Pediatric Cochlear Implantation Research Articles

Social Determinants of Health and Language and Academic Outcomes in Pediatric Cochlear Implantation: A Systematic Review and Meta-Analysis.

Cochlear implants can restore sound and enable speech and language development for children with severe to profound sensorineural hearing loss. Long-term outcomes of pediatric cochlear implant recipients are variable. Although the association between social determinants of health (SDH) and pediatric cochlear implant outcomes has been explored, the strength of this association has not been quantitatively synthesized in the literature. To determine the association of SDH with language and academic outcomes in pediatric cochlear implant recipients. In August 2023, the following databases were searched: Embase.com, Ovid MEDLINE, Scopus, Cochrane Central Register of Controlled Trials, the Cochrane Database of Systematic Reviews, Cumulated Index to Nursing and Allied Health Literature Plus, APA PsycINFO, and ClinicalTrials.gov. Following duplicate exclusion of 8687 results, 5326 records were finalized. Abstract screening, full-text review, and risk of bias assessment was performed by 1 to 2 reviewers. Articles were included if an effect size for an SDH variable that was associated with measures of language, communication, reading, academics, and quality of life was reported. A random-effects meta-analysis was performed, with standardized regression coefficients measuring the relative direction and magnitude of a variable association with the outcome of interest. Of 5326 articles, 40 articles that included a total of 3809 children were included in the systematic review; 20 articles that included a total of 1905 children were included in the meta-analysis. Parental involvement, education level, and low socioeconomic status were moderately to strongly associated with language outcomes (β = 0.30; 95% CI, 0.13-0.48; β = 0.45; 95% CI, 0.29-0.62; β = -0.47; 95% CI, -0.83 to -0.10, respectively). Known determinants of language outcomes, such as the age of cochlear implantation and duration of cochlear implant use, demonstrated moderate to no associations with language outcomes (β = -0.30; 95% CI, -0.43 to -0.17; β = 0.19; 95% CI, -0.26 to 0.63, respectively). The results of this systematic review and meta-analysis suggest that SDH are associated with childhood language development and academic achievement. In addition to efforts to expedite cochlear implant placement in eligible children, optimal outcomes may be achieved with interventions centered on the child's home, primary medical care, and school environment.

Postoperative Auditory Progress in Cochlear-Implanted Children With Auditory Neuropathy.

This study aims to evaluate the effect of auditory neuropathy spectrum disorder (ANSD) on postoperative auditory perception and listening difficulties in pediatric cochlear implant (CI) recipients. The Children's Auditory Perception Test (CAPT) assesses auditory perception skills, and the Children's Home Inventory of Listening Difficulties (CHILD) Scale evaluates daily listening difficulties. The study involved pediatric CI recipients (n = 40) aged between 5 and 7 years, with and without diagnosis of ANSD. The research ensured homogeneity across various factors, including chronological age, age at diagnosis, age at initial implantation, bilateral simultaneous surgery, etiologies of hearing loss, and family education level. The findings have demonstrated that children without ANSD exhibited better performance in integrating visual-auditory stimuli and overall listening performance, distant sound source scores, and noisy environment scores (respectively p = .047, p = .001, p = .028, and p = .010). Additionally, children with better speech perception also have a better ability to integrate audiovisual stimuli (p = .005, r = .438). There are significant differences in postoperative listening skills and auditory perceptions between children with and without an ANSD who have CIs. Accordingly, children without an ANSD perform better.

Management of Massive Iatrogenic Temporal Lobe Meningoencephalocele and Bacterial Meningitis Following Pediatric Cochlear Implant Surgery.

