Cochlear Implantation In Children Research Articles

Auditory Development of Young Children with Profound Hearing Loss, Cochlear Implants, and Congenital CMV Infection

Background/Objectives: The aim of this study was to assess auditory development in young children with profound hearing loss, cochlear implants (CIs), and congenital cytomegalovirus (cCMV) infection and to determine the effect of comorbidities on their development. Methods: The study group (cCMV group) consisted of 47 CI children—18 girls and 29 boys—who had been diagnosed as having prelingual hearing loss due to cCMV infection (with or without comorbidities); the mean age at CI activation was 15.2 months (range: 9.7–23.8; SD = 3.5). The reference group (no cCMV) consisted of 117 similar children (57 girls and 60 boys) who had profound sensorineural hearing loss not caused by cCMV infection; they had no comorbidities. The mean age at CI activation in the second group was 14.3 months (range: 7.9–23.5; SD = 4.0). Auditory development in all children was assessed with the LittlEARS Auditory Questionnaire (LEAQ) at CI activation and at about 1, 5, 9, 14, and 24 months of CI use. Results: The mean LEAQ total score increased over a similar time frame from 9.8 pts to 28.9 pts in the cCMV group without comorbidities, from 4.5 pts to 18.5 pts in the cCMV group with comorbidities, and from 9.2 to 31.6 pts in the reference group with no cCMV infection. Conclusions: Early cochlear implantation in children with sensorineural hearing loss due to congenital CMV infection and no comorbidities promotes their early auditory development in a similar way to children without cCMV infection.

Postoperative Auditory Progress in Cochlear-Implanted Children With Auditory Neuropathy.

This study aims to evaluate the effect of auditory neuropathy spectrum disorder (ANSD) on postoperative auditory perception and listening difficulties in pediatric cochlear implant (CI) recipients. The Children's Auditory Perception Test (CAPT) assesses auditory perception skills, and the Children's Home Inventory of Listening Difficulties (CHILD) Scale evaluates daily listening difficulties. The study involved pediatric CI recipients (n = 40) aged between 5 and 7 years, with and without diagnosis of ANSD. The research ensured homogeneity across various factors, including chronological age, age at diagnosis, age at initial implantation, bilateral simultaneous surgery, etiologies of hearing loss, and family education level. The findings have demonstrated that children without ANSD exhibited better performance in integrating visual-auditory stimuli and overall listening performance, distant sound source scores, and noisy environment scores (respectively p = .047, p = .001, p = .028, and p = .010). Additionally, children with better speech perception also have a better ability to integrate audiovisual stimuli (p = .005, r = .438). There are significant differences in postoperative listening skills and auditory perceptions between children with and without an ANSD who have CIs. Accordingly, children without an ANSD perform better.

Cochlear implantation for children with single-sided deafness: parents’ perspectives

ABSTRACT Historically, cochlear implantation is reserved for those with bilateral severe to profound hearing loss. However, indications have expanded to include Single-Sided Deafness (SSD) because of the known benefits of binaural hearing and the limitations of unilateral hearing, especially for children developing linguistic and cognitive skills. This study aimed to provide a qualitative perspective of lived experiences of children with SSD who use a cochlear implant (CI), as perceived and reported by their parents. An online questionnaire was developed and distributed to evaluate outcomes of cochlear implantation in children with SSD residing in New Zealand and the United States. Qualitative content analysis of open-ended questions identified themes, including: (1) motivating factors; (2) benefits of amplification; (3) challenges associated with the CI journey; and (4) advice to parents. Parents described benefits and challenges as a result of cochlear implantation, such as access to sound and academic and psychosocial development, as well as subjective improvements in spatial hearing, reduced listening fatigue, enhanced academic performance, and changes in quality of life. The difference that bilateral hearing makes to a child’s development is difficult to precisely define but parental insights offer valuable perspective to healthcare providers when considering what some consider an elective procedure.

