, assisting mothers with lower levels of education, and incorporating adults who are deaf/hard-of-hearing in the intervention process.

BACKGROUND AND OBJECTIVES:Cytomegalovirus (CMV) is the most common congenital infection and nongenetic cause of congenital sensorineural hearing loss in the United States. Utah was the first state to pass legislation mandating CMV screening for newborns who fail newborn hearing screening (NBHS). The study objective was to present outcomes of hearing-targeted CMV screening and determine factors predicting CMV screening.METHODS:We used Utah Department of Health HiTrack and Vital Records databases to examine CMV screening from 509 infants who failed NBHS in the 24 months after implementation of the Utah legislation. Multivariate logistic regression analyses were conducted to identify predictors of compliance with CMV screening and diagnostic hearing evaluation.RESULTS:Sixty-two percent of infants who never passed hearing screening underwent CMV screening. Fourteen of 234 infants tested within 21 days were CMV positive; 6 (42.9%) had hearing loss. Seventy-seven percent of eligible infants completed a diagnostic hearing evaluation within 90 days of birth. Compliance with CMV screening was associated with sociodemographic factors, time since the law was enacted, and NBHS protocol. Infants born after the legislation showed greater odds of achieving timely diagnostic hearing evaluation than infants born before the law.CONCLUSIONS:Incorporating CMV screening into an established NBHS program is a viable option for the identification of CMV in infants failing NBHS. The addition of CMV testing can help a NBHS program attain timely audiological diagnostics within 90 days, an important early hearing detection and intervention milestone.

OBJECTIVES: Otoacoustic emissions (OAE) technology, used widely in newborn hearing screening programs and validated by professional organizations as a reliable and objective tool, is beginning to be recognized as superior to subjective methods when screening young children in a variety of settings. This study examines the efficacy of integrating OAE hearing screening into services routinely provided in health care settings. METHODS: Three federally funded clinics serving low-income and uninsured people in a metropolitan area participated in the 10-month study. Subjects included 846 children (842 in the target population RESULTS: Of the 846 children screened, 814 (96%) ultimately passed the screening or audiological assessment and 29 (3%) exited the study. Three children (1 was 5) were identified with permanent hearing loss. CONCLUSIONS: The rate of identification of permanent hearing loss in this study is similar to findings from a study of OAE screening in early childhood educational settings. OAE screening holds the potential for being an effective method for helping to identify young children with permanent hearing loss in primary care settings.

BACKGROUND: In a 2-stage neonatal hearing screening protocol, if an infant fails the first-stage screening with an otoacoustic emissions test, an automated auditory brainstem response (ABR) test is performed. The purpose of this study was to estimate the rate of hearing loss detected by first-stage otoacoustic emissions test but missed by second-stage automated ABR testing. METHODS: The data of 17 078 infants who were born at Lis Maternity Hospital between January 2013 and June 2014 were reviewed. Infants who failed screening with a transient evoked otoacoustic emissions (TEOAE) test and infants admitted to the NICU for more than 5 days underwent screening with an automated ABR test at 45 decibel hearing level (dB HL). All infants who failed screening with TEOAE were referred to a follow-up evaluation at the hearing clinic. RESULTS: Twenty-four percent of the infants who failed the TEOAE and passed the automated ABR hearing screening tests were eventually diagnosed with hearing loss by diagnostic ABR testing (22/90). They comprised 52% of all of the infants in the birth cohort who were diagnosed with permanent or persistent hearing loss >25 dB HL in 1 or both ears (22/42). Hearing loss >45 dB HL, which is considered to be in the range of moderate to profound severity, was diagnosed in 36% of the infants in this group (8/22), comprising 42% of the infants with hearing loss of this degree (8/19). CONCLUSIONS: The sensitivity of the diverse response detection methods of automated ABR devices needs to be further empirically evaluated.

