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This study investigated the effects of two temporal response properties of the auditory nerve (i.e., neural adaptation and recovery from neural adaptation) on speech perception performance in postlingually deafened adult cochlear implant (CI) users.Study participants included 18 postlingually deafened adults who were Cochlear Nucleus device users with a full electrode array insertion in the test ear(s). Neural adaptation and adaptation recovery of the auditory nerve (AN) were evaluated using electrophysiological measures of the electrically evoked compound action potential (eCAP). The amount of neural adaptation was quantified by the adaptation index within three time windows: 0 to 8.89 (window 1), 44.44 to 50.00 (window 2), and 94.44 to 100.00 ms (window 3). The speed of neural adaptation was estimated using a two-parameter power law function. To evaluate adaptation recovery of the AN, eCAPs to the last pulse of the 100-ms pulse train were recorded at masker-probe-intervals ranging from 1.054 to 256 ms in logarithmic steps. The amount of adaptation recovery was quantified by the adaptation recovery ratio. The time-constant of adaptation recovery was estimated using an exponential function with up to three components. Speech perception performance was evaluated by measuring consonant-nucleus-consonant (CNC) word scores presented in quiet and in speech-shaped noise at a signal-to-noise ratio (SNR) of +10 dB. One-tailed Pearson Product Moment correlation tests were used (1) to assess the associations among parameters of neural adaptation and adaptation recovery and (2) to evaluate the strength of association between these parameters and CNC word scores measured in quiet and in noise. The contributions of different parameters quantifying neural adaptation and adaptation recovery on speech perception scores were evaluated using multivariable linear regression analyses.The Pearson Product Moment correlation coefficient demonstrated a moderate, negative correlation between the speed of adaptation recovery and CNC word scores measured in quiet and in noise. The speed of adaptation recovery accounted for 14.1% of variability in CNC word scores measured in quiet and 16.7% of variability in CNC word scores measured in noise. The correlation strengths between CNC word scores and the adaptation index, the adaptation recovery ratio and the speed of neural adaptation ranged from negligible to weak.The speed of adaptation recovery plays a more important role than other features of neural adaptation and adaptation recovery of the AN in speech perception in postlingually deafened adult CI users. Patients with prolonged adaptation recovery tend to show poorer speech perception performance.

Purpose: The purpose of this study was to evaluate vocabulary development in Mandarin-speaking children with bilateral cochlear implants (CIs), bimodal stimulation (CI plus hearing aids [HAs]), or unilateral CIs during the first year after CI activation. Method: Participants included 23 children with simultaneous bilateral CIs, 23 children with bimodal stimulation, and 15 children with unilateral CIs. They all received CIs before 30 months of age. Parents were asked to endorse words that their child could understand only or understand and say using the Early Vocabulary Inventory for Mandarin Chinese at the day of CI activation and 1, 3, 6, 9, and 12 months after CI activation. Receptive and expressive vocabulary sizes were computed. Results: Growth curve analysis revealed that children with simultaneous bilateral CIs demonstrated faster growth of receptive vocabulary than those with bimodal stimulation, followed by those with unilateral CIs. Moreover, children with simultaneous bilateral CIs reached the 100-word mark for receptive vocabulary earlier than children with bimodal stimulation, followed by those with unilateral CIs. There were no significant differences among the three groups in expressive vocabulary. Conclusions: Bilateral CIs have an advantage over bimodal stimulation in early receptive vocabulary development in Mandarin, a tone language. HA usage is still recommended for those who receive one CI.

OBJECTIVE: This study aimed to investigate the associations between advanced age and the amount and the speed of neural adaptation of the electrically-stimulated auditory nerve (AN) in postlingually deafened adult cochlear implant (CI) users. DESIGN: Study participants included 26 postlingually deafened adult CI users, ranging in age between 28.7 and 84.0 years (mean: 63.8 years, std: 14.4 years) at the time of testing. All study participants used a Cochlear(™) Nucleus® device with a full electrode array insertion in the test ear. The stimulus was a 100-ms pulse train with a pulse rate of 500, 900, 1800 or 2400 pulses per second (pps) per channel. The stimulus was presented at the maximum comfortable level measured at 2400 pps with a presentation rate of 2 Hz. Neural adaptation of the AN was evaluated using electrophysiological measures of the electrically evoked compound action potential (eCAP). The amount of neural adaptation was quantified by the adaptation index (AI) within three time windows: around 0–8 ms (window 1), 44–50 ms (window 2) and 94–100 ms (window 3). The speed of neural adaptation was quantified using a two-parameter power law estimation. In 23 participants, four electrodes across the electrode array were tested. In three participants, three electrodes were tested. Results measured at different electrode locations were averaged for each participant at each pulse rate in order to get an overall representation of neural adaptation properties of the AN across the cochlea. Linear Mixed Models (LMMs) were used 1) to evaluate the effects of age at testing and pulse rate on the speed of neural adaptation, and 2) to assess the effects of age at testing, pulse rate, and duration of stimulation (i.e., time window) on the amount of neural adaptation in these participants. RESULTS: There was substantial variability in both the amount and the speed of neural adaptation of the AN among study participants. The amount and the speed of neural adaptation increased at higher pulse rates. In addition, larger amounts of adaptation were observed for longer durations of stimulation. There was no significant effect of age on the speed or the amount of neural adaptation. CONCLUSIONS: The amount and the speed of neural adaptation of the AN are affected by both the pulse rate and the duration of stimulation, with higher pulse rates and longer durations of stimulation leading to faster and greater neural adaptation. Advanced age does not affect neural adaptation of the AN in postlingually deafened, middle-aged and elderly adult CI users.

This study assessed the relationships between the salience of amplitude modulation (AM) cues encoded at the auditory nerve (AN), perceptual sensitivity to changes in AM rate (i.e., AM rate discrimination threshold, AMRDT), and speech perception scores in postlingually deafened adult cochlear implant (CI) users.Study participants were 18 postlingually deafened adults with Cochlear Nucleus devices, including five bilaterally implanted patients. For each of 23 implanted ears, neural encoding of AM cues at 20 Hz at the AN was evaluated at seven electrode locations across the electrode array using electrophysiological measures of the electrically evoked compound action potential (eCAP). The salience of AM neural encoding was quantified by the Modulated Response Amplitude Ratio (MRAR). Psychophysical measures of AMRDT for 20 Hz modulation were evaluated in 16 ears using a three-alternative, forced-choice procedure, targeting 79.4% correct on the psychometric function. AMRDT was measured at up to five electrode locations for each test ear, including the electrode pair that showed the largest difference in the MRAR. Consonant-Nucleus-Consonant (CNC) word scores presented in quiet and in speech-shaped noise at a signal to noise ratio (SNR) of +10 dB were measured in all 23 implanted ears. Simulation tests were used to assess the variations in correlation results when using the MRAR and AMRDT measured at only one electrode location in each participant to correlate with CNC word scores. Linear Mixed Models (LMMs) were used to evaluate the relationship between MRARs/AMRDTs measured at individual electrode locations and CNC word scores. Spearman Rank correlation tests were used to evaluate the strength of association between CNC word scores measured in quiet and in noise with (1) the variances in MRARs and AMRDTs, and (2) the averaged MRAR or AMRDT across multiple electrodes tested for each participant.There was no association between the MRAR and AMRDT. Using the MRAR and AMRDT measured at only one, randomly selected electrode location to assess their associations with CNC word scores could lead to opposite conclusions. Both the results of LMMs and Spearman Rank correlation tests showed that CNC word scores measured in quiet or at 10 dB SNR were not significantly correlated with the MRAR or AMRDT. In addition, the results of Spearman Rank correlation tests showed that the variances in MRARs and AMRDTs were not significantly correlated with CNC word scores measured in quiet or in noise.The difference in AN sensitivity to AM cues is not the primary factor accounting for the variation in AMRDTs measured at different stimulation sites within individual CI users. The AN sensitivity to AM per se may not be a crucial factor for CNC word perception in quiet or at 10 dB SNR in postlingually deafened adult CI users. Using electrophysiological or psychophysical results measured at only one electrode location to correlate with speech perception scores in CI users can lead to inaccurate, if not wrong, conclusions.

