Pediatric Cochlear Implant Recipients Research Articles

Impact of paediatric cochlear implantation on family life: a conceptual framework informed by parents

Purpose While the impact of paediatric cochlear implantation on parents and siblings are recognised, limited evidence exists regarding the effect of paediatric cochlear implantation on the entire family life (e.g., routine interactions, family activities). This study aimed to describe the impact of paediatric cochlear implantation on family life as perceived by parents, using concept mapping. Materials and methods Parents of paediatric cochlear implant (CI) recipients (n = 29) participated in this concept mapping study to generate, group, and rate statements regarding the impact of paediatric cochlear implantation on family life. Results In total, 99 unique statements described the impact of paediatric cochlear implantation on family life. The concept map revealed six concepts, namely (i) Financial Outlay and Supports; (ii) Education and Therapy; (iii) Responsibilities and Sacrifices; (iv) Extended Family and Community; (v) Spouses and Siblings and (vi) Achievements and Enrichments. Conclusions This study highlights the multiple areas of family life affected by paediatric cochlear implantation. To improve patient-and-family-centered care, these factors must be considered during pre-operative and successive counselling of paediatric CI recipients and their families.

Factors that influence performance in pediatric cochlear implant recipients with cochlear nerve deficiency

Objectives Children with cochlear nerve deficiency (CND) have wide variability in outcomes with cochlear implant (CI) use. The current study aims to report a large cohort of pediatric CI recipients with CND and to evaluate for factors that may predict improved performance. Methods The current study is a retrospective review of pediatric CI recipients with CND at a tertiary academic hospital. Variables including cochlear nerve status (hypoplasia vs aplasia), age at implantation, cochleovestibular malformation, bony cochlear nerve aperture, internal auditory canal aperture, and cognitive delay were evaluated for predictors of postoperative performance. A stepwise multinomial regression analysis was performed. Results Forty-seven CI recipients (54 ears) were included in the analysis. A majority (59%) showed auditory capabilities with their CI. Twenty percent of recipients achieved some level of open-set speech perception with their CI. The regression analysis identified cochlear nerve status and cognitive delay as predictors of performance. CI recipients with cochlear nerve hypoplasia had significantly improved performance compared to those with aplasia (p = 0.003). Recipients with cognitive delay had more limited benefit than those without cognitive delay (p = 0.033). Conclusions Children with CND can benefit from CI use, with outcomes spanning from non-use to development of spoken language. Predictive factors for improved performance include a lack of cognitive delay and cochlear hypoplasia rather than aplasia. These can be important considerations for parent counseling and decision making.

Psychosocial and auditory factors that influence successful music-based auditory training in pediatric cochlear implant recipients.

Cochlear implants (CIs), which are designed to support spoken communication of persons with severe to profound hearing loss, can provide improved hearing capability through passive exposure. However, auditory training may optimize perception of spectrally complex sounds such as music or speech. Reviews of music-based training for pediatric CI users have reported modest though variable benefits, as well as problems with attrition. It is presumed that more substantial changes may result from longer, more intensive training; however, the development of protocols sufficiently motivating for sustained intensity is challenging. This article examined the experiences of star pediatric CI users, whose years of music training have yielded exceptional auditory benefits. Greater understanding of their experiences and attitudes may suggest best practices for music-based training. Research aims included: (a) characterizing the musical behaviors and perceptual learning processes of music-centric (Music-centric, for purposes of this paper, refers to CI users who engage in sustained and successful music making such as music lessons and ensembles and focused music listening over a period of years, and who derive deep satisfaction from those experiences.) pediatric CI users, and (b) identifying psychosocial and auditory factors that motivated persistence in auditory training. We used qualitative and patient-engaged research methodologies, gathering data through questionnaires with open-ended questions. The participants, six music-centric CI users and five parents, described their experiences and attitudes regarding music training, and factors that supported or undermined those experiences. Data were analyzed using reflexive thematic analysis. The codes were consolidated into five themes and organized into a Model of Music-Based Learning for Pediatric Cochlear Implant Recipients. Sustained participation in music training was perceived as a dynamic process including varied musical stimuli, and moderated by intrinsic (attitude, perceived behavioral control) and extrinsic (parents, teachers, peers) influences, hearing status, sound access and background factors. These themes highlighted motivational factors that pediatric CI users and parents considered important to sustained, intensive and successful music learning throughout childhood and adolescence. These factors should be considered in the development of music-based training for pediatric CI recipients.

