Categories Of Auditory Performance Research Articles

Age as an important variable that affects auditory and language results after cochlear implant

AbstractCochlear implant (CI) benefits deaf children's speech perception, language development and speech production. Early fitting of an implant results in improved outcomes. Over a period of more than 3 decades, cochlear implantation (CI) is firmly known as safe and effective treatment for children with bilateral severe to profound sensorineural hearing loss (SNHL) who develop minimal or no benefit from conventional hearing aid use. The primary aim of CI is to improve auditory perception of the child and hence the spoken language. The development of functional spoken language would be the major long-term benefits of CI. Children who received a cochlear implant before a real delay in spoken language development (i.e., between 12–16 months) were more able to achieve age-appropriate spoken language. These results strengthen the idea of doing cochlear implantation before 24 months of age, especially for children with a better ear aided pure tone with average thresholds greater than 65 dB prior to surgery.The main aim is to study the effect of age on CI patients results by applying simple questioners on 31 CI children, that are easy to assess the auditory perception and discrimination as an important prerequisite for language development.Results There was an inverse correlation between; age at CI, categories of auditory performance (CAPS), and meaningful auditory integration scale (MAIS) scores. While; there was direct correlation between MAIS & CAPS scores.Conclusions The auditory perception development after CI in children being 3 years old or younger is much better than the older children which would be reflected on their speech perception and acquiring their 1st word and sentence, but language development did not differ between both groups for this period of follow up. With a need of further detailed full language aspects assessment and follow up to ensure these results.

Reading achievement and deaf students with cochlear implants

Objectives The purpose of this study was to investigate the reading outcomes of a Canadian cohort of school-aged deaf learners with cochlear implants (CIs). The goal was to investigate whether achievement approached that of hearing age peers and identify demographic factors influencing performance. Methods Participants represent a subset of 13 students with CIs from a larger sample of 70 deaf students in grades four through 12 educated in inclusive settings within a large school board in central Canada. Data sources included demographic information, teachers’ ratings on the Categories of Auditory Performance (CAP), and scores from the Woodcock-Johnson III Diagnostic Reading Battery [WJ III-DRB]. Results/Discussion: Participants performed within the low average range in all areas except for Phonological Awareness, which was in the low range; however, there was wide variability in scores across participants. None of the demographic variables (e.g. home language, additional disabilities) had a statistically significant association with performance, although older students had higher mean scores on the Phonological Awareness cluster. Conclusion These findings add to the body of research on literacy achievement and cochlear implantation, providing evidence that this technology has a significant positive effect on outcomes for a population that has heretofore underperformed in this area.

The use of a computer-based program focused on the syllabic method to support early literacy in children with cochlear implants

BackgroundChildren with cochlear implants (CIs) often lag behind children with normal hearing (NH) in early literacy skills. Furthermore, the development of language skills associated with their emergent literacy skills seems to depend on good auditory access. Supporting language acquisition and early literacy in children with CIs may prevent difficulties in primary school. The use of technology may facilitate auditory and speech recovery in children with CIs, but evidence on computer-based early literacy programs is limited. ObjectiveThis study investigates (a) the effects of a computer-based program focusing on the syllabic method on the literacy skills of children with CIs (CIs group), comparing them with the literacy skills of a group of age-matched NH (normal hearing) peers (NHs group); (b) the associations between language and early literacy skills in the NHs group and between language, auditory and early literacy skills in the CIs group. MethodNine prelingually deaf children with CIs (M = 61.11, SD = 6.90) with severe to profound sensorineural hearing loss and nine age-matched NH children participated in the program. Categories of Auditory Performance (CAP) as measures of children's auditory skills were collected. All participants were tested on phonological, morphosyntax (grammatical comprehension and repetition), and early literacy skills (syllable blending and segmentation, syllable and word reading) (T1). Next, all children participated in the computer-based program for 12 weeks. After the program was completed (T2), only early literacy tests were administered to the children. ResultsAlthough, on average, both groups obtained higher scores in all literacy tasks at T2, the CIs group scored lower than the NHs group. In the CIs group, at T2 we found significant improvements in syllable segmentation (p = 0.042) and word reading (p = 0.035). In the NHs group, at T2 we found significant improvements in syllable segmentation (p = 0.034), syllable blending (p = 0.022), syllable reading (p = 0.008), and word reading (p = 0.009). We also found significant associations in both groups between measures of morphosyntax at T1 and measures of early literacy at T2. In addition, for the CIs group, we found significant associations between children's auditory performance at T1 and measures of morphosyntax at T1 and early literacy at T2. Conclusiona computer-based program focused on the syllabic method could support children with CIs in acquiring emergent literacy abilities. The auditory performance of children with CIs seems to influence their morphosyntax and later early literacy skills.

