Childhood Listening and Associated Cognitive Difficulties Persist Into Adolescence.

OBJECTIVE: Listening difficulty (LiD) refers to the challenges individuals face when trying to hear and comprehend speech and other sounds. LiD can arise from various sources, such as hearing sensitivity, language comprehension, cognitive function, or auditory processing. Although some children with LiD have hearing loss, many have clinically normal audiometric thresholds. To determine the impact of hearing and cognitive factors on LiD in children with a clinically normal audiogram, we conducted a longitudinal study. The Evaluation of Children's Listening & Processing Skills (ECLiPS), a validated and standardized caregiver evaluation tool, was used to group participants as either LiD or typically developing (TD). Our previous study aimed to characterize LiD in 6- to 13-year-old children during the project's baseline, cross-sectional phase. We found that children with LiD needed a higher signal-to-noise ratio during speech-in-speech tests and scored lower on all assessed components of the NIH Cognition Toolbox than TD children. The primary goal of this study was to examine if the differences between LiD and TD groups are temporary or enduring throughout childhood. DESIGN: This longitudinal study had three data collection waves for children with LiD and TD aged 6 to 13 years at Wave 1, followed by assessments at 2-year (Wave 2) and 4-year (Wave 3) intervals. Primary analysis focused on data from Waves 1 and 2. Secondary analysis encompassed all three waves despite high attrition at Wave 3. Caregivers completed the ECLiPS, while participants completed the Listening in Spatialized Noise-Sentences (LiSN-S) test and the NIH-Toolbox Cognition Battery during each wave. The analysis consisted of (1) examining longitudinal differences between TD and LiD groups in demographics, listening, auditory, and cognitive function; (2) identifying functional domains contributing to LiD; and (3) test-retest reliability of measures across waves. Mixed-effect models were employed to analyze longitudinal data. RESULTS: The study enrolled 169 participants, with 147, 100, and 31 children completing the required testing during Waves 1, 2, and 3, respectively. The mean ages at these waves were 9.5, 12.0, and 14.0 years. On average, children with LiD consistently underperformed TD children in auditory and cognitive tasks across all waves. Maternal education, auditory, and cognitive abilities independently predicted caregiver-reported listening skills. Significant correlations between Waves 1 and 2 confirmed high, long-term reliability. Secondary analysis of Wave 3 was consistent with the primary analyses of Waves 1 and 2, reinforcing the enduring nature of listening difficulties. CONCLUSION: Children with LiD and clinically normal audiograms experience persistent auditory, listening, and cognitive challenges through at least adolescence. The degree of LiD can be independently predicted by maternal education, cognitive processing, and spatial listening skills. This study underscores the importance of early detection and intervention for childhood LiD and highlights the role of socioeconomic factors as contributors to these challenges.

Extended High-Frequency Audiometry using the Wireless Automated Hearing Test System Compared to Manual Audiometry in Children and Adolescents.

Objectives: Reliable wireless automated audiometry that includes extended high frequencies (EHF) outside a sound booth would increase access to monitoring programs for individuals at risk for hearing loss, particularly those at risk for ototoxicity. The purpose of the study was to compare thresholds obtained with 1) standard manual audiometry to automated thresholds measured with the Wireless Automated Hearing Test System (WAHTS) inside a sound booth, and 2) automated audiometry in the sound booth to automated audiometry outside the sound booth in an office environment. Design: Cross-sectional, repeated measures study. Twenty-eight typically developing children and adolescents (mean = 14.6 yrs; range = 10 to 18 yrs). Audiometric thresholds were measured from 0.25 to 16 kHz with manual audiometry in the sound booth, automated audiometry in the sound booth, and automated audiometry in a typical office environment in counterbalanced order. Ambient noise levels were measured inside the sound booth and the office environment were compared to thresholds at each test frequency. Results: Automated thresholds were overall about 5 dB better compared to manual thresholds, with greater differences in the extended high frequency range (EHF;10-16 kHz). The majority of automated thresholds measured in a quiet office were within ± 10 dB of automated thresholds measured in a sound booth (84%), while only 56% of automated thresholds in the sound booth were within ± 10 dB of manual thresholds. No relationship was found between automated thresholds measured in the office environment and the average or maximum ambient noise level. Conclusions: These results indicate that self-administered, automated audiometry results in slightly better thresholds overall than manually administered audiometry in children, consistent with previous studies in adults. Ambient noise levels in a typical office environment did not have an adverse effect on audiometric thresholds measured using noise attenuation headphones. Thresholds measured using an automated tablet with noise attenuating headphones could improve access to hearing assessment for children with a variety of risk factors. Additional studies of extended high frequency automated audiometry in a wider age range are needed to establish normative thresholds.