* Abbreviations: CMV — : cytomegalovirus cCMV — : congenital cytomegalovirus NBHS — : newborn hearing screening SNHL — : sensorineural hearing loss Congenital cytomegalovirus (cCMV) infection is a common and yet underappreciated cause of hearing loss and neurodevelopment disability in US children.1 Any opportunity to achieve early detection of cCMV and provide interventions warrants careful consideration. Diener et al2 in this issue document the experience with targeted screening for cCMV in Utah among infants who do not pass newborn hearing screening (NBHS). Since July 2013, Utah has required referral for testing for cytomegalovirus (CMV) within 21 days of birth for newborns who do not pass NBHS and follow-up outpatient screening.3 Most notably, the introduction of targeted screening for cCMV, along with state-funded public education about cCMV, was associated with an increase from 56% to 77% in timely diagnostic audiology follow-up (

This statement endorses the implementation of universal newborn hearing screening. In addition, the statement reviews the primary objectives, important components, and recommended screening parameters that characterize an effective universal newborn hearing screening program.

OBJECTIVE. This study was conducted to determine whether incidental jaundice affects automated auditory brainstem response results.METHODS. We reviewed the medical charts of jaundiced newborns of ≥34 weeks of gestation who underwent automated auditory brainstem response testing within 4 hours of plasma total bilirubin concentration and unbound bilirubin concentration measurements. We tested the hypothesis that the likelihood of abnormal automated auditory brainstem response results would increase as total bilirubin and unbound bilirubin concentrations increased.RESULTS. Forty-four infants with proximate total bilirubin concentration, unbound bilirubin concentration, and automated auditory brainstem response measurements were identified, and 4 (9%) had bilateral refer automated auditory brainstem response results. The mean total bilirubin concentration of 21.4 mg/dL (SD: 4.0 mg/dL; range: 14.4–29.5 mg/dL) for the 40 infants with bilateral pass automated auditory brainstem response results was not significantly different from that of 23.0 mg/dL (range: 14.9–33.1 mg/dL) for the 4 infants with bilateral refer automated auditory brainstem response results. However, the mean unbound bilirubin concentration of 1.32 μg/dL (range: 0.22–2.99 μg/dL) for the 40 infants with bilateral pass results was significantly lower than the mean of 2.62 μg/dL (range: 0.88–4.41 μg/dL) for the 4 infants with bilateral refer results. Logistic regression showed that increasing unbound bilirubin concentrations but not increasing total bilirubin concentrations were associated with of bilateral refer automated auditory brainstem response results.CONCLUSIONS. The probability of bilateral refer automated auditory brainstem response results increases significantly with increasing unbound bilirubin concentrations but not with increasing total bilirubin concentrations. Because unbound bilirubin concentrations are also more closely correlated with bilirubin neurotoxicity than are total bilirubin concentrations, bilateral refer automated auditory brainstem response results for jaundiced newborns may indicate increased risk of bilirubin neurotoxicity, in addition to the possibility of congenital deafness.

Every year, 4000 to 8000 children are born in the United States with permanent hearing losses that pose a risk to their speech and language development.1–3 If children with milder losses and losses that affect only 1 ear are included, that number is almost doubled. Until just a decade ago, late diagnosis was the norm, with the average age at identification of congenital hearing loss reported as 2½ to 3 years or even later.1,4 A valuable window of opportunity for early intervention was being lost. Intervention in the first 6 months of life may be particularly important for speech and language development,5,6 and parents and professionals overwhelmingly prefer early diagnosis.7,8 Technologic advances in automated newborn hearing-screening technology, followed by a series of successful demonstration projects,9 resulted in the introduction of universal newborn hearing screening across the United States in the mid-to-late 1990s. More than 95% of newborns are now screened for hearing loss shortly after birth, which represents one of the most successful and rapid examples of research moving into practice in pediatric public health. Newborn hearing screening is 1 of only 7 preventive services for children recommended by the US Preventive Services Task Force.10 The success of the screening program depends on far more than the initial screen. The Joint Committee on Infant Hearing has set 3 goals: screening should be completed by 1 month of age, diagnosis should be made by 3 months, and intervention and treatment should commence by 6 months.11 Although the first goal is close to being met, the other goals are not. Recent data from the Centers for Disease Control and Prevention suggest that up to 60% of newborns who do not pass the hearing screen do not have … Address correspondence to Shirley A. Russ, MD, MPH, Division of Academic Primary Care Pediatrics, 8700 Beverly Blvd, Room 1165W, Los Angeles, CA 90048. E-mail: shirlyruss{at}aol.com