Cochlear reimplantation outcomes over 20 years: Expertise in reimplantation surgery and auditory-speech rehabilitation

ObjectivesThe aim of this study was to present an institution's experience with cochlear reimplantation (CRI), to assess surgical challenges and post-operative outcomes and to increase the success rate of CRI. Study designRetrospective single-institution study. SettingTertiary medical center. MethodsWe retrospectively evaluated data from 76 reimplantation cases treated in a tertiary center between 2001 and 2022. Clinical features including etiology of hearing loss, type of failure, surgical issues, and auditory speech performance were analyzed. Categorical Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were used to evaluate pre- and post-CRI outcomes. ResultsThe CRI population comprises of 7 patients from our institute,69 referred patients from other centers. Device failure was the most common reason (68/76, 89.5 %) for CRI; in addition, there were 7 medical failures and 1 had both soft device failure. Medical failures included flap rupture and device extrusion, magnet migration, auditory neuropathy, leukoencephalopathy, foreign-body residue and meningitis. In 21/76 patients, the electrode technology was upgraded. The mean time to failure was 0.58–13 years, with a mean of 4.97 years. The mean (± SD) CAP and SIR scores before and after CRI were 5.2 ± 1.2 versus 5.5 ± 1.1 and 3.4 ± 1.1 versus 3.5 ± 1.1, respectively. Performance was poor in six patients with severe cochlear malformation, auditory nerve dysplasia, leukoencephalopathy, and epilepsy. ConclusionCRI surgery is a challenging but relatively safe procedure, and most reimplanted patients experience favorable postoperative outcomes. Medical complications and intracochlear damage are the main causes of poor postoperative results. Therefore, adequate preoperative preparation and atraumatic CRI should be carried out for optimal results.

Early auditory processing of speech in prelingually-deafened children with cochlear implants based on event-related potentials

Objective: To investigate the early auditory discrimination of vowels, consonants and lexical tones in prelingually-deafened children with cochlear implants (CI) using auditory event-related potentials. Methods: Nineteen prelingually-deafened CI children and 19 normal hearing (NH) children were recruited in this study. A multi-deviant oddball paradigm was constructed using the monosyllable/ta1/as the standard stimulus and monosyllables/tu1/,/te1/, /da1/,/ra1/,/ta4/and/ta2/as the deviant stimuli. The event-related potentials evoked by vowel, consonant and lexical tone contrasts were recorded and analyzed in the two groups. Results: NH children showed robust mismatch negativities (MMNs) to vowel, consonant and lexical tone contrasts (P<0.05), whereas CI children only showed positive mismatch responses (pMMRs) and P3a responses to the vowel (P<0.05) and consonant contrasts (P<0.05) and no significant event-related potential to the lexical tone contrasts (P>0.05). The longer pMMR and P3a peak latencies (P<0.01) but similar amplitudes (P>0.05) were found in CI children than in NH children. CI children showed weaker phase synchronization of θ oscillations than NH children (P<0.05). The duration of CI use was positively correlated with the scores of Categories of Auditory Performance (CAP) (P=0.004), Speech Intelligibility Rate (SIR) (P=0.044) and Meaningful Auditory Integration Scale (MAIS) (P=0.001) in CI children. Conclusions: Prelingually-deafened CI children can process vowels and consonants at an early stage. However, their ability of processing speech, especially lexical tones, is still more immature compared with their NH peers. The event-related potentials could be objective electrophysiological indicators reflecting the maturity of CI children's auditory speech functions. Long-term CI use is beneficial for prelingually-deafened children to improve auditory and speech performance.

Efficacy of cochlear implantation in cochlear nerve deficiency children – A single center study

PurposesThe objective of this research was to interpret and analyze the imaging, audiological features and cochlear implantation outcomes in cochlear nerve deficiency children. Material and methodsRetrospective analysis. Results25 prelingual hearing loss children diagnosed cochlear nerve deficiency (CND), the age range from 1 to 15 (mean age, 5.04), underwent cochlear implantation at Ear Nose and Throat Hospital - Ho Chi Minh City (ENT hospital – HCMC) from 2016 to 2023. All children had sensorineural hearing loss (SNHL) from severe to profound degree. Magnetic resonance imaging (MRI) showed cochlear nerve hypoplasia in 76 % and cochlear nerve aplasia in 24 % of cases. Inner ear malformations were found in 52 % of cases. The mean Categories of Auditory Performance (CAP) score at 1 year after surgery was 4.8. At 6 months and 1 year after surgery, the mean CAP score of the aplasia group was significantly lower than that of the hypoplasia group (p < 0.05). ConclusionsIn cochlear nerve deficiency children, auditory perception and speech performance still improved after cochlear implantation. However, this progress was significantly limited in cochlear nerve aplasia group.

