Meaningful Auditory Integration Scale Research Articles

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.

Hearing and Language Skills in Children Using Hearing Aids: Experimental Intervention Study.

Hearing loss in childhood compromises a child's auditory, linguistic, and social skill development. Stimulation and early intervention through therapy and the use of personal sound amplification devices (PSAPs) are important for improving communication. To verify the effectiveness of speech therapy intervention on the auditory and linguistic skills of Brazilian children aged between 6 and 8 years using PSAPs. Experimental study analyzing the intervention process in children aged between 6 and 8 years with mild to severe bilateral hearing loss and prelingual deafness who are PSAP users. Diagnostic information was analyzed, and assessments and interventions were carried out using the Glendonald Auditory Screening Procedure (GASP), a phoneme discrimination test with figures (TFDF), an expressive language category classification test, and an Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) questionnaire. Sixteen children participated in the study; they were divided into a control group (CG) of six children and an intervention group (IG) of ten children. All research subjects underwent two protocol application sessions, and the IG underwent six speech therapy intervention sessions. In the IT-MAIS, the CG had a 9% increase in score, and the IG had an increase of 3% after intervention. The TFDF obtained a 5% increase in the IG in terms of phonemic discrimination ability. The expressive language category classification tests and GASP were considered not sensitive enough to modify the parameters of auditory and linguistic skills. The study found a significant improvement amongst the IG in the TFDF protocol and an increase in IT-MAIS scores in both groups.

Adaptation and Validation for European Portuguese of the Auditory Performance Categories-II and Infant-Toddler Meaningful Auditory Integration Scale for Children with Cochlear Implant

The Categories of Auditory Performance II (CAP-II) scale and the Infant-Toddler Meaningful Audit Integration Scale (IT-MAIS) are simple and quick questionnaires that allow assessment of the auditory performance of children with cochlear implant (CI). The aim of this study was to translate, adapt and validate the European Portuguese version of the CAP-II and IT-MAIS scales. A total of 85 participants completed the European Portuguese version of the CAP-II and IT-MAIS questionnaires, of which 45 were parents of children with pediatric cochlear implants (9.84 ± 4.22 years) and another 40 were parents of children with normal hearing (8.35 ± 3.56 years). Inter-rater reproducibility, test-retest reproducibility, comparison of study group versus control group results, internal consistency and correlation of the new scales were evaluated. The CAP-II and IT-MAIS scales showed high reliability and reproducibility, respectively, with an intraclass correlation coefficient (ICC) of 0.979 (p < 0.001) and a Spearman's correlation of 0.924 for the CAP-II scale, and an ICC of 0.932 (p < 0.001) and Spearman's correlation coefficient of 0.732 for the IT-MAIS scale. The IT-MAIS and CAP-II versions showed strong internal consistency (Cronbach's α coefficient value of 0.887 for the CAP-II scale and Spearman's positive correlation of 0.677 for the IT-MAIS scale, respectively) and allowed for the differentiation between children with normal hearing and post-implantation children (p = 0.001 and p < 0.001 respectively for each of the scales). There was no association between parental education and the results on the scales (p > 0.05). The findings demonstrated that the European Portuguese version of these scales is a valid and reliable tool for assessing auditory performance in European Portuguese-speaking children with hearing loss.

Time course from cochlear implant surgery to non-use for congenitally deaf recipients implanted as children over ten years ago.

To determine the time-course from first cochlear implantation to non-use, to characterise non-users' receptive and expressive communication, and document known risk factors for inconsistent use, for congenitally deaf non-users of cochlear implants implanted as children at least ten years ago. Retrospective service evaluation. All congenitally deaf patients who received a first cochlear implant as children at least ten years ago at a regional service, and were currently non-users, were identified. They were characterised in terms of ages at implantation and non-use, known risk factors for inconsistent CI use or CI non-use, and outcome measures were the Meaningful Auditory Integration Scale (MAIS) and Meaningful Use of Speech Scale (MUSS) scores. Seventeen patients met the inclusion criteria. They were implanted from 1990 to 2006. Median age at implantation was 4 years (range: 2-11), median age at non-use was 17 years (range: 9-31), and median duration of use was 8.5 years (range: 4-25). All used sign or gesture as their primary expressive and receptive communication modes. In addition, each child had at least one other known risk factor for inconsistent CI use. At 3 years post-implantation, mean Parent-rated MAIS scores were 76.5% (N = 14), and mean MUSS scores were 43.1% (N = 9). This cohort included cases where CI use was rejected following longer periods of time than previously reported, highlighting a need for long-term support, particularly around the ages of life transitions. Studies conducted when the earliest cohort of paediatric CI users were younger, and studies reliant on parent or patient reports, may under-estimate long-term non-use rates. No non-users were identified among congenitally-deaf children implanted 10-15 years ago. Further research is warranted to explore relationships between risk factors, including communication mode, and non-use to inform expectation setting and candidacy selection.