Are children with unilateral sensorineural hearing loss receiving cochlear implants?

IntroductionThe US Food and Drug Administration (FDA) granted its first approval for cochlear implants (CI) in children with bilateral sensorineural hearing loss (SNHL) in 1990. In 2019, the FDA expanded CI indications to include children with unilateral SNHL. ObjectiveThe aim of this study was to assess the prevalence of children with unilateral SNHL in the population of new pediatric CI recipients between 2012 and 2021. MethodsA retrospective analysis using the American College of Surgeons National Surgical Quality Improvement Pediatric database examined patients under 18 years of age with bilateral or unilateral SNHL who underwent CI between 2012 and 2021. Current Procedural Terminology code 69930 identified patients with ‘cochlear device implantation, with or without mastoidectomy.’ The percentage of children undergoing CI for unilateral versus bilateral SNHL during the study period was calculated and subjected to statistical analysis. Results9863 pediatric CI patients were included with a mean age of 5.1 (95 % CI 5.1–5.2) years at the time of implantation. 7.5 % (N = 739) of patients had unilateral SNHL and 92.5 % (N = 9124) had bilateral SNHL. Children with bilateral SNHL undergoing CI were significantly younger (5.0 years versus 6.9 years for those with unilateral SNHL, p < .001). There was a statistically significant difference in the percentage of children receiving CI for unilateral versus bilateral SNHL (3.3 % in 2012 to 14.3 % in 2021, p < .001) before and after the FDA changes. ConclusionsThe proportion of CIs placed for unilateral SNHL has increased annually even before 2019 when the FDA expanded its CI indications to include children with unilateral SNHL for the first time.

Factors Affecting Early and Late Cochlear Implantation.

This study aimed to describe the factors affecting early and late cochlear implantation. A total of 159 patients from the Hospital Canselor Tuanku Muhriz (HCTM) Cochlear Implant Programme were recruited in this retrospective cross-sectional study. All paediatric Cochlear Implant (CI) recipients with pre-lingual deafness were included in this retrospective study. The study was conducted from January 2019 until December 2020. The pre-lingual cochlear implant recipients' data were analysed based on demographics and interval from diagnosis to hearing aid fitting and implantation. The association between the dependent variables with early and late cochlear implantation was compared. A total of 83 (52%) patients were female. Chinese race constituted most of the patients, which was 90/159 (57%). The majority were from middle-income families (M40); 89 (56%). The most common aetiology of Hearing Loss (HL) was idiopathic; 139 (87%), followed by intrauterine infections, which comprised of congenital CMV; 8 (5%) and congenital Rubella; 1 (1%) and nonspecific intrauterine infection 2 (1%). The relationship between the universal neonatal hearing screening and the interval between diagnosis to implantation was significant (p=0.033). Other variables were not significant. UNHS was a significant factor contributing to early and late implantation. The median age of diagnosis of hearing loss was 18 months (interquartile range; 15); the age of CI was 34 months (interquartile range; 24); the interval from diagnosis to hearing aid was 2 months (interquartile range; 5), and the interval from diagnosis to CI was 16 months (interquartile range; 14).

Predictors of Emotional Prosody Identification by School-Age Children With Cochlear Implants and Their Peers With Normal Hearing.

