Categories Of Auditory Performance Scores Research Articles

Clinical Portrait of Cochlear Implantation in Patients With CHARGE Syndrome.

To examine the clinical characteristics and auditory performance of patients with CHARGE syndrome following cochlear implantation (CI), as well as the prognostic factors affecting auditory outcomes. Retrospective cohort. Tertiary academic center. A retrospective chart review was performed in patients with CHARGE syndrome who underwent CI from 2007 to 2022. The category of auditory performance (CAP) score was used to assess the CI outcomes, and factors that may affect the speech outcomes were also evaluated. In 14 children with CHARGE syndrome, 22 CIs were performed, 6 unilaterally and 8 bilaterally. The mean age at CI was 25.9 months (range: 10-62). All patients had ear abnormalities and developmental delays, and cochlear nerve deficiency (CND) was present in all ears. At the last follow-up (mean: 49.6 months), the mean CAP score improved significantly compared to the preoperative measure (from 0.36 ± 0.81 to 3.21 ± 1.70, P = .001), with 6 patients (42.9%) achieving a CAP score of 4 points or higher. However, between the unilateral and bilateral CI groups, the final CAP score or change in CAP score was similar. Factors including age, coloboma, and CND did not significantly affect speech outcomes (all P > .05). Even though CHARGE syndrome features challenging anomalies, CI can be conducted safely and can offer effective contribution to significant speech improvement. Patients with CHARGE syndrome should be given the opportunity to undergo CI to maximize their audiological progress.

The Role of Measuring Preoperative Social Maturation Score in Children With Additional Needs Who Underwent Cochlear Implantation.

Additional needs refer to specific requirements or support for individuals with disabilities or syndromes. Intellectual ability is a crucial outcome determinant of a cochlear implant. The social quotient (SQ) is an indirect predictor of intellectual capacity and social skills. This study aimed to investigate the clinical significance of the SQ on children with additional needs who received cochlear implants. Subjects and. This study included 24 patients with diagnosed developmental delays and syndromes, who demonstrated SQ scores of <70. Preoperative social skills were evaluated using the SQ. All patients underwent cochlear implantation (CI) surgery before 7 years of age. Outcomes were evaluated using the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) and Categories of Auditory Performance (CAP) scores. Data were collected through a retrospective chart review. Children were categorized into three groups based on their SQ. There were no correlations between the preoperative SQ and IT-MAIS or CAP scores at 2 and 5 years of follow-up postoperatively. The CI outcomes of children with low SQ (<70) differed from those with normal development (SQ>70). In the low-SQ group, inner ear anomalies were observed in 10 (41.7%) patients. Although not statistically significant, these children exhibited a trend of lower average outcomes than children without inner ear anomalies. CI outcomes in children with additional needs positively affected auditory performance. Postoperative auditory and language skills tended to improve slowly in children with additional needs and a lower SQ. Over time, development gradually became more comparable to the other groups of children. However, this improvement was less than that observed in children without additional needs. Our findings support CI for children with additional needs as part of long-term auditory rehabilitation following surgery.

Auditory Brainstem Implant Outcomes in Tumor andNontumor Patients: A Systematic Review.

To elucidate the differences in auditory performance between auditory brainstem implant (ABI) patients with tumor or nontumor etiologies. PubMed, Embase, and Web of Science Core Collection from 1990 to 2021. We included published studies with 5or more pediatric or adult ABI users. Auditory outcomes and side effects were analyzed with weighted means for closed-set, open-set speech, and categories of auditory performance (CAP) scores. Overall performance was compared using an Adult Pediatric Ranked Order Speech Perception (APROSPER) scale created for this study. Thirty-six studies were included and underwent full-text review. Data were extracted for 662 tumor and 267 nontumor patients. 83% were postlingually deafened and 17% were prelingually deafened. Studies that included tumor ABI patients had a weighted mean speech recognition of 39.2% (range: 19.6%-83.3%) for closed-set words, 23.4% (range: 17.2%-37.5%) for open-set words, 21.5% (range: 2.7%-48.4%) for open-set sentences, and 3.1 (range: 1.0-3.2) for CAP scores. Studies including nontumor ABI patients had a weighted mean speech recognition of 79.8% (range: 31.7%-84.4%) for closed-set words, 53.0% (range: 14.6%-72.5%) for open-set sentences, and 2.30 (range: 2.0-4.7) for CAP scores. Mean APROSPER results indicate better auditory performance among nontumor versus tumor patients (3.5 vs 3.0, P = .04). Differences in most common side effects were also observed between tumor and nontumor ABI patients. Auditory performance is similar for tumor and nontumor patients for standardized auditory test scores. However, the APROSPER scale demonstrates better ABI performance for nontumor compared to tumor patients.