TCOF1 Gene variation in Treacher Collins syndrome and evaluation of speech rehabilitation after bone bridge surgery

Objective:By analyzing the clinical phenotypic characteristics and gene sequences of two patients with Treacher Collins syndrome（TCS）, the biological causes of the disease were determined. Then discuss the therapeutic effect of hearing intervention after bone bridge implantation. Methods:All clinical data of the two family members were collected, and the patients signed the informed consent. The peripheral blood of the proband and family members was extracted, DNA was extracted for whole exome sequencing, and Sanger sequencing was performed on the family members for the mutation site.TCOF1genetic mutations analysis was performed on the paitents. Then, the hearing threshold and speech recognition rate of family 2 proband were evaluated and compared under the sound field between bare ear and wearing bone bridge. Results:In the two pedigrees, the probands of both families presented with auricle deformity, zygomatic and mandibular hypoplasia, micrognathia, hypotropia of the eye fissure, and hypoplasia of the medial eyelashes. The proband of Family 1 also presents with specific features including right-sided narrow anterior nasal aperture and dental hypoplasia, which were consistent with the clinical diagnosis of Treacher Collins syndrome. Genetic testing was conducted on both families, and two heterozygous mutations were identified in the TCOF1 gene: c. 1350_1351dupGG（p. A451Gfs*43） and c. 4362_4366del（p. K1457Efs*12）, resulting in frameshift mutations in the amino acid sequence. Sanger sequencing validation of the TCOF1 gene in the parents of the proband in Family 1 did not detect any mutations. Proband 1 TCOF1 c. 1350_1351dupGG heterozygous variants have not been reported previously. The postoperative monosyllabic speech recognition rate of family 2 proband was 76%, the Categories of Auditory Performance（CAP） score was 6, and the Speech Intelligibility Rating（SIR） score was 4. Assessment using the Meaningful Auditory Integration Scale（MAIS） showed notable improvement in the patient's auditory perception, comprehension, and usage of hearing aids. Evaluation using the Glasgow Children's Benefit Inventory and quality of life assessment revealed significant improvements in the child's self care abilities, daily living and learning, social interactions, and psychological well being, as perceived by the parents. Conclusion:This study has elucidated the biological cause of Treacher Collins syndrome, enriched the spectrum of TCOF1 gene mutations in the Chinese population, and demonstrated that bone bridge implantation can improve the auditory and speech recognition rates in TCS patients.

Correlation between cortical auditory evoked potential and auditory speech performance in children with cochlear implants.

This study aimed to explore the correlation between the characteristics of cortical auditory evoked potential (CAEP) of children with cochlear implants (CIs) and auditory and speech rehabilitation performance by an objective evaluation technique and subjective auditory and speech skills measurements. All participants were recruited from Beijing Children's Hospital, Beijing, China. 19 children with CIs had their responses to the CAEP and MMN recorded. The LittlEARs® Auditory Questionnaire (LEAQ), Categories of Auditory Performance (CAP), Speech Intelligibility Rating Scale (SIR), Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), and Meaningful Use of Speech Scale (MUSS) measures were taken to assess the children's speech and hearing abilities. P1 and MMN of CAEP were negatively related to the duration of CI usage. The duration of CI usage and scores of auditory-verbal assessment questionnaires all showed significant relationships. Additionally, scores of these questionnaires were significantly inversely associated with the latency of P1 and MMN. P1 and MMN could be used as objective methods to evaluate the effectiveness of hearing and speech rehabilitation in children with CIs. In particular to those who cannot give effectively feedback of auditory and verbal effects, these methods might have a certain guiding significance.

Association of Cochlear Implant Management Skills of Parents and Outcomes in Their Cochlear Implanted Wards: An Indian Perspective.