To review the management of meningitis, large lateral skull base defect, and meningoencephalocele following pediatric cochlear implant (CI) surgery. Case report. Patient demographics, medical and surgical history, computed tomography (CT). An 8-year-old male with congenital severe to profound sensorineural hearing loss underwent uncomplicated right CI surgery at 18 months of age. He returned to his home country and received a bilateral sequential left ear CI at age 2. Five years following his second CI surgery, he presented to his local emergency room with worsening fever, headache, back pain, and vomiting. Lumbar puncture confirmed bacterial meningitis, and he was managed with intravenous antibiotics. The hospital course was complicated by a right-sided facial nerve paresis, prompting a temporal bone CT. Imaging demonstrated a tegmen defect measuring 15 mm × 10 mm with soft tissue opacification involving the entire mastoid and CI harness. He was then referred by family back to our institution. Intraoperatively, we identified a massive temporal lobe meningoencephalocele involving the entire mastoid cavity and engulfing the entire proximal electrode harness. Following middle cranial fossa craniotomy and revision mastoidectomy, proximal and distal array were separated, encephalocele transected, and remaining temporal lobe reduced. The lateral skull base was reconstructed with calvarial bone, temporalis fascia, and bovine collagen graft. The remnant electrode array was removed, a new CI receiver stimulator was fixed postero-superiorly to the cranial defect, and a new multichannel array inserted. A titanium cranioplasty completed the repair. A lumbar drain was placed for 3 days. Recovery and CI activation occurred uneventfully. Risk of encephalocele and meningitis underscores the importance of early identification, adequate repair, and long-term radiologic follow-up of iatrogenic tegmen defects during pediatric CI surgery.

Some Challenging Questions About Outcomes in Children With Cochlear Implants

Purpose: Cochlear implants (CIs) have improved the quality of life for many children with severe-to-profound sensorineural hearing loss. Despite the reported CI benefits of improved speech recognition, speech intelligibility, and spoken language processing, large individual differences in speech and language outcomes are still consistently reported in the literature. The enormous variability in CI outcomes has made it challenging to predict which children may be at high risk for limited benefits and how potential risk factors can be improved with interventions. In this article, we consider four challenging, unresolved issues that exist in the field of pediatric cochlear implantation (age at implantation, comorbid diagnoses, preimplant functioning in infants, and communication modality) to illustrate the value of adopting a broader integrated multidisciplinary approach to explaining variability and individual differences in outcomes. Conclusions: Four challenging and unresolved issues concerning our ability to predict individual differences before implantation were discussed. In order to address some of the current barriers to progress in dealing with these unresolved issues, a biopsychosocial system model is proposed as a possible unifying approach to conceptualizing these issues. Additionally, several new research directions to better understand and explain variability in pediatric CI outcomes are suggested.

Associated factors and outcomes for quality of life in children receiving cochlear implantation before seven years of age.

To evaluate the relationship of cochlear implant-related factors with quality of life (QOL) outcomes in pediatric cochlear implantation (CI) recipients. In this cross-sectional study, data from 146 children who received CI before 7 years of age were collected. QOL was measured using the Children using Hearing Implants Quality of Life (CuHI-QoL) questionnaire. Auditory and language abilities were measured using categories of auditory performance II(CAP-II) scale and speech intelligibility rating (SIR) scale. The reliability and validity of the CuHI-QOL scale were tested using internal consistency tests and correlation analysis, respectively. Bivariate correlations were used to compare CuHI-QOL scores and educational placements to cochlear implant-related factors. QOL scores were further compared using ANOVA in different groups based on age at CI with different durations of implant use. The mean total CuHI-QOL scores was 60.13 (SD 8.97). The Cronbach's alpha of overall CuHI-QOL scale was 0.820. The CuHI-QOL total score was strongly to moderately correlated with CAP score (r = 0.542), SIR score (r = 0.545), duration of implant use (r = 0.403), and educational placement (r = 0.478). ANOVA showed the CuHI-QOL scores after 5 years post-CI were higher than those less than 2 years post-CI in children implanted ≤ 3 years of age. Good QOL could be obtained for children with CI and were significantly associated with young age at implantation, good auditory and speech abilities, speech rehabilitation training pre-CI, long duration of cochlear implant use, and mainstream educational placement.

Inclusive Soundscapes: How Race, Socioeconomic Status and Maternal Age Influence the Pediatric Cochlear Implant Journey.

