Two-Year Outcomes of Cochlear Implant Use for Children With Unilateral Hearing Loss: Benefits and Comparison to Children With Normal Hearing.

OBJECTIVES: Children with severe-to-profound unilateral hearing loss, including cases of single-sided deafness (SSD), lack access to binaural cues that support spatial hearing, such as recognizing speech in complex multisource environments and sound source localization. Listening in a monaural condition negatively impacts communication, learning, and quality of life for children with SSD. Cochlear implant (CI) use may restore binaural hearing abilities and improve outcomes as compared to alternative treatments or no treatment. This study investigated performance over 24 months of CI use in young children with SSD as compared to the better hearing ear alone and to children with bilateral normal hearing (NH). DESIGN: Eighteen children with SSD who received a CI between the ages of 3.5 and 6.5 years as part of a prospective clinical trial completed assessments of word recognition in quiet, masked sentence recognition, and sound source localization at regular intervals out to 24-month postactivation. Eighteen peers with bilateral NH, matched by age at the group level, completed the same test battery. Performance at 24-month postactivation for the SSD group was compared to the performance of the NH group. RESULTS: Children with SSD have significantly poorer speech recognition in quiet, masked sentence recognition, and localization both with and without the use of the CI than their peers with NH. The SSD group experienced significant benefits with the CI+NH versus the NH ear alone on measures of isolated word recognition, masked sentence recognition, and localization. These benefits were realized within the first 3 months of use and were maintained through the 24-month postactivation interval. CONCLUSIONS: Young children with SSD who use a CI experience significant isolated word recognition and bilateral spatial hearing benefits, although their performance remains poorer than their peers with NH.

Cochlear Nerve Deficiency in Pediatric Unilateral Hearing Loss and Asymmetric Hearing Loss.

INTRODUCTION: The rates of cochlear nerve abnormalities and cochlear malformations in pediatric unilateral hearing loss (UHL) are conflicting in the literature, with important implications on management. The aim of this study was to investigate the incidence of cochlear nerve deficiency (CND) in pediatric subjects with UHL or asymmetric hearing loss (AHL). METHODS: A retrospective chart review of pediatric subjects <18 years of age evaluated for UHL or AHL with fine-cut heavily T2-weighted magnetic resonance imaging (MRI) between January 2014 and October 2019 (n = 291) at a tertiary referral center was conducted. MRI brain and computed tomography temporal bone were reviewed for the presence of inner ear malformations and/or CND. Status of the ipsilateral cochlear nerve and inner ear was evaluated. Pure tone average (PTA) at 500, 1,000 and 2,000 Hz was assessed. RESULTS: 204 subjects with UHL and 87 subjects with AHL were included. CND (aplasia or hypoplasia) was demonstrated in 61 pediatric subjects with UHL (29.9%) and 10 with AHL (11.5%). Ipsilateral cochlear malformations were noted in 25 subjects with UHL (12.3%) and 11 with AHL (12.6%), and ipsilateral vestibular malformations in 23 (11.3%) and 12 (13.8%) ears, respectively. Median PTA was statistically significantly higher in ears with CND (98.33) than ears with normal nerves (90.84). DISCUSSION/CONCLUSION: Imaging demonstrated a high incidence of inner ear malformations, particularly CND, in pediatric subjects with UHL. Auditory findings indicated CND cannot be ruled out by thresholds alone as some CND ears did demonstrate measurable hearing. Radiologic evaluation by MRI should be performed in all patients within this population to guide counseling and management of hearing loss based on etiology, with implications on candidacy for cochlear implantation.

American Cochlear Implant Alliance Task Force Guidelines for Clinical Assessment and Management of Cochlear Implantation in Children With Single-Sided Deafness.

