Benefits and risks related to cochlear implantation for children with residual hearing: a systematic review.

OBJECTIVE: This study aimed to synthesise information concerning the potential benefits and risks related to cochlear implants (CIs) versus hearing aids (HAs) in children with residual hearing. DESIGN: A systematic review of articles published from January 2003 to January 2019 was conducted. STUDY SAMPLE: Our review included studies that compared the benefits and risks of CIs versus HAs in children (≤18 years old) with residual hearing. A total of 3265 citations were identified; 8 studies met inclusion criteria. RESULTS: Children with CIs showed significantly better speech perception scores post-CI than pre-CI. There was limited evidence related to improvement in everyday auditory performance, and the results showed non-significant improvement in speech intelligibility. One study on social-emotional functioning suggested benefits from CIs. In four studies, 37.2% (16/43) of children showed loss of residual hearing and 14.0% (8/57) had discontinued or limited use of their device. CONCLUSIONS: Children with CIs showed improvement in speech perception outcomes compared to those with HAs. However, due to the limited number of studies and information to guide decision-making related to other areas of development, it will be important to conduct further research of both benefits and risks of CIs in this specific population to facilitate decision-making.

Clinical characteristics and outcomes of children with cochlear implants who had preoperative residual hearing.

OBJECTIVE: Cochlear implants (CI) candidacy criteria have expanded to include children with more residual hearing. This study explored the clinical profiles and outcomes of children with CIs who had preoperative residual hearing in at least one ear. DESIGN: A retrospective chart review was conducted to collect clinical characteristics and speech perception data. Pre- and post-CI auditory and speech perception data were analysed using a modified version of the Pediatric Ranked Order Speech Perception (PROSPER) score. STUDY SAMPLE: This study included all children with residual hearing who received CIs in one Canadian paediatric centre from 1992 to 2018. RESULTS: A total of 100 of 389 (25.7%) children with CIs had residual hearing (median 77.6 dB HL, better ear). The proportion of children with residual hearing increased from 1992 to 2018. Children who had auditory behaviour and speech perception tests (n = 83) showed higher modified PROSPER scores post-CI compared to pre-CI. Phonologically Balanced Kindergarten (PBK) test scores were available for 71 children post-CI; 81.7% (58/71) of children achieved > 80% on the PBK. CONCLUSIONS: One in four children who received CIs had residual hearing, and most of them had severe hearing loss at pre-CI. These children showed a high level of speech perception with CIs.

A follow-up study of cognitive function in young adults who had resective epilepsy surgery in childhood.

This study examined cognitive function in young adults who had epilepsy surgery in childhood. Thirty-seven individuals with medically intractable epilepsy with onset at 16 years or younger who had resective epilepsy surgery at least two years in the past (mean follow-up duration of 8.5 years) were assessed; of these, 13 had seizures within the year prior to the study, and the remainder had none. A comparison group of 16 individuals with childhood-onset intractable epilepsy who had not had surgery, all of whom had experienced at least one seizure in the past 12 months, was also included. The cognitive tests included measures of vocabulary, visuoconstructive ability, memory, and concept formation. Group differences were found only for the vocabulary and verbal memory tests, with the surgical group with seizures having the lowest performance. A subset of the surgical patients had preoperative data available on comparable tests, allowing for an examination of performance over time. Vocabulary scores were higher at follow-up, a finding which was present irrespective of seizure status. The results suggest that after epilepsy surgery in childhood or adolescence, few improvements in cognitive skills related to surgery or seizure outcome are to be expected.

Long-term outcome after cochlear implantation in children with additional developmental disabilities.

OBJECTIVE: Candidacy criteria for cochlear implants have expanded to include children with complex developmental disabilities. The aim of this study was to determine the long-term benefits of cochlear implantation for this clinical population. DESIGN: The study involved a retrospective chart review. STUDY SAMPLE: The review identified 21 children with complex disabilities who had received cochlear implants in a pediatric center prior to 2004. Length of cochlear implant use was between 7.3 and 19.0 years. Long-term functional auditory abilities were assessed pre and post-operatively using measures appropriate to the child's level of functioning. Cognitive assessments and developmental data were also available for the children. RESULTS: Children's long-term speech recognition outcomes depended highly on their developmental status. Children with severe developmental delay showed no open-set speech recognition abilities while children with mild to moderate delays achieved open-set scores ranging from 48 to 94% on open-set word testing. Five of 13 (38%) children with complex needs had discontinued use of their cochlear implant. CONCLUSIONS: Long-term speech recognition abilities following cochlear implantation for children with complex developmental issues seem to be highly related to their developmental profile. Developmental status is an important consideration in counselling families as part of the cochlear implant decision process.

Determining cochlear implant candidacy in children with residual hearing: A scoping review.

