Outcomes Of Implantation In Children Research Articles

Cochlear implantation outcomes in children with multiple disabilities: a topic that’s worth revisiting

ObjectivesTo determine the benefits of cochlear implantation in hearing loss children with multiple disabilities (MD) in terms of auditory outcomes, speech performance, and their quality of life. MethodsThis was a cross sectional study from January 2019 to December 2020 in which thirty-one children with hearing loss and multiple disabilities were evaluated. Their improvement in auditory and speech performances were assessed using Categories of Auditory Performance version II (CAP-II) and the Speech Intelligibility Rating (SIR) scales. The assessment was done at 6-month intervals, with the baseline evaluation done at least six months after activation of the implant. Parents were asked to fill the Parents Evaluation of Aural/Oral Performance of Children (PEACH) diary and Perceived Benefit Questionnaire (PBQ) to evaluate the child’s quality of life. ResultsAll 31 children have Global Developmental Delay (GDD), with 11 having an additional disability. Both mean CAP-II and SIR scores showed significant improvement with increased hearing age (p < 0.05) after 6-month intervals. In addition, 20 out of 31 children (64.5%) have achieved verbal communication after implantation. The mean PEACH score in quiet was significantly better than in noise (p = 0.007) and improved with the increased of hearing age. The majority of parents (96%‒100%) perceived a cochlear implant as beneficial to their child in terms of auditory response, awareness, interaction, communication, and speech development. ConclusionsCochlear implantation had shown benefits in children with multiple disabilities. Outcome measures should not only focus on auditory and speech performances but the improvement in quality of life. Hence, individualized each case with realistic expectation from families must be emphasized in this group of children. Level of evidenceLevel 3.

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 implantation outcomes in children with global developmental delay.

As the number of hearing loss cochlear implant candidates who suffer from global developmental delay has dramatically increased, we aimed to study the prognosis of implantation in this group. In this cross-sectional case-control study, we utilized the Ages and Stages Questionnaire third edition (ASQ-3) to investigate the prognosis of cochlear implantation and its rehabilitation in 26 congenitally deaf children who suffered from global developmental delay compared with those in 25 non-delayed cases with the same conditions in two time periods, namely the first diagnosis of hearing loss and 18 months after the surgery and rehabilitation program. The data were analyzed using Statistical Package for Social Sciences, version 21 (SPSS-21). By the time of hearing loss diagnosis (six months old), the performance of all the global developmentally delayed hearing loss children in five subtests of the ASQ-3 scale was significantly lower than that of their non-delayed peers. Meanwhile, they improved significantly in two gross motor and social development subtests 18 months after the surgery and rehabilitation. Along with the general improvement of delay developed children with sensorineural hearing loss after cochlear implantation, global developmental assessment in the process of candidacy and after implantation is an essential factor that needs to be considered.

Relationship between parental stress and attitude towards cochlear implantation outcomes in children in an Indian context.

The study was aimed to investigate the relationship between parental stress and attitude of parents towards the outcomes of cochlear implantation in an Indian scenario. A total of 59 parents of children with cochlear implantation participated in the study. The outcomes of cochlear implant was measured using Parental attitudes of various aspects of cochlear implantation questionnaire and parental stress was measured using parental stress scale. The questionnaires were circulated to participants and data was collected in the form of e-survey. The present study showed that the parental stress level was similar among mothers and fathers. Further, the parental attitude towards communication abilities of children and education were positively correlated with the duration of cochlear implantation. Finally, a significant positive correlation was found between the parental stress and the parental attitude towards communication abilities of children and social skills. The present study showed a positive relationship between parental stress and parental attitude towards the outcomes of cochlear implantation for aspects of communication abilities and social skills.

Cochlear Implantation Outcomes in Children With CDH23 Mutations-Associated Hearing Loss.

