Outcomes Of Cochlear Implantation Research Articles

Safety and Early Outcomes of Cochlear Implantation of Nucleus Devices in Infants: A Multi-Centre Study.

This multi-center study examined the safety and effectiveness of cochlear implantation of children between 9 and 11 months of age. The intended impact was to support practice regarding candidacy assessment and prognostic counseling of pediatric cochlear implant candidates. Data in the clinical chart of children implanted at 9-11 months of age with Cochlear Ltd devices at five cochlear implant centers in the United States and Canada were included in analyses. The study included data from two cohorts implanted with one or two Nucleus devices during the periods of January 1, 2012-December 31, 2017 (Cohort 1, n = 83) or between January 1, 2018 and May 15, 2020 (Cohort 2, n = 50). Major adverse events (requiring another procedure/hospitalization) and minor adverse events (managed with medication alone or underwent an expected course of treatment that did not require surgery or hospitalization) out to 2 years post-implant were monitored and outcomes measured by audiometric thresholds and parent-reports on the IT-MAIS and LittlEARS questionnaires were collected. Results revealed 60 adverse events in 41 children and 227 ears implanted (26%) of which 14 major events occurred in 11 children; all were transitory and resolved. Improved hearing with cochlear implant use was shown in all outcome measures. Findings reveal that the procedure is safe for infants and that they show clear benefits of cochlear implantation including increased audibility and hearing development.

Auditory and speech outcomes of cochlear implantation in patients with Waardenburg syndrome: a meta-analysis.

This study aims to assess the potential efficacy of cochlear implantation as a treatment for patients with Waardenburg syndrome (WS) and to guide clinical work by comparing the effect of auditory and speech recovery after cochlear implantation in patients with WS and non-WS. PubMed, the Cochrane Library, CNKI, and Wanfang Data were sources for retrieving literature on cochlear implantation in WS, and clinical data meeting the inclusion criteria were meta-analyzed using RevMan5.41. A total of nine articles were included in this study, including 132 patients with WS and 815 patients in the control group. Meta-analysis showed that there are no significant differences in the scores for categories of audit performance (CAP), speech intelligibility rating (SIR), and parents' evaluation of aural/oral performance of children (PEACH) between the WS group and the control group. Cochlear implantation demonstrates comparable auditory and speech recovery outcomes for WS patients and non-WS patients.

Enhancing cochlear duct length estimation by incorporating second-turn parameters

Estimating insertion depth, cochlear duct length (CDL), and other inner ear parameters is vital to optimizing cochlear implantation outcomes. Most current formulas use only the basal turn dimensions for CDL prediction. In this study, we investigated the importance of the second turn parameters in estimating CDL. Two experienced neuro-otologists blindly used segmentation software to measure (in mm) cochlear parameters, including basal turn diameter (A), basal turn width (B), second-turn diameter (A2), second-turn width (B2), CDL, first-turn length, and second-turn length (STL). These readings were taken from 33 computed tomography (CT) images of temporal bones from anatomically normal ears. We constructed regression models using A, B, A2, and B2 values fitted to CDL, two-turn length, and five-fold cross-validation to ensure model validity. CDL, A value, and STL were longer in males than in females. The mean B2/A2 ratio was 0.91 ± 0.06. Adding A2 and B2 values improved CDL prediction accuracy to 86.11%. Therefore, we propose a new formula for more accurate CDL estimation using A, B, A2, and B2 values. In conclusion, the findings of this study revealed a notable improvement in the prediction of two-turn length (2TL), and CDL by clinically appreciable margins upon adding A2 and B2 values to the prediction formulas.

