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Some children with unilateral hearing loss or mild bilateral hearing loss are at risk for developmental delays, educational difficulties, and other adverse effects. However, these children face several challenges in receiving services that can prevent such problems. Many do not meet existing eligibility requirements for early intervention services in their state. Information disseminated to professionals and parents often does not convey the significance of unilateral and mild bilateral hearing loss. Some professionals indicate that there are insufficient guidelines defining appropriate intervention services for these children. Factors that influence developmental outcomes in this population are not well understood, making it difficult to determine which children can benefit from intervention services. Additional data are needed about how to minimize or prevent adverse outcomes in these children. This article presents suggestions for intervention and future research that were developed by participants of the 2005 National Workshop on Mild and Unilateral Hearing Loss.

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More than 90% of newborns in the United States are now being screened for hearing loss. A large fraction of cases of unilateral hearing loss and mild bilateral hearing loss are not currently identified through newborn hearing screening. This is of concern because a preponderance of research has demonstrated that unilateral hearing loss and mild bilateral hearing loss can lead to developmental delays and educational problems for some children. To help address this probable underidentification of unilateral hearing loss and mild bilateral hearing loss among infants and children, the Centers for Disease Control and Prevention Early Hearing Detection and Intervention program and the Marion Downs Hearing Center convened a workshop in Breckenridge, Colorado, in July 2005. During this workshop, several issues related to screening and diagnosing unilateral hearing loss and mild bilateral hearing loss were identified, as well as recommendations for future research in this area. Issues identified included the lack of standardized definitions for permanent unilateral hearing loss and mild bilateral hearing loss; the use of screening protocols that are primarily designed to identify bilateral and unilateral hearing losses of a moderate degree or greater (eg, above 40 dB); calibration of screening equipment; availability of facilities that can provide the full range of audiologic, diagnostic, and management services to this pediatric population; and an overall lack of awareness by many professionals and families about the potential effect of unilateral hearing loss and mild bilateral hearing loss. Suggestions for future research, such as identifying ways to improve the identification of cases of unilateral hearing loss and mild bilateral hearing loss, were also discussed.

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Children with minimal or mild bilateral hearing loss and unilateral hearing loss are at higher risk for academic, speech-language, and social-emotional difficulties than their normal hearing peers. The choice to fit infants with moderate or greater degrees of bilateral hearing loss has been standard practice for most clinicians, but for those with minimal or mild bilateral hearing loss or unilateral hearing loss, the fitting of hearing technology must be based on limited data. Evidence does not yet exist to support all the management decisions that an audiologist must make upon identifying an infant with minimal or mild bilateral hearing loss or unilateral hearing loss. It is not yet known which children are at the greatest risk for educational problems nor is it known if the provision of early amplification in this population will help a child avoid later difficulties. Some of these considerations and current hearing technology options for children with minimal or mild bilateral hearing loss or unilateral hearing loss are reviewed in this article.

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OBJECTIVES: To examine the effect of conductive hearing loss (HL) secondary to otitis media with effusion (OME) in the first 3 years of life on physiologic, peripheral, and higher-order behavioral auditory measures examined at school age. METHODS: Peripheral hearing sensitivity for conventional and extended high-frequency audiometric ranges, physiologic (distortion product otoacoustic emissions, contralateral and ipsilateral acoustic middle ear muscle reflexes), auditory brain stem response (ABR), and higher-order auditory processing measures (masking level difference; Virtual Auditory Localization, Speech Intelligibility Gain; adaptive Pediatric Speech Intelligibility task) were examined at the end of the second grade of elementary school in two cohorts (North Carolina, N = 73, and New York, N = 59). All participants (mean age, 8 years) were followed prospectively in infancy and early childhood (7 to 39 months) for middle ear status and hearing loss (using pneumatic otoscopy/tympanometry and repeated conditioned behavioral audiometric response procedures). Multivariate analyses were conducted to address whether early OME and early conductive HL were related to physiologic, peripheral, and higher-order auditory processes. RESULTS: Early hearing loss and OME were significantly associated with peripheral hearing at school age; extended high-frequency thresholds accounted for the result. Similarly, hearing loss in early life and OME were significantly associated with the acoustic middle ear muscle reflex: The contralateral stimulation condition accounted for the association. Significant associations with both early OME and early HL were also found for the auditory brain stem response measure and were explained by the correlations between early hearing loss and the ABR Wave V latency but not other ABR indices. There were no reliable associations between either early OME or early HL on any other auditory processes evaluated at the end of second grade. CONCLUSIONS: Extended high-frequency hearing and brain stem auditory pathway measures in childhood were significantly associated with children's experiences with OME and hearing loss from 7 to 39 months of age. However, no significant associations were found for psychoacoustic measures of binaural processing or a behavioral adaptive speech-in-noise test at school age.