More children with single-sided deafness (SSD) are receiving cochlear implants (CIs) due to the expansion of CI indications. This unique group of pediatric patients has different needs than the typical recipient with bilateral deafness and requires special consideration and care. The goal of cochlear implantation in these children is to provide bilateral input to encourage the development of binaural hearing. Considerations for candidacy and follow-up care should reflect and measure these goals. The purpose of this document is to review the current evidence and provide guidance for CI candidacy, evaluation, and management in children with SSD.

Delaying Cochlear Implantation Impacts Postoperative Speech Perception of Nontraditional Pediatric Candidates.

INTRODUCTION: As pediatric cochlear implant (CI) candidacy expands, children with greater degrees of residual hearing are receiving CIs. These nontraditional candidates have audiometric thresholds that meet adult manufacturer labeling but are better than current pediatric guidelines allow. The purpose of this study was to determine the impact of delayed cochlear implantation on speech perception in nontraditional pediatric CI recipients. METHODS: Pediatric CI recipients with a history of progressive hearing loss and a preoperative 4-frequency pure-tone average of ≤75 dB HL at the time of implantation were considered for this retrospective study. Preoperative serial audiograms and word recognition scores were reviewed, and a method was created to establish a date when each individual ear 1st met nontraditional candidacy. The length of time between the date of candidacy and implantation was calculated and defined as the "delay time." A multiple linear regression investigated delay time, age at surgery, surgery type (1st vs. 2nd side), and array type as predictive factors of maximum postoperative Consonant-Nucleus-Consonant (CNC) word scores. A one-way ANCOVA was performed comparing the postoperative CNC scores between subjects grouped by delay time. RESULTS: A significant regression was found (F(4, 38) = 5.167, p = 0.002, R2 = 0.353). Both age at implantation (p = 0.023) and delay time (p = 0.002) predicted CNC word scores. Longer delay time was associated with poorer word recognition scores, while older age at implantation correlated with higher CNC word scores in this progressive hearing loss group. A significant difference was noted between subjects implanted with <1 year of delay and those with 3 or more years of delay (p = 0.003). All ears implanted within a year of candidacy achieved word recognition abilities that are generally accepted as above average (M = 84.91). CONCLUSION: CI candidacy for adults has evolved to allow for greater degrees of residual hearing, while audiometric guidelines for children have not changed since 2000. Our findings suggest that delay of cochlear implantation, even for children with significant levels of residual hearing, leads to poorer outcomes. Modified candidacy guidelines for children should be established to expedite referral to multidisciplinary CI teams and minimize delays in this population.

Recommendations for Measuring the Electrically Evoked Compound Action Potential in Children With Cochlear Nerve Deficiency.

OBJECTIVES: This study reports a method for measuring the electrically evoked compound action potential (eCAP) in children with cochlear nerve deficiency (CND). DESIGN: This method was developed based on experience with 50 children with CND who were Cochlear Nucleus cochlear implant users. RESULTS: This method includes three recommended steps conducted with recommended stimulating and recording parameters: initial screen, pulse phase duration optimization, and eCAP threshold determination (i.e., identifying the lowest stimulation level that can evoke an eCAP). Compared with the manufacturer-default parameters, the recommended parameters used in this method yielded a higher success rate for measuring the eCAP in children with CND. CONCLUSIONS: The eCAP can be measured successfully in children with CND using recommended parameters. This specific method is suitable for measuring the eCAP in children with CND in clinical settings. However, it is not suitable for intraoperative eCAP recordings due to the extensive testing time required.

Electric-Acoustic Stimulation Outcomes in Children.