This study aimed to assess how race, social vulnerability, and maternal age influence pediatric cochlear implant access and usage. Retrospective cohort. Tertiary Pediatric University Hospital. This study included individuals aged 0 to 18 who received a cochlear implant at our center between the years 2000 and 2022. Social vulnerability data from 2020 was obtained from the Centers for Disease Control and Prevention. Of the 302 patients included in our study, 43% were black and 50% were white. Patients from the highest to lowest social vulnerability quintiles comprised 31%, 25%, 18%, 10%, and 14% of our sample, respectively. Race was associated with social vulnerability index (SVI) (P < .001), with a mean score of 0.70 (±0.26) and 0.49 (±0.27) for black and white patients, respectively. Later age at hearing loss (HL) diagnosis and cochlear implantation (CI) were associated with more and most vulnerable SVI (P < .05). Delayed diagnosis was also associated with black and other racial groups (P = .041), and adolescent maternal age (P = .03). Greater SVI was associated with less daily cochlear implant usage (P = .004). The most vulnerable patients were more likely to be lost to follow-up (P = .03) despite no difference based on maternal age (P = .59) and insurance status (P = .47). This study underscores the significance of mitigating disparities in timely diagnosis of HL, consistent CI usage, and appropriate follow-up care. This is a first step toward the formulation of novel strategies aimed at overcoming barriers and developing appropriate intervention programs.

New Frontiers in Pediatric Cochlear Implant Surgery - A Single Center Experience with the 3-Dimensional Exoscope.

Since its introduction by Wullstein, the binocular surgical microscope has remained the gold standard of visualization in the field of otology. However, in the last decade, new technology became available in the form of the three-dimensional (3D) exoscope. In this article, we describe our experience thus far in pediatric cochlear implantation with the 3D exoscope. This article is about prospective descriptive study of all exoscopic cochlear implant (CI) cases in a quaternary pediatric CI center performed with the Vitom® 3D system. All pediatric patients (age <18) were included without exclusion criteria, and our experience and conversion to microscope rates are reported. Since the introduction of the exoscope to our unit, we have successfully performed 68 cases, of which 53 were bilateral cochlear implantations. The age of the patient varied between 10 months and 209 months (average: 64 months; median 46.5 months). There were a total of 121 implantations (96 primary implantations, 24 revision implantations). There were 2 conversions to the traditional microscopic technique. The exoscope provides a 3D high-definition (4K) images in CI surgery. There is limited data regarding its use, and we identified a number of advantages including efficiency, occupational health, theater utilization, surgical training, and safety. Although there are areas still for improvement, such as loss of signal-to-noise ratio at higher magnification, manual focus, and lack of electromagnetic articulation in the holding arm, we have found it to be a useful addition to the surgical armamentarium within pediatric cochlear implantation.

EVALUATION OF REMOTE CHECK IN CHILDREN WITH COCHLEAR IMPLANTS

ObjectiveThis study evaluates the feasibility of reducing in-person appointments by utilizing Remote Check (RC) in pediatric cochlear implant (CI) recipients and investigates the most suitable age range for implementing RC. MethodsFifty-eight pediatric CI recipients, aged 1 to 17 years, were evaluated using a web-based RC application by audiologists. Most (84.5%) used verbal communication. Inclusion criteria required stable map status, caregiver tech proficiency, and a compatible phone. ResultsRemote assessments were beneficial, with 89.7% of participants successfully completing RC. Aided threshold tests (ATT) were completed in 73.2% of participants, while speech-in-noise tests (Digit Triplet Test, DTT) were completed in 47.3%. Children as young as 4 successfully completed ATT, while a minimum age of 7 was required for DTT. A significant relationship between age and test success was demonstrated. Verbally communicative children had higher completion success rates. Parental education was significantly correlated with DTT success, with higher success rates in children whose parents had undergraduate degrees. With RC use, 69% of children did not need clinic visits, emphasizing the need for personalized and remote support for optimal cochlear implant performance. In-person appointments were primarily required due to concerns about the duration of device usage. ConclusionThe study confirms the effectiveness and feasibility of using RC in pediatric CI users, even as young as one year old with stable aided hearing. The findings advocate for the integration of RC into routine follow-up protocols, offering a more accessible option for enhanced patient care.

High variability phonetic training facilitates perception-to-production transfer in Mandarin-speaking children with cochlear implants: An acoustic investigation.

