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Purpose: The purpose of this study is to provide updated national estimates on the annual number, educational environments, and secondary school outcomes of students who are D/deaf and hard of hearing (D/HH) receiving special education (SpEd) and related services in the United States. Method: We performed a retrospective cross-sectional descriptive analysis of Individuals with Disabilities Education Act, Part B, Section 618 data from 2012 to 2018. Participants included students 6–21 years old in SpEd with “hearing impairment” reported as their primary disability. The general population of students in secondary school served as a comparator, via Current Population Survey data. We described the annual number of students (a) overall, (b) by educational environment, and (c) by reason for exiting SpEd, including the proportion graduating from and dropping out of secondary school. We described variation over time. Results: The median annual number of students was 67,655, with minimal variation by year. The proportion in general education (GenEd) for ≥ 80% of the day increased by 4.2% over 6 years from 57.8% to 62.0%, whereas the proportions in GenEd for < 40% and 40%–79% of the day decreased by 1.6% and 1.3%, respectively. Proportions in the remainder of the environments changed < 1.0% each. Of exiters, 86.8% of students graduated, whereas 3.9% dropped out, compared to a dropout rate of 5.0% in the general population. Conclusion: From 2012 to 2018, students who are D/HH receiving SpEd in the United States have spent increasingly more time in GenEd, most graduated from high school, and few dropped out, with dropout patterns appearing similar to the general population.

Objective: The Deaf community is an ethnolinguistic minority group. Low sensitivity to Deaf culture contributes to health disparities among Deaf patients. This study determines the level of sensitivity to Deaf culture among otolaryngology-head and neck surgery (OHNS) and audiology trainees. Methods: Cross-sectional survey study of OHNS and audiology trainees from 10 large US institutions. Trainees were queried on their exposure to and comfort with Deaf patients and their education on, attitude toward, and awareness and knowledge of Deaf culture. Sensitivity to Deaf culture was operationalized as awareness and knowledge of Deaf culture. These were assessed using a 35-item instrument that was previously developed using a d/Deaf community-based participatory approach to research. We used T-tests to compare the sample to previous samples of medical students with training in Deaf culture (MS-TDCs) and general practitioners (GPs). Results: There were 91 completed surveys (response rate 44.5%). Almost all were aware of Deaf culture (97.8%). The mean knowledge score was 55.0% (standard deviation (SD) 13.4%), which was significantly higher than that for GPs at 43.0% (SD 15.0%) (95% confidence interval 8.1%, 15.8%, P Conclusion: This sample of OHNS and audiology trainees was more sensitive to Deaf culture than GPs but less sensitive than MS-TDCs. Developing specialty-specific education may be warranted. Level of evidence: 4.

Objective: The study aim was to assess behavioral adjustment in preschool children with and without craniofacial microsomia (CFM). Design: Multisite cohort study of preschoolers with CFM (“cases”) or without CFM (“controls”). Participants: Mothers (89%), fathers (9%), and other caregivers (2%) of 161 preschoolers. Outcome Measure: Child Behavior Check List (CBCL 1.5-5); linear regressions with standardized effect sizes (ES) adjusted for sociodemographic confounds. Results: Child Behavior Check Lists for 89 cases and 72 controls (average age 38.3 ± 1.9 months). Children were male (54%), white (69%), and of Latino ethnicity (47%). Cases had microtia with mandibular hypoplasia (52%), microtia only (30%), or other CFM-associated features (18%). Nearly 20% of cases had extracranial anomalies. Composite CBCL scores were in the average range compared to test norms and similar for cases and controls. On the subscales, cases’ parents reported higher Anxious/Depressed scores (ES = 0.35, P = .04), Stress Problems (ES = 0.40, P = .04), Anxiety Problems (ES = 0.34, P = .04), and Autism Spectrum Problems (ES = 0.41, P = .02); however, the autism subscale primarily reflected speech concerns. Among cases, more problems were reported for children with extracranial anomalies and certain phenotypic categories with small ES. Conclusions: Behavioral adjustment of preschoolers with CFM was comparable to peers. However, parental reports reflected greater concern for internalizing behaviors; thus, anxiety screening and interventions may benefit children with CFM. Among cases, more problems were reported for those with more complex presentations of CFM. Craniofacial microsomia–related speech problems should be distinguished from associated psychosocial symptoms during developmental evaluations.

