Cochlear Implantation Outcome in Children with DFNB1 locus Pathogenic Variants.
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ABSTRACT:
SUBMITTER: Ozieblo D
PROVIDER: S-EPMC7019930 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Background and Objectives: Many otologists face a dilemma in the decision-making process of surgical management of patients with cochlear nerve (CN) aplasia. The goal of this study is to provide fresh evidence on cochlear implantation (CI) results in patients with CN aplasia. Materials and Methods: We scrutinized functional outcomes in 37 ears of 21 children with bilateral CN aplasia who underwent unilateral or bilateral CI based on cross-sectional and longitudinal assessments. Results: The Categories of Auditory Performance (CAP) scores gradually improved throughout the 3-year follow-up; however, variable outcomes existed between individuals. Specifically, 90% of recipients with a 1-year postoperative CAP score ≤1 could not achieve a CAP score over 1 even at 3-year postoperative evaluation, while the recipients with a 1-year postoperative CAP score >1 had improved auditory performance, and 72.7% of them were able to achieve a CAP score of 4 or higher. Meanwhile, intraoperative electrically evoked compound action potential was not correlated with postoperative CAP score. Conclusions: Our results further refine previous studies on the clinical feasibility of CI as the first treatment modality to elicit favorable auditory performance in children with CN aplasia. However, special attention should be paid to pediatric patients with an early postoperative CAP score ≤1 for identification of unsuccessful cochlear implants and switching to auditory brainstem implants.
| S-EPMC9607978 | biostudies-literature
Project description:Cochlear implantation is a surgical alternative to traditional amplification (hearing aids) that can facilitate spoken language development in young children with severe to profound sensorineural hearing loss (SNHL).To prospectively assess spoken language acquisition following cochlear implantation in young children.Prospective, longitudinal, and multidimensional assessment of spoken language development over a 3-year period in children who underwent cochlear implantation before 5 years of age (n = 188) from 6 US centers and hearing children of similar ages (n = 97) from 2 preschools recruited between November 2002 and December 2004. Follow-up completed between November 2005 and May 2008.Performance on measures of spoken language comprehension and expression (Reynell Developmental Language Scales).Children undergoing cochlear implantation showed greater improvement in spoken language performance (10.4; 95% confidence interval [CI], 9.6-11.2 points per year in comprehension; 8.4; 95% CI, 7.8-9.0 in expression) than would be predicted by their preimplantation baseline scores (5.4; 95% CI, 4.1-6.7, comprehension; 5.8; 95% CI, 4.6-7.0, expression), although mean scores were not restored to age-appropriate levels after 3 years. Younger age at cochlear implantation was associated with significantly steeper rate increases in comprehension (1.1; 95% CI, 0.5-1.7 points per year younger) and expression (1.0; 95% CI, 0.6-1.5 points per year younger). Similarly, each 1-year shorter history of hearing deficit was associated with steeper rate increases in comprehension (0.8; 95% CI, 0.2-1.2 points per year shorter) and expression (0.6; 95% CI, 0.2-1.0 points per year shorter). In multivariable analyses, greater residual hearing prior to cochlear implantation, higher ratings of parent-child interactions, and higher socioeconomic status were associated with greater rates of improvement in comprehension and expression.The use of cochlear implants in young children was associated with better spoken language learning than would be predicted from their preimplantation scores.
| S-EPMC3073449 | biostudies-literature
Project description:ObjectivesTo determine the breadth of outcome domains used in the reporting of adult cochlear implant surgery for the purpose of registry and quality measure development.Data sourcesSystematic review of randomized controlled trials.Review methodsIn consultation with a medical librarian, search strategies were constructed to identify randomized controlled trials studying adults undergoing cochlear implantation. MEDLINE, EMBASE, Scopus, CINAHL, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, and the Database of Abstracts of Reviews of Effects were searched from database inception to July 2015. Studies were evaluated for level of evidence and risk of bias with the Cochrane Collaboration's Risk of Bias Tool, and outcome domains were extracted from each study.ResultsOf 4473 unique citations found, 8 studies were included in this review. All 8 trials were evidence level 1B. Risk of bias was low in 2 trials and high in the other 6. Reported outcome domains included speech perception in quiet and noise, speech tracking, quality of life, timbre perception, hearing preservation, vestibular function, electrode insertion technique, functional measures, functional imaging, fitting time, and tinnitus.ConclusionsAn analysis of randomized controlled trials studying cochlear implantation in adults yielded a wide spectrum of outcome domains. This is the first study to comprehensively describe the breadth of outcome domains in adult cochlear implantation. Validated instruments from these domains could be considered for potential inclusion as quality measures and registry use.