Mutations in the cadherin 23 gene (CDH23) have been reported to cause cochlear damage, but few studies have investigated the auditory and speech outcome of patients after cochlear implantation. Here, we describe the genetic, auditory, and postoperative outcomes of patients with CDH23 mutations who received cochlear implants. Retrospective case review. Tertiary referral center. Targeted deafness-related gene panels were sequenced in Chinese families with profound sensorineural hearing loss. The clinical features of subjects carrying potentially pathogenic CDH23 mutations were analyzed. Between 2017 and 2019, we identified 5 children with prelinguistically profound hearing loss at our center who harbored 6 variants of CDH23 that segregated with the disease. Of these, 4 variants were novel (c.2591G>T, c.4785G>C, c.5765A>G, and c.9280_9281insTT). All affected individuals had a loss of outer hair cell function, with an average residual hearing level of 3 to 10 dB SPL. Cochlear implantations were arranged for the patients at 11 to 36 months of age. All children made gains in their hearing, language, and speech performances 14 to 120 months after surgery. Their auditory outcomes improved during follow-up intervals. This study revealed that children with congenital cochlear defects caused by CDH23 variants can acquire an acceptable auditory and speech outcome after cochlear implantation. Early genetic detection and prenatal counseling for rare deafness genes such as CDH23 remain a priority for the future.

Cochlear Implantation Outcomes in Children with Agenesis of the Corpus Callosum: A Retrospective Study and A Review of the Literature.

The aim of the present study was to analyze the outcomes of cochlear implantation (CI) in patients with agenesis of the corpus callosum (CCA). A literature review and a retrospective analysis of our cochlear implant database were performed. To the best of our knowledge, in the English literature, there was only one case reported with CCA who had undergone CI surgery. This case had Donnai-Barrow syndrome. In the Cukurova University School of Medicine Department of Otorhinolaryngology database, 5 of the 1317 patients who underwent CI surgery who had CCA were selected. The patients' demographic characteristics, operative findings, surgical outcomes, and additional disabilities were investigated. The patients' preoperative and postoperative Listening Progress Profile (LiP) and Meaningful Auditory Integration Scale (MAIS) tests were done to analyze the auditory performances. The participants of the study were 5 (0.38%) individuals (2 male and 3 female patients; ages 5.5, 7.5, 8, 9, and 12 years). Two of the patients had total agenesis, and the other three had partial agenesis of the CCA. In the histories of the patients, one patient had parental consanguinity, and one had febrile convulsion. No patient had an additional disability. None had experienced device failure. No patients were non-users or limited users of cochlear implants. Postoperative LiP and MAIS test scores were improved for all patients nearly as the patients without any deformity. They showed normal auditory performance in the analysis in their postoperative 48 months of follow-up. Patients who had CCA are good candidates for CI surgery.

Relationship Between Parental Stress and their Attitudes Towards Cochlear Implantation Outcomes in Children Referred to Besat Cochlear Implant Center in Hamadan 2018

PurposeThis study aimed to investigate the relationship between parental stress and their attitudes towards post-cochlear implantation outcomes in children referred to Besat cochlear implant center in Hamadan during 2018. Design and methodsIn this descriptive cross-sectional study, 100 parents of children with cochlear implantation were selected from Besat cochlear implant center in Hamadan through census method from April to October 2018. The data were collected using a demographic characteristic questionnaire, parental stress scale developed by Berry and Jones, and Parental Attitudes of Various Aspects of Cochlear Implantation. The data were analyzed using SPSS software (version 16) through correlation coefficient, one-way ANOVA, and t-test. P-value <0.05 was considered statistically significant. ResultsThe results of this study indicated that parental stress negatively correlated with their attitudes towards the outcomes of cochlear implantation (r = −0.420), including the aspects of communication (r = −0.462), educational (r = −0.398), and social skills (r = −0.445), as well as services provided by the health care centers (r = −0.277), and the efficiencies of cochlear implantation center (r = −0.118) (P < 0.05). ConclusionAccording to the results, parents with more positive attitudes towards the areas of communication, educational, and social skills, as well as services provided by the health care centers, and the efficiencies of cochlear implantation center experienced lower levels of stress. Practice implicationsNurses can measure the stress and attitudes towards cochlear implantation outcomes in children to accurately assess the needs of the family in order to improve their nursing process and advance the child's rehabilitation program.