A Long-Term Follow-Up Study on the Auditory Performance and Speech Intelligibility of Mandarin-Speaking Prelingually Deaf Children With Isolated Large Vestibular Aqueduct Syndrome After Cochlear Implantation.

The purpose of this study was to evaluate the auditory performance and speech perception of 104 children with isolated large vestibular aqueduct syndrome (LVAS) and 523 children with no inner ear malformation (IEM) for 5 years after cochlear implantation, in order to explore whether isolated LVAS can affect the long-term hearing and speech rehabilitation of deaf children after cochlear implantation. A cohort study was established consisting of 627 children who underwent cochlear implantation at Beijing Tongren Hospital from 1999 to 2016. The children were examined at 0, 6, 12, 24, 36, 48, and 60 months after cochlear implantation to assess their auditory performance and speech perception using the Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) questionnaires. The CAP scores of the isolated LVAS group increased significantly during the baseline to the 24th month, after which they gradually rose until reaching the plateau during the 24th to the 60th month. The CAP scores of the non-IEM group increased significantly during the baseline to the 36th month and then increased steadily. The SIR scores went up significantly during the baseline to the 48th month, and increased in a gradual manner in other stages of isolated LVAS evaluation. In comparison, The SIR scores of non-IEM group grew remarkably from the baseline to the 60th month. There were no significant differences in the CAP or SIR scores between isolated LVAS and non-IEM groups in each stage of evaluation, with the only exception being the CAP score at baseline month after cochlear implantation. The CAP and SIR questionnaires are helpful tools for quantifying the early hearing and speech skills of younger prelingually deafened cochlear implant recipients. This long-term follow-up study shows that the speech and hearing development of children in isolated LVAS and non-IEM groups follow similar patterns, and isolated LVAS does not affect the long-term rehabilitation of deaf children after cochlear implantation.

Electrically evoked auditory brainstem responses in deaf children with cochlear nerve canal stenosis

Background Deaf children with cochlear nerve canal stenosis (CNCs) are always considered poor candidates for cochlear implantation. Objectives To investigate the function of the peripheral auditory pathway in deaf children with CNCs, as revealed by the electrically evoked auditory brainstem response (EABR), and postoperative cochlear implants (CIs) outcomes. Materials and Methods Thirteen children with CNCs and 13 children with no inner ear malformations (IEMs) who received CIs were recruited. The EABR evoked by electrical stimulation from the CI electrode was recorded. Postoperative CI outcomes were assessed using Categories of Auditory Performance (CAP) and Speech Intelligibility Rate (SIR). Results Compared with children with no IEMs, children with CNCs showed lower EABR extraction rates, higher thresholds, a longer wave V (eV) latency and lower CAP and SIR scores. The auditory and speech performance was positively correlated with the diameter of the cochlear nerve canal and the number of channels showing wave III (eIII) and eV in children with CNCs. Conclusions and Significance The physiological function of the peripheral auditory pathway in children with CNCs is poorer than that in children with no IEMs. Postoperative auditory and speech abilities may depend on the severity of cochlear nerve malformation and auditory conduction function.

Outcomes of cochlear implantation in 75 patients with auditory neuropathy.