Brainstem auditory physiology in children with listening difficulties.

Children who have listening difficulties (LiD) despite having normal audiometry are often diagnosed as having an auditory processing disorder. A lack of evidence regarding involvement of specific auditory mechanisms has limited development of effective treatments for these children. Here, we examined electrophysiologic evidence for brainstem pathway mechanisms in children with and without defined LiD. We undertook a prospective controlled study of 132 children aged 6-14 years with normal pure tone audiometry, grouped into LiD (n = 63) or Typically Developing (TD; n = 69) based on scores on the Evaluation of Children's Listening and Processing Skills (ECLiPS), a validated caregiver report. The groups were matched on age at test, sex, race, and ethnicity. Neither group had diagnoses of major neurologic disorder, intellectual disability, or brain injuries. Both groups received a test battery including a measure of receptive speech perception against distractor speech, Listening in Spatialized Noise - Sentences (LiSN-S), along with multiple neurophysiologic measures that tap afferent and efferent auditory subcortical pathways. Group analysis showed that participants with LiD performed significantly poorer on all subtests of the LiSN-S. The LiD group had significantly greater wideband middle ear muscle reflex (MEMR) growth functions in the left ear, and shorter Wave III and Wave V latencies in auditory brainstem responses (ABR). Across individual participants, shorter latency ABR Wave V correlated significantly with poorer parent report of LiD (ECLiPS composite). Greater MEMR growth functions also correlated with poorer ECLiPS scores and reduced LiSN-S talker advantage. The LiD and TD groups had equivalent summating potentials, compound action potentials, envelope-following responses, and binaurally activated medial olivocochlear reflexes. In conclusion, there was no evidence for auditory synaptopathy for LiD. Evidence for brainstem differences in the LiD group was interpreted as increased central gain, with shorter ABR Wave III and V latencies and steeper MEMR growth curves. These differences were related to poorer parent report and speech perception in competing speech ability.

Within- and across-frequency temporal processing and speech perception in cochlear implant users.

OBJECTIVE: Cochlear implant (CI) recipient's speech perception performance is highly variable and is influenced by temporal processing abilities. Temporal processing is commonly assessed using a behavioral task that requires the participant to detect a silent gap with the pre- and post-gap stimuli of the same frequency (within-frequency gap detection) or of different frequencies (across-frequency gap detection). The purpose of the study was to evaluate behavioral and electrophysiological measures of within- and across-frequency temporal processing and their correlations with speech perception performance in CI users. DESIGN: Participants included 11 post-lingually deafened adult CI users (n = 15 ears; Mean Age = 50.2 yrs) and 11 age- and gender-matched normal hearing (NH) individuals (n = 15 ears; Mean Age = 49.0 yrs). Speech perception was assessed with Consonant-Nucleus-Consonant Word Recognition (CNC), Arizona Biomedical Sentence Recognition (AzBio), and Bamford-Kowal-Bench Speech-in-Noise Test (BKB-SIN) tests. Within- and across-frequency behavioral gap detection thresholds (referred to as the GDTwithin and GDTacross) were measured using an adaptive, two-alternative, forced-choice procedure. Cortical auditory evoked potentials (CAEPs) were elicited using within- and across-frequency gap stimuli under four gap duration conditions (no gap, GDT, sub-threshold GDT, and supra-threshold GDT). Correlations among speech perception, GDTs, and CAEPs were examined. RESULTS: CI users had poorer speech perception scores compared to NH listeners (p < 0.05), but the GDTs were not different between groups (p > 0.05). Compared to NH peers, CI users showed increased N1 latency in the CAEPs evoked by the across-frequency gap stimuli (p < 0.05). No group difference was observed for the CAEPs evoked by the within-frequency gap (p > 0.05). Three CI ears showing the longest GDTwithin also showed the poorest performance in speech in noise. The within-frequency CAEP increased in amplitude with the increase of gap duration; while the across-frequency CAEP displayed a similar amplitude for all gap durations. There was a significant correlation between speech scores and within-frequency CAEP measures for the supra-threshold GDT condition, with CI users with poorer speech performance having a smaller N1-P2 amplitude and longer N1 latency. No correlations were found among GDTacross, speech perception, and across-frequency CAEP measures. CONCLUSIONS: Within- and across-frequency gap detection may involve different neural mechanisms. The within-frequency gap detection task can help identify CI users with poor speech performance for rehabilitation. The within-frequency CAEP is a better predictor for speech perception performance than the across-frequency CAEP.

