An evaluation of the cross-check principle using visual reinforcement audiometry, otoacoustic emissions, and tympanometry.

BACKGROUND: Early intervention to reduce the effects of congenital hearing loss requires accurate description of hearing loss. In pediatric audiology, a cross-check principle is used to compare behavioral and physiological tests. PURPOSE: The purpose of this study was to investigate the correspondence of visual reinforcement audiometry (VRA) minimal response levels (MRLs), otoacoustic emissions (OAEs), tympanometry, and VRA test reliability to determine the odds of obtaining the expected cross-check results. We hypothesized that (1) when MRLs were within normal limits (WNL), OAEs would be present; (2) in the event of normal MRLs and absent OAEs, tympanograms would be abnormal; and (3) in the event of elevated MRLs and present OAEs, the tester's confidence in the MRLs would be judged to be only fair, rather than good. RESEARCH DESIGN: This was a retrospective study. STUDY SAMPLE: A previous study provided data from 993 infants who had diagnostic audiologic evaluations at 8-12 mo of age. DATA COLLECTION AND ANALYSIS: The data were analyzed to compare VRA MRLs with OAE signal-to-noise ratios at 1, 2, and 4 kHz. Odds ratios and 95% confidence intervals were calculated to test the three hypotheses related to the correspondence among MRLs, OAEs, tympanometry, and the reliability of MRLs. RESULTS: The probability that OAEs would be present when MRLs were WNL varied from 12 to 26 to 1, depending on the test frequency. When OAEs were absent in the presence of normal MRLs, the odds of abnormal tympanometry varied from 5 to 10 to 1, depending on the test frequency. When MRLs were elevated (>20 dB HL), the odds suggested that examiners judged the MRLs at 1 and 2 kHz to lack reliability. CONCLUSION: The results suggest that the cross-check principle is effective when employing VRA, OAE, and tympanometry to rule out or determine the degree, type, and configuration of hearing loss in infants.

Effects of universal newborn hearing screening on an early intervention program for children with hearing loss, birth to 3 yr of age.

BACKGROUND: Universal Newborn Hearing Screening (UNHS) was introduced in Kansas in 1999. Prior to UNHS a small percentage of newborns were screened for and identified with hearing loss. PURPOSE: The purpose of this study was to determine the effects of UNHS on a local early intervention (EI) program for young children with hearing loss. RESEARCH DESIGN: This was a retrospective study based on the chart review of children enrolled in the EI program during target years before and after the establishment of UNHS. STUDY SAMPLE: Charts for 145 children were reviewed. DATA COLLECTION AND ANALYSIS: The chart review targeted the following aspects of the EI program: caseload size, percentage of caseload identified by UNHS, age of diagnosis, age of enrollment in EI, degree of hearing loss, etiology of hearing loss, late onset of hearing loss, age of hearing aid fit, percentage of children fit with hearing aids by 6 mo, percentage of children with profound hearing loss with cochlear implants, and percentage of children with additional disabilities. RESULTS: Changes in the EI program that occurred after UNHS were increases in caseload size, percentage of caseload identified by UNHS, percentage of children fit with hearing aids by 6 mo of age, and percentage of children with profound hearing loss with cochlear implants. There were decreases in age of diagnosis, age of enrollment in EI, and age of hearing aid fit. Before UNHS, the majority of children had severe and profound hearing loss; after UNHS there were more children with mild and moderate hearing loss. The percentage of known etiology and late-onset hearing loss was approximately the same before and after UNHS, as was the percentage of children with additional disabilities. CONCLUSION: UNHS had a positive impact on caseload size, age of diagnosis, age of enrollment in EI, and age of hearing aid fit. The percentage of the caseload identified in the newborn period was about 25% before UNHS and over 80% after its implementation. After UNHS, the EI caseload included as many children with mild and moderate hearing loss as with severe and profound loss. By the last reporting year in the study (academic year 2005-2006) all children with profound hearing losses had cochlear implants.

Current state of knowledge: implications for developmental research--key issues.

This is the final article in a series of five review articles and one editorial that summarizes the proceedings of the National Institute on Deafness and Other Communication Disorders workshop on Outcomes Research in Children with Hearing Loss held December 12 and 13, 2006. The purpose of this article is to highlight the recommendations that emerged during the workshop, addressing the developmental needs of children with hearing impairment. The information in this summary is based on the National Institute on Deafness and Other Communication Disorders workshop minutes available at http://www.nidcd.nih.gov/funding/programs/hb/outcomes/recommendations.htm. General research issues focus on funding mechanisms, access to a sufficient population, experimental cohorts and control groups, research methods and outcomes, standardization of testing across agencies and intervention programs, and facilitating access to data. Discussion of those issues is followed by an outline that presents future needs in outcomes research, including research design, sources of variance, research needs and gaps, and development of measurement tools.