Children with cochlear implants (CIs) vary widely in their ability to identify emotions in speech. The causes of this variability are unknown, but this knowledge will be crucial if we are to design improvements in technological or rehabilitative interventions that are effective for individual patients. The objective of this study was to investigate how well factors such as age at implantation, duration of device experience (hearing age), nonverbal cognition, vocabulary, and socioeconomic status predict prosody-based emotion identification in children with CIs, and how the key predictors in this population compare to children with normal hearing who are listening to either normal emotional speech or to degraded speech. We measured vocal emotion identification in 47 school-age CI recipients aged 7 to 19 years in a single-interval, 5-alternative forced-choice task. None of the participants had usable residual hearing based on parent/caregiver report. Stimuli consisted of a set of semantically emotion-neutral sentences that were recorded by 4 talkers in child-directed and adult-directed prosody corresponding to five emotions: neutral, angry, happy, sad, and scared. Twenty-one children with normal hearing were also tested in the same tasks; they listened to both original speech and to versions that had been noise-vocoded to simulate CI information processing. Group comparison confirmed the expected deficit in CI participants' emotion identification relative to participants with normal hearing. Within the CI group, increasing hearing age (correlated with developmental age) and nonverbal cognition outcomes predicted emotion recognition scores. Stimulus-related factors such as talker and emotional category also influenced performance and were involved in interactions with hearing age and cognition. Age at implantation was not predictive of emotion identification. Unlike the CI participants, neither cognitive status nor vocabulary predicted outcomes in participants with normal hearing, whether listening to original speech or CI-simulated speech. Age-related improvements in outcomes were similar in the two groups. Participants with normal hearing listening to original speech showed the greatest differences in their scores for different talkers and emotions. Participants with normal hearing listening to CI-simulated speech showed significant deficits compared with their performance with original speech materials, and their scores also showed the least effect of talker- and emotion-based variability. CI participants showed more variation in their scores with different talkers and emotions than participants with normal hearing listening to CI-simulated speech, but less so than participants with normal hearing listening to original speech. Taken together, these results confirm previous findings that pediatric CI recipients have deficits in emotion identification based on prosodic cues, but they improve with age and experience at a rate that is similar to peers with normal hearing. Unlike participants with normal hearing, nonverbal cognition played a significant role in CI listeners' emotion identification. Specifically, nonverbal cognition predicted the extent to which individual CI users could benefit from some talkers being more expressive of emotions than others, and this effect was greater in CI users who had less experience with their device (or were younger) than CI users who had more experience with their device (or were older). Thus, in young prelingually deaf children with CIs performing an emotional prosody identification task, cognitive resources may be harnessed to a greater degree than in older prelingually deaf children with CIs or than children with normal hearing.

Age and Incidence of Cochlear Implantation in the Pediatric Population With Congenital Bilateral Profound Hearing Loss.

The current study characterizes age and incidence of cochlear implantation among qualifying children with congenital bilateral profound hearing loss in the U.S. Deidentified cochlear implantation data were acquired from prospectively collected patient registries from two cochlear implant (CI) manufacturers (Cochlear Americas and Advanced Bionics). Children <36 months old were assumed to have congenital bilateral profound sensorineural hearing loss. U.S. CI centers. Children <36 months old who received CIs. Cochlear implantation. Age at implantation and incidence. A total of 4,236 children <36 months old underwent cochlear implantation from 2015 to 2019. The median age at implantation was 16 months (interquartile range, 12-24 mo) and did not change significantly during the 5-year study period ( p = 0.09). Patients residing closer to CI centers ( p = 0.03) and treated at higher-volume centers ( p = 0.008) underwent implantation at a younger age. Bilateral simultaneous implantation increased from 38% to 53% of CI surgeries in 2015 and 2019, respectively. Children who received bilateral simultaneous CIs were younger compared with those receiving unilateral or bilateral sequential CIs (median, 14 versus 18 mo; p < 0.001). The incidence of cochlear implantation increased from 7,648 per 100,000 person-years in 2015 to 9,344 in 2019 ( p < 0.001). Although the incidence of pediatric CI recipients and the frequency of bilateral simultaneous implantation increased over the study period, age at implantation did not change significantly and far exceeded current Food and Drug Administration (9 mo) and American Academy of Otolaryngology and Head and Neck Surgery position statement (6-12 mo) guidelines.

Programming Levels and Speech Perception in Pediatric Cochlear Implant Recipients With Enlarged Vestibular Aqueduct or GJB2 Mutation.