Functional Impacts of Aminoglycoside Treatment on Speech Perception and Extended High-Frequency Hearing Loss in a Pediatric Cystic Fibrosis Cohort.

Purpose The purpose of this study is to better understand the prevalence of ototoxicity-related hearing loss and its functional impact on communication in a pediatric and young adult cohort with cystic fibrosis (CF) and individuals without CF (controls). Method We did an observational, cross-sectional investigation of hearing function in children, teens, and young adults with CF (n = 57, M = 15.0 years) who received intravenous aminoglycoside antibiotics and age- and gender-matched controls (n = 61, M = 14.6 years). Participants completed standard and extended high-frequency audiometry, middle ear measures, speech perception tests, and a hearing and balance questionnaire. Results Individuals with CF were 3-4 times more likely to report issues with hearing, balance, and tinnitus and performed significantly poorer on speech perception tasks compared to controls. A higher prevalence of hearing loss was observed in individuals with CF (57%) compared to controls (37%). CF and control groups had similar proportions of slight and mild hearing losses; however, individuals with CF were 7.6 times more likely to have moderate and greater degrees of hearing loss. Older participants displayed higher average extended high-frequency thresholds, with no effect of age on average standard frequency thresholds. Although middle ear dysfunction has not previously been reported to be more prevalent in CF, this study showed that 16% had conductive or mixed hearing loss and higher rates of previous otitis media and pressure equalization tube surgeries compared to controls. Conclusions Individuals with CF have a higher prevalence of conductive, mixed, and sensorineural hearing loss; poorer speech-in-noise performance; and higher rates of multiple symptoms associated with otologic disorders (tinnitus, hearing difficulty, dizziness, imbalance, and otitis media) compared to controls. Accordingly, children with CF should be asked about these symptoms and receive baseline hearing assessment(s) prior to treatment with potentially ototoxic medications and at regular intervals thereafter in order to provide otologic and audiologic treatment for hearing- and ear-related problems to improve communication functioning.

Peripheral Auditory Involvement in Childhood Listening Difficulty.

OBJECTIVES: This study tested the hypothesis that undetected peripheral hearing impairment occurs in children with idiopathic listening difficulties (LiDs), as reported by caregivers using the Evaluation of Children"s Listening and Processing Skills (ECLiPS) validated questionnaire, compared with children with typically developed (TD) listening abilities. DESIGN: Children with LiD aged 6-14 years old (n = 60, mean age = 9.9 yr) and 54 typical age matched children were recruited from audiology clinical records and from IRB-approved advertisements at hospital locations and in the local and regional areas. Both groups completed standard and extended high-frequency (EHF) pure-tone audiometry, wideband absorbance tympanometry and middle ear muscle reflexes, distortion product and chirp transient evoked otoacoustic emissions. Univariate and multivariate mixed models and multiple regression analysis were used to examine group differences and continuous performance, as well as the influence of demographic factors and pressure equalization (PE) tube history. RESULTS: There were no significant group differences between the LiD and TD groups for any of the auditory measures tested. However, analyses across all children showed that EHF hearing thresholds, wideband tympanometry, contralateral middle ear muscle reflexes, distortion product, and transient-evoked otoacoustic emissions were related to a history of PE tube surgery. The physiologic measures were also associated with EHF hearing loss, secondary to PE tube history. CONCLUSIONS: Overall, the results of this study in a sample of children with validated LiD compared with a TD group matched for age and sex showed no significant differences in peripheral function using highly sensitive auditory measures. Histories of PE tube surgery were significantly related to EHF hearing and to a range of physiologic measures in the combined sample.