Criterion Validity of Infant-Toddler Meaningful Auditory Integration Scale for Persian Children with Cochlear Implantation

Introduction: The infant-toddler meaningful auditory integration scale (IT-MAIS) can evaluate toddlers and infants’ early auditory development in aspects of detection, discrimination, and identification of sounds. The present study aims to evaluate the reliability and criterion validity of the Persian version of the IT-MAIS (IT-MAIS-P) for children with hearing loss (HL). Materials and Methods: In this study, due to the limitations of COVID-19 and the lack of access to samples, 23 available children with HL were sampled. The cause of participants› HL was 43.5% congenital, 30.4% acquired, and 26.1% unknown. The Mean±SD of the age of these children was 21.6±6.3 months. To evaluate the predictive validity and concurrent validity, the speech intelligibility rating (SIR) and categories of auditory performance (CAP) were used, respectively. Also, to assess the inter-rater reliability, both parents of each child completed the IT-MAIS-P. Results: A significant correlation (concurrent validity) was observed between the IT-MAIS-P and the CAP scores (r=0.87, P&lt;0.001). Predictive validity was not confirmed by comparing it with the SIR (P&gt;0.05). Also, the inter-rater reliability (kappa=0.44, P&lt;0.001) of the IT-MAIS-P was confirmed. Conclusion: The IT-MAIS-P is a valid tool to investigate the progress of auditory skills in infants, although the predictive validity was not confirmed.

Cerebral cortex functional reorganization in preschool children with congenital sensorineural hearing loss: a resting-state fMRI study.

How cortical functional reorganization occurs after hearing loss in preschool children with congenital sensorineural hearing loss (CSNHL) is poorly understood. Therefore, we used resting-state functional MRI (rs-fMRI) to explore the characteristics of cortical reorganization in these patents. Sixty-three preschool children with CSNHL and 32 healthy controls (HCs) were recruited, and the Categories of Auditory Performance (CAP) scores were determined at the 6-month follow-up after cochlear implantation (CI). First, rs-fMRI data were preprocessed, and amplitude of low-frequency fluctuation (ALFF) and regional homogeneity (ReHo) were calculated. Second, whole-brain functional connectivity (FC) analysis was performed using bilateral primary auditory cortex as seed points. Finally, Spearman correlation analysis was performed between the differential ALFF, ReHo and FC values and the CAP score. ALFF analysis showed that preschool children with CSNHL had lower ALFF values in the bilateral prefrontal cortex and superior temporal gyrus than HCs, but higher ALFF values in the bilateral thalamus and calcarine gyrus. And correlation analysis showed that some abnormal brain regions were weak negatively correlated with CAP score (p < 0.05). The ReHo values in the bilateral superior temporal gyrus, part of the prefrontal cortex and left insular gyrus were lower, whereas ReHo values in the bilateral thalamus, right caudate nucleus and right precentral gyrus were higher, in children with CSNHL than HCs. However, there was no correlation between ReHo values and the CAP scores (p < 0.05). Using primary auditory cortex (PAC) as seed-based FC further analysis revealed enhanced FC in the visual cortex, proprioceptive cortex and motor cortex. And there were weak negative correlations between the FC values in the bilateral superior temporal gyrus, occipital lobe, left postcentral gyrus and right thalamus were weakly negatively correlated and the CAP score (p < 0.05). After auditory deprivation in preschool children with CSNHL, the local functions of auditory cortex, visual cortex, prefrontal cortex and somatic motor cortex are changed, and the prefrontal cortex plays a regulatory role in this process. There is functional reorganization or compensation between children's hearing and these areas, which may not be conducive to auditory language recovery after CI in deaf children.

Cochlear Implantation Outcomes: A 10-Year Single-Surgeon Experience.