Long-term outcomes of secondary intraocular lens implantation in children

Long-term outcomes of secondary intraocular lens implantation in children

Cochlear implantation outcomes in children with common cavity deformity; a retrospective study

ObjectiveA common cavity deformity (CCD) is a deformed inner ear in which the cochlea and vestibule are confluent forming a common rudimentary cystic cavity that results in profound hearing loss. There are few studies paying attention to common cavity. Our group is engrossed in observing the improvement of auditory and verbal abilities in children who have received cochlear implantation (CI), and comparing these targets between children with common cavity and normal inner ear structure. Material and methodsA retrospective study was conducted in 12 patients with profound hearing loss that were divided into a common cavity group and a control group, six in each group matched in sex, age and time of implantation, based on inner ear structure. Categories of Auditory Performance (CAP) and speech intelligibility rating (SIR) scores and aided hearing thresholds were collected and compared between the two groups. All patients wore CI for more than 1 year at the Cochlear Center of Anhui Medical University from 2011 to 2015. ResultsPostoperative CAP and SIR scores were higher than before operation in both groups (p < 0.05), although the scores were lower in the CCD group than in the control group (p < 0.05). The aided threshold was also lower in the control group than in the CCD group (p < 0.05). ConclusionEven though audiological improvement in children with CCD was not as good as in those without CCD, CI provides benefits in auditory perception and communication skills in these children.

Usefulness of radiological findings for predicting cochlear implantation outcomes in children with cochlear nerve deficiency: a pilot study

Conclusion: Children with CND received limited benefits from CIs and their results varied. The size of the vestibulocochlear nerve relative to the facial nerve could potentially be used as a predicator for CI outcomes in children with CND.Objective: This study aimed to (1) retrospectively review the outcomes of cochlear implants (CIs) in children with cochlear nerve deficiency (CND) and (2) evaluate the clinical usefulness of radiological findings as predictors for post-implantation outcomes.Methods: Study participants included 10 children with bilateral CND and profound sensorineural hearing loss. The preoperative magnetic resonance imaging and temporal bone computed tomography scans were evaluated. Auditory processing capability and speech perception performance were measured with Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scales. Aided hearing thresholds with CI were measured. The relationships between CI outcomes and the sizes of vestibulocochlear nerve and cochlear nerve canal (CNC) were analysed.Results: Although post-operative CAP scores and hearing thresholds significantly improved in children with CND, their results were worse than those measured in implanted children with normal cochlear nerve. No significant correlation was found between the CI outcomes and the vestibulocochlear nerve diameters or the CNC diameters in children with CND. However, children with larger vestibulocochlear-nerve-to-facial-nerve-ratios got better results.

Long-Term Cochlear Implant Outcomes in Children with GJB2 and SLC26A4 Mutations.

ObjectivesTo investigate speech and language outcomes in children with cochlear implants (CIs) who had mutations in common deafness genes and to compare their performances with those without mutations.Study DesignProspective study.MethodsPatients who received CIs before 18 years of age and had used CIs for more than 3 years were enrolled in this study. All patients underwent mutation screening of three common deafness genes: GJB2, SLC26A4 and the mitochondrial 12S rRNA gene. The outcomes with CIs were assessed at post-implant years 3 and 5 using the Categories of Auditory Performance (CAP) scale, Speech Intelligibility Rating (SIR) scale, speech perception tests and language skill tests.ResultsForty-eight patients were found to have confirmative mutations in GJB2 or SLC26A4, and 123 without detected mutations were ascertained for comparison. Among children who received CIs before 3.5 years of age, patients with GJB2 or SLC26A4 mutations showed significantly higher CAP/SIR scores than those without mutations at post-implant year 3 (p = 0.001 for CAP; p = 0.004 for SIR) and year 5 (p = 0.035 for CAP; p = 0.038 for SIR). By contrast, among children who received CIs after age 3.5, no significant differences were noted in post-implant outcomes between patients with and without mutations (all p > 0.05).Conclusion GJB2 and SLC26A4 mutations are associated with good post-implant outcomes. However, their effects on CI outcomes may be modulated by the age at implantation: the association between mutations and CI outcomes is observed in young recipients who received CIs before age 3.5 years but not in older recipients.