Cochlear implant depth of insertion affects audiologic outcomes and can be measured in adults using plain films obtained in the "cochlear view." The objective of this study was to assess interrater and intrarater reliability of measuring depth of insertion using cochlear view radiography.Prospective, observational.Tertiary referral pediatric hospital.Patients aged 11 months to 20 years (median, 4 years; interquartile range [IQR], 1-8 years) undergoing cochlear implantation at our institution were studied over 1 year. Children underwent cochlear view imaging on postoperative day 1. Films were deidentified and 1 image per ear was selected. Two cochlear implant surgeons and 2 radiologists evaluated each image and determined angular depth of insertion. Images were re-reviewed 6 weeks later by all raters. Inter- and intrarater reliability were calculated with intraclass correlation coefficients (ICCs).Fifty-seven ears were imaged from 42 children. Forty-nine ears (86%) had successful cochlear view x-rays. Median angular depth of insertion was 381° (minimum, 272°; maximum, 450°; IQR, 360°-395°) during the first round of measurement. Measurements of the same images reviewed 6 weeks later showed median depth of insertion of 382° (minimum, 272°; maximum, 449°; IQR, 360°-397°). Interrater and intrarater reliability ICCs ranged between 0.81 and 0.96, indicating excellent reliability.Postoperative cochlear view radiography is a reliable tool for measurement of cochlear implant depth of insertion in infants and children. Further studies are needed to determine reliability of intraoperatively obtained cochlear view radiographs in this population.

COVID-19 is a rapidly growing global pandemic caused by a novel coronavirus. With no vaccine or definitive treatment, public health authorities have recommended a strategy of "social distancing," reducing individual interaction, canceling elective procedures, and limiting nonessential services. Health care providers must determine what procedures are considered "elective," balancing risk of treatment delays with that of coronavirus exposure to patient, family, and providers. Given critical periods for language development and the long-term impact of auditory deprivation, some audiologic and otologic services should be considered essential. In this article, we describe the experience of a quaternary referral pediatric hospital in Seattle, the epicenter of COVID-19 in the United States, and share strategies for risk minimization employed by Seattle Children's Hospital. We hope that this work can be a reference for other centers continuing care for children who are deaf and hard of hearing during the COVID-19 and future resource-limiting crises.

Objective To evaluate the association between craniofacial phenotype and hearing loss in children with craniofacial microsomia. Design Retrospective cohort study. Setting Tertiary care children's hospital. Patients Individuals with craniofacial microsomia. Main Outcome Measures Ear-specific audiograms and standardized phenotypic classification of facial characteristics. Results A total of 79 participants were included in the study. The mean age was 9 years (range, 1 to 23 years) and approximately 60% were boys. Facial anomalies were bilateral in 39 participants and unilateral in 40 participants (24 right, 16 left). Microtia (hypoplasia of the ear) was the most common feature (94%), followed by mandibular hypoplasia (76%), soft tissue deficiency (60%), orbital hypoplasia or displacement (53%), and facial nerve palsy (32%). Sixty-five individuals had hearing loss (12 bilateral and 53 unilateral). Hearing loss was conductive in 73% of affected ears, mixed in 10%, sensorineural in 1%, and indeterminate in 16%. Hypoplasia of the ear or mandible was frequently associated with ipsilateral hearing loss, although contralateral hearing loss occurred in 8% of hemifaces. Conclusions Hearing loss is strongly associated with malformations of the ipsilateral ear in craniofacial microsomia and is most commonly conductive. Hearing loss can occur contralaterally to the side with malformations in children with apparent hemifacial involvement. Children with craniofacial microsomia should receive early diagnostic hearing assessments.

Objective:The Craniofacial microsomia: Longitudinal Outcomes in Children pre-Kindergarten (CLOCK) study is a longitudinal cohort study of neurobehavioral outcomes in infants and toddlers with craniofacial microsomia (CFM). In this article, we review the data collection and methods used to characterize this complex condition and describe the demographic and clinical characteristics of the cohort.Setting:Craniofacial and otolaryngology clinics at 5 study sites.Participants:Infants with CFM and unaffected infants (controls) ages 12 to 24 months were recruited from the same geographical regions and followed to age 36 to 48 months.Methods:Phenotypic, neurodevelopmental, and facial expression assessments were completed during the first and third waves of data collection (time 1 and time 3, respectively). Medical history data were taken at both of these time points and during an intermediate parent phone interview (time 2).Results:Our cohort includes 108 cases and 84 controls. Most cases and controls identified as white and 55% of cases and 37% of controls identified as Hispanic. Nearly all cases had microtia (95%) and 59% had mandibular hypoplasia. Cases received extensive clinical care in infancy, with 59% receiving care in a craniofacial clinic and 28% experiencing at least one surgery. Study visits were completed at a study site (92%) or at the participant’s home (8%).Conclusions:The CLOCK study represents an effort to overcome the challenges of characterizing the phenotypic and neurodevelopmental outcomes of CFM in a large, demographically and geographically diverse cohort.

Objective The U.S. Food and Drug Administration guidelines for cochlear implantation (CI) include age greater than 12 months. Studies have suggested that implantation in children younger than 12 months with congenital deafness may be associated with better spoken language outcomes. Compare auditory comprehension (AC) outcomes for children with congenital deafness who received CI less than 12 months of age to those implanted at 12 to 24 months of age. Methods Retrospective review of prospectively collected data in consecutively implanted patients under 2 years of age who received CI and had post-CI Preschool Language Scale (PLS)-AC scores. Receptive language was assessed with the AC subtest of the PLS. Patients without pre-CI PLS-AC scores were excluded. The association between age at implantation and post-CI PLS-AC scores up to 2 years after CI surgery was modeled using a linear mixed-effects model. Time from CI surgery, number of implants, risk factors for language delay, pre-CI PLS-AC score, and sex were included in the model. Patients implanted less than 12 months of age were compared to those implanted between 12 and 24 months. Results Twenty-nine patients who had CI surgery by 12 months and 82 who had CI surgery between 12 and 24 months were included in the analysis. Younger age at implantation and better pre-CI PLS-AC scores were significantly associated with better post-CI PLS-AC scores. Conclusion Cochlear implantation in children with congenital deafness less than 12 months of age was associated with better PLS-AC than in children implanted over 12 months of age up to 2 years after implantation. Level of evidence 4 Laryngoscope, 130:776-781, 2020.

