A Predictive Model for Cochlear Implant Outcome in Children with Cochlear Nerve Deficiency.
Ontology highlight
ABSTRACT:
SUBMITTER: Han JJ
PROVIDER: S-EPMC6362156 | biostudies-literature | 2019 Feb
REPOSITORIES: biostudies-literature
Publications
A Predictive Model for Cochlear Implant Outcome in Children with Cochlear Nerve Deficiency.
Han Jae Joon JJ Suh Myung-Whan MW Park Moo Kyun MK Koo Ja-Won JW Lee Jun Ho JH Oh Seung Ha SH
Scientific reports 20190204 1
The outcome of cochlear implantation (CI) in patients with cochlear nerve deficiency (CND) is variable, resulting in a wide range of speech perception performance, from degrees of environmental sound perception to conversation without lip-reading. Twenty-five cochlear implantees with CND were enrolled retrospectively to determine the factors correlated with CI outcome in patients with CND and to develop a predictive model for CI outcome. CI outcome was evaluated using the Categories of Auditory ...[more]
Similar Datasets
Project description: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.
| S-EPMC5748379 | biostudies-literature
Project description:ObjectivesThis 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.DesignStudy 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.ResultsChildren 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.ConclusionsIncreasing 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.
| S-EPMC7211427 | biostudies-literature
Project description:This article summarizes the available evidence on pediatric cochlear implantation to provide current guidelines for clinical protocols and candidacy recommendations in the United States. Candidacy determination involves specification of audiologic and medical criteria per guidelines of the Food and Drug Administration. However, recommendations for a cochlear implant evaluation also should maintain flexibility and consider a child's skill progression (i.e., month-for-month progress in speech, language, and auditory development) and quality of life with appropriately fit hearing aids. Moreover, evidence supports medical and clinical decisions based on other factors, including (a) ear-specific performance, which affords inclusion of children with asymmetric hearing loss and single-sided deafness as implant candidates; (b) ear-specific residual hearing, which influences surgical technique and device selection to optimize hearing; and (c) early intervention to minimize negative long-term effects on communication and quality of life related to delayed identification of implant candidacy, later age at implantation, and/or limited commitment to an audiologic rehabilitation program. These evidence-based guidelines for current clinical protocols in determining pediatric cochlear implant candidacy encourage a team-based approach focused on the whole child and the family system.
| S-EPMC8862774 | biostudies-literature
Project description:To determine the rate of long-term cochlear implant (CI) use in children.Consecutive case series.Tertiary referral center.Approximately 474 patients younger than 18 years who received a first CI from 1999 to 2011.Cochlear implantation.Regular CI use, defined as using the CI for 8 hours or greater per day.We successfully contacted and obtained follow-up data on 402 patients (85%) via email, telephone, and postal survey. The rate of regular CI use was 93.2% (95% CI, 90.0-95.4) at 5 years postimplantation and 87.7% (95% CI, 82.9-91.3) at 10 years postimplantation. The mean number of hours of use per day was 12.0 hours (SD, 4.1 h). Cox proportional hazard regression analysis demonstrated a linear association between the age at implantation and the risk of discontinuing regular CI use. Rates of CI discontinuation increased by 18.2% per year of age at implantation (95% CI, 7.2%-30.4%). Reported reasons for CI use less than 8 hours per day include poor hearing benefit (53.2%), social pressure (21.3%), and recurrent displacement of the transmitter coil (17.0%).High rates of regular CI use are sustained after childhood implantation, and younger age at implantation is associated with a higher rate of continued device usage.
| S-EPMC3927162 | biostudies-literature
Project description:Spectral ripple discrimination (SRD) has been widely used to evaluate the spectral resolution in cochlear implant (CI) recipients based on its strong correlation with speech perception performance. However, despite its usefulness for predicting speech perception outcomes, SRD performance exhibits large across-subject variabilities even among subjects implanted with the same CIs and sound processors. The potential factors of this observation include current spread, nerve survival, and CI mapping. Previous studies have found that the spectral resolution reduces with increasing distance of the stimulation electrode from the auditory nerve fibers (ANFs), attributable to increasing current spread. However, it remains unclear whether the spread of excitation is the only cause of the observation, or whether other factors such as temporal interaction also contribute to it. In this study, we used a computational model to investigate channel interaction upon non-simultaneous stimulation with respect to the electrode-ANF distance, and evaluated the SRD performance for five electrode-ANF distances. The SRD performance was determined based on the similarity between two neurograms in response to standard and inverted stimuli and used to evaluate the spectral resolution in the computational model. The spread of excitation was observed to increase with increasing electrode-ANF distance, consistent with previous findings. Additionally, the preceding pulses delivered from neighboring channels induced a channel interaction that either inhibited or facilitated the neural responses to subsequent pulses depending on the electrode-ANF distance. The SRD performance was also found to decrease with increasing electrode-ANF distance. The findings of this study suggest that variation of the neural responses (inhibition or facilitation) with the electrode-ANF distance in CI users may cause spectral smearing, and hence poor spectral resolution. A computational model such as that used in this study is a useful tool for understanding the neural factors related to CI outcomes, such as cannot be accomplished by behavioral studies alone.