Outcomes of cochlear implantation in children with CHARGE syndrome

Conclusion: Regardless of the extent of inner ear anomalies and intellectual faculties, cochlear implantation (CI) with careful treatment planning can be a highly effective option for hearing rehabilitation in children with sensorineural hearing loss and CHARGE syndrome. Objective: CHARGE is a mnemonic term for coloboma, heart defects, choanal atresia, retarded growth and development, genital abnormalities, and ear anomalies. CHARGE syndrome is one of the leading causes of congenital deafness and blindness in children. We evaluated the language performance of six pediatric patients with CHARGE syndrome. Methods: Six patients with CHARGE syndrome underwent CI at the Asan Medical Center in Seoul between 2002 and 2012. The mean age of these CI patients was 4.9 years (range 2–9.8 years). All six patients had inner ear malformations; five children had an auricle anomaly and otitis media with effusion; three of these cases showed ossicle anomalies and four patients had downward displacement of the facial nerve (FN) toward the promontory; five patients showed delayed development; four of these children had cardiovascular malformations; three patients had cryptorchidism; two had choanal atresia; and there was one case of coloboma. Results: In the initial operations, two patients each were implanted with a CI512, CI24R, or CI24RE device. One patient initially received a CI512 device in the right ear, followed by a CI24RE device in the left ear. Another patient (case 1) first received a CI24R and then CI24RST device in the right ear due to poor performance, but this was eventually removed due to exposure of the electrode from adhesive otitis media and continuing poor performance. Assessment of the meaningful auditory integration scale (MAIS) showed that four patients reached a score above 95% and one patient achieved 60%. The categories of auditory performance (CAP) score showed that one patient reached CAP 7 at 2 years postoperatively and two cases showed CAP 5.5 at 1 year after implantation, while the remaining two patients had CAP scores of 3 at 12 months and 4.5 at 4 years, respectively. The speech intelligibility rating (SIR) score at 18 months post-surgery was 4.5 in two patients, 2.5 in two patients, and 1.5 in one case. The remaining patient (case 1) who was explanted showed no meaningful speech improvement.

Cochlear implantation outcomes in children with Waardenburg syndrome

Waardenburg syndrome (WS) is an autosomal dominant disease, characterized by dystopia canthorum, hyperplasia of the eyebrows, heterochromia iridis, white forelock, and congenital sensori-neural hearing loss (SNHL). The aim of this study was to evaluate the outcome of cochlear implantation in children with WS and compare it with children with pure SNHL. In a prospective study we evaluated 336 cochlear implanted children from 2008 to 2010. The WS was diagnosed by its established criteria and for control group children without any dysmorphic features, anatomical, behavioral, and developmental disorders were also enrolled. We evaluated children of both groups 1 year after cochlear implantation by categories of auditory performance (CAP) and speech intelligibility rating (SIR) tests. Eighty-one children out of the total 336 who had SNHL were included in study. Out of these 75 (22.3%) were healthy and six (1.78%) had WS. Of the 75 healthy children 40 (53.3%) were girls, while of the six children with WS, three (50%) were girls. There was a significant difference in SIR between WS and cases with pure SNHL (2.67 ± 1.03 vs. 3.79 ± 1.11, p = 021) however, the difference was not significant in CAP (4 ± 1.26 vs. 5.13 ± 1.13, p = 0.082). Prevalence of WS was 1.78% at Baqiyatallah Cochlear Implant Center. One year after implantation there was no significant difference in auditory outcome; however, the difference in speech outcome was significant between WS and cases with pure SNHL.