Subjective and functional outcome measures to evaluate performance of pediatric cochlear implant

BackgroundThe majority of research on the effects of cochlear implants has been on clinical evaluations of efficacy (hearing and speech skills and auditory thresholds). But it is unclear how much clinical efficacy metrics (speech, hearing, and language tests) actually demonstrate how beneficial cochlear implants (CI) are in everyday circumstances. In this study, we aim to assess the performance of a child’s current CI with both subjective and functional outcomes measures. Also, we aimed to assess factors affecting these outcomes after CI.MethodsThe study group included 45 cochlear-implanted children, and their chronological age ranges between 4 and 12 years. Children were divided according to their language age into three groups: group 1 (G1) includes15 children with a language age of less than 2 years, group 2 (G2) includes 15 children with a language age of 2 to less than 4 years, and group 3 (G3) includes 15 children with language age 4 to 6 years. Assessment was done in two sessions: one for phoniatric evaluation and another for audiological evaluation.ResultsCI enabled children to develop speech perception in a closed manner evidenced by the early speech perception (ESP) test with pattern perception being the easiest and monosyllabic words being the most difficult and the least to develop. Most children with CI were able to control their voice, to a lesser extent to use speech in meaningful use in social situations and to develop communication strategies in oral situations which were the least to develop, and this was evident by Meaningful Use of Speech Scale. Children with CI were able to acquire language, and their receptive language was better than expressive language, but their language age was below their chronological age.ConclusionThe cochlear implant is an effective rehabilitative tool for the treatment of prelingually deafened children, and this was evident by our results. A hierarchal approach of speech assessment according to the language age of the child and selection of age-appropriate speech perception test is very important to assess cochlear-implanted children Speech recognition had a high degree of variability. Early implantation age and increased duration of implant experience were the most important factors associated with good performance with CI, better speech perception, and better language outcomes.

A Systematic Review and Meta-analysis Examining Outcomes of Cochlear Implantation in Children With Bilateral Cochlear Nerve Deficiency.

To assess hearing and speech outcomes in pediatric patients with bilateral cochlear nerve deficiency (CND) who underwent cochlear implantation (CI) and to identify factors associated with improved outcomes. PubMed, Embase, Web of Science, and Cochran databases were searched following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Studies that reported hearing and speech outcomes of pediatric patients with bilateral CND who underwent CI were included. Demographics, comorbidities, inner ear abnormalities, CND classification (aplasia or hypoplasia), details of diagnostic workup, and outcomes data were extracted. Outcomes were assessed using the four-level auditory performance level (APL) scale. Meta-analysis, using Cochran-Armitage tests, was performed on patients with individual data to assess factors associated with performance. A total of 314 papers were screened, and 40 papers with 378 total patients met inclusion criteria. A total of 339 patients had patient-level data and were included in the meta-analysis. Of the 339 patients, 19% (n = 63) of patients had no measurable stimulation, 28% (n = 95) had improved detection, 22% (n = 76) achieved closed-set speech perception, and 31% (n = 105) achieved open-set speech perception. Patients with cochlear nerve aplasia (p = 0.016) and syndromes (p < 0.001) had significantly worse APL scores relative to patients with cochlear nerve hypoplasia and patients without syndromes, respectively. While most patients with bilateral CND benefit from CI and almost one-third of patients achieved open-set speech perception, outcomes were heterogenous and one-fifth of patients did not experience measurable benefit from CI.

Rehabilitation with cochlear implants in children with malformations of the inner ear

Cochlear implants (CIs) are the treatment of choice for hearing rehabilitation in children with congenital or acquired profound hearing loss or deafness in order to ensure appropriate speech development and avoid social deprivation. However, in the case of aradiologically detectable malformation of the inner ear structures and potentially associated hypo- or aplasia of the vestibulocochlear nerve, application of aCI is either not possible, or the functional outcome may be of limited predictability. In addition, the risk of surgical complications is also increased in these patients. Counseling parents and developing an appropriate individual therapeutic decision can therefore be amajor challenge for the medical team. The current paper is intended to provide support in this regard. It presents criteria for various inner ear malformations and discusses possible treatment options.

Indications for Cochlear Implants in Children: Where is the Journey Heading?

The indications for cochlear implants in children were gradually expended during the last 30 years. This concerns both the age at implantation and the audiological criteria, unilateral or bilateral treatment, the treatment of unilateral deafness and of children with multiple disabilities. Due to the improvement in hearing preservation and new technical developments, it is to be expected that the indication limits will continue to shift in the coming decades.