ObjectivesThis preliminary study identified a missense variant inACTG1(NM_001614.5) in a family with autosomal dominant non-syndromic hearing loss (ADNSHL). The responsiveness of the electrically-stimulated cochlear nerve (CN) in two implanted participants with this missense change was also evaluated and reported.DesignGenetic testing was done using a custom capture panel (MiamiOtoGenes) and whole exome sequencing. The responsiveness of the electrically-stimulated CN was evaluated in two members of this family (G1 and G4) using the electrically evoked compound action potential (eCAP). eCAP results from these two participants were compared with those measured three implanted patient populations: children with cochlear nerve deficiency, children with idiopathic hearing loss and normal-sized cochlear nerves, and postligually deafened adults.ResultsSequencing ofACTG1identified a missense c.737A>T (p. Gln246Leu) variant inACTG1(NM_001614.5) which is most likely the genetic cause of ADNSHL in this family. eCAP results measured in these two participants showed substantial variations.ConclusionThe missense c.737A>T (p. Gln246Leu) variant inACTG1(NM_001614.5) co-segregated with hearing loss in this family. The responsiveness of the electrically-stimulated CN can vary among patients with the same genetic variants, which suggests the importance of evaluating the functional status of the CN for individual CI patients.

Purpose: The aim of this study is to evaluate whether Mandarin-speaking children with cochlear implants (CIs) demonstrated early lexical composition similar to their hearing peers who were at the same vocabulary level and the extent to which children with CIs were sensitive to linguistic and conceptual properties when developing early lexicon. Method: Participants were 77 Mandarin-speaking children with CIs who received CIs before 30 months of age. Their expressive vocabulary was documented using the Infant Checklist of the Early Vocabulary Inventory for Mandarin Chinese 9 or 12 months after CI activation. Percent social words, common nouns, predicates (verbs, adjectives), and closed-class words in total vocabulary were computed for children at different vocabulary levels. Common nouns and verbs were further coded for their word class (noun, verb), word frequency, word length, and imageability to predict how likely a given noun or verb would be produced by children with CIs. Results: Like children with typical hearing, social words were the most dominant category when vocabulary size in children with CIs was smaller than 20 words; common nouns became the most dominant category when the vocabulary size reached 21 words. The difference in percent common nouns and percent predicates (i.e., noun bias) was similar in children with CIs and their hearing peers. In addition, verbs, common words, monosyllabic words, and more imageable words were more likely to be produced by children with CIs than their counterparts. Conclusions: Mandarin children with CIs showed language-specific patterns in early lexical composition like their hearing peers. They were able to use multiple linguistic and conceptual cues when approaching early expressive vocabulary despite perceptual and processing constraints. Supplemental Material: https://doi.org/10.23641/asha.21357723

ObjectiveThis study assessed the relationships between the auditory nerve’s ability to recovery from neural adaptation, cortical encoding of and perceptual sensitivity to within-channel temporal gaps in postlingually deafened adult cochlear implant (CI) users.DesignStudy participants included 11 postlingually deafened adults with Cochlear™ Nucleus® devices, including three bilaterally implanted participants. In each participant, recovery from neural adaptation of the auditory nerve (AN) was measured using electrophysiological measures of the electrically evoked compound action potential (eCAP) at up to four electrode locations. The electrode pair with the largest difference in the speed of adaptation recovery was selected for assessing within-channel temporal gap detection threshold (GDT). GDTs were measured using both psychophysical and electrophysiological procedures. Psychophysical GDTs were evaluated using a three-alternative, forced-choice procedure, targeting 79.4% correct on the psychometric function. Electrophysiological GDTs were measured using the electrically evoked auditory change complex (eACC) evoked by electrical pulse trains that contained temporal gaps. Objective GDT was defined as the shortest temporal gap that could evoke an eACC. Related-samples Wilcoxon Signed Rank testwas used to compare psychophysical GDTs and objective GDTs measured at all electrode locations. It was also used to compare psychophysical GDTs and objective GDTs measured at the two electrode locations with different speeds or amounts of adaptation recovery of the AN. A Kendall Rank correlation test was used to assess the correlation between GDTs measured using psychophysical or electrophysiological procedures.ResultsObjective GDTs were significantly larger than those measured using psychophysical procedures. There was a significant correlation between objective and psychophysical GDTs. GDTs could not be predicted based on the amount or the speed of adaptation recovery of the AN.ConclusionsElectrophysiological measure of the eACC can potentially be used to assess within-channel GDT in CI users who cannot provide reliable behavioral responses. The difference in adaptation recovery of the AN is not the primary factor accounting for the across-electrode variation in GDT in individual CI users.

ObjectiveThis study assessed the relationship between electrophysiological measures of the electrically evoked compound action potential (eCAP) and speech perception scores measured in quiet and in noise in post-lingually deafened adult cochlear implant (CI) users. It tested the hypothesis that how well the auditory nerve (AN) responds to electrical stimulation is important for speech perception with a CI in challenging listening conditions.DesignStudy participants included 24 post-lingually deafened adult CI users. All participants used Cochlear® Nucleus™ CIs in their test ears. In each participant, eCAPs were measured at multiple electrode locations in response to single-pulse, paired-pulse, and pulse-train stimuli. Independent variables included six parameters calculated from the eCAP recordings: the electrode-neuron interface (ENI) index, the neural adaptation (NA) ratio, NA speed, the adaptation recovery (AR) ratio, AR speed, and the amplitude modulation (AM) ratio. The ENI index quantified the effectiveness of the CI electrodes in stimulating the targeted AN fibers. The NA ratio indicated the amount of NA at the AN caused by a train of constant-amplitude pulses. NA speed was defined as the speed/rate of NA. The AR ratio estimated the amount of recovery from NA at a fixed time point after the cessation of pulse-train stimulation. AR speed referred to the speed of recovery from NA caused by previous pulse-train stimulation. The AM ratio provided a measure of AN sensitivity to AM cues. Participants’ speech perception scores were measured using Consonant-Nucleus-Consonant (CNC) word lists and AzBio sentences presented in quiet, as well as in noise at signal-to-noise ratios (SNRs) of +10 and +5 dB. Predictive models were created for each speech measure to identify eCAP parameters with meaningful predictive power.ResultsThe ENI index and AR speed had significant bivariate relationships with speech perception scores measured in this study, while the NA ratio, NA speed, the AR ratio, and the AM ratio did not. The ENI index was identified as the only eCAP parameter that had unique predictive power for each of the speech test results. The amount of variance in speech perception scores (both CNC words and AzBio sentences) explained by the eCAP parameters increased with increased difficulty in the listening condition. Over half of the variance in speech perception scores measured in +5 dB SNR noise (both CNC words and AzBio sentences) was explained by a model with only three eCAP parameters: the ENI index, NA speed, and AR speed.ConclusionsThe ENI index is the most informative predictor for speech perception performance in CI users. The response characteristics of the AN to electrical stimulation is more important for speech perception with a CI in noise than it is in quiet.

