Identification of neonatal hearing impairment: a multicenter investigation.

OBJECTIVES: This article describes the design of a multicenter study sponsored by the National Institutes of Health. The purpose of this study was to determine the accuracy of three measures of peripheral auditory system status (transient evoked otoacoustic emissions, distortion product otoacoustic emissions, and auditory brain stem responses) applied in the perinatal period for predicting behavioral hearing status at 8 to 12 mo corrected age. The influences of the infant's medical status, the test environment, and test and response parameters on test performance were examined. DESIGN: Seven institutions participated in this study. There were 7179 infants evaluated in the perinatal period. All graduates of the neonatal intensive care unit (4478) and well babies with one or more risk factor for hearing loss (353) were targeted for follow-up testing using visual reinforcement audiometry (VRA) at 8 to 12 mo corrected age. Well babies without any risk indicators (N = 2348) were not targeted for follow-up VRA testing. However, 80 of these well babies did not pass the screening protocol and thus were targeted for follow-up VRA testing as well. Perinatal test performance was evaluated using the VRA data as the "gold standard." RESULTS: The results of this study are described in a series of 11 articles following this introductory article. CONCLUSIONS: The evaluation of newborn hearing tests required a longitudinal study in which newborn test results were compared with a gold standard based on behavioral audiometric assessment. Such an evaluation was possible because all newborns, passes as well as refers, were followed up long enough to permit reliable behavioral measurements. In addition, prenatal, perinatal, and maternal history information, test environment, and test parameter information were collected to provide data that led to a complete description of factors affecting test outcomes. All of these data were obtained in a sample of sufficient ethnic, medical, and geographic diversity in efforts to increase the generalizability of the results. Finally, the data were combined in a relational data base to examine the factors that influence test performance. Specific information related to these issues is presented in the articles that follow.

Identification of neonatal hearing impairment: experimental protocol and database management.

OBJECTIVE: The purposes of this article are to describe the overall protocol for the Identification of Neonatal Hearing Impairment (INHI) project and to describe the management of the data collected as part of this project. A well-defined protocol and database management techniques were needed to ensure that data were 1) collected accurately and in the same way across sites; 2) maintained in a database that could be used to provide feedback to individual sites regarding enrollment and the extent to which the protocol was complete on individual subjects; and 3) available to answer project questions. This article describes techniques that were used to meet these needs. DESIGN: This study was a prospective, randomized study that was designed to evaluate auditory brain stem responses, transient evoked otoacoustic emissions, and distortion product otoacoustic emissions as hearing-screening tools, and to relate neonatal test findings to hearing status, defined by visual reinforcement audiometry at 8 to 12 mo of age. Measures of middle-ear function also were obtained at some sites as part of the neonatal test battery. In addition, other clinical and demographic data were gathered to determine the extent to which factors, other than auditory status, influenced test behavior. Three groups were evaluated: neonatal intensive care unit (NICU) infants (those who spent 3 or more days in a NICU), well babies with risk factors for hearing loss, and well babies without risk factors. Six centers participated in the trial. The testers for the project included audiologists, technicians, audiology graduate students, and medical research staff. The same computerized neonatal test program was applied at each center. This program generated the neonatal test database automatically. Clinical and demographic data were collected by means of concise data collection forms and were entered into a database at each site. After the neonatal test, subjects from the NICU and at-risk well babies were evaluated with visual reinforcement audiometry starting at 8 to 12 mo of age. All data were electronically transmitted to the core site where they were merged into one overall database. This database was exercised to provide feedback and to identify discrepancies throughout the course of the study. In its final form, it served as the database on which all analyses were performed. RESULTS AND CONCLUSION: The protocol was a departure from typical hearing screening procedures in terms of 1) its regimented application of three screening measures; 2) the detailed information that was obtained regarding subject clinical and demographic factors; and 3) its application of the same procedures across six centers having diverse geographic location and subject demographics. A learning curve for successfully executing the study protocols was observed. Throughout the study, monthly reports were generated to monitor subject enrollment, check for data completeness, and to perform data integrity checks. In combination with monthly data reports and checks that occurred throughout the progression of the study, miscellaneous data audits were performed to check accuracy of neonatal testing programs and to cross-check information entered in the clinical and demographic database. The data management techniques used in this project helped to ensure the quality of the data collection process and also allowed for detailed analyses once data were collected. This was particularly important because it enabled us to evaluate not only the performance of individual measures as screening tools, but also permitted an evaluation of the influence of other variables on screening test results.