This study primarily aimed to evaluate the effectiveness of high variability phonetic training (HVPT) for children with cochlear implants (CIs) via the cross-modal transfer of perceptual learning to lexical tone production, a scope that has been largely neglected by previous training research. Sixteen CI participants received a five-session HVPT within a period of three weeks, whereas another 16 CI children were recruited without receiving any formal training. Lexical tone production was assessed with a picture naming task before the provision (pretest) and immediately after (posttest) and ten weeks after (follow-up test) the completion of the training protocol. The production samples were coded and analyzed acoustically. Despite considerable distinctions from the typical baselines of normal-hearing peers, the trained CI children exhibited significant improvements in Mandarin tone production from pretest to posttest in pitch height of T1, pitch slope of T2, and pitch curvature of T3. Moreover, the training-induced acoustic changes in the concave characteristic of the T3 contour was retained ten weeks after training termination. This study represents an initial acoustic investigation on HVPT-induced benefits in lexical tone production for the pediatric CI population, which provides valuable insights into applying this perceptual training technique as a viable tool in clinical practices.

Hearing preservation in pediatric cochlear implantation.

Pediatric cochlear implantation has evolved considerably over the past three decades to include more patients at earlier ages with greater degrees of residual hearing. As an extension, a significant focus of research over the past decade has surrounded preservation of existing acoustic hearing. Multiple studies published within the last 5 years demonstrate aidable acoustic hearing preservation in 60-90% of pediatric patients, with 40-60% experiencing complete hearing preservation following cochlear implantation. Durability of preserved hearing varies among patients, with some patients losing residual hearing within 1 year of surgery whereas others maintain acoustic hearing through at least 5 years of follow-up. Speech outcomes appear superior among patients with preserved acoustic hearing, particularly in the presence of background noise. Several recent studies suggest a music appreciation advantage in children with preserved acoustic hearing following cochlear implantation. Hearing preservation rates during cochlear implantation in children matches, if not often exceeds, hearing preservation rates observed among adults. Preservation of acoustic hearing during cochlear implantation confers multiple advantages for the pediatric population. Beyond improved speech understanding and music appreciation, minimizing intracochlear trauma and resultant scarring facilitates potential future regenerative treatments or revision surgery.

Factors impacting outcomes of cochlear implantation in children at two University centres in China: Multi-year analysis from the Paediatric Implanted Recipient Observational Study (P-IROS)

Objectives To identify factors affecting functional hearing performance and quality of life (QoL) outcomes in paediatric cochlear implantation (CI) recipients at two University centres in mainland China. Methods Two university centres in mainland China, part of the prospective longitudinal Paediatric Implanted Recipient Observational Study (P-IROS), contributed participant data. Participants were aged under 10 years at time of CI. Functional hearing performance and QoL measures were collected prior to device activation, and at 6-monthly intervals for 2 years post-implantation. Functional hearing endpoints including Categories of Auditory Performance-II (CAP-II) and QoL were evaluated and analysed using ordinal mixed-effects regression models. Results Data were from 288 children with a mean age at implant of 2.74 years. Overall follow-up at 1 year was 59% and 51% at 2 years. Younger age at implantation (p<0.001) and hearing aid use preimplantation (p=0.026) were associated with significant benefit. Bilateral device users (both CI and bimodal) achieved significantly better functional hearing performance on the CAP-II than unilateral CI users (p<0.001). Slower functional hearing improvements were observed in those with lower parental expectations compared to higher expectations (p<0.001). QoL improved over time but followed a different initial trajectory between centres. Conclusion All participants demonstrated significant improvements in auditory performance and QoL over time. Younger age at CI, and bilateral/bimodal device fitting contributed to earlier improvements. Other potential factors that could help inform families, professionals, and health authorities about choice of hearing device and educational supports required included aetiology of hearing loss and level of maternal education.

Electrophysiological Characteristics in Pediatric Cochlear Implantation.

Cochlear implantation is a potential intervention for individuals with severe to profound hearing loss, in particular in the pediatric population. This literature review aims to comprehensively evaluate the applications of electrophysiological tests in enhancing cochlear implant (CI) outcomes for children. A literature review searched Medline and PubMed databases for articles on electrophysiological tests in CI children, using the terms "electrophysiological tests," "children," and "cochlear implant." The systematic search leads to 72 eligible texts. Electrophysiological tests can be used to test CI children without the need for their active participation. These tests can be helpful in identifying and improving the health of deaf children in various ways, such as determining the CI functional status, the semantic integration effects in CI children, the effect of central auditory structures in speech stimulus processing, the development of lexical-semantic in CI children, and tracking the maturation of the central auditory system. CI enhances central auditory nervous system (CANS) maturation and auditory/language skills. The quality of electrophysiological tests can be improved to enhance hearing outcome prediction, postoperative physiology understanding, and hearing loss mechanisms. Electrophysiological tests study CANS maturation, identify lesions, aid CI programming, determine prognosis, and treatment outcomes.

