Susan J. Norton

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 ≥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 mo corrected age. Although perfect test performance was never achieved, sensitivity for each measure increased with the magnitude of hearing loss. This latter finding is important because it suggests that all three tests performed better at identifying hearing losses for which intervention would be immediately recommended.

Identification of Neonatal Hearing Impairment: Ear-canal Measurements of Acoustic Admittance and Reflectance in Neonates

Objectives 1) To describe broad bandwidth measurements of acoustic admittance (Y) and energy reflectance (R) in the ear canals of neonates. 2) To describe a means for evaluating when a YR response is valid. 3) To describe the relations between these YR measurements and age, gender, left/right ear, and selected risk factors. Design YR responses were obtained at four test sites in well babies without risk indicators, well babies with at least one risk indicator, and graduates of neonatal intensive care units. YR responses were measured using a chirp stimulus at moderate levels over a frequency range from 250 to 8000 Hz. The system was calibrated based on measurements in a set of cylindrical tubes. The probe assembly was inserted in the ear canal of the neonate, and customized software was used for data acquisition. Results YR responses were measured in over 4000 ears, and half of the responses were used in e-ploratory data analyses. The particular YR variables chosen for analysis were energy reflectance, equivalent volume and acoustic conductance. Based on the view that unduly large negative equivalent volumes at low frequencies were physically impossible, it was concluded that appro-imately 13% of the YR responses showed evidence of improper probe seal in the ear canal. To test how these outliers influenced the overall pattern of YR responses, analyses were conducted both on the full data set (N = 2081) and the data set excluding outliers (N = 1825). The YR responses averaged over frequency varied with conceptional age (conception to date of test), gender, left/right ear, and selected risk factors; in all cases, significant effects were observed more frequently in the data set excluding outliers. After excluding outliers and controlling for conceptional age effects, the dichotomous risk factors accounting for the greatest variance in the YR responses were, in rank order, cleft lip and palate, aminoglycoside therapy, low birth weight, history of ventilation, and low APGAR scores. In separate analyses, YR responses varied in the first few days after birth. An analysis showed that the use of a YR test criterion to assess the quality of probe seal may help control the false-positive rate in evoked otoacoustic emission testing. Conclusions This is the first report of wideband YR responses in neonates. Data were acquired in a few seconds, but the responses are highly sensitive to whether the probe is fully sealed in the ear canal. A real-time acoustic test of probe fit is proposed to better address the probe seal problem. The YR responses provide information on middle-ear status that varies over the neonatal age range and that is sensitive to the presence or absence of risk factors, ear, and gender differences. Thus, a YR test may have potential for use in neonatal screening tests for hearing loss.

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%. Si-ty-si- 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 appro-imately 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: 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 ototo-ic 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 si- 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%), ototo-ic 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.

The effects of aging on otoacoustic emissions.

Otoacoustic emissions were measured in 42 normal hearing subjects ranging from 20 to 80 years old. For each subject spontaneous, click-evoked, tone-burst-evoked, stimulus frequency and distortion product emissions were measured across a wide intensity range for frequencies between 1 and 3 kHz. Although there are significant differences between age groups, the results indicate no age effect independent of hearing sensitivity on any type or parameter of otoacoustic emissions (OAE). The effect of increasing age is confounded with the effect of decreasing sensitivity such that post hoc analyses are inadequate to separate the effects of sensitivity and age on otoacoustic emissions. Even within the range of audiometrically normal hearing, OAE characteristics vary with threshold for all age groups. The conclusion is that hearing sensitivity must be included as a controlled variable in order to accurately assess intrinsic aging effects.

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: 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 : 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: 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 (verte- to mastoid or channel A and verte- 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 Fsp ≥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 Fsp 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.