Identification of neonatal hearing impairment: distortion product otoacoustic emissions during the perinatal period.

OBJECTIVES: 1) To describe distortion product otoacoustic emission (DPOAE) levels, noise levels and signal to noise ratios (SNRs) for a wide range of frequencies and two stimulus levels in neonates and infants. 2) To describe the relations between these DPOAE measurements and age, test environment, baby state, and test time. DESIGN: DPOAEs were measured in 2348 well babies without risk indicators, 353 well babies with at least one risk indicator, and 4478 graduates of neonatal intensive care units (NICUs). DPOAE and noise levels were measured at f2 frequencies of 1.0, 1.5, 2.0, 3.0, and 4.0 kHz, and for primary levels (L1/L2) of 65/50 dB SPL and 75/75 dB SPL. Measurement-based stopping rules were used such that a test did not terminate unless the response was at least 3 dB above the mean noise floor + 2 SDs (SNR) for at least four of five test frequencies. The test would terminate, however, if these criteria were not met after 360 sec. Baby state, test environment, and other test factors were captured at the time of each test. RESULTS: DPOAE levels, noise levels and SNRs were similar for well babies without risk indicators, well babies with risk indicators, and NICU graduates. There was a tendency for larger responses at f2 frequencies of 1.5 and 2.0 Hz, compared with 3.0 and 4.0 kHz; however, the noise levels systematically decreased as frequency increased, resulting in the most favorable SNRs at 3.0 and 4.0 kHz. Response levels were least and noise levels highest for an f2 frequency of 1.0 kHz. In addition, test time to achieve automatic stopping criteria was greatest for 1.0 kHz. With the exception of "active/alert" and "crying" babies, baby state had little influence on DPOAE measurements. Additionally, test environment had little impact on these measurements, at least for the environments in which babies were tested in this study. However, the lowest SNRs were observed for infants who were tested in functioning isolettes. Finally, there were some subtle age affects on DPOAE levels, with the infants born most prematurely producing the smallest responses, regardless of age at the time of test. CONCLUSIONS: DPOAE measurements in neonates and infants result in robust responses in the vast majority of ears for f2 frequencies of at least 2.0, 3.0 and 4.0 kHz. SNRs decrease as frequency decreases, making the measurements less reliable at 1.0 kHz. When considered along with test time, there may be little justification for including an f2 frequency at 1.0 kHz in newborn screening programs. It would appear that DPOAEs result in reliable measurements when tests are conducted in the environments in which babies typically are found. Finally, these data suggest that babies can be tested in those states of arousal that are most commonly encountered in the perinatal period.

Identification of neonatal hearing impairment: characteristics of infants in the neonatal intensive care unit and well-baby nursery.