A Preliminary Study of the Effects of Attentive Music Listening on Cochlear Implant Users' Speech Perception, Quality of Life, and Behavioral and Objective Measures of Frequency Change Detection.

INTRODUCTION: Most cochlear implant (CI) users have difficulty in listening tasks that rely strongly on perception of frequency changes (e.g., speech perception in noise, musical melody perception, etc.). Some previous studies using behavioral or subjective assessments have shown that short-term music training can benefit CI users' perception of music and speech. Electroencephalographic (EEG) recordings may reveal the neural basis for music training benefits in CI users. OBJECTIVE: To examine the effects of short-term music training on CI hearing outcomes using a comprehensive test battery of subjective evaluation, behavioral tests, and EEG measures. DESIGN: Twelve adult CI users were recruited for a home-based music training program that focused on attentive listening to music genres and materials that have an emphasis on melody. The participants used a music streaming program (i.e., Pandora) downloaded onto personal electronic devices for training. The participants attentively listened to music through a direct audio cable or through Bluetooth streaming. The training schedule was 40 min/session/day, 5 days/week, for either 4 or 8 weeks. The pre-training and post-training tests included: hearing thresholds, Speech, Spatial and Qualities of Hearing Scale (SSQ12) questionnaire, psychoacoustic tests of frequency change detection threshold (FCDT), speech recognition tests (CNC words, AzBio sentences, and QuickSIN), and EEG responses to tones that contained different magnitudes of frequency changes. RESULTS: All participants except one finished the 4- or 8-week training, resulting in a dropout rate of 8.33%. Eleven participants performed all tests except for two who did not participate in EEG tests. Results showed a significant improvement in the FCDTs as well as performance on CNC and QuickSIN after training (p < 0.05), but no significant improvement in SSQ scores (p > 0.05). Results of the EEG tests showed larger post-training cortical auditory evoked potentials (CAEPs) in seven of the nine participants, suggesting a better cortical processing of both stimulus onset and within-stimulus frequency changes. CONCLUSION: These preliminary data suggest that extensive, focused music listening can improve frequency perception and speech perception in CI users. Further studies that include a larger sample size and control groups are warranted to determine the efficacy of short-term music training in CI users.

Extended high frequency hearing and speech perception implications in adults and children.

Extended high frequencies (EHF), above 8 kHz, represent a region of the human hearing spectrum that is generally ignored by clinicians and researchers alike. This article is a compilation of contributions that, together, make the case for an essential role of EHF in both normal hearing and auditory dysfunction. We start with the fundamentals of biological and acoustic determinism - humans have EHF hearing for a purpose, for example, the detection of prey, predators, and mates. EHF hearing may also provide a boost to speech perception in challenging conditions and its loss, conversely, might help explain difficulty with the same task. However, it could be that EHF are a marker for damage in the conventional frequency region that is more related to speech perception difficulties. Measurement of EHF hearing in concert with otoacoustic emissions could provide an early warning of age-related hearing loss. In early life, when EHF hearing sensitivity is optimal, we can use it for enhanced phonetic identification during language learning, but we are also susceptible to diseases that can prematurely damage it. EHF audiometry techniques and standardization are reviewed, providing evidence that they are reliable to measure and provide important information for early detection, monitoring and possible prevention of hearing loss in populations at-risk. To better understand the full contribution of EHF to human hearing, clinicians and researchers can contribute by including its measurement, along with measures of speech in noise and self-report of hearing difficulties and tinnitus in clinical evaluations and studies.

High frequency transient-evoked otoacoustic emission measurements using chirp and click stimuli.