| S-EPMC6422673 | biostudies-literature
Project description:Johanson-Blizzard syndrome (JBS) is a rare autosomal recessive hereditary disorder characterized by multi-system involvement and facial dysmorphic features. One of the most common symptoms in JBS patients is bilateral severe to profound sensorineural hearing loss. The objective of this report is to highlight the performance of those patients after receiving cochlear implants (CI) as a management for their hearing loss. In this study, we reviewed the medical records of one female child diagnosed with JBS before and after cochlear implantation, with a particular focus on their auditory and language performance. After receiving the cochlear implant, our patient showed substantial improvement in her hearing threshold and communication abilities when compared to the preoperative condition. In conclusion, although cochlear implantation is considered a good approach for the management of JBS patients, the development of spoken language is not always achieved.
| S-EPMC8571962 | biostudies-literature
Project description:This study examines the last 10 years of medical literature on the benefits of cochlear implantation in children who are deaf or hard of hearing (DHH) with additional disabilities. The most recent literature concerning cochlear implants (CIs) in DHH children with additional disabilities was systematically explored through PubMed, Embase, Scopus, PsycINFO, and Web of Science from January 2012 to July 2023. Our two-stage search strategy selected a total of 61 articles concerning CI implantation in children with several forms of additional disabilities: autism spectrum disorder, cerebral palsy, visual impairment, motor disorders, developmental delay, genetic syndromes, and intellectual disability. Overall, many children with additional disabilities benefit from CIs by acquiring greater environmental sound awareness. This, in turn, improves non-verbal communication and adaptive skills, with greater possibilities to relate to others and to be connected with the environment. Instead, despite some improvement, expressive language tends to develop more slowly and to a lesser extent compared to children affected by hearing loss only. Further studies are needed to better appreciate the specificities of each single disability and to personalize interventions, not restricting the analysis to auditory and language skills, but rather applying or developing cross-culturally validated instruments able to reliably assess the developmental trajectory and the quality of life of DHH children with additional disabilities before and after CI.
| S-EPMC10605071 | biostudies-literature
Project description:BACKGROUND:Cochlear implantation in patients with functional residual low-frequency hearing is performed according to an established hearing-preserving surgical technique in order to cause minimal trauma of inner ear structures. Due to the increasing number of cochlear implants in children, the preservation of residual hearing is becoming increasingly important in this patient collective. OBJECTIVES:Short- and mid-term hearing preservation outcome in pediatric patients is investigated. MATERIALS AND METHODS:A group of 9 children (12 ears) between 5 and 12 years of age were examined after hearing-assisted cochlear implantation with respect to the pure tone audiometric thresholds. Retrospectively, short-term hearing preservation (up to 3 months after surgery) was examined. In a subgroup of 5 children, mid-term hearing preservation (7.5 to 16 months after surgery) was also analyzed. The mean values of hearing preserved (HL%) and hearing loss (HL) due to electrode insertion were calculated as measured values. RESULTS:In the whole group, the mean values of the preoperative PTAlow were 29.8?dB and the short-term postoperative PTAlow 42.6?dB. The mean value of the HL% was 73.6%, corresponding to an HL of 9.4?dB. In the subgroup, the mean PTAlow postoperatively was 46.0?dB in the mid-term and the HL% at 80.7% with a HL of 6.6?dB. CONCLUSIONS:The results in children are consistent with the results in adults. Electric-acoustic stimulation (EAS) should be used in the treatment of children with existing low-frequency residual hearing, as good residual hearing preservation can also be achieved in children after implantation.
| S-EPMC7062660 | biostudies-literature
Project description:ObjectivesIn this study, we aimed to (1) review the long-term outcomes of cochlear implantation in children with cochlear nerve aplasia and (2) compare the development of their auditory and speech abilities to children with normal-sized cochlear nerves.DesignThis is a retrospective case-control study. Patients who underwent unilateral cochlear implant (CI) surgery in a tertiary referral center from September 2012 to December 2018 were reviewed. The study group included 55 children with cochlear nerve aplasia diagnosed using preoperative images. The control group included 35 children with normal-sized cochlear nerves. The control group did not differ from the study group in terms of age at implantation, pre-implantation auditory and speech abilities, or the electrode array type. Cochlear implantation outcomes were assessed using a test battery, including the Categories of Auditory Performance (CAP) score, the Speech Intelligibility Rating (SIR) score, behavioral audiometry, and closed- or open-set speech recognition tests. The development of auditory and speech abilities was compared between the two groups using Generalized Linear Mixed-effect Models.ResultsThe mean duration of CI usage was 4.5 years (SD = 1.5, range = 2.0 to 9.5) in the study group. The CAP scores, SIR scores, and aided hearing thresholds improved significantly post-implantation in the study group, but were significantly poorer than those in the control group. Generalized Linear Mixed-effect Models showed that the development of CAP and SIR scores was significantly slower in the study group than in the control group. Overall, 27 (49%) children with cochlear nerve aplasia had some degree of open-set speech perception skills, but the monosyllabic and bisyllabic word recognition rates were significantly lower than those in the control group.ConclusionFor children with cochlear nerve aplasia, auditory perception and speech intelligibility continued to improve in the long-term follow-up, but this progress was significantly slower than in children with normal-sized cochlear nerves. Most children with cochlear nerve aplasia could obtain the ability of common phrase perception and understanding simple spoken language with consistent CI usage and auditory rehabilitation.