OBJECTIVE: The objective of this study was to describe the demographic data, medical status, and incidence of risk factors for hearing impairment in the neonatal intensive care unit (NICU) and well-baby populations in a multicenter prospective study designed to assess neonatal hearing impairment and to evaluate factors that might affect neonatal hearing test performance. DESIGN: This was a prospective multicenter study funded by the National Institutes of Health-National Institute on Deafness and Other Communication Disorders to evaluate the effectiveness of auditory brain stem response, transient evoked otoacoustic emissions, and distortion product otoacoustic emissions for newborn hearing screening. Research staff at each site obtained informed consent and detailed demographic and medical data, including information on established risk factors for hearing loss on 4478 high-risk infants cared for in the NICU, 2348 infants from the well-baby nurseries with no risk factor, and 353 infants from the well-baby nurseries with risk factors. For follow-up purposes the sample was divided further to include a subgroup called selects. Selects were either infants from the well-baby nursery who had an established risk factor for hearing impairment (N = 353) or did not pass the neonatal hearing screen protocol (N = 80). In this study, we focus on the distribution of infants by nursery and risk factors only. Particular effort was made to enroll infants with risk factors for hearing loss in both the NICU and well-baby nurseries. Descriptive analyses are used to describe characteristics of this sample. RESULTS: All 10 of the risk factors established by the Joint Committee on Infant Hearing in 1994 were identified in the NICU population. The four most common were ototoxic medications (44.4%), very low birth weight (17.8%), assisted ventilation > 5 days (16.4%), and low Apgar scores at 1 or 5 min (13.9%). In contrast, only six risk factors were present in the well-baby nurseries: family history (6.6%), craniofacial abnormalities (3.4%), low Apgar scores (2.8%), syndromes (0.5%), ototoxic medications (0.2%), and congenital infection (0.1%). CONCLUSION: These descriptive risk factor data reflect both the newborn populations at the study sites and the bias for enrolling infants at risk for hearing loss. The high-risk NICU sample reflects the characteristics typically found in graduates of the NICU. The data summarized in this study will be used to assess the relationships between risk factor and hearing test outcome.

Identification of neonatal hearing impairment: transient evoked otoacoustic emissions during the perinatal period.

OBJECTIVES: 1) To describe transient evoked otoacoustic emission (TEOAE) levels, noise levels and signal to noise ratios (SNRs) for a range of frequency bands in three groups of neonates who were tested as a part of the Identification of Neonatal Hearing Impairment multi-center consortium project. 2) To describe the relations between these TEOAE measurements and age, test environment, baby state, and test time. DESIGN: TEOAEs were measured in 4478 graduates of neonatal intensive care units (NICUs), 353 well babies with at least one risk indicator, and 2348 well babies without risk factors. TEOAE and noise levels were measured for frequency bands centered at 1.0, 1.5, 2.0, 3.0, and 4.0 kHz for a click stimulus level of 80 dB SPL. For those ears not meeting "passing" stopping criteria at 80 dB pSPL, a level of 86 dB pSPL was included. Measurement-based stopping rules were used such that a test did not terminate unless the response revealed a criterion SNR in four out of five frequency bands or no response occurred after a preset number of averages. Baby state, test environment, and other test factors were captured at the time of test. RESULTS: TEOAE levels, noise levels and SNRs were similar for NICU graduates, well babies with risk factors and well babies without risk factors. There were no consistent differences in response quality as a function of test environment, i.e., private room, unit, open crib, nonworking isolette, or working isolette. Noise level varied little across risk group, test environment, or infant state other than crying, suggesting that the primary source of noise in TEOAE measurements is infant noise. The most significant effect on response quality was center frequency. Responses were difficult to measure in the half-octave band centered at 1.0 kHz, compared with higher frequencies. Reliable responses were measured routinely at frequencies of 1.5 kHz and higher. CONCLUSIONS: TEOAEs are easily measured in both NICU graduates and well babies with and without risk factors for hearing loss in a wide variety of test environments. Given the difficulties encountered in making reliable measurements for a frequency band centered at 1.0 kHz, its inclusion in a screening program may not be justified.

Identification of neonatal hearing impairment: auditory brain stem responses in the perinatal period.