Background: Children with steeply sloping sensorineural hearing loss (SNHL) lack access to critical high-frequency cues despite the use of advanced hearing aid technology. In addition, their auditory-only aided speech perception abilities often meet Food and Drug Administration criteria for cochlear implantation. Purpose: The objective of this study was to describe hearing preservation and speech perception outcomes in a group of young children with steeply sloping SNHL who received a cochlear implant (CI). Research Design: Retrospective case series. Study Sample: Eight children with steeply sloping postlingual progressive SNHL who received a unilateral traditional CI at Seattle Children’s Hospital between 2009 and 2013 and had follow-up data available up to 24 mo postimplant were included. Data Collection and Analysis: A retrospective chart review was completed. Medical records were reviewed for demographic information, preoperative and postoperative behavioral hearing thresholds, and speech perception scores. Paired t tests were used to analyze speech perception data. Hearing preservation results are reported. Results: Rapid improvement of speech perception scores was observed within the first month postimplant for all participants. Mean monosyllabic word scores were 76% and mean phoneme scores were 86.7% at 1-mo postactivation compared to mean preimplant scores of 19.5% and 31.0%, respectively. Hearing preservation was observed in five participants out to 24-mo postactivation. Two participants lost hearing in both the implanted and unimplanted ear, and received a sequential bilateral CI in the other ear after progression of the hearing loss. One participant had a total loss of hearing in only the implanted ear. Results reported in this article are from the ear implanted first. Bilateral outcomes are not reported. Conclusions: CIs provided benefit for children with steeply sloping bilateral hearing loss for whom hearing aids did not provide adequate auditory access. In our cohort, significant improvements in speech understanding occurred rapidly postactivation. Preservation of residual hearing in children with a traditional CI electrode is possible.

Objective To report and compare medical, radiological, and audiological outcomes in pediatric cochlear implant recipients who underwent 1.5 and 3 Tesla strength MRI with and without retained magnet. Methods Retrospective chart review at a tertiary care pediatric hospital and review of literature. Patients were identified via electronic medical records database search and were included if they had MRI after cochlear implant. Results Of twelve instances of MRI in pediatric cochlear implant recipients at our institution, two minor complications and one major complication were recorded. The rate of complication was equal between patients who underwent MRI with and without retained magnet. All minor complications resulted from MRI with retained magnet whereas the only major complication resulted from magnet removal. Two novel complications are reported, including: magnet removal resulting in silastic tear necessitating reimplantation and magnet dislocation with spontaneous reduction. Magnet removal significantly decreased the size of artifact, but did not alter the diagnostic utility of the MRI. While audiological measures varied chronologically from MRI scans, they did not appear to be appreciably altered by MRI. Conclusion MRI with and without magnet retention appear to carry risks of both major and minor complications. For the regions of interest for each scan, MRI quality was not appreciably altered by magnet status. Audiological measures appear unaffected by magnet status during MRI however, this may reflect natural variation.

Objective The goal of this study was to estimate psychophysical levels using the electrically evoked compound action potential (EAP), measured with the Neural Response Telemetry capabilities of Cochlear Corporation's Nucleus CI24M cochlear implant system. Design Twelve postlingually deafened adults with at least 3 mo of implant experience with the CI24M were subjects in this study. EAP growth functions were successfully quantified on each active electrode of every subject. Correlation and regression analyses were performed between EAP measures and cochlear implant fitting psychophysics. Other information including performance, etiology and duration of hearing loss, and individual electrode impedance was considered. Results EAP thresholds were found to be highly correlated with psychophysical thresholds. The rate of EAP growth with increasing stimulation levels was also found to be correlated with the dynamic range of loudness limits and psychophysical thresholds in some subjects. No relationship was evident between EAP measures and speech perception tests. Conclusions Information from EAP growth function measurements may be used to estimate psychophysical information used in cochlear implant fitting but not to predict performance with the device.