During the last 20 years, the number of infants evaluated for permanent hearing loss at birth has increased dramatically with universal newborn hearing screening and intervention (UNHSI) programs operating in all US states and many territories. One of the most urgent challenges of UNHSI programs involves loss to follow-up among families whose infants screen positive for hearing loss. We surveyed 55 state and territorial UNHSI programs and conducted site visits with 8 state programs to evaluate progress in reaching program goals and to identify barriers to successful follow-up. We conclude that programs have made great strides in screening infants for hearing loss, but barriers to linking families of infants who do not pass the screening to further follow-up remain. We identified 4 areas in which there were barriers to follow-up (lack of service-system capacity, lack of provider knowledge, challenges to families in obtaining services, and information gaps), as well as successful strategies used by some states to address barriers within each of these areas. We also identified 5 key areas for future program improvements: (1) improving data systems to support surveillance and follow-up activities; (2) ensuring that all infants have a medical home; (3) building capacity beyond identified providers; (4) developing family support services; and (5) promoting the importance of early detection.

To the Editor. Dr Paradise's commentary appearing in the March 1999 issue of Pediatrics raises to my mind 2 questions regarding universal neonatal hearing screening: Is this an appropriate time to institute universal neonatal screening? Would it be more appropriate instead to institute a 2-step screening process consisting of a high-risk questionnaire followed up with neonatal screening when indicated?1 In general, decisions regarding the selection of screening techniques have largely been made by the test developers or specialty groups, such as the American Speech-Language-Hearing Association, and the rationale for their decisions is often unknown. However, since society is either benefiting from the test or paying the costs of errors, decisions regarding the use of the screening tests should reflect the values of society. This point is of particular significance, as some states have already mandated universal neonatal hearing screening. Therefore, it would behoove health professionals making such decisions to incorporate societal values in the decision-making process. One approach to achieve this is through an approach called decision analysis.2,3 Although the costs of screening, rescreening, diagnosis, and treatment may not be so difficult to obtain from the literature, the economic gain is more difficult to assess in view of the fact that about 30% of those with sensory neural hearing loss may have concomitant developmental disabilities such as mental retardation and cerebral palsy.4 Factors such as intrinsic gain, parental anxiety, and the value of identifying normal individuals can be obtained by sampling a cross-section of society with questionnaires. The second step is to sum the positive and negative factors in each of the 4 cells. The third step is review the literature for large-scale clinical trials of a test such as one for universal neonatal hearing screening to determine the relative frequency with which subjects fall into each …

Across the nation pediatricians are being importuned, and indeed propelled, to implement universal newborn hearing screening, despite a total lack of information concerning ultimate costs and, particularly, risks. Without question, early detection of severe sensorineural hearing loss is a laudable goal, to be pursued devoutly. However, I would contend again, as Fred Bess and I argued in a commentary in Pediatrics 5 years ago,1 that universal newborn hearing screening in our present state of knowledge is not necessarily the only, or the best, or the most cost-effective way to achieve that goal, and more importantly, that the benefits of universal newborn hearing screening may be outweighed by its risks. The nagging question of ultimate costs and risks that Bess and I posed in that commentary remains unanswered, and in fact, virtually unaddressed. Advocacy for screening on a national scale gained momentum after the 1993 publication of a National Institutes of Health (NIH) Consensus Statement that arose out of a Consensus Development Conference convened jointly by the National Institute on Deafness and Other Communication Disorders and the NIH Office of Medical Applications of Research, and cosponsored by the National Institute of Child Health and Human Development and the National Institute of Neurological Disorders and Stroke. The NIH Consensus Statement recommended that all infants be screened for hearing impairment within the first 3 months of life, and preferably before discharge from the hospital newborn nursery.2 Bess's and my commentary disputing the wisdom of that recommendation provoked a flurry of mainly dissenting, and in some cases, indignant letters to the editor, mainly from members of the audiology community ( Pediatrics 1994;94:948–959). These provided us with the opportunity, in our responses, to explain and expand on our position in greater detail.3 Together, the letters and our replies provide a comprehensive and still …

DESCRIPTION. This is the 2008 update of the 2001 US Preventive Services Task Force recommendation on universal newborn hearing screening. METHODS. The US Preventive Services Task Force weighed the benefits and harms of universal newborn hearing screening, incorporating new evidence addressing gaps identified in the 2001 US Preventive Services Task Force recommendation statement. Published literature on this topic was identified (by using Medline and Cochrane databases) and systematically reviewed. RECOMMENDATION. Screen for hearing loss in all newborn infants (B recommendation).