Objectives Amplitudes of electrically evoked compound action potentials (eCAPs) as a function of the stimulation level constitute the eCAP amplitude growth function (AGF). The slope of the eCAP AGF (i.e., rate of growth of eCAP amplitude as a function of stimulation level), recorded from subjects with cochlear implants (CIs), has been widely used as an indicator of survival of cochlear nerve fibers. However, substantial variation in the approach used to calculate the slope of the eCAP AGF makes it difficult to compare results across studies. In this study, we developed an improved slope-fitting method by addressing the limitations of previously used approaches and ensuring its application for the estimation of the maximum slopes of the eCAP AGFs recorded in both animal models and human listeners with various etiologies. Design The new eCAP AGF fitting method was designed based on sliding window linear regression. Slopes of the eCAP AGF estimated using this new fitting method were calculated and compared with those estimated using four other fitting methods reported in the literature. These four methods were nonlinear regression with a sigmoid function, linear regression, gradient calculation, and boxcar smoothing. The comparison was based on the fitting results of 72 eCAP AGFs recorded from 18 acutely implanted guinea pigs, 46 eCAP AGFs recorded from 23 chronically implanted guinea pigs, and 2094 eCAP AGFs recorded from 200 human CI users from 4 patient populations. The effect of the choice of input units of the eCAP AGF (linear versus logarithmic) on fitting results was also evaluated. Results The slope of the eCAP AGF was significantly influenced by the slope-fitting method and by the choice of input units. Overall, slopes estimated using all five fitting methods reflected known patterns of neural survival in human patient populations and were significantly correlated with speech perception scores. However, slopes estimated using the newly developed method showed the highest correlation with spiral ganglion neuron density among all five fitting methods for animal models. In addition, this new method could reliably and accurately estimate the slope for 4 human patient populations, while the performance of the other methods was highly influenced by the morphology of the eCAP AGF. Conclusions The novel slope-fitting method presented in this study addressed the limitations of the other methods reported in the literature and successfully characterized the slope of the eCAP AGF for various animal models and CI patient populations. This method may be useful for researchers in conducting scientific studies and for clinicians in providing clinical care for CI users.

Minimally traumatic surgical techniques and advances in cochlear implant (CI) electrode array designs have allowed acoustic hearing present in a CI candidate prior to surgery to be preserved post-operatively. As a result, these patients benefit from combined electric-acoustic stimulation (EAS) post-operatively. However, 30-40% of EAS CI users experience a partial loss of hearing up to 30 dB after surgery. In the present study, electrocochleography (ECoG) was used to study cochlear microphonic (hair cell response) and auditory nerve neurophonic (neural response) in patients with preserved hearing and patients with loss of hearing. These measures were obtained longitudinally over the course of CI use. At each test session, ECoG amplitude growth functions for several low-frequency stimuli were obtained. The threshold, slope, and suprathreshold amplitude at a fixed stimulation level was obtained from each growth function at each time point. Subjects were categorized as having stable hearing or loss of hearing. Longitudinal linear mixed effects models were used study trends in ECoG thresholds, slopes, and amplitudes for these two categories of subjects. Results showed that CM and ANN thresholds and amplitudes were stable in CI users with preserved residual hearing. CM and ANN thresholds increased (worsened) while CM and ANN amplitudes decreased (worsened) for those with delayed hearing loss. The slope did not distinguish between subjects with stable hearing and subjects with delayed loss of hearing. These results provide a new application of post-operative ECoG as an objective tool to monitor residual hearing and understand the pathophysiology of delayed hearing loss.

Background Congenital hearing loss is remarkably heterogeneous, with over 130 deafness genes and thousands of variants, making for innumerable genotype/phenotype combinations. Understanding both the pathophysiology of hearing loss and molecular site of lesion along the auditory pathway permits for significantly individualized counseling. Electrophysiologic techniques such as electrocochleography (ECochG) and electrically-evoked compound action potentials (eCAP) are being studied to localize pathology and estimate residual cochlear vs. neural health. This review describes the expanding roles of genetic and electrophysiologic evaluation in the precision medicine of congenital hearing loss. The basics of genetic mutations in hearing loss and electrophysiologic testing (ECochG and eCAP) are reviewed, and how they complement each other in the diagnostics and prognostication of hearing outcomes. Used together, these measures improve the understanding of insults to the auditory system, allowing for individualized counseling for CI candidacy/outcomes or other habilitation strategies. Conclusion Despite tremendous discovery in deafness genes, the effects of individual genes on neural function remain poorly understood. Bridging the understanding between molecular genotype and neural and functional phenotype is paramount to interpreting genetic results in clinical practice. The future hearing healthcare provider must consolidate an ever-increasing amount of genetic and phenotypic information in the precision medicine of hearing loss.

Objective This study reports two potential biomarkers of the physiological status of the cochlear nerve (CN) in cochlear implant users. Design This project represents a complementary analysis on a subset of electrophysiological data from our recently published study. This study compared changes in N1 latency and stimulation level (i.e., N1 latency offset and stimulation level offset) with increasing interphase gap of a biphasic pulse between children with cochlear nerve deficiency and children with normal-sized CNs. Results Children with cochlear nerve deficiency showed smaller N1 latency offsets and larger stimulation level offsets than children with normal-sized CNs at all electrode locations tested. Conclusions The N1 latency and stimulation level offsets differ in two patient populations with different physiological statuses of the CN. These parameters may be useful for evaluating CN function in individual cochlear implant patients.

OBJECTIVES The aim of this study is to (1) investigate the effects of increasing the pulse phase duration (PPD) on the neural response of the electrically stimulated cochlear nerve (CN) in children with CN deficiency (CND) and (2) compare the results from the CND population to those measured in children with normal-sized CNs. DESIGN Study participants included 30 children with CND and 30 children with normal-sized CNs. All participants used a Cochlear Nucleus device in the test ear. For each subject, electrically evoked compound action potential (eCAP) input/output (I/O) functions evoked by single biphasic pulses with different PPDs were recorded at three electrode locations across the electrode array. PPD durations tested in this study included 50, 62, 75, and 88 μsec/phase. For each electrode tested for each study participant, the amount of electrical charge corresponding to the maximum comfortable level measured for the 88 μsec PPD was used as the upper limit of stimulation. The eCAP amplitude measured at the highest electrical charge level, the eCAP threshold (i.e., the lowest level that evoked an eCAP), and the slope of the eCAP I/O function were measured. Generalized linear mixed effect models with study group, electrode location, and PPD as the fixed effects and subject as the random effect were used to compare these dependent variables measured at different electrode locations and PPDs between children with CND and children with normal-sized CNs. RESULTS Children with CND had smaller eCAP amplitudes, higher eCAP thresholds, and smaller slopes of the eCAP I/O function than children with normal-sized CNs. Children with CND who had fewer electrodes with a measurable eCAP showed smaller eCAP amplitudes and flatter eCAP I/O functions than children with CND who had more electrodes with eCAPs. Increasing the PPD did not show a statistically significant effect on any of these three eCAP parameters in the two subject groups tested in this study. CONCLUSIONS For the same amount of electrical charge, increasing the PPD from 50 to 88 μsec for a biphasic pulse with a 7 μsec interphase gap did not significantly affect CN responsiveness to electrical stimulation in human cochlear implant users. Further studies with different electrical pulse configurations are warranted to determine whether evaluating the eCAP sensitivity to changes in the PPD can be used as a testing paradigm to estimate neural survival of the CN for individual cochlear implant users.