To determine the relationship between hearing loss etiology, cochlear implant (CI) programming levels, and speech perception performance in a large clinical cohort of pediatric CI recipients. Retrospective chart review. Tertiary care hospitals. A total of 136 pediatric CI recipients (218 ears) were included in this study. All patients had diagnoses of either enlarged vestibular aqueduct (EVA) or GJB2 (Connexin-26) mutation confirmed via radiographic data and/or genetic reports. All patients received audiologic care at either Boston Children's Hospital or Massachusetts Eye and Ear in Boston, MA, between the years 1999 and 2020. Electrode impedances and programming levels for each active electrode and speech perception scores were evaluated as a function of etiology (EVA or GJB2 mutation). Children with EVA had significantly higher impedances and programming levels (thresholds and upper stimulation levels) than the children with GJB2 mutation. Speech perception scores did not differ as a function of etiology in this sample; rather, they were positively correlated with duration of CI experience (time since implantation). Differences in electrode impedances and CI programming levels suggest that the electrode-neuron interface varies systematically as a function of hearing loss etiology in pediatric CI recipients with EVA and those with GJB2 mutation. Time with the CI was a better predictor of speech perception scores than etiology, suggesting that children can adapt to CI stimulation with experience.

Factors that influence outcomes and device use for pediatric cochlear implant recipients with unilateral hearing loss.

Candidacy criteria for cochlear implantation in the United States has expanded to include children with single-sided deafness (SSD) who are at least 5 years of age. Pediatric cochlear implant (CI) users with SSD experience improved speech recognition with increased daily device use. There are few studies that report the hearing hour percentage (HHP) or the incidence of non-use for pediatric CI recipients with SSD. The aim of this study was to investigate factors that impact outcomes in children with SSD who use CIs. A secondary aim was to identify factors that impact daily device use in this population. A clinical database query revealed 97 pediatric CI recipients with SSD who underwent implantation between 2014 and 2022 and had records of datalogs. The clinical test battery included speech recognition assessment for CNC words with the CI-alone and BKB-SIN with the CI plus the normal-hearing ear (combined condition). The target and masker for the BKB-SIN were presented in collocated and spatially separated conditions to evaluate spatial release from masking (SRM). Linear mixed-effects models evaluated the influence of time since activation, duration of deafness, HHP, and age at activation on performance (CNC and SRM). A separate linear mixed-effects model evaluated the main effects of age at testing, time since activation, duration of deafness, and onset of deafness (stable, progressive, or sudden) on HHP. Longer time since activation, shorter duration of deafness, and higher HHP were significantly correlated with better CNC word scores. Younger age at device activation was not found to be a significant predictor of CNC outcomes. There was a significant relationship between HHP and SRM, with children who had higher HHP experiencing greater SRM. There was a significant negative correlation between time since activation and age at test with HHP. Children with sudden hearing loss had a higher HHP than children with progressive and congenital hearing losses. The present data presented here do not support a cut-off age or duration of deafness for pediatric cochlear implantation in cases of SSD. Instead, they expand on our understanding of the benefits of CI use in this population by reviewing the factors that influence outcomes in this growing patient population. Higher HHP, or greater percentage of time spent each day using bilateral input, was associated with better outcomes in the CI-alone and in the combined condition. Younger children and those within the first months of use had higher HHP. Clinicians should discuss these factors and how they may influence CI outcomes with potential candidates with SSD and their families. Ongoing work is investigating the long-term outcomes in this patient population, including whether increasing HHP after a period of limited CI use results in improved outcomes.

Observational Study of Pediatric Cochlear Implant Recipients: Two-year Follow-up Outcomes.

The aim of this study was to evaluate the improvements in quality of life and auditory performance via a descriptive report using patient-related outcomes measures in a group of children with cochlear implants (CI). This was a longitudinal and retrospective study based on the analysis of patient records of a total of 28 children with CI, 18 of whom had bilateral (64.3%) and 10 had unilateral (35.7%). The study included repeated within-subject measures of Hearing Implants Quality of Life (CuHI-QoL), Categories of Auditory Performance (CAP-II), Speech, Spatial, and Qualities of Hearing scale-Parents' version (SSQ-P) scales, and Implant Recipient Follow-up (IRF) form at 6-month intervals from baseline up to 24 months. During the 2-year follow-up assessments, all children with CI showed statistically significant improvement over time in their CAPII, SSQ-P, CuHI-QoL, and IRF scores (p<0.001). The most significant increase in scores occurred within the first six months following the baseline and then continued to increase gradually at a slower pace over time. A moderate negative relationship was found between the age of implantation and SSQ-P and family satisfaction scores of IRF (p<0.05). Family expectations, overall well-being, quality of life, auditory, and verbal skills increase positively with earlier CI intervention. Moreover, the subjective assessment results showed that parents-positive attitudes toward implantation, and willingness to recommend it to others have steadily increased in time. Although the cohort is relatively small and variable, the results offer a descriptive view to outcomes in real-world practice.