The deafwaddler ( dfw ) mouse mutant is caused by a spontaneous mutation in the gene that encodes a plasma membrane Ca 2+ ATPase (type 2), PMCA2 (Street et al., 1998. Nat. Genet. 19, 390–394), which is expressed in cochlear and vestibular hair cells. Distortion product otoacoustic emission (DPOAE) amplitudes and latencies were examined in control mice, deafwaddler mutants, and controls treated with the drug furosemide. Furosemide causes a transient reduction of DPOAEs (Mills et al., 1993. J. Acoust. Soc. Am. 94, 2108–2122). We wanted to determine whether DPOAEs obtained in furosemide-treated mice were similar or different from results obtained in +/ dfw mice. DPOAE amplitude and phase were measured as a function of f 2 / f 1 ratio. These data were converted into waveforms using inverse fast Fourier transform, and their average latency was used to estimate DPOAE group delay. Homozygous deafwaddlers did not produce DPOAEs. Heterozygous deafwaddlers (+/ dfw ) had increased DPOAE thresholds and reduced amplitudes at high frequencies, compared to controls. To the extent that DPOAEs depend on functional outer hair cells (OHCs), abnormal DPOAEs in +/ dfw mice suggest that PMCA2 is important for OHC function at high frequencies. Similar to the effects of furosemide, the mutation reduced DPOAEs for low-level stimuli; in contrast to furosemide, the mutation altered DPOAEs elicited by high levels.

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.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.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.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.

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.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.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 thresholdsor =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.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

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.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.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 ventilation5 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%).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.

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.

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 non-compliance 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-months behavioral hearing assessment protocols for validation purposes.

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.

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.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.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.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.

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 f 2 frequencies of 1.0, 1.5, 2.0, 3.0, and 4.0 kHz, and for primary levels (L 1 /L 2 ) 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 f 2 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 f 2 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 f 2 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 f 2 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.

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 e-amined. 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 e-amine the factors that influence test performance. Specific information related to these issues is presented in the articles that follow.

This study was undertaken to define the developmental period of maximal sensitivity to cisplatin ototoxicity in gerbils. Five groups were established based upon post-natal age (P) at exposure to cisplatin, P10 (n = 8), P14 (n = 8), P18 (n = 6), P22 (n = 7) and P42 (n = 7). Animals were given cisplatin, 1 mg/kg/day intraperitoneal for 4 days. In the first four groups, P10, P14, P18 and P22, distortion product otoacoustic emissions were measured at 45 days of age, when responses were expected to be developmentally stable. Distortion product grams and input-output functions were measured. There was a statistically significant difference only between P14 and P42 (P0.01). There was a significant interaction of age and frequency in the P14 group only (P0.01). A secondary analysis compared distortion product grams of P14 animals, exposed to cisplatin, and age-matched saline-treated animals (n = 6). There was a significant treatment effect. In summary, there was an effect of age on the cisplatin ototoxicity in gerbils. Also, there was an effect of the frequency on DPOAE levels in P14 gerbils. These data support the presence of a 'sensitive' period to cisplatin ototoxicity in gerbils.

OBJECTIVE Transient evoked otoacoustic emissions (TEOAEs) were measured in children with normal hearing and in children with hearing loss to investigate the characteristics of TEOAEs as they relate to overall amplitude and amplitude spectra of evoking stimuli, and to audiometric status. DESIGN Three parameters of response measure (signal to noise ratio, amplitude and reproducibility) were assessed to determine accuracy of identification of varying degrees of hearing loss for broadband clicks, frequency-specific click bands and tone bursts. Forty-four children (66 ears) between 4 and 13 yr of age were evaluated for participation in the study. Fifty-nine ears with intact tympanic membranes were included in the final analyses: 14 ears with normal hearing and 45 ears with hearing loss. RESULTS Children with normal hearing had robust responses that displayed nonlinear growth functions for broadband clicks and for tone bursts. Children with hearing loss had responses that decreased rapidly with decreasing stimulus level, if emissions were present at all. Data were analyzed using clinical decision analysis and receiver operator characteristic curves. Broadband clicks presented at 80 and 86 dB peSPL identified a hearing loss > or = 30 dB HL with a high degree of accuracy. Click responses filtered into octave bands centered at 500 and 1000 Hz did not improve classification of hearing loss, in fact, the 500 Hz band was particularly inaccurate. Results for click responses filtered into half-octave bands centered at 2000 and 4000 Hz were comparable with those for the broadband click, although the 2000 Hz band was superior for identification of hearing loss > or = 20 dB HL for an 80 dB peSPL click, and > or = 30 dB HL for an 86 dB peSPL click. Results for tone bursts centered at 500, 1000, 2000 and 4000 Hz, presented at 80 dB peSPL, were similar to results of the filtered click bands. Accuracy for identifying hearing loss increased with increasing center frequency. The 2000 and 4000 Hz tone bursts provided the best separation between normal-hearing and hearing-impaired ears, with 4000 Hz being slightly better. CONCLUSIONS Data from this study suggest that TEOAEs in children can separate ears with normal hearing from those with hearing loss using a variety of stimulus and response conditions. Moreover, by using multiple stimulus levels it may be possible to distinguish between mild and moderate hearing losses.