Parental involvement in the rehabilitation process is one of the critical factors for the success of cochlear implants in their wards. Skills to use a cochlear implant (CI) device appropriately and manipulate the different settings are essential to gain maximum benefits. There was a need to have a tool in Hindi to assess the skills of parents to manage the CI device. The study was conducted with the following aims: To translate and adapt Self-administered Cochlear Implant Management Skills (CIMSSelf) into the Hindi language. To find an association between the CI management skills of parents and outcomes in their implanted wards. To compare pre-post retraining scores on CIMS-self in Hindi (HN) and to find out factors affecting the scores. CIMS-self (English) was translated into Hindi using the forward and backward translation method. CIMS-self (HN) was administered to 22 parents of cochlear implanted children to evaluate their CI device management skills in the pretraining phase. The questionnaire was re-administered between 2 and 4weeks to check the reliability. The outcome of CI in the children was assessed using the test tools viz. Categories of Auditory Perception, Integrated Scales of Development, Speech Intelligibility Rating, and Meaningful Auditory Integration Scale (MAIS). Subjects with less than 100% score received retraining on CI device management skills. CIMS-self (HN) was readministered two weeks after completing training, and results were compared between pre-post training sessions. There was no significant correlation between CIMS-self (HN) scores and demographic of the parents and their implanted wards, CI device factors, and clinical outcomes. A significant correlation was found between the CIMS-self (HN) and MAIS scores (p < .05). The Cronbach's alpha for test-retest reliability of the CIMS-self (HN) survey was 0.998. Participants showed a significant improvement in CIMS-self (HN) scores following the intervention, demonstrating that the CIMS-self (HN) is sensitive enough to detect changes in CI device management following retraining. A client who self-reports difficulty may benefit from consultation if it helps to improve their confidence in CI device management. The CIMS-self (HN) survey can be used to evaluate and re-evaluate CI device management skills at regular intervals and may save clinical time.

Development of Auditory and Speech Language Performance Following Bimodal Cochlear Implantation in a Pre-lingual Late Implantee: A Case Report

Objective - To describe the performance in terms of auditory and speech-language development in a late implanted patient with bimodal cochlear implantation.Study Design - Prospective Case study.Subject – A girl with congenital bilateral sensorineural hearing loss with speech and language delay having left ear profound SNHL and right ear severe to profound SNHL.Intervention – Unilateral cochlear implant with neubio BOLD CI system done at the age of 15 years following auditory verbal therapy.Main Outcome Measures – “Meaningful auditory integration scale (MAIS)”, “Revised categories of auditory performance (Revised CAP)”, “Speech intelligibility rating (SIR)”, “Child using hearing devices QOL (cuHDQOL)”, 3D- language acquisition test (3D- LAT), “Parent evaluation aural/oral performance of children (PEACH)” and “Teacher evaluation aural/oral performance of children (TEACH)” is used to assess the outcomes of the cochlear implantation.Results – “Revised CAP,” “SIR,” “MAIS,” “cuHDQOL,” “3D-LAT”, “PEACH,” and “TEACH” scores showed improvement in the implanted teenager over time.Conclusion - The findings represent the evidence for better outcomes of late cochlear implanted patients with bimodal stimulation. It may also provide convincing results about the benefits with the use of Neubio BOLD Cochlear Implant system with a contralateral hearing aid.

Intraoperative Neural Response Telemetry and Auditory Outcomes in Pediatric Cochlear Implantation.

To evaluate the relationship between intraoperative neural response telemetry (NRT) and postoperative auditory testing outcomes in children. Retrospective study. Tertiary-care academic center. Children who underwent cochlear implantation using the Cochlear Corporation device between 2010 and 2019 were included. Associations of average NRT and the slope of amplitude with postoperative auditory outcomes including functional auditory measure Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), and speech perception testing (consonant-nucleus-consonant [CNC], Pediatric AzBio [BABY BIO], Hearing In Noise Test [HINT], and Northwestern University Children's Perception of Speech [NU-CHIPS]), measured between 6 and 57 months after implantation, were assessed using Spearman's rank correlation (ρ). Thirty-eight patients (19 female, 19 male) and 54 ears were included. The median age of implantation was 20.6 months (range 9.6 months to 10.6 years). Eight (21%) children had neurologic disorders such as stroke, epilepsy, cerebral palsy, and other causes. Thirteen (34%) children had connexin mutations. Average NRT was not significantly correlated with postoperative auditory outcomes (IT-MAIS [ρ = -0.08, p = .74], CNC [ρ = 0.19, p = .32], BABY BIO [ρ = 0.21, p = .29], HINT [ρ = 0.05, p = .83])and NU-CHIPS (ρ = 0.21, p = .28). The average slopes of amplitude and comfort level were not strongly correlated with any auditory outcomes (p > .05). Intraoperative NRT was not correlated with any postoperative functional auditory outcomes. Patient counseling should include discussions that a subpar intraoperative cochlear response does not preclude favorable speech and auditory outcomes.