OBJECTIVES: 1) To describe the auditory brain stem response (ABR) measurement system and optimized methods used for study of newborn hearing screening. 2) To determine how recording and infant factors related to the screening, using well-defined, specific ABR outcome measures. DESIGN: Seven thousand one hundred seventy-nine infants, 4478 from the neonatal intensive care unit (NICU) and the remaining from the well-baby nursery, were evaluated with an automated ABR protocol in each ear. Two channel recordings were obtained (vertex to mastoid or channel A and vertex to nape of neck or channel B) in response to click stimuli of 30 and 69 dB nHL in all infants as well as 50 dB nHL in infants who did not meet criteria for response at 30 dB. Criteria for response included F(SP) > or =3.1 and a tester-judgment of response. Criteria could be met in the first or repeat test with a maximum of 6144 accepted sweeps per test. RESULTS: More than 99% of infants could complete the ABR protocol. More than 90% of NICU and well-baby nursery infants "passed" given the strict criteria for response, whereas 86% of those with high risk factors met criterion for ABR response detection. The number of infants who did not meet ABR response criteria in one or both ears was systematically related to stimulus level with the largest group not meeting criteria at 30 dB followed by 50 and 69 dB nHL. Meeting criteria on the ABR was positively correlated with the amplitude of wave V, with low noise and low electrode impedance. Factors that predicted how many sweeps would be needed to reach criterion F(SP) included noise level of the test site, state of the baby (for example, quiet sleep versus crying), recording noise, electrode impedance and response latency. Channel A (vertex to mastoid) reached criterion more often than channel B (vertex to nape of neck) due to higher noise in channel B. Average total test time for 30 dB nHL screening in both ears was under 8 minutes. Well babies with risk factors took slightly longer to evaluate than other groups with this automated ABR procedure and have higher noise levels. CONCLUSIONS: ABR implemented with an automated detection algorithm using a 30 dB nHL click stimulus is reliable technique for rapid assessment of auditory status in newborns. Factors other than hearing loss that influenced the test result include infant state, electrode location and impedance, testing site, and infant risk status. Even so, ABRs were reliably recorded in the vast majority of babies under circumstances in which most babies are found in the perinatal period.

Identification of neonatal hearing impairment: recruitment and follow-up.

OBJECTIVE: The purpose of this study is to describe the recruitment and retention strategies as well as the sample demographics for families with infants completing the neonatal examination and returning for follow-up. These data are compared to those infants inactivated from the study. DESIGN: This study was a prospective, randomized clinical study. All infants who were confined to the neonatal intensive care unit (NICU) and well babies with at least one risk indicator were targeted for behavioral audiometric follow-up testing. In addition, infants without risk factors from the well-baby nursery, but who failed a newborn test, were also followed. Several variables were evaluated to determine those factors, if any, that might predict which families returned for follow-up testing. RESULTS: Recruitment was achieved as per study design with 4911 high-risk infants and 2348 well-baby nursery infants (without risk indicators for hearing) enrolled. Of the 4911 high-risk infants enrolled, 64% were successfully recruited into the follow-up portion of the study. This was less than the projected rate of 80%. Factors predicting noncompliance with the study protocol for follow-up were predominantly sociodemographic and included nonwhite race, no insurance, substance abuse, young maternal age, more than two children at home, and late onset of prenatal care. CONCLUSIONS: Factors related to low socioeconomic status and increased social risk were the strongest predictors of poor study protocol compliance. Despite retention challenges, 64% of the targeted, high-risk infants subsequently returned for the 8-to 12-mo behavioral hearing assessment protocols for validation purposes.

Identification of neonatal hearing impairment: hearing status at 8 to 12 months corrected age using a visual reinforcement audiometry protocol.