In this study, a preparation was developed which continuously monitored the f2−f1, 2 f1−f2, and 3f1−2 f2 distortion products in guinea pigs, in response to various contralateral and ipsilateral tones and broadband noise. A consistent decline over time in the quadratic distortion product, f2−f1, with continuous ipsilateral stimulation was observed, confirming previous findings by Kirk and Johnstone [Hearing Res. 67, 20–34 (1993)]. Broadband noise applied to the contralateral ear simultaneously increased both the magnitude and the rate of decline. This effect was shown for primaries across all frequencies tested (f2 from 2–16 kHz, f2/f1 ratio of 1.26), though a maximal effect was found for f2 of 4 kHz. Experiments using contralateral tones demonstrated a half-octave shift in the frequency specificity of this effect. A more “immediate” suppression effect on 2 f1−f2 (0.6 dB) was found in animals awakening from anesthesia, though this was even larger for f2−f1 (5.1 dB). Extended ipsilateral stimulation with co...

Ototoxicity associated with cis-platinum administration commonly presents as hearing loss and tinnitus. The hearing loss is usually an irreversible, high-frequency sensorineural loss. Histologic studies in humans and animals suggest that the outer hair cells (OHCs) are most susceptible to cis-platinum. Evoked otoacoustic emissions (EOAE), as a measure of outer hair cell function, are potentially useful in following ototoxic insults involving OHCs. Distortion-product otoacoustic emissions (DPOAE) test frequency-specific regions of the cochlea and therefore may be particularly well suited for monitoring ototoxic injuries. We measured distortion product otoacoustic emissions, at f2 = 2, 4, 6, 8, 10, and 12 kHz, in gerbils after a single large dose of cis-platinum. Animals treated with saline served as controls. The findings were compared to auditory brain stem evoked response (ABR) thresholds, using tone pips of the same frequencies. The DPOAE and ABR thresholds were measured before treatment and again 2, 5, and 14 days after drug administration. The changes in DPOAE were compared with the changes in ABR. No treatment effect was noted in the 2-day group. Animals treated with c/s-platinum demonstrated significant elevation of DPOAE and ABR thresholds compared with control animals at 5 and 14 days. There was no significant difference between the threshold changes in the 5-and 14-day groups.

Distortion product otoacoustic emissions (DPOAE) were measured serially in guinea pigs before and following 4-h exposures to a half-octave band of noise centered at 6 kHz. Stimulus parameters used to elicit the DPOAE were f2/f1 = 1.26 and L2 = L1-10. The 80 dB SPL exposures resulted in attenuation of emissions, which was maximal at the frequency one-half octave above the exposure when referenced to the f2 stimulus, and which recovered back to baseline after 2 days. The 90 dB SPL exposures resulted in a permanent deficit in emissions elicited by high-frequency stimuli, as measured after 8 days of recovery. A statistically significant difference was also found between animals exposed continuously for 4 h versus animals given two 2-h exposures separated by a 1-h break. Measures of f2-f1 and 3f1-2f2 indicated that they were more sensitive than 2f1-f2 to alterations in cochlear function after noise exposure.

Objectives: To determine hearing loss prevalence among a group of Third World children, to describe the physical examination and audiometric findings in the hearing-impaired children, and to assess the association of hearing loss with several known risk factors. It was hypothesized that chronic otorrhea would be the risk factor most strongly associated with hearing loss. Design: Community screening program to identify and evaluate hearing-impaired children. Case-control analysis of hearing loss risk factors. A survey taker, blinded to the children's hearing status, ascertained risk factors. Setting: Rural community in eastern province of Sierra Leone, West Africa. Patients: Population-based sample of 2015 children aged 5 to 15 years. Risk factor analysis was performed in 184 children and an equal number of matched controls. Main Outcome Measure: Hearing loss determined according to World Health Organization, Geneva, Switzerland, definition. Results: A total of 184 (9.1%) of 2015 children were found to have mild or greater hearing loss. The prevalence of bilateral profound hearing impairment was 4.0 per 1000. We assessed physical examination and audiometric findings. The risk factor most strongly associated with hearing loss was a history of otorrhea persisting longer than 1 month (odds ratio, 23.3; 95% confidence limits, 12.11, 45.40). Conclusions: A high prevalence of hearing loss was identified. Much of this impairment may result from chronic untreated or unrecognized ear infections. Further community-based studies of hearing-impaired children are necessary for planning preventive and curative programs. (Arch Otolaryngol Head Neck Surg. 1995;121:853-858)

The number of pediatric cochlear implant (CI) recipients has increased substantially over the past 10 years, and it has become more important to understand the underlying mechanisms of the variable outcomes in this population. In this study, psychoacoustic measures of spectral-ripple and Schroeder-phase discrimination, the Clinical Assessment of Music Perception, and consonant-nucleus-consonant (CNC) word recognition in quiet and spondee reception threshold (SRT) in noise tests have been presented to 11 prelingually deafened CI users, aged 8–16 years with at least 5 years of CI experience. The children’s performance was compared to the previously reported results of postlingually deafened adult CI users. The average spectral-ripple threshold (n = 10) was 2.08 ripples/octave. The average Schroeder-phase discrimination was 67.3% for 50 Hz and 56.5% for 200 Hz (n = 9). The Clinical Assessment of Music Perception test showed that the average complex pitch direction discrimination was 2.98 semitones. The mean melody score was at a chance level, and the mean timbre score was 34.1% correct. The mean CNC word recognition score was 68.6%, and the mean SRT in steady noise was –8.5 dB SNR. The children’s spectral-ripple resolution, CNC word recognition, and SRT in noise performances were, within statistical bounds, the same as in a population of postlingually deafened adult CI users. However, Schroeder-phase discrimination and music perception were generally poorer than in the adults. It is possible then that this poorer performance seen in the children might be partly accounted for by the delayed maturation in their temporal processing ability, and because of this, the children’s performance may have been driven more by their spectral sensitivity.