For a clamp-on ultrasonic flowmeter based on transit-time differential method, the flow rate calculation model with flow velocity correction is established and verified by experiments carried out in a flow standard facility. Based on established flow rate calculation model, a systematic methodology incorporating the parametric uncertainty distribution is proposed to analyze the influences of multiple parameters with uncertainty on measurement error by establishing the sample-based stochastic model. Monte Carlo sampling (MCS) method is used to randomly select the value of each parameter from its prescribed Gaussian distribution and then combining them together as one sample, and the number of selected samples is determined by conducting stochastic convergence analysis of mean value and the standard deviation of input and output parameters. And finally the influence degrees of multiple parameters are analyzed and compared quantitatively on the same dimension to find out which parameters are influential and which are negligible. The results reveal the different influences of multiple parameters with uncertainty, which can be used as reference for performance improvement and measuring error analysis of the clamp-on ultrasonic flowmeter in industrial local measurement.

This study aimed to determine the effect of advanced age on how effectively a cochlear implant (CI) electrode stimulates the targeted cochlear nerve fibers (i.e., the electrode-neuron interface [ENI]) in postlingually deafened adult CI users. The study tested the hypothesis that the quality of the ENI declined with advanced age. It also tested the hypothesis that the effect of advanced age on the quality of the ENI would be greater in basal regions of the cochlea compared to apical regions.Study participants included 40 postlingually deafened adult CI users. The participants were separated into two age groups based on age at testing in accordance with age classification terms used by the World Health Organization and the Medical Literature Analysis and Retrieval System Online bibliographic database. The middle-aged group included 16 participants between the ages of 45 and 64 years and the elderly group included 24 participants older than 65 years. Results were included from one ear for each participant. All participants used Cochlear Nucleus CIs in their test ears. For each participant, electrophysiological measures of the electrically evoked compound action potential (eCAP) were used to measure refractory recovery functions and amplitude growth functions (AGFs) at three to seven electrode sites across the electrode array. The eCAP parameters used in this study included the refractory recovery time estimated based on the eCAP refractory recovery function, the eCAP threshold, the slope of the eCAP AGF, and the negative-peak (i.e., N1) latency. The electrode-specific ENI was evaluated using an optimized combination of the eCAP parameters that represented the responsiveness of cochlear nerve fibers to electrical stimulation delivered by individual electrodes along the electrode array. The quality of the electrode-specific ENI was quantified by the local ENI index, a value between 0 and 100 where 0 and 100 represented the lowest- and the highest-quality ENI across all participants and electrodes in the study dataset, respectively.There were no significant age group differences in refractory times, eCAP thresholds, N1 latencies or local ENI indices. Slopes of the eCAP AGF were significantly larger in the middle-aged group compared to the elderly group. There was a significant effect of electrode location on each eCAP parameter, except for N1 latency. In addition, the local ENI index was significantly larger (i.e., better ENI) in the apical region than in the basal and middle regions of the cochlea for both age groups.The model developed in this study can be used to estimate the quality of the ENI at individual electrode locations in CI users. The quality of the ENI is affected by the location of the electrode along the length of the cochlea. The method for estimating the quality of the ENI developed in this study holds promise for identifying electrodes with poor ENIs that could be deactivated from the clinical programming map. The ENI is not strongly affected by advanced age in middle-aged and elderly CI users.

Cochlear implants (CIs) provide acoustic information to implanted patients by electrically stimulating nearby auditory nerve fibers (ANFs) which then transmit the information to higher-level neural structures for further processing and interpretation. Computational models that simulate ANF responses to CI stimuli enable the exploration of the mechanisms underlying CI performance beyond the capacity of in vivo experimentation alone. However, all ANF models developed to date utilize to some extent anatomical/morphometric data, biophysical properties and/or physiological data measured in non-human animal models. This review compares response properties of the electrically stimulated auditory nerve (AN) in human listeners and different mammalian models. Properties of AN responses to single pulse stimulation, paired-pulse stimulation, and pulse-train stimulation are presented. While some AN response properties are similar between human listeners and animal models (e.g., increased AN sensitivity to single pulse stimuli with long interphase gaps), there are some significant differences. For example, the AN of most animal models is typically more sensitive to cathodic stimulation while the AN of human listeners is generally more sensitive to anodic stimulation. Additionally, there are substantial differences in the speed of recovery from neural adaptation between animal models and human listeners. Therefore, results from animal models cannot be simply translated to human listeners. Recognizing the differences in responses of the AN to electrical stimulation between humans and other mammals is an important step for creating ANF models that are more applicable to various human CI patient populations.

Objectives This study aimed to create an objective predictive model for assessing the functional status of the cochlear nerve (CN) in individual cochlear implant (CI) users. Design Study participants included 23 children with cochlear nerve deficiency (CND), 29 children with normal-sized CNs (NSCNs), and 20 adults with various etiologies of hearing loss. Eight participants were bilateral CI users and were tested in both ears. As a result, a total of 80 ears were tested in this study. All participants used Cochlear Nucleus CIs in their test ears. For each participant, the CN refractory recovery function and input/output (I/O) function were measured using electrophysiological measures of the electrically evoked compound action potential (eCAP) at three electrode sites across the electrode array. Refractory recovery time constants were estimated using statistical modeling with an exponential decay function. Slopes of I/O functions were estimated using linear regression. The eCAP parameters used as input variables in the predictive model were absolute refractory recovery time estimated based on the refractory recovery function, eCAP threshold, slope of the eCAP I/O function, and negative-peak (i.e., N1) latency. The output variable of the predictive model was CN index, an indicator for the functional status of the CN. Predictive models were created by performing linear regression, support vector machine regression, and logistic regression with eCAP parameters from children with CND and the children with NSCNs. One-way analysis of variance with post hoc analysis with Tukey's honest significant difference criterion was used to compare study variables among study groups. Results All three machine learning algorithms created two distinct distributions of CN indices for children with CND and children with NSCNs. Variations in CN index when calculated using different machine learning techniques were observed for adult CI users. Regardless of these variations, CN indices calculated using all three techniques in adult CI users were significantly correlated with Consonant-Nucleus-Consonant word and AzBio sentence scores measured in quiet. The five oldest CI users had smaller CN indices than the five youngest CI users in this study. Conclusions The functional status of the CN for individual CI users was estimated by our newly developed analytical models. Model predictions of CN function for individual adult CI users were positively and significantly correlated with speech perception performance. The models presented in this study may be useful for understanding and/or predicting CI outcomes for individual patients.

Hypotheses Adult cochlear implant (CI) outcomes depend on demographic, sensory, and cognitive factors. However, these factors have not been examined together comprehensively for relations to different outcome types, such as speech recognition versus quality of life (QOL). Three hypotheses were tested: 1) speech recognition will be explained most strongly by sensory factors, whereas QOL will be explained more strongly by cognitive factors. 2) Different speech recognition outcome domains (sentences versus words) and different QOL domains (physical versus social versus psychological functioning) will be explained differentially by demographic, sensory, and cognitive factors. 3) Including cognitive factors as predictors will provide more power to explain outcomes than demographic and sensory predictors alone. Background A better understanding of the contributors to CI outcomes is needed to prognosticate outcomes before surgery, explain outcomes after surgery, and tailor rehabilitation efforts. Methods Forty-one adult postlingual experienced CI users were assessed for sentence and word recognition, as well as hearing-related QOL, along with a broad collection of predictors. Partial least squares regression was used to identify factors that were most predictive of outcome measures. Results Supporting our hypotheses, speech recognition abilities were most strongly dependent on sensory skills, while QOL outcomes required a combination of cognitive, sensory, and demographic predictors. The inclusion of cognitive measures increased the ability to explain outcomes, mainly for QOL. Conclusions Explaining variability in adult CI outcomes requires a broad assessment approach. Identifying the most important predictors depends on the particular outcome domain and even the particular measure of interest.