A multicenter evaluation of how many infants with permanent hearing loss pass a two-stage otoacoustic emissions/automated auditory brainstem response newborn hearing screening protocol.

OBJECTIVE: Ninety percent of all newborns in the United States are now screened for hearing loss before they leave the hospital. Many hospitals use a 2-stage protocol for newborn hearing screening in which all infants are screened first with otoacoustic emissions (OAE). No additional testing is done with infants who pass the OAE, but infants who fail the OAE next are screened with automated auditory brainstem response (A-ABR). Infants who fail the A-ABR screening are referred for diagnostic testing to determine whether they have permanent hearing loss (PHL). Those who pass the A-ABR are considered at low risk for hearing loss and are not tested further. The objective of this multicenter study was to determine whether a substantial number of infants who fail the initial OAE and pass the A-ABR have PHL at approximately 9 months of age. METHODS: Seven birthing centers with successful newborn hearing screening programs using a 2-stage OAE/A-ABR screening protocol participated. During the study period, 86634 infants were screened for hearing loss at these sites. Of those infants who failed the OAE but passed the A-ABR in at least 1 ear, 1524 were enrolled in the study. Data about prenatal, neonatal, and socioeconomic factors, plus hearing loss risk indicators, were collected for all enrolled infants. When the infants were an average of 9.7 months of age, diagnostic audiologic evaluations were done for 64% of the enrolled infants (1432 ears from 973 infants). RESULTS: Twenty-one infants (30 ears) who had failed the OAE but passed the A-ABR during the newborn hearing screening were identified with permanent bilateral or unilateral hearing loss. Twenty-three (77%) of the ears had mild hearing loss (average of 1 kHz, 2 kHz, and 4 kHz < or =40-decibel hearing level). Nine (43%) infants had bilateral as opposed to unilateral loss, and 18 (86%) infants had sensorineural as opposed to permanent conductive hearing loss. CONCLUSIONS: If all infants were screened for hearing loss using the 2-stage OAE/A-ABR newborn hearing screening protocol currently used in many hospitals, then approximately 23% of those with PHL at approximately 9 months of age would have passed the A-ABR. This happens in part because much of the A-ABR screening equipment in current use was designed to identify infants with moderate or greater hearing loss. Thus, program administrators should be certain that the screening program, equipment, and protocols are designed to identify the type of hearing loss targeted by their program. The results also show the need for continued surveillance of hearing status during childhood.

A multisite study to examine the efficacy of the otoacoustic emission/automated auditory brainstem response newborn hearing screening protocol: introduction and overview of the study.

PURPOSE: This article is the 1st in a series of 4 articles on a recently completed multistate study of newborn hearing screening. METHOD: The study examined the efficacy of the 2-stage otoacoustic emission/automated auditory brainstem response (OAE/A-ABR) protocol for identifying hearing loss in newborns. RESULTS: The study found that the 2-stage OAE/A-ABR protocol did miss a significant number of babies who exhibited a permanent hearing loss by 1 year of age. Three subsequent articles will describe the research design and results in detail, discuss the behavioral assessment of infants, and summarize the implications of the study for policy, practice, and research.

A multisite study to examine the efficacy of the otoacoustic emission/automated auditory brainstem response newborn hearing screening protocol: research design and results of the study.

PURPOSE: Most newborns are screened for hearing loss, and many hospitals use a 2-stage protocol in which all infants are screened first with otoacoustic emissions (OAEs). In this protocol, no additional testing is done for those passing the OAE screening, but infants failing the OAE are also screened with automated auditory brainstem response (A-ABR). This study evaluated how many infants who failed the OAE and passed the A-ABR had permanent hearing loss (PHL) at 8-12 months of age. METHOD: A total of 86,634 infants were screened at 7 birthing centers using a 2-stage OAE/A-ABR hearing screening protocol. Of infants who failed the OAE but passed the A-ABR, 1,524 were enrolled in the study. Diagnostic audiologic evaluations were performed on 64% of the enrolled infants (1,432 ears from 973 infants) when they were 8-12 months old. RESULTS: Twenty-one infants (30 ears) who passed the newborn A-ABR hearing screening were identified with PHL when they were 8-12 months old. Most (71%) had mild hearing loss. CONCLUSIONS: If all infants were screened for hearing loss using a typical 2-stage OAE/A-ABR protocol, approximately 23% of those with PHL at 8-12 months of age would have passed the A-ABR.