Pediatric cochlear implant explantation and reimplantation over a 32-year period.

Cochlear implantation is indicated for pediatric patients with bilateral severe to profound sensorineural hearing loss. The literature reports large variability in cochlear implant (CI) device survival and rates of explantation and reimplantation. This retrospective chart review summarizes the CI survival and rates of explantation and reimplantation in pediatric CI recipients at a Canadian tertiary pediatric hospital over 32 years. A retrospective chart review of all pediatric patients who received a Cochlear Corporation® CI between April 1988 and June 2020 was undertaken. Rates of explantation/reimplantation were collected and categorized based on device type and reason for failure (medical, device, and inconclusive failure). Device survival analysis based on implant model was also completed utilizing Kaplan-Meier curves. 512 CIs were implanted over the 32-year period by four surgeons (77.1%, 18.16%, 4.49%, and 0.20%, respectively). Patient age ranged from 7 months to 20.4 years. The overall explantation and reimplantation rate was 3.32% (17/512 implants), with seven as a result of device failure (1.37%), nine events of medical failure (1.76%), and one inconclusive failure (0.20%). Cumulative CI survival rates at 5, 10, 15, and 20 years were 98.15%, 96.33%, 95.53%, and 94.39%. The overall institutional CI failure, explantation, and reimplantation rates are lower than the average reported rate in the literature.

Efficacy of adenotonsillectomy in the treatment of obstructive apnea in children: A 2-year follow-up.

The main aim of this study was to estimate the volume of the Scala Tympani (ST) of our pediatric cochlear implant (CI) recipients from the computed tomography (CT) images. Then, to study the association between ST volume and both demographic characteristics and cochlear parameters.A retrospective study on the CT scans of pediatric CI patients at a tertiary referral CI center. Congenital or acquired cochlear defects were excluded. Two reviewers, with the same level of experience, blindly measured the main cochlear parameters and studied its anatomy. Then, the interrater reliability was tested to measure any differences between the two readings. After that, the ST volume of the included patients was calculated and analyzed. Furthermore, the correlations between the main cochlear parameters and ST volume were studied to propose a formula for estimating the ST volume from the cochlear duct length (CDL).The mean predicted ST volume among our pediatric CI recipients was 38.51 ± 5.54 μl (range; 24.47–52.57 μl). The statistical analysis revealed that all cochlear parameters (A, B, H, and CDL values) could be significant predictors of the ST volume (p=<0.0001).The main cochlear parameters along with the CDL are positively linked to the ST volume. There are considerable differences in cochlear size and scala tympani volume among our pediatric population. These findings confirm the importance of pre-operative planning for proper electrode array selection.

Sustainable Benefits of High Variability Phonetic Training in Mandarin-speaking Kindergarteners With Cochlear Implants: Evidence From Categorical Perception of Lexical Tones.