Association of domain-general speed of information processing with spoken language outcomes in prelingually-deaf children with cochlear implants

Spoken language development after pediatric cochlear implantation requires rapid and efficient processing of novel, degraded auditory signals and linguistic information. These demands for rapid adaptation tax the information processing speed ability of children who receive cochlear implants. This study investigated the association of speed of information processing ability with spoken language outcomes after cochlear implantation in prelingually deaf children aged 4–6 years. Two domain-general (visual, non-linguistic) speed of information processing measures were administered to 21 preschool-aged children with cochlear implants and 23 normal-hearing peers. Measures of speech recognition, language (vocabulary and comprehension), nonverbal intelligence, and executive functioning skills were also obtained from each participant. Speed of information processing was positively associated with speech recognition and language skills in preschool-aged children with cochlear implants but not in normal-hearing peers. This association remained significant after controlling for hearing group, age, nonverbal intelligence, and executive functioning skills. These findings are consistent with models suggesting that domain-general, fast-efficient information processing speed underlies adaptation to speech perception and language learning following implantation. Assessment and intervention strategies targeting speed of information processing may provide better understanding and development of speech-language skills after cochlear implantation.

Cochlear implantation in pediatrics: Impact of newborn hearing screening on intervention time

ObjectivesThe newborn hearing screening (NHS) program was globally established for early hearing loss (HL) identification and intervention. Early intervention is essential to minimize or prevent the negative consequences of HL. In Saudi Arabia, the NHS was officially implemented in 2016. Currently, its impact on the timing of cochlear implantations (CIs) in Saudi Arabia remains unclear, and information on potential hospital-related delays affecting early implantation is lacking. Thus, this study aimed to evaluate the effect of implementing the NHS on age at CI in children with prelingual deafness in a CI center in Saudi Arabia, and to evaluate the hospital timing in the CI process. MethodsAll pediatric CI users who presented for the first time to the CI committee (CIC) at a tertiary center and received their implants between 2015 and 2022 were enrolled in this study. Date of birth (DOB), date of presentation to the CI committee (DOCIC), and date of CI surgery (DOCIS) were retrospectively reviewed. ResultsIn total, 304 CI children were included in the analysis. Approximately 55 % of the children (n = 167) were screened for HL through the NHS, whereas 45 % of the children (n = 137) were born before the launch of the NHS. Both age at the presentation to the CIC (i.e. difference between DOCIC and DOB) and age at implantation (i.e. difference between DOCIS and DOB) were significantly earlier in children who were screened for HL through the NHS than those who were not screened (P < 0.0001). The time difference between the DOCIC and DOCIS was not significantly different between the screened and unscreened children (P > 0.05). ConclusionThe implementation of the NHS in the tertiary center has a significant positive effect on age at presentation to the CIC and age at implantation, but not on the actual CI surgery. Further research is needed to reduce the hospital delays before the actual surgery in order to increase the likelihood of children receiving implantation early in their life.

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

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

Successful Pediatric Cochlear Implantation In Persistent Stapedial Artery: First Reported Case In India WithLiterature Review AndRelevance of Preoperative Imaging Checklist.

We report the first successful pediatric cochlear implantation in bilateral persistent stapedial artery (pSA) in India. Another child with unilateral pSA, the contralateral side was initially implanted. Both children were preoperatively diagnosed. Preoperative imaging checklist for pSA, facilitates counselling for bilateral and decision-making for the contralateral side in unilateral abnormality.