Objective: This study evaluated cortical encoding of voice onset time (VOT) in quiet and noise, and their potential associations with the behavioral categorical perception of VOT in children with auditory neuropathy spectrum disorder (ANSD). Design: Subjects were 11 children with ANSD ranging in age between 6.4 and 16.2 years. The stimulus was an /aba/-/apa/ vowel-consonant-vowel continuum comprising eight tokens with VOTs ranging from 0 ms (voiced endpoint) to 88 ms (voiceless endpoint). For speech in noise, speech tokens were mixed with the speech-shaped noise from the Hearing In Noise Test at a signal-to-noise ratio (SNR) of +5 dB. Speech-evoked auditory event-related potentials (ERPs) and behavioral categorization perception of VOT were measured in quiet in all subjects, and at an SNR of +5 dB in seven subjects. The stimuli were presented at 35 dB SL (re: pure tone average) or 115 dB SPL if this limit was less than 35 dB SL. In addition to the onset response, the auditory change complex (ACC) elicited by VOT was recorded in eight subjects. Results: Speech evoked ERPs recorded in all subjects consisted of a vertex positive peak (i.e., P1), followed by a trough occurring approximately 100 ms later (i.e., N2). For results measured in quiet, there was no significant difference in categorical boundaries estimated using ERP measures and behavioral procedures. Categorical boundaries estimated in quiet using both ERP and behavioral measures closely correlated with the most-recently measured Phonetically Balanced Kindergarten (PBK) scores. Adding a competing background noise did not affect categorical boundaries estimated using either behavioral or ERP procedures in three subjects. For the other four subjects, categorical boundaries estimated in noise using behavioral measures were prolonged. However, adding background noise only increased categorical boundaries measured using ERPs in three out of these four subjects. Conclusions: VCV continuum can be used to evaluate behavioral identification and the neural encoding of VOT in children with ANSD. In quiet, categorical boundaries of VOT estimated using behavioral measures and ERP recordings are closely associated with speech recognition performance in children with ANSD. Underlying mechanisms for excessive speech perception deficits in noise may vary for individual patients with ANSD. Junior Faculty Career Development Award from School of Medicine, The University of North Carolina at Chapel Hill; National Institute on Deafness and Other Communication Disorders (NIDCD)United States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute on Deafness & Other Communication Disorders (NIDCD) [R01DC017846] This work was supported by the Junior Faculty Career Development Award from School of Medicine, The University of North Carolina at Chapel Hill, and the R01 grant from National Institute on Deafness and Other Communication Disorders (NIDCD; R01DC017846 for supporting SH's effort on this project and for the publication cost of this article).

This study aimed to (1) investigate the effect of pulse polarity on neural response of the electrically stimulated cochlear nerve in children with cochlear nerve deficiency (CND) and children with normal-sized cochlear nerves and (2) compare the size of the pulse polarity effect between these two subject groups.The experimental and control group included 31 children with CND and 31 children with normal-sized cochlear nerves, respectively. For each study participant, evoked compound action potential (eCAP) input/output (I/O) functions for anodic-leading and cathodic-leading biphasic stimuli were measured at three electrode locations across the electrode array. The dependent variables of interest included the eCAP amplitude measured at the maximum comfortable level of the anodic stimulus, the lowest level that could evoke an eCAP (i.e., the eCAP threshold), the slope of the eCAP I/O function estimated based on linear regression, the negative-peak (i.e., N1) latency of the eCAP, as well as the size of the pulse polarity effect on these eCAP measurements. Generalized linear mixed effect models were used to compare the eCAP amplitude, the eCAP threshold, the slope of the eCAP I/O function, and the N1 latency evoked by the anodic-leading stimulus with those measured for the cathodic-leading stimulus for children with CND and children with normal-sized cochlear nerves. Generalized linear mixed effect models were also used to compare the size of the pulse polarity effect on the eCAP between these two study groups. The one-tailed Spearman correlation test was used to assess the potential correlation between the pulse phase duration and the difference in N1 latency measured for different pulse polarities.Compared with children who had normal-sized cochlear nerves, children with CND had reduced eCAP amplitudes, elevated eCAP thresholds, flatter eCAP I/O functions, and prolonged N1 latencies. The anodic-leading stimulus led to higher eCAP amplitudes, lower eCAP thresholds, and shorter N1 latencies than the cathodic-leading stimulus in both study groups. Steeper eCAP I/O functions were recorded for the anodic-leading stimulus than those measured for the cathodic-leading stimulus in children with CND, but not in children with normal-sized cochlear nerves. Group differences in the size of the pulse polarity effect on the eCAP amplitude, the eCAP threshold, or the N1 latency were not statistically significant.Similar to the normal-sized cochlear nerve, the hypoplastic cochlear nerve is more sensitive to the anodic-leading than to the cathodic-leading stimulus. Results of this study do not provide sufficient evidence for proving the idea that the pulse polarity effect can provide an indication for local neural health.

This study demonstrated the effects of using different quantification methods and parameter scales on the sensitivity of the electrically evoked compound action potential (eCAP) to changes in the interphase gap (IPG). The IPG effect measured in two groups of cochlear implant (CI) users with different cochlear nerve (CN) health on seven eCAP measures was quantified using an absolute and a proportional difference method. The IPG effect provides an indicator for the functional status of the CN in human CI users. Specifying how the IPG effect is quantified is critical for accurate result interpretation.

OBJECTIVES The objectives of this study were to demonstrate the safety of auditory brainstem implant (ABI) surgery and document the subsequent development of auditory and spoken language skills in children without neurofibromatosis type II (NFII). DESIGN A prospective, single-subject observational study of ABI in children without NFII was undertaken at the University of North Carolina at Chapel Hill. Five children were enrolled under an investigational device exemption sponsored by the investigators. Over 3 years, patient demographics, medical/surgical findings, complications, device mapping, electrophysiologic measures, audiologic outcomes, and speech and language measures were collected. RESULTS Five children without NFII have received ABIs to date without permanent medical sequelae, although 2 children required treatment after surgery for temporary complications. All children wear their device daily, and the benefits of sound awareness have developed slowly. Intra-and postoperative electrophysiologic measures augmented surgical placement and device programming. The slow development of audition skills precipitated limited changes in speech production but had little impact on growth in spoken language. CONCLUSIONS ABI surgery is safe in young children without NFII. Benefits from device use develop slowly and include sound awareness and the use of pattern and timing aspects of sound. These skills may augment progress in speech production but progress in language development is dependent upon visual communication. Further monitoring of this cohort is needed to better delineate the benefits of this intervention in this patient population.