OBJECTIVES: 1) To describe the hearing status of the at-risk infants in the National Institutes of Health-Identification of Neonatal Hearing Impairment study sample at 8 to 12 mo corrected age (chronologic age adjusted for prematurity). 2) To describe the visual reinforcement audiometry (VRA) protocol that was used to obtain monaural behavioral data for the sample. DESIGN: All neonatal intensive care unit infants and well babies with risk factors (including well babies who failed neonatal tests) were targeted for follow-up behavioral evaluation once they had reached 8 mo corrected age. Three thousand one hundred and thirty-four (64.4%) of the 4868 surviving infants returned for at least one behavioral hearing evaluation, which employed a well-defined VRA protocol. VRA thresholds or minimum response levels (MRLs) were determined for speech and pure tones of 1.0, 2.0, and 4.0 kHz for each ear using insert earphones. RESULTS: More than 95% of the infants were reliably tested with the VRA protocol; 90% provided complete tests (four MRLs for both ears). Ninety-four percent of the at-risk infants were found to have normal hearing sensitivity (MRLs of 20 dB HL) at 1.0, 2.0, and 4.0 kHz in both ears. Of the infants, 2.2% had bilateral hearing impairment, and 3.4% had impairment in one ear only. More than 80% of the impaired ears had losses of mild-to-moderate degree. CONCLUSIONS: This may be the largest study to attempt to follow all at-risk infants with behavioral audiometric testing, regardless of screening outcome, in an effort to validate the results of auditory brain stem response, distortion product otoacoustic emission, and transient evoked otoacoustic emission testing in the newborn period. It is one of only a few studies to report hearing status of infants at 1 yr of age, using VRA on a clinical population. Successful testing of more than 95% of the infants who returned for the VRA follow-up documents the feasibility of obtaining monaural behavioral data in this population.

Identification of neonatal hearing impairment: infants with hearing loss.

OBJECTIVE: This article describes the audiologic findings and medical status of infants who were found to have hearing loss, detected as part of the Identification of Neonatal Hearing Impairment (INHI) project. In addition, the neonatal and maternal health variables for the group of infants who could not be tested with visual reinforcement audiometry (VRA) due to developmental and visual disability are presented. DESIGN: The overall goal of the INHI project was to evaluate the test performance of auditory brain stem response and evoked otoacoustic emission (OAE) tests given in the newborn period. These tools were evaluated on the basis of the infants' hearing when tested behaviorally with VRA at 8 to 12 mo corrected age. The neonatal test results, VRA results, medical history information and a record of intercurrent events occurring between the neonatal period and the time of VRA were collated and reviewed. The purpose of this article is to review the characteristics of those infants who were found to have hearing loss. RESULTS: Of 2995 infants who had VRA tests judged to be of good or fair reliability, 168 had a finding of hearing loss for at least one ear, an incidence of 5.6%. Sixty-six infants had bilateral losses, an incidence of 2%, and 22 infants had bilateral hearing losses in the moderate to profound range, an incidence 0.7%. The prevalence of middle ear problems was greater than 50% among these infants with hearing loss. From the larger group of 168 infants with hearing loss, a group of 56 infants (86 ears) was chosen as those with a low probability that the hearing loss was due to transient middle ear pathology and was more likely hearing loss of a permanent nature. These were the infants used for the analyses of neonatal test performance (Norton et al., 2000). In this selected group there were 30 infants with bilateral impairment of at least mild degree, which is an incidence of 1%. There were approximately equal numbers of ears in the mild, moderate, severe and profound range of hearing loss. Risk factors associated with hearing loss were reviewed for the total sample of infants tested with VRA and for those infants with hearing loss. A history of treatment with aminoglycosides was the risk factor most often reported in the entire sample; however, there was no difference in prevalence of this risk factor for the normal-hearing and hearing-impaired groups. The risk factor associated with the highest incidence of hearing loss was stigmata of syndromes associated with sensorineural hearing loss and other neurosensory disorders. Sixty-seven infants who returned for follow-up could not be tested with VRA due to severe developmental delay or visual disability. Many of these infants had medical histories indicating the sequelae of extreme prematurity and/or very low birthweight. CONCLUSIONS: Most of the hearing losses found in this study were mild and, based on clinical history and tympanometry tests, many of the mild and some of the moderate impairments may have been acquired in early infancy due to middle ear effusion. In the group of infants used for determination of neonatal test performance there were approximately equal numbers of mild, moderate, severe and profound losses. Only a small percentage of infants with a conventional risk indicator for hearing loss actually had a hearing loss, and there were a significant number of infants with hearing loss who did not have a risk indicator. These findings support the need for an early identification program based on universal neonatal hearing screening rather than by targeted testing of those with risk indicators.

Identification of neonatal hearing impairment: summary and recommendations.