OBJECTIVES. The objectives were to determine the frequency of congenital cytomegalovirus infection among newborns who did not pass hearing screening tests or had confirmed hearing loss and to determine how often abnormal hearing screening results were the only manifestation of congenital cytomegalovirus infection.METHODS. Retrospective chart review was performed for newborns who had abnormal hearing screening results and positive urine cytomegalovirus culture results at Parkland Memorial Hospital between September 1, 1999, and August 31, 2004.RESULTS. During the 5-year study period, 572 of 79047 newborns (7 of 1000 live births) did not pass hearing screening tests. Cytomegalovirus infection was identified in 24 (5%) of 483 tested infants and 16 (6%) of the 256 infants with subsequently confirmed hearing impairment. Of those 16 infants, 12 (75%) were identified as having congenital cytomegalovirus infection only because of failure to pass newborn hearing screening tests.CONCLUSIONS. Congenital cytomegalovirus infection was present for 6% of newborns with confirmed hearing impairment, and the majority of those infants were identified on the basis of abnormal newborn hearing screening results.

OBJECTIVE. The objective of this study was to determine the effectiveness in routine practice of the first phase of a national population-based newborn hearing screening and follow-up program that seeks to identify infants with bilateral permanent hearing loss of ≥40-dB hearing loss. METHODS. The study was a part of the independent evaluation of the 23 first phase sites (annual birth population ∼120000) of the national newborn hearing screening program in England. For each infant identified with the defined hearing loss, the measures of interest were degree and type of hearing loss, presence of risk factors, age of first audiologic assessment, age of identification of hearing loss, age of enrollment in an early support program, and age of hearing aid fitting. Data collection took place over the first 2 years of the program. RESULTS. Data were provided on 169 infants with permanent bilateral moderate or greater hearing loss identified through screening 169487 infants. Fifty-four percent of all cases were from an “at-risk” population. Three fourths of these “at-risk” infants spent ≥48 hours in the NICU. For the whole sample, the median age at first audiologic assessment was 5 weeks; the median age of identification of the hearing loss and of enrollment in early support program was 10 weeks irrespective of the degree of hearing loss; and the median age at hearing aid fitting was 16 weeks. Infants with moderate hearing loss were fitted with hearing aids significantly later than those with severe and profound hearing loss. CONCLUSIONS. Properly implemented, a newborn hearing screening program based on whole populations and routine service provision can deliver satisfactory outcomes in terms of age of referral, identification, and intervention. The distribution of degree and type of hearing loss and proportion with risk factors was similar to that expected. The numbers identified were such as to suggest that very few cases were missed by the screening program.

Objectives. As a result of universal newborn hearing screening and improved evaluation tools, many children with severe to profound hearing loss are being diagnosed as infants. This affords the opportunity to provide these children access to cochlear implantation, although medical and audiologic challenges must be addressed. The purpose of this study was to investigate the safety and efficacy of cochlear implantation in children who are younger than 1 year. Methods. A prospective study was conducted of 18 children who had confirmed severe to profound sensorineural hearing loss and received cochlear implants at our medical center before 12 months of age. The length of device usage ranged from 6 months to 4 years, 5 months. The main outcomes measured were perioperative and postoperative surgical/medical aspects, the Infant-Toddler Meaningful Auditory Integration Scale and age-appropriate phoneme, and word and sentence recognition tests, when appropriate. Results. All children had full insertions of the electrode array without surgical complications and are developing age-appropriate auditory perception and oral language skills. Conclusions. Early implantation is feasible and beneficial in some children who are younger than 12 months and should be considered with attention to variables involved in the decision-making process, including possible increased surgical risk, skull size and scalp thickness, and mastoid development.