Transient-evoked otoacoustic emissions (TEOAEs) at high frequencies are a non-invasive physiological test of basilar membrane mechanics at the basal end, and have clinical potential to detect risk of hearing loss related to outer-hair-cell dysfunction. Using stimuli with constant incident pressure across frequency, TEOAEs were measured in experiment 1 at low frequencies (0.7-8 kHz) and high frequencies (7.1-14.7 kHz) in adults with normal hearing up to 8 kHz and varying hearing levels from 9 to 16 kHz. In combination with click stimuli, chirp stimuli were used with slow, medium and fast sweep rates for which the local frequency increased or decreased with time. Chirp TEOAEs were transformed into equivalent click TEOAEs by inverse filtering out chirp stimulus phase, and analyzed similarly to click TEOAEs. To improve detection above 8 kHz, TEOAEs were measured in experiment 2 with higher-level stimuli and longer averaging times. These changes increased the TEOAE signal-to-noise ratio (SNR) by 10 dB. Slower sweep rates were investigated but the elicited TEOAEs were detected in fewer ears compared to faster rates. Data were acquired in adults and children (age 11-17 y), including children with cystic fibrosis (CF) treated with ototoxic antibiotics. Test-retest measurements revealed satisfactory repeatability of high-frequency TEOAE SNR (median of 1.3 dB) and coherence synchrony measure, despite small test-retest differences related to changes in forward and reverse transmission in the ear canal. The results suggest the potential use of such tests to screen for sensorineural hearing loss, including ototoxic loss. Experiment 2 was a feasibility study to explore TEOAE test parameters that might be used in a full-scale study to screen CF patients for risk of ototoxic hearing loss.

Cochlear Microphonic and Summating Potential Responses from Click-Evoked Auditory Brain Stem Responses in High-Risk and Normal Infants.

BACKGROUND: Examination of cochlear and neural potentials is necessary to assess sensory and neural status in infants, especially those cared for in neonatal intensive care units (NICU) who have high rates of hyperbilirubinemia and thus are at risk for auditory neuropathy (AN). PURPOSE: The purpose of this study was to determine whether recording parameters commonly used in click-evoked auditory brain stem response (ABR) are useful for recording cochlear microphonic (CM) and Wave I in infants at risk for AN. Specifically, we analyzed CM, summating potential (SP), and Waves I, III, and V. The overall aim was to compare latencies and amplitudes of evoked responses in infants cared for in NICUs with infants in a well-baby nursery (WBN), both of which passed newborn hearing screening. RESEARCH DESIGN: This is a prospective study in which infants who passed ABR newborn hearing screening were grouped based on their birth history (WBN and NICU). All infants had normal hearing status when tested with diagnostic ABR at about one month of age, corrected for prematurity. STUDY SAMPLE: Thirty infants (53 ears) from the WBN [mean corrected age at test = 5.0 weeks (wks.)] and thirty-two infants (59 ears) from the NICU (mean corrected age at test = 5.7 wks.) with normal hearing were included in this study. In addition, two infants were included as comparative case studies, one that was diagnosed with AN and another case that was diagnosed with bilateral sensorineural hearing loss (SNHL). DATA COLLECTION AND ANALYSIS: Diagnostic ABR, including click and tone-burst air- and bone-conduction stimuli were recorded. Peak Waves I, III, and V; SP; and CM latency and amplitude (peak to trough) were measured to determine if there were differences in ABR and electrocochleography (ECochG) variables between WBN and NICU infants. RESULTS: No significant group differences were found between WBN and NICU groups for ABR waveforms, CM, or SP, including amplitude and latency values. The majority (75%) of the NICU group had hyperbilirubinemia, but overall, they did not show evidence of effects in their ECochG or ABR responses when tested at about one-month corrected age. These data may serve as a normative sample for NICU and well infant ECochG and ABR latencies at one-month corrected age. Two infant case studies, one diagnosed with AN and another with SNHL demonstrated the complexity of using ECochG and otoacoustic emissions to assess the risk of AN in individual cases. CONCLUSIONS: CM and SPs can be readily measured using standard click stimuli in both well and NICU infants. Normative ranges for latency and amplitude are useful for interpreting ECochG and ABR components. Inclusion of ECochG and ABR tests in a test battery that also includes otoacoustic emission and acoustic reflex tests may provide a more refined assessment of the risks of AN and SNHL in infants.

Optimizing Clinical Interpretation of Distortion Product Otoacoustic Emissions in Infants.