Cochlear implantation (CI) outcomes in patients with auditory neuropathy (AN) are variable, which hampers patients' decisions on CI. This study aims to assess the outcomes of CI in individuals diagnosed with AN and to examine the various factors that may influence the effectiveness of this intervention. A total of 75 patients diagnosed with AN were included in the study. The hearing threshold, the score of categories of auditory performance (CAP), speech intelligibility rating (SIR), and speech audiometry were tested. Genetic testing was conducted by medical exome sequencing in 46 patients. After CI, the average aided hearing threshold for patients with prelingual and post-lingual onset was 38.25 ± 6.63 dB and 32.58 ± 9.26 dB, respectively; CAP score improved to 5.52 ± 1.64 (p < 0.001) and 6.00 ± 0.96 (p < 0.001), respectively; SIR score increased to 3.57 ± 1.22 (p < 0.001) and 4.15 ± 0.95 (p < 0.001), respectively. Maximum speech recognition ranged from 58 to 93% for prelingual onset patients and 43 to 98% for those with post-lingual onset. Speech outcomes of CI in cases with cochlear nerve (CN) deficiency were significantly poorer (p = 0.008). Molecular etiologies, including TWIST1, ACTG1, m.A7445G, and a copy-number variant (CNV) carrying ACTB, were related to AN here. CI is a viable therapy option for patients with AN; CN deficiency might impact outcomes of CI.

Developmental Auditory and Speech-Language Performance in Pediatric Cochlear Implantation Recipients with Stable White Matter Lesions.

To analyze the association between stable asymptomatic white matter lesions (WMLs) and the cochlear implantation (CI) effect in congenitally deaf children, 43 CI children with stable asymptomatic WMLs determined via preoperative assessments and 86 peers with normal white matter were included. Outcome measurements included closed-set Mandarin Chinese (tone, disyllable, and sentence) recognition tests; categories of auditory performance (CAPs); and speech intelligibility rating (SIR) scales at 1, 12, and 24 months post-CI. Generalized estimating equation (GEE) models were used to analyze the association between WML and outcomes. In the WML group (control group), median CAP and SIR scores were 5 (5) and 4 (4) with mean rates of tone, disyllable, and sentence recognition of 84.8% (89.0%), 87.9% (89.7%), and 85.8% (88.0%) at 24 months post-CI, respectively. Auditory and speech performance improved significantly with implant use. Compared to their peers in the control group, for the participants with stable asymptomatic WMLs, auditory and speech abilities were not significantly different (p > 0.05). Stable asymptomatic WMLs might not be associated with poor auditory and speech intelligibility post-CI, which indicates that it is feasible to use comprehensive assessments to screen suitable candidates with WMLs who are likely to present with a good prognosis.

Cochlear Implantation in the Elderly: Speech Performance, Associated Factor, Complication, and Surgical Safety.

The guidelines for cochlear implantation (CIs) are expanding, and the number of CI procedures performed on the elderly is increasing. The purpose of this study was to analyze the results and safety of cochlear implantation in the elderly, as well as to evaluate the predictive factors on CI outcomes. The study included 56 patients aged ≥40 years, who received CIs between 2009 and 2020. They were divided into two groups: 27 younger adults (40-64 years) and 29 elderly (>64 years). The study compared their pre- and postoperative speech perception and category of auditory performance (CAP) scores, surgical complications, and hospitalization periods. It also evaluated associated factors in the elderly group by examining categorical and continuous variables and postoperative CAP score. There was a significant improvement in speech recognition tests (both word and sentence) and CAP scores in both groups compared to the pre-implantation scores (p<0.001). Postoperative results were slightly lower in the elderly group than in younger adults for sentence recognition and CAP scores, except for word recognition. No significant associated factors were found on postoperative CAP scores, except for etiology. Postoperative CAP significantly improved in the sudden hearing loss group compared to the groups with other etiologies (p=0.045). The elderly group had more comorbidities than that in the younger adult group (p=0.026), but there were no significant differences in postoperative complications and hospitalization periods. While speech recognition and CAP scores were relatively lower in the elderly group compared to the younger adults, the elderly group showed significant improvements in audiological results after CI. Moreover, CI was safe and well tolerated in elderly patients.