Cochlear Implant Outcomes: Quality of Life in Prelingually Deafened, Late-Implanted Patients

Cochlear Implant Outcomes: Quality of Life in Prelingually Deafened, Late-Implanted Patients

An Investigation of the Impact of Expanding High-Deductible Health Plans on Patient Decision for Cochlear Implant Surgery and Postoperative Outcomes

HypothesisAfter the expansion of high deductibles, patients will delay cochlear implant (CI) surgery to the end of the year, and the risk of postoperative known risks will increase.BackgroundThe Affordable Care Act was associated with increased enrollment in high-deductible health plans (HDHPs), which resulted in rising health insurance deductibles. Health insurance plans can cover a patient's cost of healthcare once the deductible is met. Patients have been shown to be economic rational decision makers and make decisions based on cost rather than health. They wait for their deductible to be met, typically at the end of the year, then proceed to have costly care. The goal of this study was to evaluate the impact of rising health insurance deductibles on the rate and postoperative outcomes of cochlear implantation and to assess changes by the Tax Cuts and Jobs Act.MethodsTriNetX was used to accumulate summary data on patients who obtained a CI between 2005 and 2022 at the beginning (quarter 1) and the end of the year (quarter 4) from the electronic medical records of 75 healthcare organizations. The trends in average rate of cochlear implantation and resultant postoperative known risks or complications were statistically evaluated.ResultsAfter expansion of HDHPs, the rate of cochlear implantation between quarter 4 (19 cases per year) and quarter 1 (17 cases/year) was similar (p= 0.18). For all patient groups, the case volume significantly increased. Between quarter 4 and quarter 1, postoperative tinnitus was more common in the beginning of the year (risk ratio, 0.68; 95% confidence interval, 0.46–0.99).ConclusionsThe number of patients receiving CIs significantly increased despite the expansion of HDHPs. Tinnitus was a rare postoperative known risk in the beginning of the year. Patients are less likely concerned about cost of CI surgery because of the impact of hearing loss on quality of life.

NADH improves AIF dimerization and inhibits apoptosis in iPSCs-derived neurons from patients with auditory neuropathy spectrum disorder

Auditory neuropathy spectrum disorder (ANSD) is a hearing impairment involving disruptions to inner hair cells (IHCs), ribbon synapses, spiral ganglion neurons (SGNs), and/or the auditory nerve itself. The outcomes of cochlear implants (CI) for ANSD are variable and dependent on the location of lesion sites. Discovering a potential therapeutic agent for ANSD remains an urgent requirement. Here, 293T stable transfection cell lines and patient induced pluripotent stem cells (iPSCs)-derived auditory neurons carrying the apoptosis inducing factor (AIF) p.R422Q variant were used to pursue a therapeutic regent for ANSD. Nicotinamide adenine dinucleotide (NADH) is a main electron donor in the electron transport chain (ETC). In 293T stable transfection cells with the p.R422Q variant, NADH treatment improved AIF dimerization, rescued mitochondrial dysfunctions, and decreased cell apoptosis. The effects of NADH were further confirmed in patient iPSCs-derived neurons. The relative level of AIF dimers was increased to 150.7 % (P = 0.026) from 59.2 % in patient-neurons upon NADH treatment. Such increased AIF dimerization promoted the mitochondrial import of coiled-coil-helix-coiled-coil-helix domain-containing protein 4 (CHCHD4), which further restored mitochondrial functions. Similarly, the content of mitochondrial calcium (mCa2+) was downregulated from 136.7 % to 102.3 % (P = 0.0024) in patient-neurons upon NADH treatment. Such decreased mCa2+ levels inhibited calpain activity, ultimately reducing the percentage of apoptotic cells from 30.5 % to 21.1 % (P = 0.021). We also compared the therapeutic effects of gene correction and NADH treatment on hereditary ANSD. NADH treatment had comparable restorative effects on functions of ANSD patient-specific cells to that of gene correction. Our findings offer evidence of the molecular mechanisms of ANSD and introduce NADH as a potential therapeutic agent for ANSD therapy.

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.