Although pitch reception poses a great challenge for individuals with cochlear implants (CIs), formal auditory training (e.g., high variability phonetic training [HVPT]) has been shown to provide direct benefits in pitch-related perceptual performances such as lexical tone recognition for CI users. As lexical tones in spoken language are expressed with a multitude of distinct spectral, temporal, and intensity cues, it is important to determine the sources of training benefits for CI users. The purpose of the present study was to conduct a rigorous fine-scale evaluation with the categorical perception (CP) paradigm to control the acoustic parameters and test the efficacy and sustainability of HVPT for Mandarin-speaking pediatric CI recipients. The main hypothesis was that HVPT-induced perceptual learning would greatly enhance CI users' ability to extract the primary pitch contours from spoken words for lexical tone identification and discrimination. Furthermore, individual differences in immediate and long-term gains from training would likely be attributable to baseline performance and duration of CI use. Twenty-eight prelingually deaf Mandarin-speaking kindergarteners with CIs were tested. Half of them received five sessions of HVPT within a period of 3 weeks. The other half served as control who did not receive the formal training. Two classical CP tasks on a tonal continuum from Mandarin tone 1 (high-flat in pitch) to tone 2 (mid-rising in pitch) with fixed acoustic features of duration and intensity were administered before (pretest), immediately after (posttest), and 10 weeks posttraining termination (follow-up test). Participants were instructed to either label a speech stimulus along the continuum (i.e., identification task) or determine whether a pair of stimuli separated by zero or two steps from the continuum was the same or different (i.e., discrimination task). Identification function measures (i.e., boundary position and boundary width) and discrimination function scores (i.e., between-category score, within-category score, and peakedness score) were assessed for each child participant across the three test sessions. Linear mixed-effects (LME) models showed significant training-induced enhancement in lexical tone categorization with significantly narrower boundary width and better between-category discrimination in the immediate posttest over pretest for the trainees. Furthermore, training-induced gains were reliably retained in the follow-up test 10 weeks after training. By contrast, no significant changes were found in the control group across sessions. Regression analysis confirmed that baseline performance (i.e., boundary width in the pretest session) and duration of CI use were significant predictors for the magnitude of training-induced benefits. The stringent CP tests with synthesized stimuli that excluded acoustic cues other than the pitch contour and were never used in training showed strong evidence for the efficacy of HVPT in yielding immediate and sustained improvement in lexical tone categorization for Mandarin-speaking children with CIs. The training results and individual differences have remarkable implications for developing personalized computer-based short-term HVPT protocols that may have sustainable long-term benefits for aural rehabilitation in this clinical population.

Cochlear implantation: Predicting the scala tympani volume of the pediatric recipients

The main aim of this study was to estimate the volume of the Scala Tympani (ST) of our pediatric cochlear implant (CI) recipients from the computed tomography (CT) images. Then, to study the association between ST volume and both demographic characteristics and cochlear parameters. A retrospective study on the CT scans of pediatric CI patients at a tertiary referral CI center. Congenital or acquired cochlear defects were excluded. Two reviewers, with the same level of experience, blindly measured the main cochlear parameters and studied its anatomy. Then, the interrater reliability was tested to measure any differences between the two readings. After that, the ST volume of the included patients was calculated and analyzed. Furthermore, the correlations between the main cochlear parameters and ST volume were studied to propose a formula for estimating the ST volume from the cochlear duct length (CDL). The mean predicted ST volume among our pediatric CI recipients was 38.51±5.54μl (range; 24.47-52.57μl). The statistical analysis revealed that all cochlear parameters (A, B, H, and CDL values) could be significant predictors of the ST volume (p=<0.0001). The main cochlear parameters along with the CDL are positively linked to the ST volume. There are considerable differences in cochlear size and scala tympani volume among our pediatric population. These findings confirm the importance of pre-operative planning for proper electrode array selection.

Psychoacoustic abilities in pediatric cochlear implant recipients: The relation with short-term memory and working memory capacity.

The aim was to investigate school-age children with cochlear implants (CIs) and their typically developing peers in terms of auditory short-term memory (ASTM), auditory working memory (AWM), visuospatial short-term memory (VSTM), visuospatial working memory (VWM), spectral resolution and monosyllabic word recognition in noise. Twenty-three prelingually deaf CI users and twenty-three typically developing (TD) peers aged 7-10 years participated. Twelve children with CI were earlier-implanted (i.e., age at implantation ≤24 months). Children with CIs were compared to typically developing peers and correlations between cognitive and psychoacoustic abilities were computed separately for the groups. Besides, regression analyses were conducted to develop models that could predict SMRT (spectral-temporally modulated ripple test) and speech recognition scores. The AWM scores of the later-implanted group were significantly lower than both earlier-implanted and TD groups. ASTM scores of TD children were significantly higher than both earlier-implanted and later-implanted participants. There was no statistically significant difference between groups in terms of VSTM and VWM. AWM performance was positively correlated with ASTM, SMRT scores, and speech recognition under noisy conditions for pediatric CI recipients. The AWM was a statistically significant predictor of the SMRT score and the SMRT score was an indicator of speech recognition score under 0dB SNR condition. Most of children using CI are at risk for clinically remarkable deficits across cognitive abilities such as AWM and ASTM. While evaluating cognitive and psychoacoustic abilities in the clinic routine, it should be kept in mind that they can be influenced by each other.