Cochlear Implantation in Children with Meningitis: A Multicenter Study on Auditory Performance and Speech Production Outcomes.

This study aims to evaluate speech production outcomes and auditory performance in children with post-meningitis deafness who were treated with cochlear implants. Additionally, the study assesses the impact of electrode insertion depth on surgical outcomes.". We conducted a study on 66 pediatric patients with bilateral postmeningitis hearing loss who were being prepared for cochlear implantation at four tertiary referral academic institutions. The speech intelligibility rating (SIR) and categories of auditory performance (CAP) were evaluated after the first and second years following implantation. The patients were divided into two groups based on electrode insertion depth: one group had full electrode insertion (more than two-thirds), while the other had partial electrode insertion (less than two-thirds). We compared the SIR and CAP scores between the two groups to assess the impact of electrode insertion depth on outcomes. Before implantation, the median CAP score was one, but it improved significantly to six within two years after the procedure (P-value < 0.001). Similarly, the median SIR score before implantation was one, but it improved significantly to three within two years after surgery (P-value < 0.001). However, there was no significant difference between the partial and full electrode insertion groups in terms of CAP and SIR scores during the follow-up evaluations conducted after the first and second years. The study found that cochlear implantation significantly improved speech production skills and auditory performance in children with postmeningitis deafness. Importantly, the amount of electrode insertion at the time of implantation did not have a significant impact on the outcomes.

Long-term speech perception performance in prelingually deafened adult cochlear implant recipients

Objective To investigate the postoperative long-term outcomes after an average of 9.2 years following cochlear implantation (CI) in prelingually deafened adults, along with preimplantation factors predicting postoperative outcomes. Methods Twenty-six prelingually deafened adults who underwent CI at >18 years were compared with those who had undergone CI in childhood (<9 years) and were >10 years old. Outcome measures includedhearing thresholds, preoperative and postoperative aided hearing level (HL), speech discrimination score (SDS), and Categories of Auditory Performance (CAP) scores. Correlation analyses were performed on the following: SDS results, aided HL, school attendant status, implant manufacturers, and speech processor models. Results Improvement was achieved in the aided HL and SDS results, although these results were not better than those of the child group. CAP score was also statistically significantly improved after CI. Statistically significant correlation between the preoperative SDS and postoperative HL with CI results was observed. In other words, the better the preoperative SDS results, the better the postoperative SDS results. Conclusion Prelingually deafened adults achieved considerable improvement through CI. It is important to understand that patients achieving better hearing with a well-fitted hearing aid and good SDS performance before surgery may be good candidates for CI.

Analysis of rehabilitation effects of cochlear implantation in elderly patients with prelingual deafness

Objective:The auditory and speech rehabilitation effects were assessed by the Categories of Auditory Performance（CAP） and the speech intelligibility rating scale（SIR） after cochlear implantation（CI） in prelingually elderly patients by telephone follow-up or face-to-face conversation. Methods:The clinical data of the prelingually deaf patients who underwent unilateral CI in the Department of Otorhinolaryngology and Head and Neck Surgery, Shanxi People's Hospital, from December 2016 to December 2021 were collected. Thirty-eight patients were divided into Group A（SIR 1, 17 cases）, Group B（SIR 2, 10 cases） and Group C（SIR 3, 11 cases） according to the preoperative SIR Score. Nineteen patients with post-lingual hearing impairment were selected as the control group（Group D, 19 cases）. The effects of hearing and speech rehabilitation were evaluated using CAP and SIR Scores before surgery, 6 months after startup, and 1 year after startup. Results:There were no significant differences in CAP scores among the three groups of patients with prelingually deaf patients at 6 months and 1 year after startup（P>0.05）, but there were significant differences between group A and group D at 6 months and 1 year after startup（P<0.05）; the SIR Score of group A had statistical difference before surgery and 6 months after startup（P<0.05）, group B had statistical difference before surgery and 1 year after startup（P<0.05）, and group C and D had no statistical difference before surgery and 6 months and 1 year after startup, respectively（P>0.05）. Conclusion:For the prelingually deaf elderly patients, hearing will develop rapidly 6 months after startup, and the effect of postoperative auditory rehabilitation was positively correlated with the preoperative speech ability. In the aspect of speech, the prelingually dear elderly patients who have poor preoperative speech ability could benefit more from CI early after surgery. CI is not contraindicated in prelingually deaf elderly patients, even those with poor preoperative speech function.