OBJECTIVES: This study investigates outcomes in children fit with electric-acoustic stimulation (EAS) and addresses three main questions: (1) Are outcomes with EAS superior to outcomes with conventional electric-only stimulation in children? (2) Do children with residual hearing benefit from EAS and conventional electric-only stimulation when compared with the preoperative hearing aid (HA) condition? (3) Can children with residual hearing derive benefit from EAS after several years of listening with conventional electric-only stimulation? DESIGN: Sixteen pediatric cochlear implant (CI) recipients between 4 and 16 years of age with an unaided low-frequency pure tone average of 75 dB HL in the implanted ear were included in two study arms. Arm 1 included new recipients, and Arm 2 included children with at least 1 year of CI experience. Using a within-subject design, participants were evaluated unilaterally with the Consonant-Nucleus-Consonant (CNC) word list in quiet and the Baby Bio at a +5 dB SNR using an EAS program and a conventional full electric (FE) program. Arm 1 participants' scores were also compared with preoperative scores. RESULTS: Speech perception outcomes were statistically higher with the EAS program than the FE program. For new recipients, scores were significantly higher with EAS than preoperative HA scores for both the CNC and Baby Bio in noise; however, after 6 months of device use, results in the FE condition were not significantly better than preoperative scores. Long-term FE users benefited from EAS over their FE programs based on CNC word scores. CONCLUSIONS: Whether newly implanted or long-term CI users, children with residual hearing after CI surgery can benefit from EAS. Cochlear implantation with EAS fitting is a viable option for children with HAs who have residual hearing but have insufficient access to high-frequency sounds and poor speech perception.

Residual Cochlear Function in Adults and Children Receiving Cochlear Implants: Correlations With Speech Perception Outcomes.

OBJECTIVES: Variability in speech perception outcomes with cochlear implants remains largely unexplained. Recently, electrocochleography, or measurements of cochlear potentials in response to sound, has been used to assess residual cochlear function at the time of implantation. Our objective was to characterize the potentials recorded preimplantation in subjects of all ages, and evaluate the relationship between the responses, including a subjective estimate of neural activity, and speech perception outcomes. DESIGN: Electrocochleography was recorded in a prospective cohort of 284 candidates for cochlear implant at University of North Carolina (10 months to 88 years of ages). Measurement of residual cochlear function called the "total response" (TR), which is the sum of magnitudes of spectral components in response to tones of different stimulus frequencies, was obtained for each subject. The TR was then related to results on age-appropriate monosyllabic word score tests presented in quiet. In addition to the TR, the electrocochleography results were also assessed for neural activity in the forms of the compound action potential and auditory nerve neurophonic. RESULTS: The TR magnitude ranged from a barely detectable response of about 0.02 µV to more than 100 µV. In adults (18 to 79 years old), the TR accounted for 46% of variability in speech perception outcome by linear regression (r = 0.46; p < 0.001). In children between 6 and 17 years old, the variability accounted for was 36% (p < 0.001). In younger children, the TR accounted for less of the variability, 15% (p = 0.012). Subjects over 80 years old tended to perform worse for a given TR than younger adults at the 6-month testing interval. The subjectively assessed neural activity did not increase the information compared with the TR alone, which is primarily composed of the cochlear microphonic produced by hair cells. CONCLUSIONS: The status of the auditory periphery, particularly of hair cells rather than neural activity, accounts for a large fraction of variability in speech perception outcomes in adults and older children. In younger children, the relationship is weaker, and the elderly differ from other adults. This simple measurement can be applied with high throughput so that peripheral status can be assessed to help manage patient expectations, create individually-tailored treatment plans, and identify subjects performing below expectations based on residual cochlear function.

Acoustically evoked auditory change complex in children with auditory neuropathy spectrum disorder: a potential objective tool for identifying cochlear implant candidates.