The Preliminary Study of the Effects of Cochlear Implantation on Developmental Outcome in Thai Children

Background: Children born with severe or profound hearing loss usually have delays in receptive and expressive language development that affect academic achievement and social functioning. Cochlear implantation is the treatment of choice for these children. There were very few studies on developmental outcome of the children with severe sensory neural hearing loss who had gone through cochlear implantation. Objective: 1) To assess developmental outcome of cochlear implantation in children aged 9 to 66 months. 2) To study attitude and Parenting Styles and Dimensions Questionnaire (PSDQ) of the parents. Materials and Methods: A cross-sectional descriptive study of 27 children aged 9 to 66 months who received cochlear implantation at Rajavithi Hospital. The data were collected online using the Ages &amp; Stages Questionnaires, Third Edition, Thai-Version (ASQ-3 Thai), Parents’ attitudes and PSDQ, Thai version. Results: The age of cochlear implant was 30.52±12.22 months with 29.6% of the patients getting the cochlear implantation before two years of age. There was 70%, and 37% of children suspected of having delays in language and problem solving. Relationship between language development with age at diagnosis of hearing impairment, age of receive hearing aids, age of auditory-verbal therapy, age of cochlear implantation, and duration of auditory verbal training after cochlear implantation were statistically significant. There was a significant relationship between problem solving and reading with children family type. Most parenting style was authoritative. Parental knowledge and attitudes were very good at 96.3%. Conclusion: Children with severe sensory neural hearing loss who received early diagnosis and cochlear implantation at the age younger than 2 years old, as well as long consecutive auditory and speech skills training, results in better language developmental outcomes. Keywords: Cochlear implantation; Development outcome; Language; Sensory neural hearing loss

Evolving a Model for Cochlear Implant Outcome.

Cochlear implantation is an efficient treatment for postlingually deafened adults who do not benefit sufficiently from acoustic amplification. Implantation is indicated when it can be foreseen that speech recognition with a cochlear implant (CI) is superior to that with a hearing aid. Especially for subjects with residual speech recognition, it is desirable to predict CI outcome on the basis of preoperative audiological tests. The purpose of the study was to extend and refine a previously developed model for CI outcome prediction for subjects with preoperative word recognition to include subjects with no residual hearing by incorporating additional results of routine examinations. By introducing the duration of unaided hearing loss (DuHL), the median absolute error (MAE) of the prediction was reduced. While for subjects with preoperative speech recognition, the model modification did not change the MAE, for subjects with no residual speech recognition before surgery, the MAE decreased from 23.7% with the previous model to 17.2% with the extended model. Prediction of word recognition with CI is possible within clinically relevant limits. Outcome prediction is particularly important for preoperative counseling and in CI aftercare to support systematic monitoring of CI fitting.

Two Discrete Types of Tip Fold-Over in Cochlear Implantation Using Slim Modiolar Electrodes: Influence of Cochlear Duct Length on Tip Fold-Over.

Precise electrode positioning is crucial for achieving optimal audiological outcomes in cochlear implantation. The slim modiolar electrode (SME), a thin, flexible, and precurved electrode, exhibits favorable modiolar proximity. However, tip fold-over can affect optimal electrode placement. Herein, we share our experiences with tip fold-over in SMEs and present an analysis of conditions that may predispose to tip fold-over. Retrospective medical record review. In total, 475 patients (671 ears) underwent cochlear implantation using SMEs (Nucleus CI532 or CI632 from Cochlear) performed by a single surgeon at a tertiary center between June 14, 2018, and December 1, 2022. Intraoperative x-ray scans (cochlear view), operative records, and cochlear duct length (CDL) were reviewed. Tip fold-over patterns on plain x-ray images (proximal versus distal). Electrode tip fold-over was observed in 18 (2.7%) of the 671 ears with SMEs. This fold-over occurred more frequently in cases with long CDL (>36 mm). Among the 14 cases with available initial x-rays before correction of the tip fold-over, half were classified as proximal and the other half as distal. A predilection for proximal tip fold-over was found in those with a CDL of 36 mm or longer, and longer CDLs were observed for proximal cases than for distal cases. Our pilot data suggest that identifying the type of tip fold-over can aid in correcting it more efficiently. Tip fold-over of SME does not occur uniformly and is more common in ears with long CDL. This tendency is particularly pronounced for the proximal type of tip fold-over. Therefore, preoperative measurement of the CDL and meticulous examination of intraoperative imaging are essential for customized correction.