Cochlear Implant Outcomes in Children with Aplasia/Hypoplasia of the Cochleovestibular Nerve

Objective: Cochlear implant (CI) candidates undergo a preoperative workup including radiologic, audiologic, and neurologic testing to determine if they are candidates for implantation. A percentage of these patients are found to have aplasia/hypoplasia of the cochleovestibular nerve (CVN). Past literature has debated if this population of children benefit from CI. Method: Retrospective review evaluating 17 children with radiographically diagnosed aplasia/hypoplasia of CVN. Eight of these children underwent cochlear implantation during the period from 2002 to 2009. The remaining 9 children were treated nonsurgically with hearing aids. Medical records, audiologic testing, imaging studies, and speech and language evaluations were reviewed. Results: Meaningful Auditory Integration Scale (MAIS) scores were available for 6 out of 8 CI patients. The average MAIS score pre-CI was 6.16. The average scores post-CI were 46.6, 72.8, 70.25, and 75. Auditory Preschool Language Scale (PLS-4) pre-CI was 4.75, and post-CI score was 23. The pre- and post-CI Expressive PLS-4 scores improved from 11.25 to 23.5. Phonetic inventory scores were 3.7 for vowel sounds and 3.17 for consonant sounds. Scores post-CI were 10.8 for vowel sounds and 12.5 for consonants. Linear regression analysis revealed a significantly faster acquisition of consonant sounds with the CI group. Conclusion: Children with CVN aplasia/hypoplasia who received a CI had improved audiologic and speech and language assessments post-CI. The CI group averaged higher scores in vowel and consonant sound production, which was statistically significant. Long term follow-up is needed to further evaluate outcomes of CI with CVN aplasia/hypoplasia.

More About Cochlear Implantation in Children with Charge Association

CHARGE is the acronym for Coloboma, Heart malformation, Choanal Atresia, Retardation of growth and/or development, Genital and Ear anomalies. It is a rare genetic syndrome, usually with no family history. From an otological point of view, it is characterized by semicircular canal agenesia, Mondini-type cochlear hypoplasia, frequent vestibular dysfunction, and hearing impairment (ranging from transient OME to profound deafness) (Abadie et al., 2000).Speech and language delay in this syndrome is due not only to hearing problems, but also to mental retardation, facial palsy, oro-lingual motor deficit, and swallowing disorders, which often require enteral feeding or gastrostomy. Moreover, anatomical cochlear or facial nerve anomalies have been described.Due to these aggravating factors, few children affected by CHARGE have undergone cochlear implantation. However, several cochlear implantation centres, including ours, consider that children affected by this syndrome and profound hearing impairment might obtain benefit from a cochlear implant (CI), at least to improve communication, although not necessarily to develop spoken language (Bauer et al., 2002; McArdle et al., 2002; Lanson et al., 2007). This point of view was supported by a previous work about four children with CHARGE and sensorineural hearing loss: communication mode and language reception and expression progressed in a similar way in the two implanted profoundly deaf children as in the two of them with severe hearing impairment and hearing aids (Lina-Granade et al., 2006). Hence, we undertook this case study with more children and a longer follow-up, in order to have a more accurate description of the surgical particularities, fitting, and outcomes of implantation in children with CHARGE.