OBJECTIVES: The overall aim of the study was to evaluate the feasibility of using electrophysiological measures of the auditory change complex (ACC) to identify candidates for cochlear implantation in children with auditory neuropathy spectrum disorder (ANSD). To achieve this overall aim, this study (1) assessed the feasibility of measuring the ACC evoked by temporal gaps in a group of children with ANSD across a wide age range and (2) investigated the association between gap detection thresholds (GDTs) measured by the ACC recordings and open-set speech-perception performance in these subjects. DESIGN: Nineteen children with bilateral ANSD ranging in age between 1.9 and 14.9 years (mean: 7.8 years) participated in this study. Electrophysiological recordings of the auditory event-related potential (ERP), including the onset ERP response and the ACC, were completed in all subjects and open-set speech perception was evaluated for a subgroup of 16 subjects. For the ERP recordings, the stimulus was a Gaussian noise presented through ER-3A insert earphones to the test ear. Two stimulation conditions were used. In the "control condition," the stimulus was an 800-msec Gaussian noise. In the "gapped condition," the stimuli were two noise segments, each being 400 msec in duration, separated by one of five gaps (i.e., 5, 10, 20, 50, or 100 msec). The interstimulation interval was 1200 msec. The aided open-set speech perception ability was assessed using the Phonetically Balanced Kindergarten (PBK) word lists presented at 60 dB SPL using recorded testing material in a sound booth. For speech perception tests, subjects wore their hearing aids at the settings recommended by their clinical audiologists. For a subgroup of five subjects, psychophysical GDTs for the Gaussian noise were also assessed using a three-interval, three-alternative forced-choice procedure. RESULTS: Responses evoked by the onset of the Gaussian noise (i.e., onset responses) were recorded in all stimulation conditions from all subjects tested in this study. The presence/absence, peak latency and amplitude, and response width of the onset response did not correlate with aided PBK word scores. The objective GDTs measured with the ACC recordings from 17 subjects ranged from 10 to 100 msec. The ACC was not recorded from two subjects for any gap durations tested in this study. There was a robust negative correlation between objective GDTs and aided PBK word scores. In general, subjects with prolonged objective GDTs showed low-aided PBK word scores. GDTs measured using electrophysiological recordings of the ACC correlated well with those measured using psychophysical procedures in four of five subjects who were evaluated using both procedures. CONCLUSIONS: The clinical application of the onset response in predicting open-set speech-perception ability is relatively limited in children with ANSD. The ACC recordings can be used to objectively evaluate temporal resolution abilities in children with ANSD having no severe comorbidities, and who are older than 1.9 years. The ACC can potentially be used as an objective tool to identify poor performers among children with ANSD using properly fit amplification, and who are thus, cochlear implant candidates.

Long-Term Perceived Benefit of Pediatric Cochlear Implant Users with Unilateral Hearing Loss.

OBJECTIVE: To assess the perceived benefit of cochlear implant (CI) use for children with unilateral hearing loss (UHL) and evaluate whether perceived abilities are associated with performance on measures of speech recognition and spatial hearing. METHOD: Nineteen children with moderate-to-profound UHL underwent cochlear implantation. The Speech Spatial and Qualities of Hearing Questionnaire modified for children (SSQ-C) were completed by parental proxy pre-operatively and at 3, 6, 9, 12, 18, and 24 months post-activation. Linear mixed models evaluated perceived benefits over the study period. Pearson correlations assessed the association between subjective report and performance on measures of word recognition with the CI alone and spatial hearing (speech recognition in spatially-separated noise and sound source localization) in the combined condition (CI plus contralateral ear). RESULTS: For the SSQ-C, parents reported significant improvements with CI use as compared to pre-operative perceptions (p < 0.001); improved perceptions were either maintained or continued to improve over the 2-year post-activation period. Perceived benefit did not significantly correlate with word recognition with the CI alone or spatial hearing outcomes in the combined condition. CONCLUSION: Families of children with UHL observed benefits of CI use early after cochlear implantation that was maintained with long-term device use. Responses to subjective measures may broaden our understanding of the experiences of pediatric CI users with UHL in addition to outcomes on typical measures of CI performance. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:919-925, 2024.

Effect of Age and Unaided Acoustic Hearing on Pediatric Cochlear Implant Users' Ability to Distinguish Yes/No Statements and Questions.