Long-Term Outcome of Cochlear Implantation in Children With Congenital, Perilingual, and Postlingual Single-Sided Deafness.

We investigated the long-term outcomes of children with single-sided deafness (SSD) after cochlear implant (CI) surgery, during and after rehabilitation, and compared the results of children with congenital, perilingual, and postlingual SSD. We evaluated the impact of SSD at age at onset and duration of deafness on their performance. Thirty-six children with SSD treated with CI participated in the study: 20 had congenital, seven perilingual (defined: >0 to 4 years), and nine had postlingual deafness (defined as >4 years of age). Their outcome with CI were measured on both subjective and objective scales: duration of device use, speech intelligibility in noise and in quiet, bilateral hearing and localization ability, quality of life and hearing, presence and loudness of tinnitus, and hearing ability of the better hearing ear. After a mean follow-up time of 4.75 years, 32 of the 36 children used their CI on a regular basis. The remaining four children were nonusers. These children had congenital SSD and were older than three years at the time of CI surgery. Overall, for congenital/perilingual and postlingual SSD, speech intelligibility in noise and the Speech, Spatial and Qualities of Hearing Scale (SSQ) speech subscore were significantly improved, as were their subjective and objective localization ability and hearing-related quality of life. Children with postlingual SSD benefited from the CI with regard to speech intelligibility, SSQ speech/spatial/total score, and localization error, and children with congenital SSD showed better results with a short duration of deafness of less than 3 years compared with those with a longer deafness period. Cochlear implantation is a successful treatment for children with congenital/perilingual or postlingual SSD. Results largely differed with respect to the onset and duration of deafness, and better outcomes were achieved by children with postlingual SSD and with a short duration of deafness. Our data also confirmed that children with congenital SSD should be implanted with a CI within three years of age.

Implementation of the International Classification of Functioning, Disability and Health model in cochlear implant recipients: a multi-center prospective follow-up cohort study

IntroductionNo standard protocol based on a multidisciplinary framework currently exists for the evaluation, follow-up and interpretation of Cochlear Implant (CI) outcomes in clinical routine. Therefore, this study aimed to develop and clinically implement a multidimensional Cochlear Implant (CI) outcome assessment protocol for adult CI users based on the International Classification of Functioning, Disability and Health (ICF).MethodsAn international multidisciplinary core group of CI experts selected the most relevant ICF categories and codes for adult CI users out of the existing ICF core set for hearing loss. A well-known, commonly used instrument or method was identified and assigned to each ICF category along with quantified ICF qualifiers. These qualifiers facilitate the interpretation of CI outcomes by categorizing the outcome on a rating scale between 0 and 4, based on the severity of participants' impairment/restriction/limitation/barrier. The ICF-based CI outcome assessment protocol was applied in clinical routine in a prospective longitudinal multicenter study assessing a consecutive sample of 72 adult CI candidates before and 6 months after implantation. For each relevant ICF category, the participants' improvement, stability or deterioration was specified in a descriptive approach using ICF qualifiers.ResultsParticipants either showed improved or stable ICF qualifiers after cochlear implantation across all ICF domains (e.g., Body Functions and Structures, Activities, and Participation and Environmental factors). The ICF qualifiers for environmental sound tolerance (n = 30 [45%]) and vestibular functions (n = 21 [33%]) deteriorated most frequently after implantation. ICF qualifiers for falling (n = 53 [83%]) and dizziness (n = 40 [62%]) remained stable in most participants. Auditory perception-related and communication-related categories generally improved in most participants after cochlear implantation, with 46 to 73% (n = 34 to n = 48) of CI users perceiving an improvement postoperatively.ConclusionThis study is the first to propose and apply a ready-to-use CI outcome assessment protocol based on the ICF model, which provides a common language and comprehensive assessment protocol for the description and measurement of CI outcomes worldwide. Its straightforward outcome description and rating enables CI outcome interpretation by non-experts, enhancing multidisciplinary communication and knowledge on individual healthcare needs in CI users.