| S-EPMC10097487 | biostudies-literature
Project description:Introduction The cochlear implants centers in the world are studying the cochlear implantation in children with multiple handicaps. Objective To develop a questionnaire to analyze the subjective benefits of the cochlear implantation in multiple handicapped children according to their parent's perspective. Methods A questionnaire was applied to 14 families of multiple handicapped children, aging from 2-12 years old and having from 11 months to 11 years of implant use. Results The social-emotional abilities were improved because of many factors, such as: auditory exposure, which happened in 84% of the children; recognition of their own names, which increased in 56%; and development of eye contact, in 28% of the subjects. Other benefits appeared to be: music appreciation and more attention and adherence to other therapies and school activities. Besides, some children became interested in objects, playing with other children, and more adapted to daily routines. Thirty-five percent of the children acquired oral language, mainly the bilaterally implanted, while 14% of them were engaged in sign language. Although all of the children showed a significant improvement in communication, the emotional issues of some families and the severity of the handicaps negatively impacted the outcomes. In spite of the families' acknowledgement of some benefits, the diagnosis of autism spectrum disorder caused frustration, requiring a readjustment of the expectations. Conclusion The questionnaire turned out to be an adequate tool to reveal the social-emotional benefits of cochlear implantation. Although oral language was not the major outcome in these cases, the cochlear implant benefits involved the whole family. All of the families recommended the implant to other children in a similar situation.
| S-EPMC6197970 | biostudies-literature
Project description:The outcome of cochlear implantation (CI) in patients with cochlear nerve deficiency (CND) is variable, resulting in a wide range of speech perception performance, from degrees of environmental sound perception to conversation without lip-reading. Twenty-five cochlear implantees with CND were enrolled retrospectively to determine the factors correlated with CI outcome in patients with CND and to develop a predictive model for CI outcome. CI outcome was evaluated using the Categories of Auditory Performance (CAP) score at 2 years after CI. Patients with negative auditory brainstem response (ABR) showed a significantly lower CAP score than those with positive ABR (2.5?±?1.7, 4.8?±?0.7; p?=?0.001). The area ratio of vestibulocochlear nerve (VCN) to facial nerve (FN) at the cerebellopontine angle on magnetic resonance images was positively correlated with CI outcome (p?<?0.001). With multiple regression analysis, a predictive equation accounting for 66% of variance of CAP score at 2 years after CI was [Formula: see text]. We found that preoperative ABR and area ratio of VCN to FN at the cerebellopontine angle could predict CI outcome in patients with CND. Preoperative counselling based on our predictive model might be helpful to determine treatment modality for auditory rehabilitation and which ear to implant.
| S-EPMC6362156 | biostudies-literature
Project description:Objective: This paper reviewed the literature on the development of and factors affecting speech perception of Mandarin-speaking children with cochlear implantation (CI). We also summarized speech outcome measures in standard Mandarin for evaluating auditory and speech perception of children with CI. Method: A comprehensive search of Google Scholar and PubMed was conducted from March to June 2021. Search terms used were speech perception/lexical tone recognition/auditory perception AND cochlear implant AND Mandarin/Chinese. Conclusion: Unilateral CI recipients demonstrated continuous improvements in auditory and speech perception for several years post-activation. Younger age at implantation and longer duration of CI use contribute to better speech perception. Having undergone a hearing aid trial before implantation and having caregivers whose educational level is higher may lead to better performance. While the findings that support the use of CI to improve speech perception continue to grow, much research is needed to validate the use of unilateral and bilateral implantation. Evidence to date, however, revealed bimodal benefits over CI-only conditions in lexical tone recognition and sentence perception in noise. Due to scarcity of research, conclusions on the benefits of bilateral CIs compared to unilateral CI or bimodal CI use cannot be drawn. Therefore, future research on bimodal and bilateral CIs is needed to guide evidence-based clinical practice.
| S-EPMC8712552 | biostudies-literature