Translation, adaptation, and validation of the Arabic version of the meaningful auditory integration scale

Objectives The main objectives of this study were to translate and adapt the infant-toddler meaningful integration scale (IT-MAIS) into Arabic and to establish the psychometric properties of the translated scale in children with a cochlear implant. Methods The translation and cross-cultural adaptation of this questionnaire were completed in multiple steps and following standard translation protocols. In total, twenty-eight parents of young cochlear implant recipients completed IT-MAIS. Data were collected postoperatively and at 3-, 6-, 9-, and 12-month post-device activation. Data were examined for the validity and reliability of the scale. The internal consistency and reliability of the scale were analyzed using Cronbach α, split-half reliability, and the corrected item-total correlation coefficients. Results Findings demonstrated that the scale exhibited good face and content validity, suggesting that the scale is a one-dimensional measure. Additionally, the reliability analysis for the scale indicated high reliability and correlation among test items. IT-MAIS scores consistently improved over time for all participants and this improvement. correlated negatively with the duration of deafness. Conclusion Current findings indicated that the translated Arabic version of the IT-MAIS scale could serve as a valid instrument for assessing the development of auditory skills in Arabic-speaking children with cochlear implants.

A relação entre estilos parentais e desenvolvimento auditivo em crianças com implante coclear

ABSTRACT Purpose The purpose of this study was to investigate the relationship between parenting styles and hearing skills in children with hearing loss who use cochlear implants. Methods This is an observational analytical study. The participants were the children’s main caregivers divided into a study group (N=50) and a control group (N=28). The children’s ages were between 3 and 7 years old. Subjects in the study group provided a past medical history (PMH), and completed the Parenting Styles and Dimensions Questionnaire (PSDQ) and the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) or the Meaningful Auditory Integration Scale (MAIS). Subjects in the control group also provided a PMH and completed the PSDQ. The Mann Whitney test, the Spearman coefficient, and the Kruskal-Wallis test were used for data analysis, utilizing the JASP 0.8 and SPSS 23 softwares. Results Results showed that The authoritarian parenting style and punishment dimension revealed a statistical significance, with higher scores for children with altered auditory development. Conclusion It was concluded that the practice of authoritarian parenting style with punitive dimensions and physical coercion were related to altered hearing development. Regulation dimension presented a significant correlation with hearing development. The outcome suggested the importance of psychotherapeutic strategies for caregivers who are responsible for children with hearing loss.

A 3-Year Follow-Up and Radiological Analysis of Cochlear Implantation Patients with Cochlear Nerve Deficiency and Modiolar Deficiency-Type Malformations.

Cochlear nerve deficiency (CND) is often combined with modiolar deficiency-type inner ear malformations, which cause variable cochlear implantation (CI) outcomes. We aimed to assess the postoperative development of auditory and speech perception in CND patients with modiolar deficiency-type malformations after 3 years of follow-up to determine the factors correlated with CI outcomes. Sixty-seven CND patients with modiolar deficiency-type malformations who underwent CI surgery were retrospectively reviewed. Modiolar deficiency-type malformations included common cavity (CC), cochlear hypoplasia (CH) (including CH-I and CH-II) and incomplete partition-I (IP-I). Categorical auditory performance (CAP) and the infant-toddler meaningful auditory integration scale (MAIS) were used to assess auditory ability. The speech intelligibility rating (SIR) and meaningful use of speech scale (MUSS) were used to assess the speech intelligibility of these CI patients. The CI outcomes were evaluated at 0, 12, 24 and 36 months after implant activation. All patients demonstrated improvements in auditory ability and speech intelligibility after CI. There were no significant differences in CI outcomes at any time point according to the malformation type. The number of nerve bundles within the internal auditory canal (IAC) showed significant differences at 12, 24 and 36 months after CI ( p < 0.05). Patients with one nerve bundle had relatively poor CI outcomes. CND patients with modiolar deficiency-type malformations showed continuous improvement in auditory and speech abilities after CI. Compared with malformations, the number of nerve bundles should be given more attention when selecting the side for CI.