PURPOSE: The purpose of this study was to evaluate the ability to discriminate yes/no questions from statements in three groups of children: bilateral cochlear implant (CI) users, nontraditional CI users with aidable hearing preoperatively in the ear to be implanted, and controls with normal hearing. Half of the nontraditional CI users had sufficient postoperative acoustic hearing in the implanted ear to use electric-acoustic stimulation, and half used a CI alone. METHOD: Participants heard recorded sentences that were produced either as yes/no questions or as statements by three male and three female talkers. Three raters scored each participant response as either a question or a statement. Bilateral CI users (n = 40, 4-12 years old) and normal-hearing controls (n = 10, 4-12 years old) were tested binaurally in the free field. Nontraditional CI recipients (n = 22, 6-17 years old) were tested with direct audio input to the study ear. RESULTS: For the bilateral CI users, performance was predicted by age but not by 125-Hz acoustic thresholds; just under half (n = 17) of the participants in this group had measurable 125-Hz thresholds in their better ear. For nontraditional CI recipients, better performance was predicted by lower 125-Hz acoustic thresholds in the test ear, and there was no association with participant age. Performance approached that of the normal-hearing controls for some participants in each group. CONCLUSIONS: Results suggest that a 125-Hz acoustic hearing supports discrimination of yes/no questions and statements in pediatric CI users. Bilateral CI users with little or no acoustic hearing at 125 Hz develop the ability to perform this task, but that ability emerges later than for children with better acoustic hearing. These results underscore the importance of preserving acoustic hearing for pediatric CI users when possible.

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.

Gap detection measured with electrically evoked auditory event-related potentials and speech-perception abilities in children with auditory neuropathy spectrum disorder.

OBJECTIVES: This study aimed (1) to investigate the feasibility of recording the electrically evoked auditory event-related potential (eERP), including the onset P1-N1-P2 complex and the electrically evoked auditory change complex (EACC) in response to temporal gaps, in children with auditory neuropathy spectrum disorder (ANSD); and (2) to evaluate the relationship between these measures and speech-perception abilities in these subjects. DESIGN: Fifteen ANSD children who are Cochlear Nucleus device users participated in this study. For each subject, the speech-processor microphone was bypassed and the eERPs were elicited by direct stimulation of one mid-array electrode (electrode 12). The stimulus was a train of biphasic current pulses 800 msec in duration. Two basic stimulation conditions were used to elicit the eERP. In the no-gap condition, the entire pulse train was delivered uninterrupted to electrode 12, and the onset P1-N1-P2 complex was measured relative to the stimulus onset. In the gapped condition, the stimulus consisted of two pulse train bursts, each being 400 msec in duration, presented sequentially on the same electrode and separated by one of five gaps (i.e., 5, 10, 20, 50, and 100 msec). Open-set speech-perception ability of these subjects with ANSD was assessed using the phonetically balanced kindergarten (PBK) word lists presented at 60 dB SPL, using monitored live voice in a sound booth. RESULTS: The eERPs were recorded from all subjects with ANSD who participated in this study. There were no significant differences in test-retest reliability, root mean square amplitude or P1 latency for the onset P1-N1-P2 complex between subjects with good (>70% correct on PBK words) and poorer speech-perception performance. In general, the EACC showed less mature morphological characteristics than the onset P1-N1-P2 response recorded from the same subject. There was a robust correlation between the PBK word scores and the EACC thresholds for gap detection. Subjects with poorer speech-perception performance showed larger EACC thresholds in this study. CONCLUSIONS: These results demonstrate the feasibility of recording eERPs from implanted children with ANSD, using direct electrical stimulation. Temporal-processing deficits, as demonstrated by large EACC thresholds for gap detection, might account in part for the poor speech-perception performances observed in a subgroup of implanted subjects with ANSD. This finding suggests that the EACC elicited by changes in temporal continuity (i.e., gap) holds promise as a predictor of speech-perception ability among implanted children with ANSD.

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

Introduction: 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. Methods: 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. Results: 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. Conclusion: 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.

Effects of frequency compression hearing aids for unilaterally implanted children with acoustically amplified residual hearing in the nonimplanted ear.