Objective. Although previous studies have documented the feasibility and benefits of universal newborn hearing screening in selected hospitals, none have reviewed the effectiveness of regionally mandated participation of large numbers of hospitals with variable levels of motivation to succeed. The purpose of this study was to measure hospital participation and overall screening success in a statewide program for universal newborn hearing screening and to track improvements in program establishment and outpatient follow-up over time. Methods. Four Colorado hospitals began voluntarily performing hearing screening before hospital discharge on all newborns in 1992. By 1996, 26 Colorado hospitals were participating in universal newborn hearing screening. The publication of screening results from these early years served as a catalyst for legislation requiring increased hospital participation in establishing universal screening programs. Data systems were subsequently developed to improve statistical tracking and follow-up. Eight years’ worth of cumulative study data as well as the results from calendar year 1999 (the year of greatest hospital participation) were reviewed for collective measures of successful screening and follow-up. Three hospitals did not initiate newborn hearing screening programs until after the study period ended in 1999. Of the 57 hospitals that were screening newborns in 1999, the chosen method of screening at 52 hospitals was automated auditory brainstem response testing; 3 hospitals used otoacoustic emission testing, and the remaining 2 hospitals used 2-stage screening. Hearing loss was defined as a threshold of 35 decibels or greater in 1 or both ears at the time of confirmatory testing. Results. During the full 8-year study period, 1992 to 1999, 148 240 newborns were screened. A total of 291 infants who were born during the study period received a diagnosis of congenital hearing loss. In this cohort of 291 children, the cumulative frequency of bilateral hearing loss was 71% (range: 48%–94% by calendar year), the frequency of sensorineural hearing loss was 82% (range: 67%–88%), and the frequency of 1 or more risk factors was 47% (range: 37%–61%). During calendar year 1999, a total of 63 590 births were recorded at 60 birthing hospitals in Colorado. The families of 263 (0.4%) of these newborns refused newborn hearing screening. Of the remaining 63 327 newborns, 87% (55 324 infants) were screened for hearing acuity before hospital discharge, a far greater percentage than the 19% of all newborns screened during the first 5 years of voluntary hospital participation, and approaching the American Academy of Pediatrics’s recommendation of 95% of newborns completing hospital-based testing in a successful screening program. As a result of this statewide hearing screening program, congenital hearing loss was diagnosed in 86 Colorado newborns during 1999, representing an occurrence rate of approximately 1 affected child in every 650 newborns. In this group of 86 infants, 59 had bilateral sensorineural hearing loss, 17 had unilateral sensorineural hearing loss, 4 had bilateral conductive hearing loss, and 6 had unilateral conductive hearing loss. Mild hearing loss was present in 6 infants, moderate hearing loss was present in 42 infants, severe hearing loss was present in 33 infants, and profound hearing loss was present in the remaining 5 infants. Only 32 of the 86 affected newborns in 1999 had 1 or more risk factors for hearing loss subsequently identified. After failing an initial hospital-based screening at 1 of the 57 participating hospitals in 1999, 2.3% of infants screened (1283 newborns) were referred for follow-up testing, easily exceeding the standard of Conclusions. Universal screening for congenital hearing loss is demonstrated to be feasible in a large regional effort of legislatively mandated participation. The success of such an endeavor is dependent on educational efforts for community professionals, commitment on the part of program planners, and data systems that more accurately track and recall infants who fail initial hospital-based screening.

Objective.A positive test result from universal newborn hearing screening (UNHS) has been suspected to cause maternal concern. However, findings so far are inconclusive. Against the background of a 2-stage UNHS protocol, we investigated the extent of maternal concern in 2 different situations: 1) mothers' immediate reactions after a positive result at the first-stage test and 2) maternal concern during a 1-month period while waiting for the infant's hearing assessment after the infant also failed the retest (ie, the screening). In addition, we checked whether mothers who are informed by an audiologist about the low predictive validity of positive test results in hearing screening are less concerned about a positive result than mothers who are not informed.Methods.A prospective study was conducted over a 1-year period, in which all mothers whose infants tested positive in the first stage or failed the screening were questioned about their level of concern attributable to the positive test result.Results.Of 85 mothers whose infants tested positive in the first-stage test (situation 1), 34 (40%) did not know the result. Of the remaining 51 mothers, 59% were not at all concerned and 27% were only slightly concerned about the result, whereas 14% stated that they were highly concerned. In an additional sample of 43 mothers whose infants failed the screening (situation 2), 42% reported not being worried and 37% only slightly worried, whereas 21% were highly concerned about the positive screening result. No effect of information about the low predictive validity of positive test results on the extent of maternal concern could be ascertained.Conclusions.The results of this study contradict the findings of some previous surveys that reported considerably higher levels of maternal concern after a positive test in UNHS. The failure to demonstrate the impact of information on maternal concern might be attributable to the fact that the number of uninformed mothers was too small to affect our results perceptibly.