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OBJECTIVE: To determine whether the peripheral audition of a group of children comprehensively diagnosed as being on the autistic spectrum was quantitatively different from that of a matched group of typically developing children. METHODS: Thirty-seven children with autism and an equal number of control participants matched for chronological age within 6 mo were examined by means of behavioral and physiologic measures of auditory function. All participants had normal hearing (thresholds <15 dB HL; 0.25 to 8 kHz) and normal middle ear function by quantitative tympanometry. Conventional behavioral audiometry, a computer-assisted threshold assessment procedure, acoustic middle ear muscle reflex thresholds, and evoked otoacoustic emissions (both transient and distortion product) tests were administered to both groups. RESULTS: No significant differences between children with autism and those developing typically on any behavioral or physiologic measure of peripheral auditory function were found. CONCLUSIONS: There was no evidence of intrinsic differences in the peripheral auditory mechanism of children with autism that would account for the auditory processing disorders and sound sensitivity that are commonly reported in this population.

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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.

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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.

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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.

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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.

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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.

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The test efficiency and reliability of loudness assessment using a computer-controlled method of cross-modality matching (CMM) between line length and loudness was investigated in children 4 to 12 years with normal hearing or mild to severe degrees of sensorineural hearing loss. Adult listeners with normal hearing served as a comparison group. Computer-generated visual and acoustic stimuli were used to derive individual loudness data. Children and adults with normal hearing presented with similar loudness functions, while children with sensorineural hearing loss had steeper functions than their normal-hearing counterparts. Retest data supported reliability of the CMM method with children within the current study and between previous studies performed with a similar, but manual, method. The computer CMM approach proved more time efficient than the manual one, halving the test time. The CMM loudness task in a computerized version may have potential in a research or clinical setting, in particular for individualizing hearing aid fittings with children.

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Otitis media with effusion (OME) often results in hearing loss for children with the condition. In order to provide appropriate and effective audiologic management, it is important to understand the impact of OME on speech recognition ability when hearing loss is present. This study examined the speech recognition abilities of normal-hearing six- and seven-year-old children (n = 12) and adults (n = 12) using monosyllabic words and nonsense syllables presented at two levels of simulated conductive hearing loss characteristic of OME. Average speech recognition scores decreased as the degree of simulated conductive hearing loss increased. Both age groups scored significantly poorer for nonsense syllables than for monosyllabic words. In general, the children performed more poorly than the adults with the exception of the easiest listening condition for word stimuli. Furthermore, children appeared less able than adults to use their knowledge of familiar words to improve performance. These findings suggest that rehabilitative strategies may best be focused on combining familiarization techniques and amplification options.

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This article examines the communication options that are available for use within families of infants and young children who are hard-of-hearing or deaf. The need for language development, regardless of the specific communication mode, is stressed. The demands of the current environment of early identification and intervention often put families in a position of needing to decide among communication methods before they are fully knowledgeable and/or emotionally ready. Specific communication options are delineated and considered within a continuum of spoken and visual language. Available research related to early acquisition of language by infants and young children who are hard-of-hearing and deaf is reviewed; outcomes, when available, are presented for specific methods from reports of older children. Factors that influence families' decisions regarding the selection of a communication option are highlighted in the context of the existing literature. An ongoing evaluative process that respects the choices of families is advocated; a context in which change(s) in communication mode through childhood is viewed as a positive circumstance. The ultimate goal in the selection of any communication approach is to ensure that infants and young children who are hard of hearing or deaf and their families are language proficient and fluent communicators.

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