The experience of device failure after cochlear implantation

BackgroundThe present study describes the treatment of patients at a tertiary institution who experienced device failure after Cochlear Implantation (CI), as well as identifying prodromic symptoms that could assist in the timely identification and management of device failure.Study designRetrospective database review (January 2000–May 2017).SettingSingle tertiary hospital.MethodsFactors recorded included the etiology of hearing loss; age at first and revision CI surgeries; surgical information, including operation time and approach; electrical outcomes after implantation; device implanted; symptoms of device failure; history of head trauma; and audiologic outcomes as determined by categories of auditory performance (CAP).ResultsFrom January 2000 to May 2017, 1431 CIs were performed, with 27 (1.9%) undergoing revision surgeries due to device failure. The most common etiology of hearing loss was idiopathic (12/27), followed by cochlear hypoplasia (5/27). Mean age at initial CI was 11.8 (1–72) years, with 21 being pre-lingual and 6 being post-lingual. Of the total devices initially implanted, 80.5% were from Cochlear, 15.9% from MED-EL, and 3.5% from Advanced Bionics. The failure rates of these devices were 1.3%, 3.1%, and 10.0%, respectively. The most suggestive symptom of device failure was intermittent loss of signal. Mean CAP scores were 5.17 before reimplantation and 5.54 and 5.81 at 1- and 3-years, respectively, after reimplantation.ConclusionThe most suggestive symptom preceding device failure was intermittent loss of signal. Patients who present with this symptom should undergo electrical examination for suspected device failure. Audiologic outcomes showed continuous development despite revision surgeries.

Cochlear Implantation Outcomes in Pediatrics with Inner Ear Malformations in a Tertiary Care Hospital in Ahvaz.

Cochlear implantation (CI) has been successful in individuals with sensorineural hearing loss (SNHL) over the last few years. This study assessed the auditory and speech performance results of pediatrics with inner ear malformations (IEMs) following cochlear implantation at the Ahvaz cochlear implantation center and compared the outcomes of various malformations. All pediatrics with IEMs undergoing CI were included in the study. This retrospective study was performed on pediatrics with congenital IEMs who underwent cochlear implantation in Ahvaz cochlear implantation center between 2014 and 2019. The Category of Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR) scores are two of the most frequently administered tests. The CAP with ranging from 0 (displays no awareness of environmental sounds) to 7 (can use the telephone with a familiar talker), was used to measure the speech perception performance of the implanted children. Furthermore, SIR consists of five performance categories ranging from "prerecognizable words in spoken language" to "connected speech is intelligible to all listeners". Finally, the study included 22 patients. The evaluation of the CT-Scan revealed three types of inner ear malformation: Incomplete Partition (IP)-I in 2 (9.1%), IP-II in 12 (54.5%), and common cavity in 8 (36.4%) individuals. The results were shown that the median CAP score preoperative was 0.5 (interquartile range (IQR): 0-2) and postoperative was 3.5 (IQR: 3-7). There were statistically significant differences in CAP scores between preoperative and second-year follow-up postoperative (p value = 0.036). The results were shown that the median SIR score preoperative was 1 (IQR: 1-5) and postoperative was 2 (IQR: 1-5). There were statistically significant differences in SIR scores between preoperative and second-year follow-up postoperative (p value = 0.001). Following a thorough preoperative screening, patients with specific IEMs can be candidates for CI and not a contraindication. There were statistically significant differences in CAP and SIR scores between preoperative and second-year follow-up postoperative in the common cavity and IP-II groups.