The relationships between language, functional hearing, social, and motor development skills in children with early cochlear implants

PurposeThe purpose of this study was to investigate the relations between functional hearing, language, social, bilateral coordination and manual dexterity skills in children with early cochlear implants (CIs).MethodsThirty children with CIs were included in this study. The manual dexterity and bilateral coordination development of the participants were evaluated with Manual Dexterity and Bilateral Coordination subtests of Bruininks-Oseretsky Motor Proficiency-2 (BOT-2). Their language skills were assessed by the Test of Early language Development-3. To assess the functional hearing of participants the Functioning After Pediatric Cochlear Implantation scale (FAPCI) was administered their caregivers. Also, the Social Skills Evaluation Scale was administered to participants’ teachers to asses their social skills.ResultsThere were significant correlations between participants’ receptive and expressive language skills, Manual Dexterity, and FAPCI scores (p < 0.05). There were also significant relationships between the SSES and FAPCI scores of the participants (p < 0.05). However, the Bilateral Coordination subtest did not show any significant correlation with any of the measurements (p > 0.05).ConclusionThe results suggest that the language, manual dexterity and functional hearing abilities of children with CIs are closely related. Although, there were no significant correlations between all of the measurement, it is important to look beyond hearing and speech evaluations to assess the whole child.

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

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

Current Issues With Pediatric Cochlear Implantation.

Cochlear implants (CIs) have demonstrated a clear functional benefit in children with severe-to-profound sensorineural hearing loss (SNHL) and thus have gained wide acceptance for treating deafness in the pediatric population. When evaluating young children for cochlear implantation, there are unique considerations beyond the standard issues addressed during surgery in adults. Because of advances in genetic testing, imaging resolution, CI technology, post-implant rehabilitation, and other factors, issues related to CI surgery in children continue to evolve. Such factors have led to changes in candidacy guidelines, vaccine requirements, and lowering of age requirement for surgery. In addition, differences in the anatomy and physiology of infants require special attention to ensure safety when operating on young children. This review summarizes these issues and provides guidance for surgeons treating children with SNHL.

Intraoperative EABR Testing Predicts Strength of Cochlear Implant Stimulation Optimized After Long-Term Use in Pediatric Malformation Ears.

This study focused on the intensities of cochlear implant (CI) stimulation in pediatric CI users with inner ear malformation or cochlear nerve deficiency (CND). In this population, CI programming is difficult because a large intensity of CI stimulation is required to achieve sufficient hearing, but the excess CI stimuli often induce facial nerve stimulation. We aimed to assess whether the results of intraoperative electrically evoked auditory brainstem responses (EABRs) testing predict maximum current levels of CI stimuli (cC levels) optimized by a behavioral-based method after long-term CI use. A retrospective case review. A tertiary referral CI center. A total of 116 ears with malformations (malformation group) and 63 control ears (control group) from patients younger than 18 years who received CI. The malformation group comprised 23 ears with a common cavity (CC), 26 with incomplete partition type 1 (IP-1), 26 with incomplete partition type 2 (IP-2), and 41 with CND. Diagnostic. Correlation between intraoperative EABR results and cC levels determined by the behavioral-based CI programming after long-term CI use. The CC, IP-1, and CND ears required significantly larger cC levels than the IP-2 ears and control groups. However, the cC levels increased to reach the plateau 1 year after surgery in all groups. Among the malformation group, 79 ears underwent intraoperative EABR testing. Greater than 80% of the CC, IP-1, and IP-2 ears and 54.8% of the CND ears exhibited evoked wave V (eV) and were included in the eV-positive category. Myogenic responses but no eV were observed in 18.2, 15.0, and 35.5% of the CC, IP-1, and CND ears, defined as the myogenic category. No eV or myogenic response was elicited in 9.7% of the CND ears. We focused on minimum current levels that elicited eV (eV levels) in the eV-positive category and maximum current levels that did not elicit any myogenic responses (myogenic levels) in the myogenic category. A significant relationship was detected between the eV levels and the cC levels. When analyzed in each malformation type, the eV levels significantly correlate with the cC levels in the CC and CND ears but not in the IP-1 and IP-2 ears, probably because of slight variation within the IP-1 group and the small number of the IP-2 group. The myogenic category did not show a significant relationship between the myogenic levels and cC levels, but the cC levels were similar to or smaller than the myogenic levels in most ears. This study confirmed that intraoperative EABR testing helps predict the optimal cC levels in malformation ears. EABR-based CI programming immediately after cochlear implantation, followed by behavioral-based CI programming, may allow us to achieve early postoperative optimization of CI maps even in young children with severe malformations.