OBJECTIVES: This article summarizes the results of a multi-center study, "Identification of Neonatal Hearing Impairment," sponsored by the National Institutes of Health. The purpose of this study was to determine the performance characteristics of three measures of peripheral auditory system status, transient evoked otoacoustic emissions (TEOAEs), distortion product otoacoustic emissions (DPOAEs), and auditory brain stem responses (ABR), applied in the neonatal period in predicting hearing status at 8 to 12 mo corrected age. DESIGN: The design and implementation of this study are described in the first two articles in this series. Seven institutions participated in this study; 7179 infants were evaluated. Graduates of the neonatal intensive care unit and well babies with one or more risk factors for hearing loss were targeted for follow-up testing using visual reinforcement audiometry (VRA) at 8 to 12 mo corrected age. Neonatal test performance was evaluated using the VRA data as the "gold standard." RESULTS: The major results of the study are described in the nine articles preceding this summary article. TEOAEs in response to an 80 dB pSPL click, DPOAEs in response to L1 = 65 and L2 = 50 dB SPL and ABR in response to a 30 dB nHL click performed well as predictors of permanent hearing loss of 30 dB or greater at 8 to 12 mo corrected age. All measures were robust with respect to infant state, test environment and infant medical status. No test performed perfectly. CONCLUSIONS: Based on the data from this study, the 1993 National Institutes of Health Consensus Conference-recommended protocol-an OAE test followed by an ABR test for those infants failing the OAE test-would result in low referral rate (96 to 98%). TEOAEs for 80 dB pSPL, ABR for 30 dB nHL and DPOAEs for L1 = 65 dB SPL and L2 = 50 dB SPL perform well in predicting hearing status based on the area under the relative operating characteristic curve. Accuracy for the OAE measurements are best when the speech awareness threshold or the pure-tone average for 2.0 kHz and 4 kHz are used as the gold standard. ABR accuracy varies little as a function of the frequencies included in the gold standard. In addition, 96% of those infants returning for VRA at 8 to 12 mo corrected age were able to provide reliable ear-specific behavioral thresholds using insert earphones and a rigorous psychophysical VRA protocol.

Identification of neonatal hearing impairment: evaluation of transient evoked otoacoustic emission, distortion product otoacoustic emission, and auditory brain stem response test performance.

OBJECTIVES: The purpose of this study was to compare the performance of transient evoked otoacoustic emissions (TEOAEs), distortion product otoacoustic emissions (DPOAEs), and auditory brain stem responses (ABRs) as tools for identification of neonatal hearing impairment. DESIGN: A total of 4911 infants including 4478 graduates of neonatal intensive care units, 353 well babies with one or more risk factors for hearing loss (Joint Committee on Infant Hearing, 1994) and 80 well babies without risk factor who did not pass one or more neonatal test were targeted as the potential subject pool on which test performance would be assessed. During the neonatal period, they were evaluated using TEOAEs in response to an 80 dB pSPL click, DPOAE responses to two stimulus conditions (L1 = L2 = 75 dB SPL and L1 = 65 dB SPL L2 = 50 dB SPL), and ABR elicited by a 30 dB nHL click. In an effort to describe test performance, these "at-risk" infants were asked to return for behavioral audiologic assessments, using visual reinforcement audiometry (VRA) at 8 to 12 mo corrected age, regardless of neonatal test results. Sixty-four percent of these subjects returned and reliable VRA data were obtained on 95.6% of these returnees. This approach is in contrast to previous studies in which, by necessity, efforts were made to follow only those infants who "failed" the neonatal screening tests. The accuracy of the neonatal measures in predicting hearing status at 8 to 12 mo corrected age was determined. Only those infants who provided reliable, monaural VRA test results were included in the analysis. Separate analyses were performed without regard to intercurrent events (i.e., events between the neonatal and VRA tests that could cause their results to disagree), and then after accounting for the possible influence of intercurrent events such as otitis media and late-onset or progressive hearing loss. RESULTS: Low refer rates were achieved for the stopping criteria used in the present study, especially when a protocol similar to the one recommended in the National Institutes of Health (1993) Consensus Conference Report was followed. These analyses, however, do not completely describe test performance because they did not compare neonatal screening test results with a gold standard test of hearing. Test performance, as measured by the area under a relative operating characteristic curve, were similar for all three neonatal tests when neonatal test results were compared with VRA data obtained at 8 to 12 mo corrected age. However, ABRs were more successful at determining auditory status at 1 kHz, compared with the otoacoustic emission (OAE) tests. Performance was more similar across all three tests when they were used to identify hearing loss at 2 and 4 kHz. No test performed perfectly. Using either the two- or three-frequency pure-tone average (PTA), with a fixed false alarm rate of 20%, hit rates for the neonatal tests, in general, exceeded 80% when hearing impairment was defined as behavioral thresholds > or =30 dB HL. All three tests performed similarly when a two-frequency (2 and 4 kHz) PTA was used as the gold standard; OAE test performance decreased when a three-frequency PTA (adding 1 kHz) was used as the gold standard definition. For both PTA and all three neonatal screening measures, however, hit rate increased as the magnitude of hearing loss increased. CONCLUSIONS: Singly, all three neonatal hearing screening tests resulted in low refer rates, especially if referrals for follow-up were made only for the cases in which stopping criteria were not met in both ears. Following a protocol similar to that recommended in the National Institutes of Health (1993) Consensus Conference report resulted in refer rates that were less than 4%. TEOAEs at 80 dB pSPL, DPOAE at L1 = 65, L2 = 50 dB SPL and ABR at 30 dB nHL measured during the neonatal period, and as implemented in the current study, performed similarly at predicting behavioral hearing status at 8 to 12