Parents' Perspectives on the Outcome of Cochlear Implantation for the Deaf Child and the Family in Saudi Arabia.

The researchers investigated parents' perspectives on the outcome of cochlear implantation on the deaf child and the family in Saudi Arabia with respect to linguistic, social, psychological, and educational aspects. They also explored potential factors influencing parents' perspectives on the outcome of a cochlear implant (CI). Seventy-seven parents completed the questionnaire, and multiple linear regression and descriptive statistics were used to answer the research questions. Most of the parents (88.5%) reported choosing a CI for their deaf child because they wanted their child to be part of the hearing world. About half of the parents (49.4%) expressed the belief that the CI would help their child find a better job in the future. The study also found that, overall, the model was not significant, and the independent variables explained little of the variance in parents' perspectives on the outcome of their child's cochlear implantation.

Outcomes of Cochlear Implantation in Age Subgroups of Veterans Over 50 Years Old.

This study aimed to determine whether there was a difference in postoperative Arizona Biomedical (AzBio) speech recognition scores in 2 differently aged subgroups of veterans 50 years and older after cochlear implantation (CI). Retrospective chart review. Tertiary referral center. Seventy-one patients aged 50 to 74 years (younger cohort) were compared with 56 patients aged 75 years and older (older cohort) at the time of CI. Patients underwent therapeutic CI. Comparison of AzBio speech recognition test scores in a quiet environment between the 2 differently aged cohorts of veterans 50 years and older. Despite no significant differences in preoperative AzBio score between the younger (mean 22.2%) and older cohorts (mean, 17.3%; P > 0.05), when examining the 6- and 12-month postoperative time points, the older cohort had significantly lower mean AzBio scores (50%, 55.8%; P < 0.05) than the younger cohort (69.8%, 71.9%; P < 0.05). All patients aged >50 years experienced significant improvement in speech recognition scores following CI, although the cohort of ages 50 to 74 years scored significantly higher in later follow-up visits. These findings suggest that CI should be offered to appropriate candidates, regardless of age, although earlier intervention may be more advantageous.

An Umbrella Review of Cochlear Implant Outcomes in Children With Auditory Neuropathy.

The objective of this overview of systematic reviews (SRs; umbrella review) was to systematically summarize and critically appraise current evidence of cochlear implant (CI) outcomes in children with auditory neuropathy spectrum disorder (ANSD). This study was guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 statement. The methodological quality and the risk of bias in the included SRs were assessed using A MeaSurement Tool to Assess systematic Reviews 2 checklist and the Risk of Bias in Systematic Reviews tool, respectively. According to eight included SRs, children with ANSD achieve CI outcomes (speech perception performance) similar to their peers with sensorineural hearing loss. In children with postsynaptic ANSD (cochlear nerve deficiency), cochlear nerve hypoplasia is associated with better speech recognition outcomes compared with cochlear nerve aplasia, especially in the absence of additional disabilities. Except for one study, the overall quality of the included SRs was critically low, and except for three studies, evidence of a high risk of bias was identified in other included SRs. Current evidence supports CI benefits for children with ANSD. To improve the quality of evidence, well-designed, prospective studies with appropriate sample sizes, using valid outcome measures, clarifying matching criteria, and taking into account the role of confounding factors are essential.