Effects of Segmental and Suprasegmental Speech Perception on Reading in Pediatric Cochlear Implant Recipients.

The aim of this study was to determine whether suprasegmental speech perception contributes unique variance in predictions of reading decoding and comprehension for prelingually deaf children using two devices, at least one of which is a cochlear implant (CI). A total of 104, 5- to 9-year-old CI recipients completed tests of segmental perception (e.g., word recognition in quiet and noise, recognition of vowels and consonants in quiet), suprasegmental perception (e.g., talker and stress discrimination, nonword stress repetition, and emotion identification), and nonverbal intelligence. Two years later, participants completed standardized tests of reading decoding and comprehension. Using regression analyses, the unique contribution of suprasegmental perception to reading skills was determined after controlling for demographic characteristics and segmental perception performance. Standardized reading scores of the CI recipients increased with nonverbal intelligence for both decoding and comprehension. Female gender was associated with higher comprehension scores. After controlling for gender and nonverbal intelligence, segmental perception accounted for approximately 4% and 2% of the variance in decoding and comprehension, respectively. After controlling for nonverbal intelligence, gender, and segmental perception, suprasegmental perception accounted for an extra 4% and 7% unique variance in reading decoding and reading comprehension, respectively. Suprasegmental perception operates independently from segmental perception to facilitate good reading outcomes for these children with CIs. Clinicians and educators should be mindful that early perceptual skills may have long-term benefits for literacy. Research on how to optimize suprasegmental perception, perhaps through hearing-device programming and/or training strategies, is needed.

Younger Age at Cochlear Implant Activation Results in Improved Auditory Skill Development for Children With Congenital Deafness.

The U.S. Food and Drug Administration indications for cochlear implantation in children is currently 9 months of age and older for children with bilateral profound sensorineural hearing loss (SNHL). Studies have shown that earlier activation of a cochlear implant (CI) can lead to better spoken language outcomes. As auditory skills are a precursor to the development of spoken language, this study was developed to investigate the influence of age at CI activation on auditory skill acquisition in young children. A secondary aim was to describe the auditory skills of children implanted prior to 9 months of age as compared to children with older ages of activation. Functional Listening Index (FLI) scores obtained during routine clinical visits were reviewed for 78 pediatric CI recipients with congenital bilateral profound hearing loss who were activated before 2 years of age. A linear mixed-effects model assessed the effect of age at CI activation on cumulative FLI scores over time. There was a significant interaction between age at activation and chronological age at the time of evaluation, indicating that children with earlier access to sound achieved a greater number of auditory skills than those with later CI activations when measured at the same chronological age. Children activated before the age of 9 months approximated scores expected of children with typical hearing, whereas children activated between 9 and 24 months of age did not. Younger age at CI activation is associated with increased auditory skills over time. Children who undergo cochlear implantation and CI activation before 9 months achieve more auditory skills by 4 years of age than children who are activated at later ages. These data suggest that reducing the approved age at cochlear implantation for children with congenital bilateral profound SNHL may support optimal auditory skill acquisition.

The effect of lexical tone experience on English intonation perception in Mandarin-speaking cochlear-implanted children.