Cochlear implantation (CI) is an appropriate management strategy for prelingual hearing loss. Rehabilitation programs are essential in enabling children to cope with devices and acquire language skills. Objectives: This study aimed to assess the hearing outcomes of CIs performed by a single surgeon in Erbil, Kurdistan region of Iraq, and compare them with the results of other CI centers worldwide. This is a prospective study implemented in Erbil from November 1, 2013, to October 30, 2023, on a convenience sample of 161 patients with prelingual hearing loss (HL) who underwent CI by a single surgeon. The following were collected: age at implantation, use of hearing aids before implantation, parent's educational level, and duration of rehabilitation. The effect of the previous variables on Categories of Auditory Performance (CAP) score and speech intelligibility rating (SIR) on the auditory levels of children was assessed by the researcher six months and one year following surgery. Implantation age showed significant associations with the CAP score examined at six months and 12 months post implantation (p-value 0.001). Speech intelligibility rating was also significantly linked to implantation age (p-value 0.001).

THE BENEFITS OF EARLY COCHLEAR IMPLANTATION FOR SPEECH DEVELOPMENT IN CHILDREN WITH USHER SYNDROME: LITERATURE REVIEW

IntroductionUsher syndrome (USH) is a rare autosomal recessive genetic disorder characterized by sensorineural hearing loss (SNHL), vision loss (retinitis pigmentosa), and occasional balance impairment. Depending on the severity and onset of hearing loss and coexisting vestibular dysfunction, USH is divided into three clinical types – USH1, USH2, USH3 – as well as atypical USH which combines features of all these three. The purpose of this review is to present the impact of cochlear implantation on speech development in children diagnosed with all types of Usher syndrome.Material and methodsAll relevant publications published in from 2013 to 2023 were retrieved from PubMed based on the keywords &lt;i&gt;Usher syndrome&lt;/i&gt;, &lt;i&gt;Usher syndrome diagnostics&lt;/i&gt;, &lt;i&gt;Usher syndrome hearing loss&lt;/i&gt;, &lt;i&gt;Usher syndrome cochlear&lt;/i&gt;, and &lt;i&gt;Usher syndrome speech&lt;/i&gt;. Exactly 67 papers were selected.ResultsBilateral cochlear implantation in children with Usher syndrome is beneficial for audiological and verbal development provided that hearing loss is detected early and implantation done promptly. In USH1 the preferred age is before 3 in the case of severe to profound congenital SNHL; in USH2 and USH3 the optimal time for implantation is hampered by the difficulty of estimating when hearing loss occurred and its rate of progression. After bilateral cochlear implantation, studies showed improvements in the categories of auditory performance (CAP), speech intelligibility rate (SIR), meaningful auditory integration scale (MAIS), meaningful use of speech scale (MUSS), and speech reception score (SRS), together with good development of speech perception and verbal communication.ConclusionsEarly diagnosis (before the onset of vision loss) and early bilateral cochlear implantation in children who have suffered severe to profound SNHL due to Usher syndrome reduces disability and maximizes auditory–oral communication skills, significantly increasing their quality of life.

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.

Autism's Impact on Cochlear Implantation Surgery Outcomes in Deaf Children.

Cochlear implants (CI) provide a hearing sense for severe to profound hearing-impaired patients, both adults and children, and they are a broadly effective and accepted therapeutic method for those patients. Also, Deaf children with comorbidities, including autism spectrum disorders (ASDs), undergo cochlear implantation. ASDs are a group of developing disorders characterized by abnormalities in social interaction and communication with limited repetitive patterns of behavior. This study aimed to assess the effect of Autism on CI surgery outcomes in Deaf Children. We followed 12 autistic patients with cochlear implantation and 12 non-autistic cochlear-implanted patients for two years. The Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were used to assess 6, 12, and 24 months after cochlear implantation surgery. During the 24-month follow-up, the CAP means scores increased in both groups, and SIR and CAP progresses were considerably greater in non-ASD children (P<0.001). However, in ASD children, the progress of CAP and SIR variables were significant, with 99% and 95% confidence, respectively, at 24 months after surgery. Although the CIs could improve hearing performance in autistic patients, speech development after CIs in autistic children could affected by several factors, including the severity of autism, and this can be effective in providing pre-implant counseling to parents. The application of the alternative communication methods could be taken into account as a potential rehab technique.

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.