OBJECTIVES: The primary objective of this study is to evaluate the benefits of nonlinear frequency compression (NLFC) hearing aids in the nonimplanted ears of children with unilateral cochlear implants (CIs). It is hypothesized that speech perception performance will benefit from complementary auditory cues provided by the CI and the hearing aid, particularly with the increased access to high-frequency sounds provided by NLFC. DESIGN: Eleven children using unilateral CIs with usable residual hearing in the nonimplanted ears were enrolled in the study and fitted with NLFC hearing aids. The test protocol included consonant-nucleus-consonant words in quiet, the Hearing in Noise Test sentences presented in speech noise and two-talker maskers, and a consonant identification task. Subjects were tested in a CI-alone condition as well as bimodally, with and without NLFC enabled. RESULTS: The results support previous work in adults and children, demonstrating the beneficial effects of bimodal listening. Frequency compression did not significantly affect performance for the children enrolled in this study, although some preferred using NLFC. The results yield suggestions regarding test methods for pediatric bimodal listeners, and considerations regarding validation and audibility of the compressed signal. CONCLUSIONS: Hearing aid use in the contralateral ear of unilaterally implanted children is beneficial. Children and young adults who are fitted bimodally should be tested both in quiet and in complex listening situations to determine bimodal benefit. In the current test battery, the inclusion of frequency compression in the hearing aid fitting does not seem to provide significant improvement beyond standard hearing aid fittings or any bilateral interference symptoms for this group of bimodal listeners.

Benefits of Cochlear Implantation in Childhood Unilateral Hearing Loss (CUHL Trial).

OBJECTIVES/HYPOTHESES: Children with unilateral sensory hearing loss (UHL) struggle to understand speech in noise and locate the origin of sound and have reduced quality of hearing. This clinical trial will determine the benefits of cochlear implantation in children with UHL. STUDY DESIGN: Prospective clinical trial. METHODS: Twenty children with at least moderate to profound sensory hearing loss and poor speech perception (word score <30%) in one ear and normal hearing in the contralateral ear participated in a Food and Drug Administration-approved clinical trial. Subjects were evaluated for speech perception in quiet, speech perception in noise, sound localization, and subjective benefits after implantation. RESULTS: CNC word score perception in quiet significantly improved (1% to 50%, P < .0001) by 12 months after activation. Speech perception in noise by BKB-SIN significantly improved in all three noise configurations; there was a 3.6 dB advantage in head shadow (P < .0001), a 1.6 dB advantage in summation (P = .003), and a 2.5 dB advantage in squelch (P = .0001). Localization improved by 26° at 9 months (P < .0001). Speech, Spatial, and Qualities (SSQ) demonstrated significant improvements in speech (5.2 to 7.4, P = .0012), qualities of hearing (5.9 to 7.5, P = .0056), and spatial hearing (2.7 to 6.6, P < .0001). SSQ subscales associated with binaural hearing were significantly improved, as was listening effort (P = .0082). Subjects demonstrated a non-significant improvement in fatigue. CONCLUSIONS: This study demonstrates that children with UHL significantly benefit from cochlear implantation. LEVEL OF EVIDENCE: Level 3 Laryngoscope, 132:S1-S18, 2022.

Frequency Mapping of a Precurved Electrode Array in the Internal Auditory Canal for a Pediatric Cochlear Implant Recipient.

OBJECTIVES: Review the effectiveness of an alternative mapping procedure of a precurved electrode array in the internal auditory canal (IAC). DESIGN: A 7-year-old bilateral cochlear implant (CI) recipient of precurved arrays transferred to the study site and demonstrated no speech recognition with the left CI. Imaging revealed bilateral incomplete partition type III malformations. For the left CI, four contacts were observed in the basal cochlear turn and 18 contacts were coiled in the IAC. The family decided against revision surgery. Pitch ranking was completed to map the contacts in the IAC that were perceptually discrete and tonotopically organized. RESULTS: For the left CI, PB-K word recognition improved from no recognition to 32% after 1 month and to 52% after 3 months. In the bilateral CI condition, performance improved from 56 to 72% after 1 month and 80% after 3 months. CONCLUSION: A precurved array in the IAC creates difficult management decisions. Direct stimulation of the auditory nerve resulted in better monaural and bilateral speech recognition, likely due to better spectral representation of the speech signal. Individualizing the map using imaging and behavioral findings may improve performance for malpositioned arrays when revision surgery is not pursued.