To examine the effect of lexical tone experience on English intonation perception in Mandarin-speaking cochlear-implanted children during second language acquisition in Taiwan. A retrospective cohort study. A tertiary referred center. Fourteen children with cochlear implant (CI) in the experimental group, and 9 normal hearing children in the control group were enrolled in this study. Cochlear implantation and hearing rehabilitation. Two speech recognition accuracies were examined: (1) Lexical tone recognition (4-alternative forced choice, AFC), (2) English Sentence Intonation (2AFC). The overall accuracies for tone perception are 61.13% (standard deviation, SD = 10.84%) for CI group and 93.82% (SD = 1.80%) for normal hearing group. Tone 4 and Tone 1 were more easily to be recognized than tone 2 and tone 3 in the pediatric CI recipients (cCI) group. In English intonation perception, the overall accuracies are 61.82% (SD = 16.85%) for CI group, and 97.59% (SD = 4.73%) for normal hearing group. Significant high correlation (R = .919, P ≦ .000) between lexical tone perception and English intonation perception is noted. There is no significant difference for English intonation perception accuracies between Mandarin-speaking cCI (61.82%) and English-speaking cCI (70.13%, P = .11). Mandarin-speaking cochlear-implanted children showed significant deficits in perception of lexical tone and English intonation relative to normal hearing children. There was no tonal language benefit in Mandarin-speaking cochlear-implanted children's English intonation perception, compared to the English-speaking cochlear-implanted peers. For cochlear-implanted children, better lexical tone perception comes with better English intonation perception. Enhancing Mandarin prosodic perception for cochlear-implanted children may benefit their command of intonation in English.

Round Window Electrocochleography to Low Frequency Tones in Pediatric Cochlear Implant Recipients with and Without Auditory Neuropathy Spectrum Disorder: Separating Hair Cell and Neural Contributions Using a Computational Model.

Characterize the contribution of the auditory nerve neurophonic (ANN) to electrocochleography (ECochG) of pediatric cochlear implant (CI) recipients with and without auditory nerve spectrum disorder (ANSD). ECochG is an emerging technique for predicting outcomes in CI recipients. Its utility may be increased by separating the cochlear microphonic (CM), produced by hair cells, from the ANN, the evoked potential correlate of neural phase-locking, which are mixed in the ongoing portion of the response to low frequency tone bursts. Responses to tone bursts of different frequency and intensities were recorded from the round window of pediatric CI recipients. Separation of the CM and ANN was performed using a model of the underlying processes that lead to the shapes of the observed waveforms. Preoperative mean pure tone amplitudes of the included ANSD (n = 36) and non-ANSD subjects (n = 123), were similar (89.5 and 93.5, p = 0.1). Total of 1,024 ECochG responses to frequency and intensity series were recorded. The mean correlation ( r ) between the input and the modeled signals was 0.973 ± 0.056 (standard deviation). The ANN magnitudes were higher in the ANSD group (ANOVAs, F = 26.5 for frequency and 21.9 for intensity, df's = 1, p 's < 0.001). However, its relative contribution to the overall signal was lower (ANOVAs, F = 25.8 and 12.1, df = 1, p 's < 0.001). ANN was detected in low frequency ECochG responses but not high frequency responses in both ANSD and non-ANSD subjects. ANSD subjects, evidence of neural contribution in responses to low frequency stimuli was highly variable and often comparable to signals recorded in non-ANSD subjects. The computational model revealed that on average the ANN comprised a lower proportion of the overall signal than in non-ANSD subjects.

Efficacy of cochlear implants in children with borderline hearing who have already achieved significant language development with hearing aids.

There are still debates about timing and effectiveness of cochlear implants (CI) in pediatric subjects with significant residual hearing who do not belong to traditional indication of CI. In this study, we aimed to investigate the outcomes of CI, specifically on improvement of pronunciation, among hearing-impaired children already with a substantial degree of language skills as evaluated by Categories of Auditory Perception (CAP) scores or sentence score. Our cohort comprised pediatric CI recipients from July 2018 through October 2020. Among them, cases with CAP scores of 5 or 6 preoperatively were defined as "borderline cases". We investigated prevalence and etiologies, and compared speech evaluation data preoperatively and postoperatively at three time points (3, 6 and 9-12 months after implantation). Among 86 pediatric CI recipients, 13 subjects (15.12%) had language development that reached CAP scores of 5 or 6 before implantation. Postoperative speech evaluation data 6 months after implantation revealed significant improvement of pronunciation (Urimal Test of Articulation and Phonation scores: UTAP), Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) and word perception scores, but not of CAP and sentence perception scores. Notably, the significant improvement of pronunciation based on UTAP scores outstripped that of other speech parameters and this continued steadily up to one-year postoperatively. The result of the study serves as evidence for what to expect from cochlear implantation in hearing-impaired children who have already achieved a substantial degree of language development in terms of CAP scores or sentence perception scores, preoperatively.