Cochlear Implantation in a Child with Patau Syndrome.

This study aimed to present the first cochlear implant surgery performed on a patient with Patau syndrome. In the auditory brainstem Response test performed on the 37th month, I-III-V waves at 100 dB were not obtained in the right ear, while I-III-V waves at 90 dB were obtained in the left ear. In the free-field audiometry test done in the first year, the threshold value of cochlear implantation was found to be 45 dB. While the Meaningful Auditory Integration Scale test result was 35/40, the Meaningful Use of Speech Scale test result was 13/40. The cochlear implantation was observed and found that hearing results are good and had a positive effect on the quality of life.

Early prelingual auditory and language development in children with simultaneous bilateral and unilateral cochlear implants.

This current study aimed to explore early prelingual auditory development (EPLAD) and early language development in Mandarin-speaking children who received simultaneous bilateral cochlear implants (BICI) during the first year of cochlear implantation and compare the performance of the children who received BICI with those received unilateral cochlear implant (UCI). 39 Mandarin-speaking children who received BICIs simultaneously and 36 children with UCIs were enrolled in this study. To access the EPLAD, the Infant-Toddler Meaningful Auditory Integration Scale (IT/MAIS) was conducted, and a subtest of the simplified short-form version of the Mandarin Communicative Development Inventory (SSF-MCDI) was used to evaluate the development of expressive and receptive vocabulary for the children at indicated time points after surgery. In both the simultaneous BICI and UCI groups, we observed significantly increased scores of the SSF-MCDI and IT/MAIS 1 year after the surgery. There are indications of early advantages in children with BICI in IT/MAIS scores (at 1, 3, and 6 months after activation). For early development of language, a great difference between the expressive vocabulary scores and the receptive vocabulary scores was observed in both groups. We found there were not significant differences between the two groups on expressive or receptive vocabulary scores, the use of more differentiated measures might be required in future research. We further found that the development of the receptive or expressive vocabulary is dramatically correlated with the age at implantation and the total scores of IT/MAIS for children with simultaneous BICIs. These results may supplement the skills development of early language and auditory in Mandarin-native children with simultaneous CIs. It is obvious that children with normal hearing have mastery of receptive vocabulary before that of expressive vocabulary, which is the same as children with unilateral and bilateral CIs in this research. IT/MAIS total scores and age at CI were important factors for early language performance in children with simultaneous BICIs.

Simultaneous Bilateral Cochlear Implantation in Very Young Children Improves Adaptability and Social Skills: A Prospective Cohort Study.

To investigate the value of using the Gesell Development Diagnosis Scale (GDDS) to predict developmental outcomes in very young children who undergo simultaneous bilateral cochlear implantation. In this prospective cohort study, a repeated-measures investigation was conducted in a tertiary referral hospital. A total of 62 children receiving simultaneous bilateral cochlear implantations were enrolled from April 2017 to August 2018. They were divided into 2 groups depending on the operative age: "Infants" group (6 to 12 months, N = 38) or "Children" group (12 to 36 months, N = 24). Data on the surgical outcomes, auditory development, speech production, and developmental indicators were collected until 2 years after the initial fitting. The primary outcome measure was the GDDS, a neuropsychological development examination. Secondary outcomes included the following: complication rate, aided pure-tone average, Infant-Toddler Meaningful Auditory Integration Scale, Categories of Auditory Performance-II, Meaningful Use of Speech Scale, Speech Intelligibility Rating, and the LittlEARS Auditory Questionnaire. The mean ages at implantation in infants and children groups were 9.2 ± 1.17 and 16.6 ± 3.60 months, respectively. Significant differences were found in the social skills ( p = 0.001) and adaptability ( p = 0.031) domains of GDDS. The younger the age of bilateral cochlear implants surgery, the higher developmental quotient of language, social skills, and adaptability the child could achieve after 2 years. The complication rates in the infants and children groups were 0% versus 2.1% ( p = 0.57). There was no surgical complication in the infants group. In the children group, 1 case with enlarged vestibular aqueduct and Mondini malformation had a receiver-implant misplacement on the right side (2%, 1/48). In the two groups, auditory performance and speech production had improved similarly. In the infants group, social skills developmental quotient at baseline had a significant positive relationship with Meaningful Use of Speech Scale after 2 years. Simultaneous bilateral cochlear implantation in younger children improves adaptability and social skills. GDDS is a sensitive tool of evaluating short-term effect of bilateral cochlear implants in neuropsychological development and constitutes a reliable predictor of speech production for the very younger pediatric cochlear implant users.