Clinical outcomes of scala vestibuli cochlear implantation in children with partial labyrinthine ossification

Conclusion. Cochlear implantation via the scala vestibuli is a viable approach in those with ossification in the scala tympani. With extended cochlear implant experience, there is no significant difference in the mapping parameters and auditory performance between those implanted via scala vestibuli and via scala tympani. Objectives. To assess the clinical outcomes of cochlear implantation via scala vestibuli. Patients and methods. In a cohort follow-up study, 11 prelingually deafened children who received cochlear implantation between age 3 and 10 years through the scala vestibuli served as participants. The mapping parameters (i.e. comfortable level (C), threshold level (T), dynamic range) and auditory performance of each participant were evaluated following initial cochlear implant stimulation, then at 3 month intervals for 2 years, then semi-annually. The follow-up period lasted for 9 years 9 months on average, with a minimum of 8 years 3 months. Results. The clinical results of the mapping parameters and auditory performance of children implanted via the scala vestibuli were comparative to those who were implanted via the scala tympani. No balance problem was reported by any of these patients. One child exhibited residual low frequency hearing after implantation.

Update: Auditory Brainstem Implant Outcomes In Children

Objective This study illustrates the auditory perceptual development and the non-verbal cognitive abilities in young deaf children fitted with auditory brainstem implant (ABI). Methods In our department, from 2000 to 2008, 31 children, aged 9 months to 16 years, received an ABI for different tumor and non-tumor diseases. Six children have elsewhere previously had a CI with no sound detection. 14 out of 31 children had associated disabilities. The retrosigmoid-transmeatal approach was used in tumor patients and the retrosigmoid approach was used in non-tumor patients. Perceptual auditory abilities were evaluated with the CAP and EARS battery tests. Cognitive evaluation was performed on 10 children using the Leiter International Performance Scale-Revised (LEITERS-R) test battery. Intraoperative EABRs and postoperative EABRs and EMLRs were performed. Results No postoperative complications were observed. All children consistently use their devices for more than 75% of waking hours and have environmental sound awareness. Their CAP scores ranged from 2 to 7 (average: 4); at MAIS they scored 2%–98.5% (average: 39%); MUSS scores ranged from 5% to 100% (average: 51%) and LIP scores from 5% to 100% (average: 46%). Scores on 2 of the 4 subtests considered for cognitive evaluation in this study increased significantly (from 2 to 10; p&gt;0.01) during the first year of ABI use in all 10 children. Conclusions The findings of the present study indicate that children with cochlear or cochlear nerve abnormalities, with or without associated cognitive deficits, should not be excluded from ABI implantation.

Predicting Cochlear Implant Outcomes in Children With Auditory Neuropathy

To examine the outcome of cochlear implantation in children with auditory neuropathy (AN) and cochlear nerve deficiency (Group A). Results are compared with a cohort of children with AN and normal cochlear nerves (Group B). Retrospective cohort study. The Sydney Cochlear Implant Centre and the Children's Hospital at Westmead. Children younger than 15 years with bilateral profound sensorineural hearing loss and the diagnosis of AN confirmed on electrophysiologic testing. All children underwent cochlear implantation with Nucleus 24 cochlear implants from 1997 to 2006. Magnetic resonance imaging was examined for deficiency of the vestibulocochlear nerve. Brain and inner ear abnormalities were recorded. Cochlear implant outcomes and demographic variables were compared. Melbourne speech perception score (MSPS) at 1 year and implant evoked electric auditory brainstem response (EABR). Group A performed significantly worse on both parameters than Group B. In Group A, median MSPS was 1, compared with a median score of 4 in Group B (z = -3.010; p = 0.003). EABR was abnormal in 13 of 15 (87%) children in Group A, compared with 9 of 39 (23%) in Group B. Children in both groups with abnormal EABR had significantly worse MSPS (z = -2.780; p = 0.005). Fourteen of 15 children with cochlear nerve deficiency had associated inner ear abnormalities. Children with AN can have associated cochlear nerve deficiency. These patients have worse speech perception scores at 1 year post cochlear implantation, higher rates of abnormal EABR, and more associated inner ear abnormalities than children with AN and normal cochlear nerves.