To compare the prevalence of congenital cytomegalovirus (CMV) infection in Washington State in children with hearing loss (HL) and the general population and to compare the characteristics of HL in children with and without congenital CMV infection.Matched case-control; case cohort.Regional pediatric hospital, Washington State Department of Health (WSDOH).Cases were children 4 years and older with HL born in Washington State. Control individuals matched for demographic characteristics were identified at random through the WSDOH.Congenital CMV status determined using quantitative polymerase chain reaction testing on newborn heel stick blood spots archived by the WSDOH. Audiologic data were used to characterize HL.Congenital CMV testing was performed for 222 matched cases and controls. Congenital CMV infection was detected in 1.4% of controls and in 9.9% of cases (odds ratio, 10.5; 95% confidence interval, 2.6-92.4). An estimated 8.9% of HL in children in Washington can be attributed to CMV infection. After inclusion of an additional 132 children with HL (for a total of 354 cases in the case cohort), we observed that children with congenital CMV had more severe HL (P.001) and higher proportions of progressive (P = .02) and unilateral (P = .002) HL compared with children without congenital CMV infection. In the 35 children with congenital CMV infection, there was no relationship between neonatal CMV load and severity of HL.In Washington State, children with HL had a far higher prevalence of congenital CMV viremia than did the general pediatric population, and CMV infection seems to be responsible for an appreciable fraction of pediatric HL in Washington State.

The endocochlear potential (EP) was reversibly decreased in adult gerbils by the intraperitoneal injection of furosemide, while cochlear functioning was monitored by measurement of distortion production otoacoustic emissions (DPE) at a range of stimulus intensities. Stimulus frequencies for DPEs were f1 = 6.8 and f2 = 8 kHz (f2/f1 = 1.18). Emissions monitored in the ear canal and scala media were 2f1-f2, 3f1-2f2, 2f2-f1, and f2-f1. Typically, the EP decreased smoothly, reached a minimum one-half hour after injection, then recovered slowly over several hours. Emissions at 2f1-f2 and 3f1-2f2 at low stimulus levels were particularly vulnerable to the change in EP. These vulnerable emissions showed characteristic trajectories in which the amplitudes changed little with the initial EP decrease, then dropped sharply as the EP continued to decrease. However, the amplitudes then began to recover even before the EP reached minimum, and recovered completely while the EP remained subnormal. The trajectories of the other odd order emissions were similar, but lacked the abrupt decrease. The variation of the even order (f2-f1) component was completely different, but appeared related to the odd order trajectories in a complex fashion. During the initial decrease for the vulnerable components, the decrease in emission amplitude (in dB) was found to be proportional to the square of the change in EP (in mV). The recovery with a subnormal EP was interpreted as an adaptive effect with a time constant of about 15 min.

Objectives: First, to establish the feasibility of the observer-based psychophysical procedure (OPP) in measuring sound detection in infant and toddler cochlear implant (CI) recipients. Second, to measure the psychometric function for detection (PFD) from individual subjects. Third, to determine whether reaction time (RT) provides information about the auditory sensitivity of young CI users. Design: Twelve CI recipients, 11 to 32 mo old, participated in our study. Initially, tones were presented in sound field, and children learned to respond when they heard tones but not at other times. Once an 80% correct criterion was met in sound field, a novel stimulation paradigm was used to present stimuli to a single electrode while the child listened to acoustic input on most other electrodes using their usual map. The PFD and RT were measured using this single-electrode stimulation paradigm. Results: Eleven subjects met criterion, 6 within the minimum possible number of trials. For eight subjects, the asymptotic level of detecting single-electrode stimuli averaged 86% correct, similar to levels achieved by normal-hearing infants and toddlers detecting pure tones. The PFD slope of infant and toddler CI recipients was less than or equal to the slope for adult CI users reported in previous studies. RT decreased significantly with stimulus level in four children. Conclusions: These preliminary results suggest that psychophysical detection data can be obtained from infant and toddler CI recipients using OPP. The PFD of young CI users may be shallower than that of adult CI users. Relatively good asymptotic detection performance implies that young CI users are more attentive to sound than has been suggested in previous studies. RT tended to be a less reliable measure of detection, but methodological changes could improve its utility.