Factors impacting outcomes of cochlear implantation in children at two University centres in China: Multi-year analysis from the Paediatric Implanted Recipient Observational Study (P-IROS)

Objectives To identify factors affecting functional hearing performance and quality of life (QoL) outcomes in paediatric cochlear implantation (CI) recipients at two University centres in mainland China. Methods Two university centres in mainland China, part of the prospective longitudinal Paediatric Implanted Recipient Observational Study (P-IROS), contributed participant data. Participants were aged under 10 years at time of CI. Functional hearing performance and QoL measures were collected prior to device activation, and at 6-monthly intervals for 2 years post-implantation. Functional hearing endpoints including Categories of Auditory Performance-II (CAP-II) and QoL were evaluated and analysed using ordinal mixed-effects regression models. Results Data were from 288 children with a mean age at implant of 2.74 years. Overall follow-up at 1 year was 59% and 51% at 2 years. Younger age at implantation (p<0.001) and hearing aid use preimplantation (p=0.026) were associated with significant benefit. Bilateral device users (both CI and bimodal) achieved significantly better functional hearing performance on the CAP-II than unilateral CI users (p<0.001). Slower functional hearing improvements were observed in those with lower parental expectations compared to higher expectations (p<0.001). QoL improved over time but followed a different initial trajectory between centres. Conclusion All participants demonstrated significant improvements in auditory performance and QoL over time. Younger age at CI, and bilateral/bimodal device fitting contributed to earlier improvements. Other potential factors that could help inform families, professionals, and health authorities about choice of hearing device and educational supports required included aetiology of hearing loss and level of maternal education.

Assessing Neural Synchrony in the Cochlear Nerve to Electrical Stimulation in Children With Auditory Neuropathy Spectrum Disorder.

This study reported phase locking values (PLVs) that quantified the trial-to-trial phase coherence of electrically evoked compound action potentials in children with auditory neuropathy spectrum disorders (ANSD) and children with Gap Junction Beta 2 (GJB2) mutations, a patient population without noticeable cochlear nerve damage. PLVs were measured at three electrode locations in 11 children with ANSD and 11 children with GJB2 mutations. Smaller PLVs indicated poorer neural synchrony. A linear mixed-effects model was used to compare PLVs measured at different electrode locations between participant groups. After controlling for the stimulation level effect, children with ANSD had smaller PLVs than children with GJB2 mutations at all three electrode locations. Cochlear-implanted children with ANSD show poorer peripheral neural synchrony than children with GJB2 mutations.

Cochlear implantation in children with single-sided deafness under the age of 5 years: a review of current literature.

Here we explore the current literature on cochlear implantation of pediatric patients under the age of 5 years with single-sided deafness (SSD). Single-sided deafness has been noted to cause developmental delays in speech, language, and cognition because of loss of binaural hearing. Currently, cochlear implantation is the only intervention capable of restoring binaural hearing for pediatric patients with SSD. Young children have been shown to have the greatest neuroplasticity of the auditory cortex before 4 years of age. Currently, only children over the age of 5 years are approved by the United States Food and Drug Administration (FDA) to undergo cochlear implantation for SSD. Cochlear implantation for SSD in patients under the age of 5 years has been performed on a limited basis and has been shown to have excellent results. Cochlear implantation is a well tolerated and effective treatment for pediatric patients under the age of 5 years with SSD. Receiving cochlear implantation under the age of 5 years is critical for child development as neuroplasticity decreases after this age.

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.

Prediction of Cochlear Implant Fitting by Machine Learning Techniques.

This study aimed to evaluate the differences in electrically evoked compound action potential (ECAP) thresholds and postoperative mapping current (T) levels between electrode types after cochlear implantation, the correlation between ECAP thresholds and T levels, and the performance of machine learning techniques in predicting postoperative T levels. Retrospective case review. Tertiary hospital. We reviewed the charts of 124 ears of children with severe-to-profound hearing loss who had undergone cochlear implantation. We compared ECAP thresholds and T levels from different electrodes, calculated correlations between ECAP thresholds and T levels, and created five prediction models of T levels at switch-on and 6 months after surgery. The accuracy of prediction in postoperative mapping current (T) levels. The ECAP thresholds of the slim modiolar electrodes were significantly lower than those of the straight electrodes on the apical side. However, there was no significant difference in the neural response telemetry thresholds between the two electrodes on the basal side. Lasso regression achieved the most accurate prediction of T levels at switch-on, and the random forest algorithm achieved the most accurate prediction of T levels 6 months after surgery in this dataset. Machine learning techniques could be useful for accurately predicting postoperative T levels after cochlear implantation in children.