Cochlear implant: factors that impact hearing outcomes

Background: Cochlear implant has been providing hearing rehabilitation to patients with severe hearing loss. However, not all patients achieve optimal results, and the goal of this study was to evaluate the factors that influence hearing outcomes in cochlear implantation. Methods: This was a retrospective analysis of the patients who underwent cochlear implantation in our institution between 2018 and 2021. Review of clinical files and complementary tests, and analysis of hearing outcomes and complications were performed. Results: Sixty-nine cochlear implants were placed in 64 patients, with an average age of 49.3 years. In initial evaluation post activation and 1 year after implantation, the average pure tone average (PTA) was 37 dB, with a vocal discrimination of 48.7% at 50 dB, and 28, 6 dB, with a discrimination of 73.8% at 50 dB, respectively. Patients with postlingual deafness had better hearing outcomes at 1 year post surgery, in both PTA (27.9 compared to 33 dB) and discrimination at 50 dB (76.5 compared to 56.1%). Only one case had a major complication. Conclusions: In conclusion, we found that increased implant use by the patient, postlingual deafness, and in-creased surgeon experience, were factors that improved hearing outcomes in patients undergoing cochlear implantation.

"Of mice and men": the relevance of Cometin and Erythropoietin origin for its effects on murine spiral ganglion neuron survival and neurite outgrowth in vitro.

Neurotrophic factors (NTF) play key roles in the survival of neurons, making them promising candidates for therapy of neurodegenerative diseases. In the case of the inner ear, sensorineural hearing loss (SNHL) is characterized over time by a degeneration of the primary auditory neurons, the spiral ganglion neurons (SGN). It is well known that selected NTF can protect SGN from degeneration, which positively influences the outcome of cochlear implants, the treatment of choice for patients with profound to severe SNHL. However, the outcome of studies investigating protective effects of NTF on auditory neurons are in some cases of high variability. We hypothesize that the factor origin may be one aspect that affects the neuroprotective potential. The aim of this study was to investigate the neuroprotective potential of human and mouse Erythropoietin (EPO) and Cometin on rat SGN. SGN were isolated from neonatal rats (P 2-5) and cultured in serum-free medium. EPO and Cometin of mouse and human origin were added in concentrations of 0.1, 1, and 10 ng/mL and 0.1, 1, and 10 μg/mL, respectively. The SGN survival rate and morphology, and the neurite outgrowth were determined and compared to negative (no additives) and positive (brain-derived neurotrophic factor, BDNF) controls. A neuroprotective effect of 10 μg/mL human Cometin comparable to that obtained with BDNF was observed in the SGN-culture. In contrast, mouse Cometin was ineffective. A similar influence of 10 μg/mL human and mouse and 1 μg/mL human Cometin on the length of regenerated neurites compared to BDNF was also detected. No other Cometin-conditions, and none of the EPO-conditions tested had neuroprotective or neuritogenic effects or influenced the neuronal morphology of the SGN. The neuroprotective effect of 10 μg/mL human Cometin on SGN indicates it is a potentially interesting protein for the supportive treatment of inner ear disorders. The finding that mouse Cometin had no effect on the SGN in the parallel-performed experiments underlines the importance of species origin of molecules being screened for therapeutic purpose.

Hearing Rehabilitation in Patients With Neurofibromatosis Type 2: The Quebec's Experience With Auditory Implants.

To review a single-center experience with hearing rehabilitation in patients with neurofibromatosis type 2 (NF2) and to describe the auditory outcomes of cochlear implants (CIs) and auditory brainstem implants (ABI) in this population. Retrospective case series. Tertiary referral center. Twelve adult patients with NF2 who received a CI (n = 10) and/or an ABI (n = 5) between 2000 and 2021. Insertion of a CI and/or an ABI in NF2 patients with bilateral vestibular schwannomas (VSs). Patients' demographic data, treatment history, hearing evolution, hearing rehabilitation methods, implant details, and auditory outcomes after implantation. Among those who received a CI, five patients had a stable untreated VS, one patient underwent a cochlear nerve preserving surgery, and four patients received radiotherapy treatments. Six patients became regular users of their device. The median open-set sentence recognition scores were as follows: 0.5% preoperatively, 60% at 1 year postoperatively, and 80% on the most recent audiological evaluation. All patients with an ABI were implanted concomitantly with VS surgical excision, and three of them also received radiotherapy treatments. The median open-set sentence recognition scores were as follows: 4% preoperatively, 26% at 1 year postoperatively, and 0% on the most recent evaluation. Three patients became regular ABI users. Despite major technological advances, auditory outcomes with ABIs remain deceiving. Considering the overall improvement in postoperative auditory performances provided by CIs compared with ABIs, cochlear implantation standouts as a primary mean of hearing rehabilitation in NF2 patients.