The development of the acoustic distortion product (ADP) 2ƒ 1 —ƒ 2 was studied in gerbils, beginning 12 days after birth (P12). ADPs were measured as a function of stimulus frequency region (1.0 to 13.0 kHz) and level (10 to 80 dB SPL). There was an orderly progression in the appearance and maturation of the emissions, with responses to high-frequency stimuli ( ƒ 2 = 13.0 kHz ) appearing first, at P13–14. Responses to mid and high frequencies ( ƒ 2 = 3.9 to 13.0 kHz ) matured earlier than responses to lower frequencies. Responses to low-frequency stimuli ( ƒ 2 = 1.3 KHz ) did not appear until P18–19 and were not mature until after one month of age. The first emissions to develop in a given frequency region had elevated thresholds, were reduced in amplitude, and displayed monotonie input-output functions. As the auditory system matured, emission growth functions became non-monotonic displaying saturation, but initially retained a reduced dynamic range. Data from the developing gerbil suggest that initially its cochlear mechanics are passive and that active elements associated with normal outer hair cell function mature first in the basal turn and last near the apex. Furthermore, the development of active nonlinear elements underlying ADP generation is consistent with the development of frequency selectivity and developmental shifts in the place code which have been demonstrated in the gerbil.

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.

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.

Objectives/Hypothesis: Congenital severe to profound sensorineural hearing loss (SNHL) is found in higher proportions of children with minority and/or lower socioeconomic status (SES). Cochlear implants were approved by the U.S. Food and Drug Administration for use in children with bilateral severe to profound SNHL in 1990. The objectives of the study were as follows: 1) to study the epidemiology of pediatric cochlear implantation, assessing whether cochlear implant technology is provided to children with severe to profound SNHL in proportion to their racial/ethnic or SES, and 2) to compare data provided by a national health care database with data provided by cochlear implant manufacturers. Study Design: Patients aged 0 to 18 years who underwent cochlear implantation in 1997 using a cross-sectional study design. Methods: Analyses were made of pediatric cochlear implant patients, using data from the 1997 Health Care and Utilization Project/Kids' Inpatient Database. Relative rates of implantation compared with rates of severe to profound SNHL were calculated using national estimates generated from census and Galludet Research Institution data. Logistic regression analysis was carried out to compare implanted children of different racial/ethnic backgrounds. A surrogate measure of socioeconomic status was used based on the median household income of the patient's home ZIP code. Information was also obtained from the two companies producing U.S. Food and Drug Administration–approved cochlear implants in 1997 and used to determine whether the data obtained from the Health Care and Utilization Project/Kids' Inpatient Database were representative of the national cohort of implanted children. Results: The Health Care and Utilization Project/Kids' Inpatient Database identified 124 children who underwent cochlear implant surgery in 1997. White and Asian children were implanted at higher rates than Hispanic and black children. Furthermore, white and Asian children received implants at greater rates than would be expected based on prevalence of severe to profound SNHL. The relative rate (RR) of implantation, defined as the proportion of children who received cochlear implants divided by the proportion of children with severe to profound SNHL (in each race/ethnicity group compared with the same ratio in white children), was similar in white (RR = 1.00) and Asian (RR = 0.93) children but markedly different in Hispanic (RR = 0.28) and black (RR = 0.10) children. Comparison of SES information from the Health Care and Utilization Project/Kids' Inpatient Database population with the manufacturers' database suggested that the Health Care and Utilization Project/Kids' Inpatient Database is representative of all implanted children in the United States. Both sources of information suggested that children receiving cochlear implants in the United States in 1997 resided in above-average SES areas. Conclusion: White and Asian children with severe to profound SNHL had higher proportionate rates of cochlear implantation than black and Hispanic children in 1997. Implanted children were more likely to live in areas (represented by ZIP codes) with higher median incomes. Although there was a disparity in rate of cochlear implantation based on race/ethnicity and surrogate measures of SES, these data did not allow the authors to determine the causes for these differences.

Problem: Pediatric cochlear implant recipients have been historically assessed postoperatively with audiometric testing in a soundproof booth by trained audiologists. Assessment of functional outcome after cochlear implantation must also consider a number of other factors including speech perception, speech intelligibility, mode of communication, educational placement, as well as the social and psychological factors that affect one’s ability to function in the mainstream environment. No uniform tool for assessing effective functional outcome in the daily life of pediatric cochlear implant recipients has been developed. Methods: Using a cross-sectional design, the COCHLEA (Children’s Outcome for Cochlear Implant Hearing, Language, and Environmental Assessment) questionnaire, designed to measure functional outcomes in the pediatric cochlear implant population, was administered. The questionnaire was tested for reliability and validity, while investigating for any possible respondent or administrative burden. The parents of children with cochlear implants completed the questionnaire. The subjects were individuals with nonsyndromic sensorineural hearing loss (SNHL) implanted at Children’s Hospital and Regional Medical Center before 12/31/2001, aged 3–12 years. Implantation must have occurred before age 7. Non-English-speaking children/families were excluded secondary to a lack of resources. Results: Thirty-one patients were enrolled in the study. Preliminary results indicate that absolute scores of this outcome measure correlate with higher global health measures, including the Child Health Questionnaire (CHQ), and predict better audiometric scores. Conclusion: The COCHLEA functional outcome measure appears to be a reliable and valid method for assessing effective cochlear implantation. More research is necessary to determine this questionnaire’s sensitivity to longitudinal change. Significance: The COCHLEA questionnaire represents the first disease-specific functional outcome measure for the pediatric cochlear implant patient population. Ultimately it may be used to compare the effectiveness of cochlear implants and rehabilitation strategies. Support: None reported.