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

PURPOSE: 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. METHOD: 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. RESULTS: 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. CONCLUSIONS: 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 impact of unilateral, simultaneous, or sequential cochlear implantation on pediatric language outcomes.

OBJECTIVES: The purpose of this study was to determine the impact of unilateral versus bilateral cochlear implantation on receptive and expressive spoken language outcomes. Secondary aims were to investigate factors timing of first and second implant placement and reliance on government funded health plans on language outcome. METHODS: This was a retrospective chart review of spoken language users with bilateral severe-to-profound hearing loss. A total of 204 children were included, 105 in the bilateral group and 99 in the unilateral group. Multiple regression analyses were completed to investigate factors that influence language outcomes at age 5. RESULTS: Recipients who received bilateral CIs performed significantly higher on measures of receptive and expressive language than those with unilateral implants. DISCUSSION: This study demonstrates that bilateral implantation has a significant impact on receptive and expressive aspects of language development, and should be strongly considered as standard practice for children with bilateral severe to profound hearing loss. CONCLUSION: These results indicate that families should be counseled that language outcomes are better with bilateral cochlear implantation than unilateral implantation. Cochlear implant teams should continue to consider the impact of socioeconomic status on outcomes and explore new methods to reduce the impacts and barriers of poverty to language development.

Comparison of test methods to assess the implanted ear alone for pediatric cochlear implant recipients with single-sided deafness.

OBJECTIVES: The purpose of this investigation was to compare three test methods for isolating the test ear for children with single-sided deafness (SSD) who use a cochlear implant (CI). METHODS: Word recognition was assessed for five CI recipients with SSD and six bilateral CI recipients with no acoustic hearing. For the SSD subjects, performance was compared: 1) in the sound field with masking in the normal-hearing ear, 2) in the sound field with an earplug and earmuff ("plug-and-muff"), and 3) via direct connect (DC). For the bilateral CI subjects, performance was compared: 1) in the sound field and 2) via DC. RESULTS: For the bilateral CI subjects, word recognition was similar when assessed in the sound field versus via DC. For the SSD subjects, performance was similar when assessed with the plug-and-muff and DC methods but was significantly poorer with masking presented to the normal-hearing ear. DISCUSSION: Masking the normal-hearing ear to isolate the CI for word recognition is problematic in this population. The plug-and-muff and DC test methods may provide a more accurate assessment. CONCLUSION: DC or plug-and-muff methods are recommended to isolate the CI-ear for word recognition testing in children with SSD. Patient specific variables should be considered.

The Limitations of FDA Criteria: Inconsistencies with Clinical Practice, Findings, and Adult Criteria as a Barrier to Pediatric Implantation.

Children require greater access to sound than adults as they are learning to communicate using hearing and spoken language. Yet when it comes to cochlear implant candidacy, currently approved Food and Drug Administration (FDA) criteria for adults are much less restrictive than those for children, allowing for greater levels of residual hearing and aided speech recognition in adults. Cochlear implant guidelines for children have changed very little in the 30 years since cochlear implants have been approved for pediatrics, and this lack of change has proven to be a barrier to implantation. Using evidence-based practice, centers have been providing off-label implantation for children who fall outside of current FDA criteria, including children with more residual hearing, children with single-side deafness younger than 5 years, and infants with bilateral profound loss younger than 9 months. The purpose of this article is to outline how these restrictions impede access to implants for children and describe the evidence supporting cochlear implantation in children who fall outside of current criteria.