Cochlear implantation in older adults: 15 years’ experience of a tertiary centre in a developing country

ABsTRACT Introduction Cochlear implant (CI) is a viable option of treatment for older patients with severe to profound deafness in resource-rich countries. Implantation is limited in developing countries. Objective To review factors and outcomes of elderly patients that underwent CI in a pioneer centre in a developing country. Material and methods An observational retrospective review of patients older than 60, implanted between 2005 to 2020. Results Eleven patients were included. Patient were aged 60–74 years old with median of 66 years old. Average duration of deafness prior to implantation is 22 years. All implantation was unilateral except for one case that was implanted sequentially after 5 years Three patients were privately funded. Analysis of the hearing aided level with CI and hearing aid showed substantial improvement provided by the CI. The Categories of Auditory performance (CAP-II) scale were in the range of 6–9. Discussion Cochlear implant is safe and stable intervention in providing improvement of hearing and self-esteem in the elderly patients. Social isolation and depression also improved with better hearing and communication. Conclusion Awareness of the CI in elderly patients must be advocated among policy- makers, clinicians, and patients to mitigate the clinical and public health burden of hearing loss among older patients.

Machine learning-based prediction of the outcomes of cochlear implantation in patients with inner ear malformation.

The objectives of this study are twofold: first, to visualize the structure of malformed cochleae through image reconstruction; and second, to develop a predictive model for postoperative outcomes of cochlear implantation (CI) in patients diagnosed with cochlear hypoplasia (CH) and incomplete partition (IP) malformation. The clinical data from patients diagnosed with cochlear hypoplasia (CH) and incomplete partition (IP) malformation who underwent cochlear implantation (CI) at Beijing Tongren Hospital between January 2016 and August 2020 were collected. Radiological features were analyzed through 3D segmentation of the cochlea. Postoperative auditory speech rehabilitation outcomes were evaluated using the Categories of Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR). This study aimed to investigate the relationship between cochlear parameters and postoperative outcomes. Additionally, a predictive model for postoperative outcomes was developed using the K-nearest neighbors (KNN) algorithm. In our study, we conducted feature selection by using patients' imaging and audiological attributes. This process involved methods such as the removal of missing values, correlation analysis, and chi-square tests. The findings indicated that two specific features, cochlear volume (V) and cochlear canal length (CDL), significantly contributed to predicting the outcomes of hearing and speech rehabilitation for patients with inner ear malformations. In terms of hearing rehabilitation, the KNN classification achieved an accuracy of 93.3%. Likewise, for speech rehabilitation, the KNN classification demonstrated an accuracy of 86.7%. The measurements obtained from the 3D reconstruction model hold significant clinical relevance. Despite the considerable variability in cochlear morphology across individuals, radiological features remain effective in predicting cochlear implantation (CI) prognosis for patients with inner ear malformations. The utilization of 3D segmentation techniques and the developed predictive model can assist surgeons in conducting preoperative cochlear structural measurements for patients with inner ear malformations. This, in turn, can offer a more informed perspective on the anticipated outcomes of cochlear implantation.

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.

Exploring cross-modal plasticity in the auditory-visual cortex post cochlear implantation: implications for auditory and speech function recovery and mechanisms.

The aim of this is to explore changes in cross-modal reorganization within the auditory-visual cortex after cochlear implantation, examining their influence on auditory and speech functions along with their underlying mechanisms. Twenty prelingually deaf children who received cochlear implantation and rehabilitation training at our hospital between February 2022 and February 2023 comprised the prelingual deaf group. Simultaneously, 20 healthy children served as the control group. The prelingual deaf group underwent brain cortical activity assessment and evaluation of auditory-speech recovery pre-surgery, at postoperative weeks 1 and 2, and at months 1, 3, 6, 9, and 12. The control group underwent parallel assessments and evaluations. We analyzed the correlation between cortical activity in the auditory-visual cortex of patients and their auditory-speech functional recovery. The group with prelingual deafness displayed elevated levels of auditory and visual cortical electromagnetic intensity compared to the control group, both prior to and 9 months after surgery. However, by the 12-month mark post-surgery, there was no discernible distinction between the two groups. Following surgery, the prelingually deaf group exhibited a progressive improvement in both Categories of Auditory Performance (CAP) and Speech Intelligibility Rate (SIR), initially lagging behind the control group. Notably, a negative correlation emerged between auditory and visual cortical electromagnetic intensity values and CAP/SIR scores at the 12-month post-surgery assessment. Cochlear implantation in prelingually deaf children results in elevated activity within the auditory and visual cortices, demonstrated by heightened electromagnetic intensity readings. Cross-modal reorganization is observed temporarily at 3 months post-surgery, which resolves to baseline levels by 12 months post-surgery. This phenomenon of reversal correlates with the restoration of auditory and speech functions in these children.

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.