Frequency of Herpes Simplex Virus Types 1 and 2 and Cytomegalovirus in Perilymph and Peripheral Blood Samples of Cochlear-Implanted Children Using the Polymerase Chain Reaction Method

Background and Aim: Inner ear infection with some viruses may be one of the possible causes of Sensorineural Hearing Loss (SNHL). This study aims to determine the frequency of Herpes Simplex Virus 1 and 2 (HSV-1, HSV-2) and Cytomegalovirus (CMV) in perilymph and peripheral blood samples of cochlear-implanted children. Methods: In this cross-sectional study, 30 children with severe-to-profound SNHL (aged 1.1–5 years) underwent cochlear implantation surgery. During surgery, their perilymph and peripheral blood samples were collected. The samples were analyzed separately for the presence of herpes HSV-1, HSV-2, and CMV by real-time Polymerase Chain Reaction (PCR) method. The load of IgG and IgM antibodies against these viruses was determined using the Enzyme-Linked Immunosorbent Assay (ELISA) method. Results: The frequency of CMV in perilymph samples was 16.7% (5 patients) and in peripheral blood samples was 3.3% (1 patient). The IgG antibody against CMV and HSV- 1 was positive in 80% and 46.7% of the patients, respectively. The IgM antibody against CMV was positive in 10%. The mean IgM serum antibody load against HSV-1, HSV-2, and CMV was 2.70, 1.70, and 5.47, respectively, and the mean IgG antibody load against these viruses was 56.07, 2.50, and 23.67, respectively. Conclusion: The IgG test is positive in cochlear-implanted children with CMV in their perilymph samples, and the CMV genome is not present in their peripheral blood. This may indicate the previous presence of this virus in the ear and its role in hearing loss. Keywords: Sensorineural hearing loss; perilymph fluid; cytomegalovirus; herpes simplex

Evaluation of Cochlear Implantation in Children With Cochlear Nerve Absence or Deficiency.

To identify associations between cochleovestibular anatomy findings and hearing outcomes found in children with imaging evidence of an absent or hypoplastic cochlear nerve treated with cochlear implantation (CI). retrospective review. Cochlear implant program at tertiary care center. A retrospective review was performed to identify children with imaging evidence of cochlear nerve absence or deficiency who underwent CI evaluation. High-resolution 3-dimensional T2-weighted magnetic resonance imaging in the oblique sagittal and axial planes were reviewed by a neuroradiologist to identify cochleovestibular anatomy. Hearing was assessed pre and postoperatively with Speech Perception Category scores. Seven CI recipients were identified (n = 10 ears) who had bilateral severe to profound sensorineural hearing loss with lack of auditory development with binaural hearing aid trial and imaging evidence of cochlear nerve aplasia/hypoplasia. All ears had 2 nerves in the cerebellopontine angle (100%, n = 10), half of the ears had evidence of 2 or less nerves in the internal auditory canal (IAC). All children showed large improvement in speech perception after CI. Our experience with CIs for children with absent or hypoplastic cochlear nerves demonstrates that CI can be a viable option in select patients who satisfy preoperative audiological criteria. Radiological identification of a hypoplastic or aplastic cochlear nerve does not preclude auditory innervation of the cochlea. CI recipients in this subgroup must be counseled on difficulty in predicting postimplantation language and speech outcomes, and cautioned about facial nerve stimulation.

Assessment of auditory perception of children with single-sided deafness after cochlear implantation

Unilateral deafness will lead to the decline of children's speech recognition rate, language development retardation and spatial positioning ability, which will have many adverse effects on children's life and study. Cochlear implantation can help children rebuild binaural hearing, and systematic audiological evaluation after operation is particularly important for clinicians to evaluate the hearing recovery of children. In this study, a variety of commonly used audiological evaluation, testing processes and methods after cochlear implantation in children with unilateral deafness are described in detail, and the related research status and results are summarized.

Functional Outcomes from Cochlear Implants in Children with Acquired Hearing Loss: Case Studies

Purpose: Early identification of postlingual hearing loss (HL) and knowing its probable cause is of great significance. Causes that result in irreversible profound HL require immediate radiological investigation and intervention to decide the best listening device.Methods: Case reports of three children with acquired HL using cochlear implant (CI) are being presented to understand the functional outcomes in domains of audition and language skills after 12 months of CI use. The age of onset of deafness and implantation were not identical. Etiology of deafness was meningitis in two cases and viral infection in the other. Consent was obtained from the parents for participating in the study. Case history review was conducted to collect demographic and audiological details of the participants. Functional benefits from CI in those children were assessed in the domains of auditory and language skills using standardized assessment tools respectively.Results: Improvements in all domains of auditory and language skills were observed. However, the degree of benefits depended on the duration of deafness, etiology of deafness, age at onset of deafness, age at implantation and effectiveness of intervention.Conclusions: The results of the study can be used during pre-implant CI counselling to facilitate development of real expectations about the functional benefits of CI in children with acquired HL due to various reasons and showed the importance of early cochlear implantation following the diagnosis. These case presentations give an understanding of the various factors that can affect the functional outcome from CI in children with post-lingual HL.