Objective.To examine the effect of test environment on recording transient-evoked otoacoustic emissions (TEOAE) in neonates.Methods.Thirty-two infants from the newborn nursery (NBN) who passed a screening auditory brainstem response (SABR) test and were at least 34 weeks' postconceptional age were studied. One ear of each newborn was tested using TEOAE in 5 different test environments: open bassinet in the NBN (E1), working isolet located in the NBN (E2), nonfunctioning isolet in the NBN (E3), nonfunctioning isolet in a quiet room off the NBN (E4), and open bassinet in a quiet room (E5). The number of high noise samples (HNS), the test duration (in seconds), the signal-to-noise ratio (SNR; in dB) measured at bandwidths centered at 1.6, 2.4, 3.2, and 4.0 kHz, and the percentage of neonates with a fail screening outcome based on a common pass–fail screening criteria were compared in the 5 test environments.Results.There were statistically significant differences in the number of HNS accumulated in the 5 test environments (F = 6.79). The use of a nonfunctioning isolet in both the NBN and within a room off the NBN (E3 and E4, respectively) resulted in significantly fewer HNS than when TEOAEs were recorded in the other 3 test environments (E1, E2, and E5). Mean test duration was significantly different among the 5 locations (F = 6.53). Posthoc analyses revealed that test time in E3 and E4 was significantly shorter than in E1 and E2. The percentage of newborns with a fail (≤3 dB SNR at 2.4, 3.2, and 4.0 kHz) outcome was lowest in E3 (6.3%) and the same in E1 and E4 (12.5%). A high percentage of infants received a fail outcome when tested in both the working isolet and in the open bassinet in a room off the nursery (21.8% and 25%, respectively). SNR (in dB) for bandwidths centered at 1.6, 2.4, and 3.2 kHz was negatively correlated with the HNS in the working isolet. SNR (in dB) at 4.0 kHz was negatively correlated with the HNS when TEOAEs were recorded in the open bassinet in a room adjacent to the NBN. The number of HNS was correlated with overall test time in each environment.Conclusions.Test environments typically used for newborn hearing screening can influence the recording of TEOAEs. Performing the TEOAE test with the neonate placed in a nonfunctioning isolet located in either the NBN or in a room off of the NBN resulted in the most desirable outcomes (shortest test times, fewest HNS, highest SNR (in dB), and fewest fail outcomes).

To the Editor. I congratulate Dr Mehl and Ms Thomson1 for their excellent analysis of newborn hearing screening in Colorado. Their results and recommendations are duly noted. In analyzing the cases of sensorineural hearing loss, I ask whether they detected any infants who had both hearing loss and congenital hypothyroidism. This question relates to the issue of Pendred syndrome, an autosomal dominant disease, that includes sensorineural deafness and hypothyroidism, which occur with variable penetrance. Thus, there may be infants within the same kindred with various combinations of mild-moderate sensorineural hearing loss and/or congenital hypothyroidism, associated with thyroid dyshormonogenesis. I actually follow a couple of these families in which there are some members with varying degrees of sensorineural deafness, some with congenital hypothyroidism, some with both, and some who are unaffected. My additional recommendation, therefore, would be that if any infant is recognized on screening to have sensorineural hearing loss, …

To the Editor .— Does universal newborn hearing screening (UNHS) save money by reducing expenditures on special education services for children with congenital hearing loss? An article by Schroeder et al1 in the April issue of Pediatrics provides the first empirical evidence to address this important question. The investigators produced an important new analysis of economic costs of medical and special education for children with permanent, bilateral congenital hearing loss (PCHL) and their families in selected areas of England. Here we situate their findings in the context of US-policy discussions on UNHS. Schroeder et al compared costs among children with PCHL aged 7 to 9 years who were born in areas with or without UNHS programs, including children who participated in the Wessex trial of UNHS.2 The authors …