OBJECTIVES: The purpose of this study was to analyze distortion product otoacoustic emission (DPOAE) level and signal to noise ratio in a group of infants from birth to 4 months of age to optimize prediction of hearing status. DPOAEs from infants with normal hearing (NH) and hearing loss (HL) were used to predict the presence of conductive HL (CHL), sensorineural HL (SNHL), and mixed HL (MHL). Wideband ambient absorbance was also measured and compared among the HL types. DESIGN: This is a prospective, longitudinal study of 279 infants with verified NH and HL, including conductive, sensorineural, and mixed types that were enrolled from a well-baby nursery and two neonatal intensive care units in Cincinnati, Ohio. At approximately 1 month of age, DPOAEs (1-8 kHz), wideband absorbance (0.25-8 kHz), and air and bone conduction diagnostic tone burst auditory brainstem response (0.5-4 kHz) thresholds were measured. Hearing status was verified at approximately 9 months of age with visual reinforcement audiometry (0.5-4 kHz). Auditory brainstem response air conduction thresholds were used to assign infants to an NH or HL group, and the efficacy of DPOAE data to classify ears as NH or HL was analyzed using receiver operating characteristic (ROC) curves. Two summary statistics of the ROC curve were calculated: the area under the ROC curve and the point of symmetry on the curve at which the sensitivity and specificity were equal. DPOAE level and signal to noise ratio cutoff values were defined at each frequency as the symmetry point on their respective ROC curve, and DPOAE results were combined across frequency in a multifrequency analysis to predict the presence of HL. RESULTS: Single-frequency test performance of DPOAEs was best at mid to high frequencies (3-8 kHz) with intermediate performance at 1.5 and 2 kHz and chance performance at 1 kHz. Infants with a conductive component to their HL (CHL and MHL combined) displayed significantly lower ambient absorbance values than the NH group. No differences in ambient absorbance were found between the NH and SNHL groups. Multifrequency analysis resulted in the best prediction of HL for the SNHL/MHL group with poorer sensitivity values when infants with CHL were included. CONCLUSIONS: Clinical interpretation of DPOAEs in infants can be improved by using age-appropriate normative ranges and optimized cutoff values. DPOAE interpretation is most predictive at higher F2 test frequencies in young infants (2-8 kHz) due to poor test performance at 1 to 1.5 kHz. Multifrequency rules can be used to improve sensitivity while balancing specificity. Last, a sensitive middle ear measure such as wideband absorbance should be included in the test battery to assess possibility of a conductive component to the HL.

Longitudinal Development of Distortion Product Otoacoustic Emissions in Infants With Normal Hearing.

OBJECTIVES: The purpose of this study was to describe normal characteristics of distortion product otoacoustic emission (DPOAE) signal and noise level in a group of newborns and infants with normal hearing followed longitudinally from birth to 15 months of age. DESIGN: This is a prospective, longitudinal study of 231 infants who passed newborn hearing screening and were verified to have normal hearing. Infants were enrolled from a well-baby nursery and two neonatal intensive care units (NICUs) in Cincinnati, OH. Normal hearing was confirmed with threshold auditory brainstem response and visual reinforcement audiometry. DPOAEs were measured in up to four study visits over the first year after birth. Stimulus frequencies f1 and f2 were used with f2/f1 = 1.22, and the DPOAE was recorded at frequency 2f1-f2. A longitudinal repeated-measure linear mixed model design was used to study changes in DPOAE level and noise level as related to age, middle ear transfer, race, and NICU history. RESULTS: Significant changes in the DPOAE and noise levels occurred from birth to 12 months of age. DPOAE levels were the highest at 1 month of age. The largest decrease in DPOAE level occurred between 1 and 5 months of age in the mid to high frequencies (2 to 8 kHz) with minimal changes occurring between 6, 9, and 12 months of age. The decrease in DPOAE level was significantly related to a decrease in wideband absorbance at the same f2 frequencies. DPOAE noise level increased only slightly with age over the first year with the highest noise levels in the 12-month-old age range. Minor, nonsystematic effects for NICU history, race, and gestational age at birth were found, thus these results were generalizable to commonly seen clinical populations. CONCLUSIONS: DPOAE levels were related to wideband middle ear absorbance changes in this large sample of infants confirmed to have normal hearing at auditory brainstem response and visual reinforcement audiometry testing. This normative database can be used to evaluate clinical results from birth to 1 year of age. The distributions of DPOAE level and signal to noise ratio data reported herein across frequency and age in normal-hearing infants who were healthy or had NICU histories may be helpful to detect the presence of hearing loss in infants.