Current Issues With Pediatric Cochlear Implantation.

Cochlear implants (CIs) have demonstrated a clear functional benefit in children with severe-to-profound sensorineural hearing loss (SNHL) and thus have gained wide acceptance for treating deafness in the pediatric population. When evaluating young children for cochlear implantation, there are unique considerations beyond the standard issues addressed during surgery in adults. Because of advances in genetic testing, imaging resolution, CI technology, post-implant rehabilitation, and other factors, issues related to CI surgery in children continue to evolve. Such factors have led to changes in candidacy guidelines, vaccine requirements, and lowering of age requirement for surgery. In addition, differences in the anatomy and physiology of infants require special attention to ensure safety when operating on young children. This review summarizes these issues and provides guidance for surgeons treating children with SNHL.

Cochlear Implant Complications in Children: Libyan Scenario and Experience

cochlear implant (CI) is considered a safe procedure; however, complications may happen, either due to the presence of the implant itself which act as a foreign body or the presence of congenital anomalies of the ear. One hundred eighty-two children implanted by cochlear implant team in Specialty surgical center (SSC)/Benghazi-Libya between May 2012 to December 2021 were recruited in this study. Demographic data: about name, age and sex, vaccination history, audiological and radiological assessment in form of CT scan temporal bone and MRI brain, surgical procedure and any difficulties encountered during the operation, are recorded. Complications encountered were grouped as major and minor ones. one hundred eighty-two patients were studied; age range was from seventeen months to twelve years. The major complications that need hospitalization or surgical interventions were seen 3.29% whereas minor complications that do not need hospitalization but they resolve spontaneously or treated in out-patient department; accounts for 13.7%. Accidental trauma was the major risk factor that seen in both major and minor complications. Cochlear implantation in children continues to be reliable and safe in experienced hands, with a low percentage of severe complications as long as long-term medical follow up is there. Major Complications of CI in children in SSC are related to trauma and hematoma formation as a major factor.

Auditory Outcomes Following Cochlear Implantation in Children with Unilateral Hearing Loss.

Unilateral hearing loss (UHL) in children is associated with speech and language delays. Cochlear implantation (CI) is currently the only rehabilitative option that restores binaural hearing. This study aims to describe auditory outcomes in children who underwent CI for UHL and to determine the association between duration of hearing loss and auditory outcomes. Retrospective case series. Three tertiary-level, academic institutions. Children <18 years with UHL who underwent CI between 2018 and 2021. Cochlear implantation. Speech perception and Speech, Spatial and Qualities of Hearing Scale (SSQ) were assessed postimplantation. Scores >50% on speech perception and SSQ scores >8 points were considered satisfactory. Associations between duration of UHL and implantation age and outcomes were assessed using Spearman's rank correlation. Of the 38 children included, mean age at CI was 7.9 ± 3.2 years and mean UHL duration was 5.0 ± 2.8 years. Mean datalogging was 8.1 ± 3.1 hours/day. Mean auditory testing scores were SSQ, 7.9 ± 1.2; BABY BIO, 68.1 ± 30.2%; CNC, 38.4 ± 28.4%; WIPI, 52.5 ± 23.1%. Scores >50% on CNC testing were achieved by 40% of patients. SSQ scores >8 points were reported by 78% (7/9) of patients. There were no significant correlations between UHL duration and auditory outcomes. Overall, children with UHL who undergo CI can achieve satisfactory speech perception scores and SSQ scores. There were no associations between duration of hearing loss and age at implantation with auditory outcomes. Multiple variables may impact auditory outcomes, including motivation, family support, access to technology, and consistent isolated auditory training postactivation and should be taken into consideration in addition to age at implantation and duration of UHL in determination of CI candidacy.

Indikationen zur Cochlea-Implantat Versorgung bei Kindern: Wohin geht die Reise?

AbstractThe indications for cochlear implants in children were gradually expended during the last 30 years. This concerns both the age at implantation and the audiological criteria, unilateral or bilateral treatment, the treatment of unilateral deafness and of children with multiple disabilities. Due to the improvement in hearing preservation and new technical developments, it is to be expected that the indication limits will continue to shift in the coming decades.