Objectives This preliminary study aimed (1) to assess longitudinal changes in electrically evoked auditory event-related potentials (eERPs) in children with auditory brainstem implants (ABIs) and (2) to explore whether these changes could be accounted for by maturation in the central auditory system of these patients. Design Study participants included 5 children (S1 to S5) with an ABI in the affected ear. The stimulus was a train of electrical pulses delivered to individual ABI electrodes via a research interface. For each subject, the eERP was repeatedly measured in multiple test sessions scheduled over up to 41 months after initial device activation. Longitudinal changes in eERPs recorded for each ABI electrode were evaluated using intraclass correlation tests for each subject. Results eERPs recorded in S1 showed notable morphological changes for five ABI electrodes over 41 months. In parallel, signs or symptoms of nonauditory stimulation elicited by these electrodes were observed or reported at 41 months. eERPs could not be observed in S2 after 9 months of ABI use but were recorded at 12 months after initial stimulation. Repeatable eERPs were recorded in S3 in the first 9 months. However, these responses were either absent or showed remarkable morphological changes at 30 months. Longitudinal changes in eERP waveform morphology recorded in S4 and S5 were also observed. Conclusions eERP responses in children with ABIs could change over a long period of time. Maturation of the central auditory system could not fully account for these observed changes. Children with ABIs need to be closely monitored for potential changes in auditory perception and unfavorable nonauditory sensations. Neuroimaging correlates are needed to better understand the emergence of nonauditory stimulation over time in these children.

Objectives This study aimed to compare the effects of increasing the interphase gap (IPG) on the neural response of the electrically stimulated cochlear nerve (CN) between children with CN deficiency (CND) and children with normal-sized CNs. Design Study participants included 30 children with CND and 30 children with normal-sized CNs. All subjects were implanted with a Cochlear Nucleus device with the internal electrode array 24RE[CA] in the test ear. The stimulus was a charge-balanced, cathodic leading, biphasic pulse with a pulse-phase duration of 50 μsec. For each subject, the electrically evoked compound action potential (eCAP) input/output (I/O) function was measured for 6 IPGs (i.e., 7, 14, 21, 28, 35, and 42 μsec) at 3 electrode locations across the electrode array. For each subject and each testing electrode, the highest stimulation used to measure the eCAP I/O function was the maximum comfortable level measured with an IPG of 42 μsec. Dependent variables (DVs) were the maximum eCAP amplitude, the eCAP threshold, and the slope of the eCAP I/O function estimated using both linear and sigmoidal regression functions. For each DV, the size of the IPG effect was defined as the proportional change relative to the result measured for the 7 μsec IPG at the basal electrode location. Generalized linear mixed effect models with subject group, electrode location, and IPG duration as the fixed effects and subject as the random effect were used to compare these DVs and the size of the IPG effect on these DVs. Results Children with CND showed smaller maximum eCAP amplitudes, higher eCAP thresholds, and smaller slopes of eCAP I/O function estimated using either linear or sigmoidal regression function than children with normal-sized CNs. Increasing the IPG duration resulted in larger maximum eCAP amplitudes, lower eCAP thresholds and larger slopes of eCAP I/O function estimated using sigmoidal regression function at all three electrode locations in both study groups. Compared with children with normal-sized CNs, children with CND showed larger IPG effects on both the maximum eCAP amplitude and the slope of the eCAP I/O function estimated using either linear or sigmoidal regression function, and a smaller IPG effect on the eCAP threshold than those measured in children with normal-sized CNs. Conclusions Increasing the IPG increases responsiveness of the electrically stimulated CN in both children with CND and children with normal-sized CNs. The maximum eCAP amplitude and the slope of the eCAP I/O function measured in human listeners with poorer CN survival are more sensitive to changes in the IPG. In contrast, the eCAP threshold in listeners with poorer CN survival is less sensitive to increases in the IPG. Further studies are warranted to identify the best parameters of eCAP results for predicting CN survival before this eCAP testing paradigm can be used as a clinical tool for evaluating neural health for individual cochlear implant patients.

OBJECTIVE: 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 (CI) 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 to 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 intra-operative eCAP recordings due to the extensive testing time required.

Objectives This study aimed to (1) investigate the effect of GJB2 and SLC26A4 gene mutations on auditory nerve function in pediatric cochlear implant users and (2) compare their results with those measured in implanted children with idiopathic hearing loss. Design Participants included 20 children with biallelic GJB2 mutations, 16 children with biallelic SLC26A4 mutations, and 19 children with idiopathic hearing loss. All subjects except for two in the SLC26A4 group had concurrent Mondini malformation and enlarged vestibular aqueduct. All subjects used Cochlear Nucleus devices in their test ears. For each subject, electrophysiological measures of the electrically evoked compound action potential (eCAP) were recorded using both anodic- and cathodic-leading biphasic pulses. Dependent variables (DVs) of interest included slope of eCAP input/output (I/O) function, the eCAP threshold, and eCAP amplitude measured at the maximum comfortable level (C level) of the anodic-leading stimulus (i.e., the anodic C level). Slopes of eCAP I/O functions were estimated using statistical modeling with a linear regression function. These DVs were measured at three electrode locations across the electrode array. Generalized linear mixed effect models were used to evaluate the effects of study group, stimulus polarity, and electrode location on each DV. Results Steeper slopes of eCAP I/O function, lower eCAP thresholds, and larger eCAP amplitude at the anodic C level were measured for the anodic-leading stimulus compared with the cathodic-leading stimulus in all subject groups. Children with GJB2 mutations showed steeper slopes of eCAP I/O function and larger eCAP amplitudes at the anodic C level than children with SLC26A4 mutations and children with idiopathic hearing loss for both the anodic- and cathodic-leading stimuli. In addition, children with GJB2 mutations showed a smaller increase in eCAP amplitude when the stimulus changed from the cathodic-leading pulse to the anodic-leading pulse (i.e., smaller polarity effect) than children with idiopathic hearing loss. There was no statistically significant difference in slope of eCAP I/O function, eCAP amplitude at the anodic C level, or the size of polarity effect on all three DVs between children with SLC26A4 mutations and children with idiopathic hearing loss. These results suggested that better auditory nerve function was associated with GJB2 but not with SLC26A4 mutations when compared with idiopathic hearing loss. In addition, significant effects of electrode location were observed for slope of eCAP I/O function and the eCAP threshold. Conclusions GJB2 and SLC26A4 gene mutations did not alter polarity sensitivity of auditory nerve fibers to electrical stimulation. The anodic-leading stimulus was generally more effective in activating auditory nerve fibers than the cathodic-leading stimulus, despite the presence of GJB2 or SLC26A4 mutations. Patients with GJB2 mutations appeared to have better functional status of the auditory nerve than patients with SLC26A4 mutations who had concurrent Mondini malformation and enlarged vestibular aqueduct and patients with idiopathic hearing loss.

Emerging Issues in Cochlear ImplantationThe American Cochlear Implant Alliance (ACI Alliance) was incorporated in the late 2011 as a non-profit organization of cochlear implant clinicians from acro...