Assessment of the correlation between residual hearing and audiologic outcomes after cochlear implantation in patients with cochlear nerve deficiency.

ObjectiveThis study aimed to assess the correlation between residual hearing and audiologic outcomes after cochlear implant (CI) surgery in patients with cochlear nerve deficiency (CND).MethodsThis retrospective study included 57 patients with CND who underwent CI surgery. Patients were divided into four groups according to hearing level (80–95, 95–110, 110–120, and >120 dB) and three groups according to residual hearing (entire spectrum hearing, partial spectrum hearing, and no spectrum hearing) based on the measured response at each frequency. Auditory performance (categorical auditory performance [CAP], Infant–Toddler Meaningful Auditory Integration Scale [IT‐MAIS]) and speech perception (speech intelligibility rating [SIR] and meaningful use of speech scale [MUSS]) were assessed before and 2 years after the surgery.ResultsForty‐seven (82.5%) patients had complete or total hearing loss (≥95 dB) and 17 (29.8%) had no spectrum hearing before CI surgery. Twenty‐nine (50.9%) patients did not exhibit residual hearing at 4 kHz. All patients demonstrated an improvement in auditory performance and speech perception: the CAP score in the 80–95 dB group was significantly higher than that in the 110–120 and >120 dB groups, and the entire spectrum hearing group showed significantly higher CAP, SIR, and IT‐MAIS scores than the partial spectrum hearing group and significantly higher CAP, SIR, IT‐MAIS, and MUSS scores than the no spectrum hearing group.ConclusionFor patients with CND, residual hearing, especially high‐frequency residual hearing, was poor and postoperative audiologic outcomes were significantly associated with the range of residual hearing.Level of Evidence4

Comprehensive Prediction Model, Including Genetic Testing, for the Outcomes of Cochlear Implantation.

Despite growing interest in the genetic contribution to cochlear implant (CI) outcomes, only a few studies with limited samples have examined the association of CI outcomes with genetic etiologies. We analyzed CI outcomes using known predictors and genetic testing results to obtain a comprehensive understanding of the impact of genetic etiologies. We retrospectively reviewed the medical records and images of patients who underwent cochlear implantation and genetic testing at a single tertiary medical institution, between May 2008 and December 2020. After excluding those whose speech test results were unavailable, and those in whom the implant was removed due to complications, such as infection or device failure, 203 patients were included in this study. The participants were categorized into adult (≥19 years), child (2-18 years), and infant (<24 months) groups. Outcomes were measured based on categories of auditory perception, monosyllable, disyllable, and sentence scores. For the infant group, the Infant-Toddler Meaningful Auditory Integration Scale score was used. Among the 203 participants, a causative genetic variant was identified in 117 (57.6%) individuals. The presence of a causative variant was significantly associated with better CI outcomes in the infant group (β = 0.23; 95% confidence interval, 0.01 to 0.47; p = 0.044), but not in the child and adult groups. In the genetically confirmed patients without cochlear malformation, genetic variants involving the spiral ganglion was a poor prognostic factor in the child group (β = -57.24; 95% confidence interval, -90.63 to -23.75; p = 0.004). The presence of known genetic etiology of hearing loss was associated with better CI outcomes in the infant group, but not in the child and adult groups. A neural-type genetic variant was a poor prognostic factor in the genetically diagnosed child subgroup without cochlear malformation. Careful genetic counseling should be performed before cochlear implantation.

Residual Hearing Improves Early Auditory Perception and Speech Intelligibility in Mandarin-Speaking Children with Cochlear Implants.