Long-Term Auditory and Speech Outcomes of Cochlear Implantation in Children With Cochlear Nerve Aplasia.

In this study, we aimed to (1) review the long-term outcomes of cochlear implantation in children with cochlear nerve aplasia and (2) compare the development of their auditory and speech abilities to children with normal-sized cochlear nerves. This is a retrospective case-control study. Patients who underwent unilateral cochlear implant (CI) surgery in a tertiary referral center from September 2012 to December 2018 were reviewed. The study group included 55 children with cochlear nerve aplasia diagnosed using preoperative images. The control group included 35 children with normal-sized cochlear nerves. The control group did not differ from the study group in terms of age at implantation, pre-implantation auditory and speech abilities, or the electrode array type. Cochlear implantation outcomes were assessed using a test battery, including the Categories of Auditory Performance (CAP) score, the Speech Intelligibility Rating (SIR) score, behavioral audiometry, and closed- or open-set speech recognition tests. The development of auditory and speech abilities was compared between the two groups using Generalized Linear Mixed-effect Models. The mean duration of CI usage was 4.5 years (SD = 1.5, range = 2.0 to 9.5) in the study group. The CAP scores, SIR scores, and aided hearing thresholds improved significantly post-implantation in the study group, but were significantly poorer than those in the control group. Generalized Linear Mixed-effect Models showed that the development of CAP and SIR scores was significantly slower in the study group than in the control group. Overall, 27 (49%) children with cochlear nerve aplasia had some degree of open-set speech perception skills, but the monosyllabic and bisyllabic word recognition rates were significantly lower than those in the control group. For children with cochlear nerve aplasia, auditory perception and speech intelligibility continued to improve in the long-term follow-up, but this progress was significantly slower than in children with normal-sized cochlear nerves. Most children with cochlear nerve aplasia could obtain the ability of common phrase perception and understanding simple spoken language with consistent CI usage and auditory rehabilitation.

Brain MRI findings of prelingually deaf children and cochlear implant outcome: Preliminary results.

To estimate the incidence of brain abnormalities in a cohort of prelingually deaf children and whether these abnormalities can impact the hearing outcomes of patients eligible for cochlear implantation (CI). We performed a retrospective review of consecutive medical charts of prelingually deaf children under 12 years of age who underwent brain magnetic resonance imaging (MRI) during their preoperative workup for CI surgery. We used the category of auditory performance (CAP) test and the speech intelligibility rating (SIR) test to assess the hearing and speech performance of the children, respectively. The MRIs of 285 patients, 174 boys and 111 girls with a mean age of 36.4 (±16) months, were evaluated for this study. We identified 31 patients with abnormal findings (10.88%): (17/31) (54.8%) had MRI brain abnormalities, (9/31) (29%) had inner ear anomalies, and (5/31) (16.1%) had both inner ear and brain abnormalities. The most frequent inner ear anomaly was an enlarged vestibular aqueduct, while white matter lesions were the most common brain abnormality. The CAP and SIR mean score of patients with inner ear anomalies was slightly, but not significantly, higher than those of patients with brain abnormalities. CAP and SIR scores were not significantly different in children with brain abnormalities than inner ear anomalies. These patients can still benefit from CI to improve their overall hearing and speech performance.

Functional Outcomes of Cochlear Implantation in Children with Bilateral Cochlear Nerve Aplasia.