In Reply .— We appreciate the comments on our article by Grosse and Ross, specifically relating it to the broader issue of early hearing detection and intervention programs that build on universal newborn hearing screening. It is particularly gratifying to see the results of the article applied to US interventions. The study reported in our article shows reliably that there are substantial increases in costs at 7 to 9 years in children with permanent childhood hearing impairment compared with …

To the Editor .— The study by Mehl and Thomson1 concerning the Colorado Newborn Hearing Screening Project, 1992–1999, is an excellent demonstration of the feasibility and benefits of setting up, operating, and improving a statewide hospital-based universal newborn hearing screening, termed the Early Hearing Detection and Intervention (EHDI) program. The success of this program should be an encouragement to other states without fully developed EHDI programs to emulate Colorado’s efforts. The March of Dimes, the American Academy of Pediatrics, the Centers for Disease Control and Prevention, the Health Resources and Services Administration, the American Academy of Audiology, the American Speech-Language-Hearing Association, and others strongly support EHDI programs that are of high quality and provide appropriate follow-up, diagnosis, treatment, and program evaluation.2 There is a pressing need for ongoing systematic evaluation of EHDI programs so that lack of …

Background and Objectives. The false-positive rates of previously reported universal newborn hearing screening (UNHS) programs range between 2.5% and 8%. Critics of UNHS programs have claimed that this rate is too high and might lead to a number of the negative effects produced by false-positive screening tests, namely emotional trauma, disease labeling, iatrogenesis from unnecessary testing, and increased expense in terms of time and money.We previously reported, based on some preliminary data, that as many as 80% of newborns who failed the initial hearing screen subsequently passed when they were retested the following day, before being discharged from the hospital. We now present the results of this intervention for our entire UNHS program during a 7-month period.Methods.We analyzed data from 3142 non-neonatal intensive care unit infants screened with an automated auditory brainstem response at the Women's Hospital of Greensboro from November 1, 1999 to May 31, 2000. A protocol was developed wherein all infants who failed the initial UNHS were rescreened with another automated auditory brainstem response before hospital discharge. Data collected included pass/fail rates during the inpatient stay as well as follow-up data and risk factors for congenital hearing loss.Results.Confirmed hearing loss occurred in 8 nonneonatal intensive care unit infants, a rate of 2.5/1000. Eighty percent of newborns who failed the initial hearing screen passed on rescreening before hospital discharge. This produced a false-positive rate of 0.8% and a corresponding positive predictive value of 24%. If inhospital rescreening had not occurred, our false-positive rate and positive predictive value would have been 3.9% and 6.1%, respectively.Conclusions.Our simple intervention of rescreening all infants who failed their initial UNHS before hospital discharge reduced the false-positive rate of UNHS to 0.8%. We suggest that this simple, inexpensive intervention should be instituted for all similar UNHS programs.

Objectives.Concern has been raised about the frequency and subsequent emotional effect of a false-positive result during universal newborn hearing screening (UNHS). This study describes: 1) the results of 1 UNHS program and a potential method to significantly reduce the false-positive rate, and 2) the effect a false-positive result has on lasting maternal anxiety toward their children as well as their views toward UNHS in general.Methods.A retrospective analysis was conducted using data from 5010 infants screened with an automated auditory brainstem response (ABR) at the Women's Hospital of Greensboro (WHOG) from July 6, 1998 to June 30, 1999. In addition, a structured telephone survey was given to mothers of infants who had failed the initial hearing screen (stage 1) and who had completed an outpatient rescreen (stage 2).Results.Confirmed hearing loss occurred in non-neonatal intensive care unit infants at a rate of 1.8/1000. A false-positive rate of 1.9% occurred during stage 1 of UNHS (screening before newborn discharge). We attribute this relatively low rate to rescreening of 51% of those newborns who failed the initial screen before hospital discharge. Eighty percent of these rescreened infants passed, thus needing no additional follow-up. If we had rescreened all infants before discharge, the false-positive rate would have approached .5%.Results of the survey were reassuring with regard to lasting emotional effects of false-positive tests. Only 9% of mothers said they “treated their child differently” before outpatient rescreening, and only 14% reported any lasting anxiety after their child passed the outpatient repeat screen. Although none reached statistical significance, potential risk factors for lasting anxiety include more educated mothers, lack of understanding of UNHS, and a false-positive result in both stage 1 and stage 2. Over 90% of all mothers believed that UNHS was a good idea.Conclusions.By rescreening all infants before hospital discharge, the false-positive rate of UNHS performed using automated ABR can be reduced to