Objective We sought to demonstrate the rate of change in distortion product otoacoustic emission (DPOE) amplitude with age in relation to hearing loss in an unselected adult population. Study design and setting We conducted a cross-sectional observation study involving the Framingham Offspring Cohort. Age changes in DPOE amplitude for frequencies of f2 from 1 to 8 kHz adjusted for pure-tone threshold level were assessed by multivariate linear regression. Results The women showed a mean hearing threshold-adjusted loss in high-frequency DPOE amplitude of 0.6, 2.1, 2.6, and 1.1 dB/per decade at the f2 frequencies of 1, 2, 4, and 8 kHz, respectively. In contrast, the men showed no effect of age on the DPOE amplitude independent of hearing loss. Emissions were reduced or absent in the noise notch frequencies. The rate of change with age in DPOE amplitude was significantly less than the rate of change in pure-tone thresholds in both the men and the women. Conclusion Women lose DPOE amplitude from both age and hearing threshold loss. Men lose more DPOE amplitude than do women, and the loss is proportional to the degree of loss of hearing threshold sensitivity. The differential effect whereby age-related hearing loss affects thresholds more than emissions suggests that strial atrophy may be a pathophysiologic factor. Significance The use of DPOE measures for screening and monitoring cochlear status of adult women should take into account the age, pure-tone thresholds, and noise exposure status of the subjects.

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.

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.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."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.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.

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.

• Objective. —Screening auditory status prior to neonatal hospital discharge to identify newborns with severe hearing impairment is an important pediatric care priority. Evoked otoacoustic emission (OAE) testing is a quick noninvasive method. The purpose of this study was to determine the relationship between external auditory canal and middle ear status with click-evoked OAE. It was hypothesized that vernix caseosa, debris in the ear canal, and middle ear fluid contribute to the OAE fail rate. Design. —All neonates had an initial OAE examination. A second investigator, "blinded" to the results, examined all ears otoscopically, cleaned any obstructing debris, and repeated with a second OAE test. Setting. —All neonates were tested in a designated nursery at a mean age of 43±21 hours. Patients. —Forty-one full-term neonates were prospectively enrolled. Intervention. —The ear canals with debris were cleaned under direct vision with a pediatric swab dampened by an alcohol wipe. Outcome Measure —The primary outcome measure was the postcleaning OAE pass rate. Results. —The preotoscopic examination pass rate of 82 ears was 76%. The OAE pass rate improved to 91% after debris removal. Conclusions. —The results indicate that the examination and cleaning of the external ear canal are important components of the neonatal screening process. (Arch Otolaryngol Head Neck Surg.1993;119:276-282)

Transient evoked otoacoustic emissions (TEOAEs) occur after presentation of brief acoustic stimuli such as clicks and tone pips. They represent physiological activity from within the cochlea, specifically from normal functioning outer hair cells. TEOAEs are frequency specific in that their spectra are determined by the spectra of the evoking stimulus and the audiometric configuration. TEOAEs are sensitive to mild to moderate degrees of cochlear hearing loss up to about 40 to 50 dB HL. They can be measured rapidly and noninvasively in infants and children. Among the potential applications in pediatric audiology are screening for hearing impairment in neonates, separating peripheral hearing loss and central auditory dysfunction, and monitoring cochlear status in children receiving ototoxic drugs.

Attempts to identify the mechanisms underlying tinnitus and to develop effective treatments have been frustrating, in part because there are no objective measures of tinnitus. Following Kemp's initial reports of evoked and spontaneous otoacoustic emissions (OAEs), many people hoped that OAEs were an objective correlate of tinnitus. The results of several studies suggest that in about 6 to 12% of normal-hearing persons with tinnitus and spontaneous otoacoustic emissions (SOAEs), the SOAEs are at least partly responsible for the tinnitus. In addition, recent work indicates that some subjects with tinnitus display oscillating or ringing evoked otoacoustic emissions (EOAEs). In these cases, it is hypothesized that the oscillating EOAEs and tinnitus are related to a common underlying pathology rather than the emissions being the source of the tinnitus.

In recent years, a number of investigators have suggested that the mammalian cochlea consists of two systems: one sharply tuned and physiologically vulnerable, and the other broadly tuned and relatively invulnerable. Davis (1983) suggested these two systems were active and passive, respectively. The majority of data supporting this hypothesis come from animal studies including Mossbauer measurements of basilar membrane displacement (e.g. Sellick. Patuzzi and Johnstone. 1982; Johnstone. Patuzzi and Sellick, 1984; Johnstone. Patuzzi and Yates, 1986) and recordings from single 8th nerve fibers (c.g. Sellick, Patuzzi and Johnstone, 1982; and sec Kiang et al., 1986 for a review).