| S-EPMC7398541 | biostudies-literature
Project description:The use and utility of cochlear implantation has rapidly increased in recent years as technological advances in the field have expanded both the efficacy and eligible patient population for implantation. This review aims to serve as a general overview of the most common hearing disorders that have favorable auditory outcomes with cochlear implants (CI). Hearing loss in children caused by congenital cytomegalovirus infection, syndromic conditions including Pendred Syndrome, and non-syndromic genetic conditions such as hearing impairment associated with GJB2 mutations have shown to be successfully managed by CI. Furthermore, cochlear implantation provides the auditory rehabilitation for the most common etiology of hearing loss in adults and age-related hearing loss (ARHL) or presbycusis. However, in some cases, cochlear implantation have been associated with some challenges. Regarding implantation in children, studies have shown that sometimes parents seem to have unrealistic expectations regarding the ability of CI to provide auditory rehabilitation and speech improvement. Given the evidence revealing the beneficial effects of early intervention via CI in individuals with hearing disorders especially hearing loss due to genetic etiology, early auditory and genetic screening efforts may yield better clinical outcomes. There is a need to better understand genotype-phenotype correlations and CI outcome, so that effective genetic counseling and successful treatment strategies can be developed at the appropriate time for hearing impaired individuals.
| S-EPMC7381205 | biostudies-literature
Project description:OBJECTIVE:This study investigated the speech perception of cochlear implant (CI) recipients with measurable preoperative ipsilateral speech perception. These data should support improved individual counselling of CI candidates. MATERIALS AND METHODS:Pre- and postoperative speech audiometric parameters were analyzed, including maximum score for phonemically balanced words (PBmax) and monosyllabic score at a normal conversational level of 65 dBSPL, with hearing aids one hand and CI on the other. Data of 284 experienced adult CI wearers were grouped and evaluated in terms of preoperative PBmax. RESULTS:The preoperative PBmax was exceeded by the postoperative monosyllabic score in 96% of cases. The overall median postoperative score was 72.5%. The groups with preoperative PBmax > 0% showed significantly better speech perception scores with CI than the group with PBmax = 0%. Median improvement compared to the preoperative monosyllabic score with hearing aids was 65 percentage points, independent of preoperative PBmax. CONCLUSION:The preoperatively measured PBmax may be used as a predictor for the minimum speech perception obtained with CI. This is of high clinical relevance for CI candidates with a PBmax above zero.
| S-EPMC6565665 | biostudies-other
Project description:ObjectivesThis study aimed to create an objective predictive model for assessing the functional status of the cochlear nerve (CN) in individual cochlear implant (CI) users.DesignStudy 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.ResultsAll 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.ConclusionsThe 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.
| S-EPMC8156737 | biostudies-literature
Project description:The use of neonatal hearing screening has enabled the identification of congenital unilateral sensorineural hearing loss (USNHL) immediately after birth, and today there are several intervention options available to minimize potential adverse effects of this disease, including cochlear implantation. This study aims to analyze the characteristics of the inner ear of a homogeneous group of congenital non-syndromic USNHL to highlight the features of the inner ear, which can help in clinical, surgical, and rehabilitative decision-making. A retrospective chart review was carried out at a tertiary referral center. Systematic diagnostic work-up and rigorous inclusion-exclusion criteria were applied to 126 children with unilateral hearing impairment, leading to a selection of 39 strictly congenital and non-syndromic USNHL cases, undergoing computed tomography (CT) and magnetic resonance (MR) imaging studies. The frequency and type of malformations of the inner ear in USNHL and unaffected contralateral ears were assessed, with an in-depth analysis of the deficiency of the cochlear nerve (CND), the internal auditory canal (IAC) and the cochlear aperture (CA). Inner ear anomalies were found in 18 out of 39 (46%) of the USNHL patients. In 1 subject, the anomalies were bilateral, and the CND resulted in the predominant identified defect (78% of our abnormal case series), frequently associated with CA stenosis. Only 3 out of 14 children with CND presented stenosis of the IAC. CND and CA stenosis (and to a much lesser extent IAC stenosis) are a frequent association within congenital and non-syndromic USNHL that could represent a distinct pathological entity affecting otherwise healthy infants. In the context of a diagnostic work-up, the evaluation with CT and MRI measurements should take place in a shared decision-making setting with thorough counseling. Both imaging techniques have proven useful in differentiating the cases that will most likely benefit from the cochlear implant, from those with potentially poor implant performance.
| S-EPMC7870947 | biostudies-literature
Project description:IntroductionDespite recent advancement in the prediction of cochlear implant outcome, the benefit of bilateral procedures compared to bimodal stimulation and how we predict speech perception outcomes of sequential bilateral cochlear implant based on bimodal auditory performance in children remain unclear.ObjectivesThis investigation was performed: (1) to determine the benefit of sequential bilateral cochlear implant and (2) to identify the associated factors for the outcome of sequential bilateral cochlear implant.MethodsObservational and retrospective study. We retrospectively analyzed 29 patients with sequential cochlear implant following bimodal-fitting condition. Audiological evaluations were performed; the categories of auditory performance scores, speech perception with monosyllable and disyllables words, and the Korean version of Ling. Audiological evaluations were performed before sequential cochlear implant with the bimodal fitting condition (CI1+HA) and one year after the sequential cochlear implant with bilateral cochlear implant condition (CI1+CI2). The good performance group (GP) was defined as follows; 90% or higher in monosyllable and bisyllable tests with auditory-only condition or 20% or higher improvement of the scores with CI1+CI2. Age at first implantation, inter-implant interval, categories of auditory performance score, and various comorbidities were analyzed by logistic regression analysis.ResultsCompared to the CI1+HA, CI1+CI2 provided significant benefit in categories of auditory performance, speech perception, and Korean version of Ling results. Preoperative categories of auditory performance scores were the only associated factor for being GP (odds ratio=4.38, 95% confidence interval - 95%=1.07-17.93, p=0.04).ConclusionsThe children with limited language development in bimodal condition should be considered as the sequential bilateral cochlear implant and preoperative categories of auditory performance score could be used as the predictor in speech perception after sequential cochlear implant.
| S-EPMC9443016 | biostudies-literature