Objective. To perform hearing screenings on all newborns before hospital discharge, using auditory brainstem evoked responses with analysis of time, cost, and failure rates to evaluate and determine the screening practicality. Method. Over a 3-year period from January 1, 1993 to December 31, 1995, auditory brainstem evoked response screenings were performed on 15 749 infants born at Saint Barnabas Medical Center, Livingston, New Jersey, before their hospital discharge by certified/licensed audiologists. The auditory brainstem evoked response screenings were conducted using the Nicolet Compass Evoked Potential System. Results. A 3-year experience of testing 15 749 infants proved to be a cost-effective program with costs less than $30.00/baby. To date, 46 babies have been identified with bilateral sensorineural hearing loss and 6 babies with unilateral sensorineural hearing loss. Conclusions. The universal newborn hearing screening program at Saint Barnabas Medical Center has proved to be effective, beneficial, and necessary for an institution with more than 5000 births, annually. Early identification of hearing loss has resulted in infants receiving early intervention, and the screening program has provided education and follow-up services to both parents and physicians. auditory brainstem evoked responses, early hospital discharge of newborns, early identification of hearing loss, screening auditory brainstem evoked responses, universal newborn hearing screening.

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.

This article provides background information on acoustic impedance testing and illustrates utilization of test results in pediatric clinical practice. Acoustic impedance measurement devices are presently being used successfully by hearing screening programs and by otorhinolaryngologists in clinical and hospital settings. As a diagnostic tool, the acoustic impedance bridge can have particular applicability in dealing with young children and other difficut-to-test populations. Impedance measurements can provide physicians with objective information about the condition of the middle ear, oftentimes more accurately than either otoscopic examination or standard audiometry. The technique described can assist physicians in diagnosing middleear diseases and in monitoring the effects of otological treatment.

The use of acoustic impedance measurement as a means of evaluating the physiological function of the middle ear system is gaining wide interest by pediatricians.1-3 The impedance technique is extremely sensitive to differentiating between normal and pathological middle ears. Children with middle ear effusions are typically identified by physicians through an otoscopic examination, or by audiologists when the child fails the traditional hearing screening test at school. Most physicians use otoscopy to visually examine the tympanic membrane, but with varying degrees of success.4 Otologists teach that pneumatic otoscopy is an absolute necessity to identify the presence of middle ear disease, yet only 25% of physicians use the pneumatic otoscope.5 In a recent study of the accuracy of otoscopic diagnoses, 15% to 20% of ears in children under 3 years of age with effusions were missed clinically. This high miss rate was noted in a group of examiners from a medical center who were aware that their diagnostic skill was being evaluated and thus should have been motivated to perform their most careful otoscopy.6 Accurate otoscopy and pneumotoscopy require skilled and experienced individuals. Our experience in training health professionals shows considerable variability in interpretation of otoscopic observations. Other problems in otoscopy include difficulty in visualizing the tympanic membrane, the removal of cerumen prior to examination, and the unwillingness of some children to cooperate with the examination. TYMPANOMETRY Tympanometry resolves many of the disadvantages of conventional audiology and otologic assessment. It requires only passive cooperation from children since no overt response is required.

The subject of this communication is a modifled technique for hearing screening in young children that is quick, easy to use, and appears to be effective. In the original method described by Hardy, et al.,1 based on earlier work by Ewing and Ewing,2 the child sits on his mother's lap, facing another adult who attracts the child's attention with a toy. A third adult crouches behind the child, out of sight, and operates a variety of noise makers. The hearing child usually responds by turning his head toward the sound. This technique is most suitable for the 9-month to 18-month age group when the patient is too old for newborn screening techniques and too young for conventional audiometry and when deafness may not yet be suspected from failure of normal language development.