This study aimed to (1) characterize temporal response properties of the auditory nerve in implanted children with auditory neuropathy spectrum disorder (ANSD), and (2) compare results recorded in implanted children with ANSD with those measured in implanted children with sensorineural hearing loss (SNHL). Participants included 28 children with ANSD and 29 children with SNHL. All subjects used cochlear nucleus devices in their test ears. Both ears were tested in 6 children with ANSD and 3 children with SNHL. For all other subjects, only one ear was tested. The electrically evoked compound action potential (ECAP) was measured in response to each of the 33 pulses in a pulse train (excluding the second pulse) for one apical, one middle-array, and one basal electrode. The pulse train was presented in a monopolar-coupled stimulation mode at 4 pulse rates: 500, 900, 1800, and 2400 pulses per second. Response metrics included the averaged amplitude, latencies of response components and response width, the alternating depth and the amount of neural adaptation. These dependent variables were quantified based on the last six ECAPs or the six ECAPs occurring within a time window centered around 11 to 12 msec. A generalized linear mixed model was used to compare these dependent variables between the 2 subject groups. The slope of the linear fit of the normalized ECAP amplitudes (re. amplitude of the first ECAP response) over the duration of the pulse train was used to quantify the amount of ECAP increment over time for a subgroup of 9 subjects. Pulse train-evoked ECAPs were measured in all but 8 subjects (5 with ANSD and 3 with SNHL). ECAPs measured in children with ANSD had smaller amplitude, longer averaged P2 latency and greater response width than children with SNHL. However, differences in these two groups were only observed for some electrodes. No differences in averaged N1 latency or in the alternating depth were observed between children with ANSD and children with SNHL. Neural adaptation measured in these 2 subject groups was comparable for relatively short durations of stimulation (i.e., 11 to 12 msec). Children with ANSD showed greater neural adaptation than children with SNHL for a longer duration of stimulation. Amplitudes of ECAP responses rapidly declined within the first few milliseconds of stimulation, followed by a gradual decline up to 64 msec after stimulus onset in the majority of subjects. This decline exhibited an alternating pattern at some pulse rates. Further increases in pulse rate diminished this alternating pattern. In contrast, ECAPs recorded from at least one stimulating electrode in six ears with ANSD and three ears with SNHL showed a clear increase in amplitude over the time course of stimulation. The slope of linear regression functions measured in these subjects was significantly greater than zero. Some but not all aspects of temporal response properties of the auditory nerve measured in this study differ between implanted children with ANSD and implanted children with SNHL. These differences are observed for some but not all electrodes. A new neural response pattern is identified. Further studies investigating its underlying mechanism and clinical relevance are warranted.

Objectives This study aimed to (1) establish the feasibility of measuring the electrically evoked auditory change complex (eACC) in response to temporal gaps in children with cochlear nerve deficiency (CND) who are using cochlear implants (CIs) and/or auditory brainstem implants (ABIs); and (2) explore the association between neural encoding of, and perceptual sensitivity to, temporal gaps in these patients. Design Study participants included 5 children (S1 to S5) ranging in age from 3.8 to 8.2 years (mean: 6.3 years) at the time of testing. All subjects were unilaterally implanted with a Nucleus 24M ABI due to CND. For each subject, two or more stimulating electrodes of the ABI were tested. S2, S3, and S5 previously received a CI in the contralateral ear. For these 3 subjects, at least two stimulating electrodes of their CIs were also tested. For electrophysiological measures, the stimulus was an 800-msec biphasic pulse train delivered to individual electrodes at the maximum comfortable level (C level). The electrically evoked responses, including the onset response and the eACC, were measured for two stimulation conditions. In the standard condition, the 800-msec pulse train was delivered uninterrupted to individual stimulating electrodes. In the gapped condition, a temporal gap was inserted into the pulse train after 400 msec of stimulation. Gap durations tested in this study ranged from 2 up to 128 msec. The shortest gap that could reliably evoke the eACC was defined as the objective gap detection threshold (GDT). For behavioral GDT measures, the stimulus was a 500-msec biphasic pulse train presented at the C level. The behavioral GDT was measured for individual stimulating electrodes using a one-interval, two-alternative forced-choice procedure. Results The eACCs to temporal gaps were recorded successfully in all subjects for at least one stimulating electrode using either the ABI or the CI. Objective GDTs showed intersubject variations, as well as variations across stimulating electrodes of the ABI or the CI within each subject. Behavioral GDTs were measured for one ABI electrode in S2 and for multiple ABI and CI electrodes in S5. All other subjects could not complete the task. S5 showed smaller behavioral GDTs for CI electrodes than those measured for ABI electrodes. One CI and two ABI electrodes in S5 showed comparable objective and behavioral GDTs. In contrast, one CI and two ABI electrodes in S5 and one ABI electrode in S2 showed measurable behavioral GDTs but no identifiable eACCs. Conclusions The eACCs to temporal gaps were recorded in children with CND using either ABIs or CIs. Both objective and behavioral GDTs showed inter- and intrasubject variations. Consistency between results of eACC recordings and psychophysical measures of GDT was observed for some but not all ABI or CI electrodes in these subjects.

Objectives This study aimed to (1) investigate the responsiveness of the cochlear nerve (CN) to a single biphasic-electrical pulse in implanted children with cochlear nerve deficiency (CND) and (2) compare their results with those measured in implanted children with normal-size CNs. Design Participants included 23 children with CND (CND1 to CND23) and 18 children with normal-size CNs (S1 to S18). All subjects except for CND1 used Cochlear Nucleus cochlear implants with contour electrode arrays in their test ears. CND1 was implanted with a Cochlear Nucleus Freedom cochlear implant with a straight electrode array in the test ear. For each subject, the CN input/output (I/O) function and the refractory recovery function were measured using electrophysiological measures of the electrically evoked compound action potential (eCAP) at multiple electrode sites across the electrode array. Dependent variables included eCAP threshold, the maximum eCAP amplitude, slope of the I/O function, and time-constants of the refractory recovery function. Slopes of I/O functions were estimated using statistical modeling with a sigmoidal function. Recovery time-constants, including measures of the absolute refractory period and the relative refractory period, were estimated using statistical modeling with an exponential decay function. Generalized linear mixed-effect models were used to evaluate the effects of electrode site on the dependent variables measured in children with CND and to compare results of these dependent variables between subject groups. Results The eCAP was recorded at all test electrodes in children with normal-size CNs. In contrast, the eCAP could not be recorded at any electrode site in 4 children with CND. For all other children with CND, the percentage of electrodes with measurable eCAPs decreased as the stimulating site moved in a basal-to-apical direction. For children with CND, the stimulating site had a significant effect on the slope of the I/O functions and the relative refractory period but showed no significant effect on eCAP threshold and the maximum eCAP amplitude. Children with CND had significantly higher eCAP thresholds, smaller maximum eCAP amplitudes, flatter slopes of I/O functions, and longer absolute refractory periods than children with normal-size CNs. There was no significant difference in the relative refractory period measured in these two subject groups. Conclusions In children with CND, the functional status of the CN varied along the length of the cochlea. Compared with children with normal-size CNs, children with CND showed reduced CN responsiveness to electrical stimuli. The prolonged CN absolute refractory period in children with CND might account for, at least partially, the observed benefit of using relatively slow pulse rate in these patients.

The electrically evoked compound action potential (eCAP) represents the synchronous firing of a population of electrically stimulated auditory nerve fibers. It can be directly recorded on a surgically exposed nerve trunk in animals or from an intra-cochlear electrode of a cochlear implant. In the past two decades, the eCAP has been widely recorded in both animals and clinical patient populations using different testing paradigms. This paper provides an overview of recording methodologies and response characteristics of the eCAP, as well as its potential applications in research and clinical situations. Relevant studies are reviewed and implications for clinicians are discussed.