Progressive neural hearing impairment: case report.

This report describes a patient whose ability to understand speech had so deteriorated over 20 years time that she was being considered for a cochlear implant, despite the fact that pure-tone sensitivity loss had not deteriorated proportionately. An unusual combination of otoacoustic emissions (OAEs) and auditory evoked potential (AEP) results are described. Click-evoked and distortion product emissions were present and normal-appearing. Auditory brainstem and middle latency responses were totally absent but the N1-P2 complex of the long-latency response was present. The case illustrates the contribution of otoacoustic emissions to site of lesion testing. It also illustrates that the manifestations of pathology can appear in certain epochs of the family of AEPs without affecting the others.

Adding objectivity to infant behavioral audiometry.

This article deals with the limitations of infant behavioral audiometry, which have led us to seek objective methods of audiometry for determining hearing sensitivity in infants. It describes efforts to add objectivity to (or reduce the subjectivity of) behavioral methods of infant audiometry. Several aspects of visual reinforcement audiometry (VRA), including the head turn response, the use of control trials, and adherence to the principles of operant conditioning, serve to reduce the subjectivity inherent in behavior observation audiometry. In addition, computer control of VRA procedures can relieve the examiner of potentially subjective decision-making during the course of evaluation and can facilitate testing with sophisticated algorithms that could not be done manually by a single examiner. Computer simulations to determine the best VRA test parameters are described.

Optimization of automated hearing test algorithms: a comparison of data from simulations and young children.

A five-up, five-down automated staircase procedure was used to explore the effects of variable starting and stopping rules as they interact with infant behavior in influencing the accuracy and efficiency of infant auditory threshold testing. Results from 146 infants were compared to results from computer simulations (see accompanying article) in order to evaluate an infant response model used in the simulations. Results indicate that the automated procedure successfully discriminated between infants with normal versus abnormal tympanograms. In addition, the model accurately predicted infant results in terms of the effects of starting and stopping rules. The model was less useful in predicting the relationship between minimum response levels and false positive rates.

Optimization of automated hearing test algorithms: simulations using an infant response model.