Background and Objectives: Many otologists face a dilemma in the decision-making process of surgical management of patients with cochlear nerve (CN) aplasia. The goal of this study is to provide fresh evidence on cochlear implantation (CI) results in patients with CN aplasia. Materials and Methods: We scrutinized functional outcomes in 37 ears of 21 children with bilateral CN aplasia who underwent unilateral or bilateral CI based on cross-sectional and longitudinal assessments. Results: The Categories of Auditory Performance (CAP) scores gradually improved throughout the 3-year follow-up; however, variable outcomes existed between individuals. Specifically, 90% of recipients with a 1-year postoperative CAP score ≤1 could not achieve a CAP score over 1 even at 3-year postoperative evaluation, while the recipients with a 1-year postoperative CAP score >1 had improved auditory performance, and 72.7% of them were able to achieve a CAP score of 4 or higher. Meanwhile, intraoperative electrically evoked compound action potential was not correlated with postoperative CAP score. Conclusions: Our results further refine previous studies on the clinical feasibility of CI as the first treatment modality to elicit favorable auditory performance in children with CN aplasia. However, special attention should be paid to pediatric patients with an early postoperative CAP score ≤1 for identification of unsuccessful cochlear implants and switching to auditory brainstem implants.

The Outcome of Cochlear Implantations in Deaf-Blind Patients: A Multicenter Observational Study.

This multicenter study aimed to evaluate the auditory and speech outcomes of cochlear implantation (CI) in deaf-blind patients compared with deaf-only patients. Retrospective cohort study. Multiple cochlear implant centers. The current study was conducted on 17 prelingual deaf-blind children and 12 postlingual deaf-blind adults who underwent CI surgery. As a control group, 17 prelingual deaf children and 12 postlingual deaf adults were selected. Cochlear implantation. Auditory and linguistic performances in children were assessed using the categories of auditory performance (CAP) and Speech Intelligibility Rating (SIR) scales, respectively. The word recognition score (WRS) was also used to measure speech perception ability in adults. The mean CAP, SIR, and WRS cores were compared between the deaf-only and deaf-blind groups before CI surgery and at "12 months" and "24 months" after device activation. Cohen's d was used for effect size estimation. We found no significant differences in the mean CAP and SIR scores between the deaf-blind and deaf-only children before the CI surgery. For both groups, SIR and CAP scores improved with increasing time after the device activation. The mean CAP scores in the deaf-only children were either equivalent or slightly higher than those of the deaf-blind children at "12 months post-CI" (3.94 ± 0.74 vs 3.24 ± 1.25; mean difference score, 0.706) and "24 months post-CI" (6.01 ± 0.79 vs 5.47 ± 1.06; mean difference score, 0.529) time intervals, but these differences were not statistically significant. The SIR scores in deaf-only implanted children were, on average, 0.870 scores greater than the deaf-blind children at "12 months post-CI" (2.94 ± 0.55 vs 2.07 ± 1.4; p = 0.01, d = 0.97) and, on average, 1.067 scores greater than deaf-blind children at "24 months post-CI" (4.35 ± 0.49 vs 3.29 ± 1.20; p = 0.002; d = 1.15) time intervals. We also found an improvement in WRS scores from the "preimplantation" to the "12-month post-CI" and "24-month post-CI" time intervals in both groups. Pairwise comparisons indicated that the mean WRS in the deaf-only adults was, on average, 10.61% better than deaf-blind implanted adults at "12 months post-CI" (62.33 ± 9.09% vs 51.71 ± 10.73%, p = 0.034, d = 1.06) and, on average, 15.81% better than deaf-blind adults at "24-months post-CI" (72.67 ± 8.66% vs 56.8 ± 9.78%, p = 0.002, d = 1.61) follow-ups. Cochlear implantation is a beneficial method for the rehabilitation of deaf-blind patients. Both deaf-blind and deaf-only implanted children revealed similar auditory performances. However, speech perception ability in deaf-blind patients was slightly lower than the deaf-only patients in both children and adults.

A novel mutation of X-linked recessive deafness gene POU3F4 in a boy with congenital deafness.