Several lines of evidence indicate that auditory temporal resolution improves over childhood, whereas other data implicate the development of processing efficiency. The present study used the masking period pattern paradigm to examine the maturation of temporal processing in normal-hearing children (4.8 to 10.7 yrs) compared to adults. Thresholds for a brief tone were measured at 6 temporal positions relative to the period of a 5-Hz quasi-square-wave masker envelope, with a 20-dB modulation depth, as well as in 2 steady maskers. The signal was a pure tone at either 1000 or 6500 Hz, and the masker was a band of noise, either spectrally wide or narrow (21.3 and 1.4 equivalent rectangular bandwidths, respectively). Masker modulation improved thresholds more for wide than narrow bandwidths, and adults tended to receive more benefit from modulation than young children. Fits to data for the wide maskers indicated a change in window symmetry with development, reflecting relatively greater backward masking for the youngest listeners. Data for children >6.5 yrs of age appeared more adult-like for the 6500- than the 1000-Hz signal. Differences in temporal window asymmetry with listener age cannot be entirely explained as a consequence of a higher criterion for detection in children, a form of inefficiency.

This study aimed to (1) characterize morphological characteristics of the electrically evoked cortical auditory event-related potentials (eERPs) and explore the potential association between onset eERP morphology and auditory versus nonauditory stimulation; (2) assess test–retest reliability of onset eERPs; (3) investigate effects of stimulation level on onset eERPs; and (4) explore the feasibility of using the onset eERP to estimate the lowest stimulation level that can be detected for individual stimulating electrodes in patients with auditory brainstem implants (ABIs). Study participants included 5 children (S1 to S5) and 2 adults (S6 to S7) with unilateral Cochlear Nucleus 24M ABIs. Pediatric ABI recipients ranged in age from 2.6 to 10.2 years (mean: 5.2 years) at the time of testing. S6 and S7 were 21.2 and 24.6 years of age at the time of testing, respectively. S6 and S7 were diagnosed with neurofibromatosis II (NF2) and implanted with an ABI after a surgical removal of the tumors. All pediatric subjects received ABIs after being diagnosed with cochlear nerve deficiency. The lowest stimulation level that could be detected (behavioral T level) and the estimated maximum comfortable level (C level) was measured for individual electrodes using clinical procedures. For electrophysiological measures, the stimulus was a 100-msec biphasic pulse train that was delivered to individual electrodes in a monopolar-coupled stimulation mode at stimulation levels ranging from subthreshold to C levels. Electrophysiological recordings of the onset eERP were obtained in all subjects. For studies evaluating the test–retest reliability of the onset eERP, responses were measured using the same set of parameters in two test sessions. The time interval between test sessions ranged from 2 to 6 months. The lowest stimulation level that could evoke the onset eERP was defined as the objective T level. Onset eERPs were recorded in all subjects tested in this study. Inter- and intrasubject variations in morphological characteristics of onset eERPs were observed. Onset eERPs with complex waveforms were recorded for electrodes that evoked nonauditory sensations, based on feedback from subjects, as well as for electrodes without any indications of nonauditory stimulations. Onset eERPs in patients with ABIs demonstrated good test–retest reliability. Increasing stimulation levels resulted in increased eERP amplitudes but showed inconsistent effects on response latencies in patients with ABIs. Objective and behavioral T levels were correlated. eERPs could be recorded in both non-NF2 and NF2 patients with ABIs. eERPs in both ABI patient groups show inter- and intrasubject variations in morphological characteristics. However, onset eERPs measured within the same subject in this study tended to be stable across study sessions. The onset eERP can potentially be used to estimate behavioral T levels in patients with ABIs. Further studies with more adult ABI recipients are warranted to investigate whether the onset eERP can be used to identify electrodes with nonauditory stimulations.

The purpose of this study was to investigate the relationship between electrophysiologic measures of the binaural interaction component (BIC) of the electrically evoked auditory brainstem response and psychophysical measures of interaural pitch comparisons in Nucleus bilateral cochlear implant users.: Data were collected for 10 postlingually deafened adult cochlear implant users. Each subject conducted an interaural pitch-comparison task using a biphasic pulse train with a pulse rate of 1000 pulses per second (pps) at high stimulation levels. Stimuli were presented in a two-interval, two-alternative forced-choice procedure with roving current variations. A subgroup of four subjects repeated the task at low stimulation levels. BICs were measured using loudness balanced, biphasic current pulses presented at a rate of 19.9 pps for each subject by pairing the electrode 12 (out of 22 intracochlear electrodes) in the right ear with each of 11 electrodes spaced across the electrode array in the left ear. The BIC was measured at high stimulation levels in 10 subjects and at low stimulation levels in 7 subjects. Because of differences in stimulation rate used in BIC measures and interaural pitch comparisons, the actual stimulation levels were different in these two measures. The relationship between BIC responses and results of interaural pitch comparisons was evaluated for each of the individual subjects and at the group level. Evaluation was carried out separately for results obtained at high and low stimulation levels.: There was no significant correlation between results of BIC measures and interaural pitch comparisons on either the individual or group levels. Lower stimulation level did not improve the relationship between these two measures.: No significant correlations between psychophysical measures of interaural pitch comparisons and electrophysiologic measures of the BIC of the electrically evoked auditory brainstem response were found. The lack of correlation may be attributed to methods used to quantify the data, small number of subjects retested at low stimulation levels, and central processing components involved in the interaural pitch-comparison task.

Background Adults with single sided deafness (SSD) have lost binaural function, which limits sound source localization, speech understanding in noise, and quality of life. For SSD patients, restoration of bilateral auditory input is possible only with a cochlear implant (CI). In this study, cortical auditory evoked potentials (CAEPs) and behavioral performance were measured in left-implanted (SSD-CI-L) and right-implanted (SSD-CI-R) patients before and after cochlear implantation. We hypothesized that improvements in behavioral performance would be accompanied by changes in CAEPs after cochlear implantation. Design Prospective longitudinal study. Setting Tertiary referral center. Method Nine right-handed adult SSD CI patients participated in the study. CAEPs were recorded before cochlear implantation and at 6 and 12 months post-implantation. CAEPs were elicited using speech stimuli (/ba/) delivered in sound field at 70 dBA. Global field power (GFP) latency and amplitude were calculated for P1, N1 and P2 peaks at each test session. CAEP were analyzed at frontocentral (Cz) and temporal (P7, P8, T7 and T8) and mastoid electrodes (M1 and M2) contralateral to the CI ear. Behavioral measures (sentence recognition in noise, with and without spatial cues) were collected at the same test sessions as for CAEPs. Speech performance and CAEPs were also measured in a control group of normal-hearing (NH) subjects. Results While increased N1 amplitude was observed in the scalp potential maps for GFP and Cz for SSD-CI-L patients after implantation, the changes were not statistically significant. Peak CAEP amplitude at electrodes to contralateral to the CI ear increased after cochlear implantation for all SSD-CI patients, but significant increases were observed only for mastoid sites. Peak latencies for some components at temporal and mastoid sites remained significantly longer than for the NH control group, even after cochlear implantation. For SSD-CI-R patients, P2 peak amplitude for baseline GFP and Cz was significantly lower than for the NH control group. A significant improvement for speech understanding in noise was observed at 12 months post-implantation when speech was presented to the CI ear and noise to the non-implanted ear. Conclusion After cochlear implantation, speech understanding significantly improved when speech and noise were spatially separated. The increased N1 amplitude for SSD-CI-L patients and the increased bilateral activation for all SSD-CI patients may reflect cortical reorganization and restoration of binaural function after one year of experience with the CI. However, because of the limited number of SSD patients, significant changes in cortical activity after cochlear implantation were often difficult to observe.