Computer simulation was used to evaluate several parameters of an automated hearing test algorithm in an attempt to optimize the algorithm for accuracy and efficiency. An infant response model was developed to guide the simulations. Test parameters of interest were starting intensity and stopping rule and their interaction with a measure that is thought to emulate infant reliability, probability of task orientation. Results indicated that stopping rule, within the ranges investigated, had little effect on accuracy but had major impact on efficiency. Starting intensity interacted with the hearing status of pseudosubjects in influencing accuracy. Accuracy was most influenced by probability of task orientation. The simulated test data are compared to data from infants and young children in the accompanying article so that the response model can be evaluated.

Computer methods for on-line hearing testing with auditory brain stem responses.

On-line computer methods were developed and evaluated for automated hearing testing with auditory brain stem responses (ABR). The system contained (1) a response recognition unit which recognized the presence or absence of an ABR response and (2) a threshold tracking unit which adjusted stimulus intensity and determined the ABR threshold. Two methods, one nontemplate (variance ratio) and one template (cross-correlation), were evaluated for response recognition while three threshold tracking methods were explored: clinical, Békésy, and PEST (parameter estimation by sequential testing). Both response recognition and threshold tracking units were evaluated by computer simulations for on-line operation using a standard set of experimentally recorded ABRs. The results indicated that while all the response recognition methods were reasonably accurate, their on-line use resulted in significant differences in test efficiency due to differences in tracking methods.

Evoked otoacoustic emissions in normal-hearing infants and children: emerging data and issues.

Evoked otoacoustic emissions (EOAEs) are a promising tool for evaluating cochlear status in children. Preliminary data from normal-hearing subjects ranging from birth to 29.9 years old are discussed. EOAEs are present and robust in infant ears. However, there is a statistically significant decrease in EOAE amplitude for a fixed stimulus level with increasing age even in a carefully screened sample. At the present time it is unclear if these age-associated changes are due to normal developmental changes in the external and/or middle ear acoustics, normal developmental changes in cochlear mechanics and/or everyday cochlear wear and tear. Issues related to further application of evoked emissions to pediatric populations are discussed.

Assessment techniques to evaluate tactual aids for hearing-impaired subjects.

In order to optimize the use of tactual aids for the deaf, it is important to have a battery of assessments to determine the potential contribution of the aids to acoustic perception and speech identification. We have designed such a battery to be used with young hearing-impaired children. The tasks were developed so that they could be implemented with standard audiometric equipment and applied to subjects of varying age and to different types of tactual aids. Illustrative results from four profoundly hearing-impaired children showed that tactual vocoders allowed detection of high frequencies that were not available to the subjects through aided audition. In most cases with these subjects, performance on simple detection and discrimination tasks showed facilitative effects with tactual vocoders. Facilitative effects were further evidenced in more complex phonemic identification tasks for all subjects. The tasks can be used to determine possible benefits of tactual aids for individual hearing-impaired children.

Auditory brainstem responses in young adults with Down syndrome.

Auditory brainstem response (ABR) wave V detection levels, latency, and amplitude were studied in a group of 15 young adults with Down syndrome for whom behavioral measures of hearing sensitivity were also obtained. Comparisons were made to a group of nonretarded control subjects matched by age and sex. Auditory brainstem response detection levels were elevated, response amplitude was reduced, and latency-intensity functions were significantly steeper for the subjects with Down syndrome than for the control group. These findings were associated with a high frequency (8000 Hz) hearing loss prevalent in the otherwise normal-hearing experimental group.

Auditory brain-stem responses in infants with Down's syndrome.

Auditory brain-stem responses to clicks were recorded from 38 infants with Down's syndrome at the ages of 3, 6, and 12 months in an attempt to delineate age-dependent and intensity-dependent latency changes in this population. Comparisons were made with 35 normally developing infants at the same age levels. Significant wave V latency differences were observed between groups for both age and intensity, with the group with Down's syndrome showing, in general, shorter absolute wave V latency values across age and steeper latency functions across intensity. The implications of this study are as follows: (1) latency-intensity curves for normally developing infants will not serve adequately as normative values for infants with Down's syndrome, particularly at the age of 12 months, and (2) cochlear function in infants with Down's syndrome may differ from normal infants by the age of 12 months.