A multisite study to examine the efficacy of the otoacoustic emission/automated auditory brainstem response newborn hearing screening protocol: results of visual reinforcement audiometry.

PURPOSE: This 3rd of 4 articles on a study of the efficacy of the 2-stage otoacoustic emission/automated auditory brainstem response (OAE/A-ABR) newborn hearing screening protocol describes (a) the behavioral audiometric protocol used to validate hearing status at 8-12 months of age, (b) the hearing status of the sample, and (c) the success of the visual reinforcement audiometry (VRA) protocol across 7 sites. METHOD: A total of 973 infants who failed OAE but passed A-ABR, in one or both ears, during newborn screening were tested with a VRA protocol, supplemented by tympanometry and OAE screening at age 8-12 months. RESULTS: VRA audiograms (1.0, 2.0, and 4.0 kHz) were obtained for 1,184 (82.7%) of the 1,432 study ears. Hearing loss was ruled out in another 100 ears by VRA in combination with OAE, for a total of 88.7% of the study sample. Permanent hearing loss was identified in 30 ears of 21 infants. Sites differed in their success with the VRA protocol. CONCLUSIONS: Continued monitoring of hearing beyond the newborn period is an important component of early detection of hearing loss. Using a structured protocol, VRA is an appropriate test method for most, but not all, infants. A battery of test procedures is often needed to adequately delineate hearing loss in infants. Examiner experience appears to be a factor in successful VRA.

A multisite study to examine the efficacy of the otoacoustic emission/automated auditory brainstem response newborn hearing screening protocol: recommendations for policy, practice, and research.

PURPOSE: This article examines whether changes in hearing screening practices are warranted based on the results of the recent series of studies by J. L. Johnson, K. R. White, J. E. Widen, J. S. Gravel, B. R. Vohr, M. James, T. Kennalley, A. B. Maxon, L. Spivak, M. Sullivan-Mahoney, Y. Weirather, and S. Meyer (Johnson, White, Widen, Gravel, James, et al., 2005; Johnson, White, Widen, Gravel, Vohr, et al., 2005; White et al., 2005; Widen et al., 2005) that found a significant number of infants who passed an automated auditory brainstem response (A-ABR) screening after failing an initial otoacoustic emission (OAE) screening later were found to have permanent hearing loss in one or both ears. METHOD: Similar to the approach used by F. H. Bess and J. Paradise (1994), this article addresses the public health tenets that need to be in place before screening programs, or in this case, a change in screening practice (use of a 2-step screening protocol) can be justified. RESULTS: There are no data to suggest that a 2-step OAE/A-ABR screening protocol should be avoided. CONCLUSION: Research is needed before any change in public policy and practice surrounding current early hearing detection and intervention programs could be supported.

Diagnostic testing for hearing loss in infants and young children.

An early, reliable diagnosis and description of hearing loss is essential in determining appropriate treatment to minimize potential developmental delays attributed to the loss. Objective assessment of auditory function is possible shortly after birth. Both objective and subjective measures of auditory status are used in a test battery approach to determine the type, degree, and configuration of the hearing loss. This paper reviews the diagnostic test procedures used to define the hearing capability of infants and young children. For the youngest infants electrophysiologic tests, primarily auditory evoked potentials, are used to estimate hearing sensitivity. Later behavioral audiometric measures based on operant conditioning techniques become the primary component of the test battery. Diagnostic audiologic assessment is an ongoing process that requires special equipment and expertise when directed to infants and young children.

Evaluating the benefit of speech recoding hearing aids in children.

Children with significant high-frequency hearing loss may be difficult to fit with hearing aids using conventional amplification. Frequency-lowering hearing aids using dynamic speech recoding (DSR) technology have been proposed as a possible means to achieve full speech audibility. The current study investigated 78 children from ages 1.3 to 21.6 years (M = 10.6 years) who wore DSR hearing aids. These hearing aids provided significant improvements in pure-tone average (PTA; mean improvement for the aided versus unaided condition of 49 dB) and high-frequency PTA (mean improvement for the aided versus unaided condition of 56 dB). A subgroup of 19 children were previous users of conventional hearing aids. These participants demonstrated a mean improvement of 11 dB in PTA and 12.5% in word recognition scores for DSR versus conventional amplification. However, DSR hearing aids required repair 3 times as often as conventional hearing aids. The greatest benefit was observed in children whose word recognition scores were poorest using conventional hearing aids.