Causes of hearing loss and implantation age in a cohort of Danish pediatric cochlear implant recipients.

Sensorineural hearing loss (SNHL) is the most common birth disorder. The cause of SNHL is heterogeneous and varies in different populations. Understanding the causes of a hearing loss (HL) predict the outcome of cochlear implantation and is of great importance in understanding the mechanism of the disease and in providing the best treatment. Undiagnosed and untreated HL has a profound effect on the acquisition of early communication skills, speech, language, academic, emotional, and psychosocial development in children. To determine the cause of HL and implantation age in pediatric cochlear implant (CI) users in a Danish population. Data of 100 children (54 females and 46 males), age 0-17 years, was analyzed. All of the children were implanted during 2020-2022. Hereditary HL was diagnosed in 44 cases (44%), with pathogenic variants in the SLC26A4 gene found in 14 cases (14%). Syndromic HL was diagnosed in 23 children (23%). Non-syndromic HL was diagnosed in 21 children (21%), where the most common genetic variation was found in the GJB2 gene. Acquired prenatal and postnatal sensory disorders TORCH risk factors were associated with HL in 25 cases (25%). Congenital CMV DNA was diagnosed in 23 samples (23%). The cause of the HL remained unknown for 31 (31%) children. In 70 (70%) of the participants the HL was diagnosed at time of newborn hearing screening (NHS). Twenty-three of the children were diagnosed with congenital severe to profound bilateral HL and were simultaneously implanted between 8 and 14 months (mean age 10.5 months). In the remaining 47 cases, the HL was progressive and the children were implanted when the HL reached the criteria for implantation. In the current study, the major causes of HL were alterations in the SLC26A4 gene: 13% with Pendred syndrome and 1% non-syndromic. Thirty-one (31%) had HL of unknown origin and almost half of these cases had inner ear malformations (n=16).

Cochlear Implant Outcomes between Patients with Sporadic and Neurofibromatosis Type 2-Associated Vestibular Schwannoma.

Compare cochlear implant (CI) performance between patients with ipsilateral sporadic vestibular schwannoma (VS) and NF2-related schwannomatosis (NF2). Compare CI performance according to VS management modality. Historical cohort. Tertiary academic center. Forty-nine patients (52 ears) undergoing cochlear implantation in the setting of ipsilateral sporadic (n = 21) or NF2-associated VS (n = 28). CI ipsilateral to VS. Auditory thresholds, consonant-nucleus-consonant (CNC) word scores, and AzBio sentences in quiet scores. Among all patients, median post-CI pure tone average was 28 dB HL (interquartile range [IQR], 21-38), CNC word score was 39% (IQR, 6-62), and AzBio sentences in quiet score was 60% (IQR, 11-83) at a median of 12.5 months postimplantation. Despite the NF2 cohort having larger tumors, when comparing patients with sporadic versus NF2-associated VS, there were no statistically significant differences in CNC word (49% [30-70] vs. 31% [0-52]) or AzBio sentences in quiet (66% [28-80] vs. 57% [5-83]) scores. Regardless of NF2 status, all patients managed with observation, and radiosurgery achieved open-set speech. In patients who underwent microsurgery, 6 (46%) of 13 with NF2 achieved open-set speech recognition compared with 4 (67%) of 6 with sporadic disease. Select patients with VS achieve successful hearing rehabilitation with a CI. In this cohort, tumor management strategy significantly influenced CI performance, whereas differences in NF2 status exhibited less effect. Specifically, all patients managed with observation or radiosurgery achieved open-set speech perception, whereas approximately half of people with NF2-related VS and two-thirds of people with sporadic VS achieved this outcome after tumor microsurgery. When disease permits, observation and radiosurgery should be considered in patients who may later pursue a CI.