PurposeTo report an interstitial deletion of Xq21.1 in chromosome X in a boy with congenital deafness.MethodsThe proband underwent a thorough physical examination and a detailed audiological and temporal bone computed tomography (CT) scan. Cochlear implantation was performed on the proband, and follow‐up was conducted. High throughput sequencing and copy number analysis was made of peripheral blood samples from the proband, family members, and control subjects.ResultsSensorineural hearing loss was present in the boy and temporal bone CT scan showed a bilateral incomplete partition type III anomaly (IP‐III). Q21.1 (79.40–83.32 Mb) of chromosome X in the proband had a copy number deletion with a fragment size of about 3.92 Mb. Categories of auditory performance scores and SIR scores of the cochlea in this child improved after surgery.ConclusionThrough the analysis of POU3F4, a novel mutation site with potentially pathogenic significance was found.Level of Evidence: 5.

Cochlear Implantation Outcomes in Patients with Auditory Neuropathy Spectrum Disorder of Genetic and Non-Genetic Etiologies: A Multicenter Study.

With diverse etiologies and clinical features, the management of pediatric auditory neuropathy spectrum disorder (ANSD) is often challenging, and the outcomes of cochlear implants (CIs) are variable. This study aimed to investigate CI outcomes in pediatric patients with ANSD of different etiologies. Thirty-six children with ANSD who underwent cochlear implantation between 2001 and 2021 were included. Comprehensive etiological analyses were conducted, including a history review, next-generation sequencing-based genetic examinations, and imaging studies using high-resolution computed tomography and magnetic resonance imaging. Serial behavioral and speech audiometry were performed before and after surgery, and the outcomes with CI were evaluated using the Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores. By etiology, 18, 1, 1, and 10 patients had OTOF-related, WFS1-related, OPA1-related, and cochlear nerve deficiency (CND)-related ANSD, respectively. Six patients had no definite etiology. The average CI-aided behavioral threshold was 28.3 ± 7.8 dBHL, and those with CND-related ANSD were significantly worse than OTOF-related ANSD. The patients’ median CAP and SIR scores were 6 and 4, respectively. Favorable CI outcomes were observed in patients with certain etiologies of ANSD, particularly those with OTOF (CAP/SIR scores 5–7/2–5), WFS1 (CAP/SIR score 6/5), and OPA1 variants (CAP/SIR score 7/5). Patients with CND had suboptimal CI outcomes (CAP/SIR scores 2–6/1–3). Identifying the etiologies in ANSD patients is crucial before surgery and can aid in predicting prognoses.

Effect of inner ear malformations on intraoperative ECAP thresholds and postoperative auditory performance.

ObjectivesThis study sought to characterize the influence of inner ear malformations (IEMs) on intraoperative electrically evoked compound action potential (ECAP) and auditory performance to better understand the underlying pathophysiology related to variabilities in cochlear implant (CI) outcomes that individuals with malformed cochlea may present.MethodsThe medical records of 222 ears implanted with Cochlear Nucleus CI were reviewed. Of the total, 64 ears had radiologic evidence of IEMs, and 158 ears were normal. Individuals with IEMs were grouped based on the severity of anomalies; 38 had mild IEMs (e.g., enlarged vestibular aqueduct, incomplete partition type II, etc.) and 26 had severe IEMs (e.g., cochlear nerve hypoplasia, common cavity, etc.). Intraoperative ECAP thresholds obtained via neural response telemetry (NRT) and the categories of auditory performance (CAP) scores measured at 12 months postoperative were compared and correlated.ResultsAbsent ECAP responses were more apparent in the IEM group. ECAP thresholds were significantly elevated in the severe IEM group, while the mild IEM group had ECAP thresholds comparable to the normal group. The mild IEM group achieved CAP scores similar to the normal control. Patients in the severe IEM group showed significantly lower CAP scores at 12 months postoperative. Significant negative relationships existed between ECAP thresholds and CAP scores obtained from all subjects.ConclusionMeasurable ECAP responses and NRT thresholds varied across groups. The inverse relationship between NRT thresholds and CAP scores may suggest that electrophysiological responses measured during surgery may potentially be indicative of postoperative performance in our CI population.Level of Evidence2b.