Background:To investigate the relationship between residual hearing and early auditory speech performance in Mandarin-speaking children with cochlear implants.Methods:Twenty-four prelingually deaf children implanted with a cochlear implants participated in the study. Auditory performance and speech intelligibility were longitudinally evaluated by categories of auditory performance, infant-toddler meaningful auditory integration scale/meaningful auditory integration scale, speech intelligibility rating, and meaningful use of speech scale. According to the postoperative pure tone average threshold, children were grouped as “better” and “worse” residual hearing.Results:Better hearing preservation was observed in 7 children (29.2%) and worse preservation in 17 children (70.8%). The scores of categories of auditory performance, infant-toddler meaningful auditory integration scale/meaningful auditory integration scale, speech intelligibility rating, and meaningful use of speech scalewere higher in children with better residual hearing. The residual hearing level was significantly associated with the performance of meaningful use of speech scale (P = .004), as well as the performance of speech intelligibility rating (P = .049).CONCLUSION:The present study showed that children with better residual hearing exhibited advantages in the early auditory and speech outcomes. The study highlighted the effects of residual hearing on early auditory performance and speech intelligibility development in Mandarin-speaking children with cochlear implants.

Machine Learning-Based Prediction of the Outcomes of Cochlear Implantation in Patients With Cochlear Nerve Deficiency and Normal Cochlea: A 2-Year Follow-Up of 70 Children.

Cochlear nerve deficiency (CND) is often associated with variable outcomes of cochlear implantation (CI). We assessed previous investigations aiming to identify the main factors that determine CI outcomes, which would enable us to develop predictive models. Seventy patients with CND and normal cochlea who underwent CI surgery were retrospectively examined. First, using a data-driven approach, we collected demographic information, radiographic measurements, audiological findings, and audition and speech assessments. Next, CI outcomes were evaluated based on the scores obtained after 2 years of CI from the Categories of Auditory Performance index, Speech Intelligibility Rating, Infant/Toddler Meaningful Auditory Integration Scale or Meaningful Auditory Integration Scale, and Meaningful Use of Speech Scale. Then, we measured and averaged the audiological and radiographic characteristics of the patients to form feature vectors, adopting a multivariate feature selection method, called stability selection, to select the features that were consistent within a certain range of model parameters. Stability selection analysis identified two out of six characteristics, namely the vestibulocochlear nerve (VCN) area and the number of nerve bundles, which played an important role in predicting the hearing and speech rehabilitation results of CND patients. Finally, we used a parameter-optimized support vector machine (SVM) as a classifier to study the postoperative hearing and speech rehabilitation of the patients. For hearing rehabilitation, the accuracy rate was 71% for both the SVM classification and the area under the curve (AUC), whereas for speech rehabilitation, the accuracy rate for SVM classification and AUC was 93% and 94%, respectively. Our results identified that a greater number of nerve bundles and a larger VCN area were associated with better CI outcomes. The number of nerve bundles and VCN area can predict CI outcomes in patients with CND. These findings can help surgeons in selecting the side for CI and provide reasonable expectations for the outcomes of CI surgery.

Efficacy of cochlear implants in children with borderline hearing who have already achieved significant language development with hearing aids.

There are still debates about timing and effectiveness of cochlear implants (CI) in pediatric subjects with significant residual hearing who do not belong to traditional indication of CI. In this study, we aimed to investigate the outcomes of CI, specifically on improvement of pronunciation, among hearing-impaired children already with a substantial degree of language skills as evaluated by Categories of Auditory Perception (CAP) scores or sentence score. Our cohort comprised pediatric CI recipients from July 2018 through October 2020. Among them, cases with CAP scores of 5 or 6 preoperatively were defined as "borderline cases". We investigated prevalence and etiologies, and compared speech evaluation data preoperatively and postoperatively at three time points (3, 6 and 9-12 months after implantation). Among 86 pediatric CI recipients, 13 subjects (15.12%) had language development that reached CAP scores of 5 or 6 before implantation. Postoperative speech evaluation data 6 months after implantation revealed significant improvement of pronunciation (Urimal Test of Articulation and Phonation scores: UTAP), Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) and word perception scores, but not of CAP and sentence perception scores. Notably, the significant improvement of pronunciation based on UTAP scores outstripped that of other speech parameters and this continued steadily up to one-year postoperatively. The result of the study serves as evidence for what to expect from cochlear implantation in hearing-impaired children who have already achieved a substantial degree of language development in terms of CAP scores or sentence perception scores, preoperatively.