OBJECTIVES: The purpose of this review was to summarize what is known about criteria, assessments, and recommendations for evaluating cochlear implant (CI) candidacy in children with residual hearing. METHODS: Peer-reviewed studies were identified through a systematic search in five electronic databases. Articles were screened and assessed for eligibility. From the eligible studies, data were extracted to summarize and present a narrative synthesis of the findings. RESULTS: A total of seven articles (two reviews and five primary studies) were included in the final analysis. Hearing levels better than the moderately severe to severe range (65-90 dB HL) tend to be supported as audiological candidacy criteria for pediatric CI. Recommendations for candidacy consideration based on audiologic thresholds range from 65 to 80 dB Hl pure-tone average as the lower boundary. Our review did not identify any specific assessment protocols. However, additional decision-making considerations related to borderline hearing loss configurations and assessment tools (the Speech Intelligibility Index and the Pediatric Minimum Speech Test Battery) were identified. Supplementary assessment considerations were also reported. CONCLUSION: There is limited information regarding specific assessment protocols for children with residual hearing. The literature is primarily focused on guidelines related to audiologic criteria, although it is widely recommended that other areas of functioning should also be considered. Most recommendations appear to be based on expert opinion, clinical expertise, and evidence from overall pediatric CI outcomes rather than empirical evidence targeting children with residual hearing. There is an ongoing need for research to further develop protocols and tools that can assist clinicians and families in making cochlear implantation decisions for children with residual hearing.

Cochlear implant decision-making for children with residual hearing: Perspectives of parents.

PURPOSE: Cochlear implant (CI) decision-making is particularly challenging for families of children who have residual hearing. Parents of these children may be uncertain about whether the potential benefits of CIs outweigh the risks. This study aimed to understand parents' decisional needs during the decision-making process for children with residual hearing. METHOD: Semi-structured interviews were conducted with parents of 11 children who had received CIs. Open-ended questions were asked to encourage parents to share their experiences about the decision-making process, their values/preferences, and their needs. The interviews were transcribed verbatim and analyzed using thematic analysis. RESULTS: Data were organized according to three key themes: (1) Parents' decisional conflict, (2) values and preferences, (3) decision support and parents' needs. We found that overall parents were satisfied with their decision-making process and the decision support from practitioners. However, parents stressed the importance of receiving more personalized information that considers their specific concerns, values and preferences related to family's circumstances. CONCLUSIONS: Our research provides additional evidence to guide the CI decision-making process for children with residual hearing. Additional collaborative research with audiology and decision-making experts specifically on facilitating shared decision-making is needed to provide better decision coaching for these families.

Cochlear Implant Decision Making for Children With Residual Hearing: Perspectives of Practitioners.

PURPOSE: Cochlear implants (CIs) are increasingly considered for children with residual hearing who benefit from hearing aids (HAs). However, the decision-making process for families of these children and for practitioners is particularly challenging because there is no clear audiological cut point for CI candidacy. This study aimed to understand Canadian practitioners' perspectives of the CI decision-making process and how they guide families of children with residual hearing. METHOD: Semistructured interviews were conducted with a total of 17 practitioners through four focus groups and one individual interview. Interviews were transcribed verbatim, and a thematic analysis was carried out. RESULTS: Data were organized into five broad domains: candidacy issues for children with residual hearing, practitioners' roles in decision support, additional considerations affecting decision making, factors facilitating decision making, and practitioners' needs. CONCLUSIONS: This study found that practitioners' confidence in determining candidacy and supporting parents has increased due to their experiences with positive outcomes for these children. Practitioners indicated that there was a need for more research to guide the decision-making process.

Trajectory of hearing loss in children with unilateral hearing loss.

Introduction: The aim of this study was to quantify the amount of deterioration in hearing and to document the trajectory of hearing loss in early identified children with unilateral hearing loss (UHL). We also examined whether clinical characteristics were associated with the likelihood of having progressive hearing loss. Methods: As part of the Mild and Unilateral Hearing Loss Study, we followed a population-based cohort of 177 children diagnosed with UHL from 2003 to 2018. We applied linear mixed models to examine hearing trends over time including the average amount of change in hearing. Logistic regression models were used to examine the relationship between age and severity at diagnosis, etiology, and the likelihood of progressive loss and amount of deterioration in hearing. Results: The median age of the children at diagnosis was 4.1 months (IQR 2.1, 53.9) and follow-up time was 58.9 months (35.6, 92.0). Average hearing loss in the impaired ear was 58.8 dB HL (SD 28.5). Over the 16-year period, 47.5% (84/177) of children showed deterioration in hearing in one or both ears from their initial diagnostic assessment to most recent assessment including 21 (11.9%) who developed bilateral hearing loss. Average deterioration in the impaired ear ranged from 27 to 31 dB with little variation across frequencies. Deterioration resulted in a change in category of severity for 67.5% (52/77) of the children. Analysis for children who were followed for at least 8 years showed that most lost a significant amount of hearing rapidly in the first 4 years, with the decrease stabilizing and showing a plateau in the last 4 years. Age and severity at diagnosis were not significantly associated with progressive/stable loss after adjusting for time since diagnosis. Etiologic factors (ENT external/middle ear anomalies, inner ear anomalies, syndromic hearing loss, hereditary/genetic) were found to be positively associated with stable hearing loss. Conclusion: Almost half of children with UHL are at risk for deterioration in hearing in one or both ears. Most deterioration occurs within the first 4 years following diagnosis. Most children did not experience sudden "large" drops in hearing but more gradual decrease over time. These results suggest that careful monitoring of UHL especially in the early years is important to ensure optimal benefit from early hearing loss detection.

Quality of life in young adults who underwent resective surgery for epilepsy in childhood.

PURPOSE: This study investigated quality of life (QOL) in young adults who had undergone epilepsy surgery before the age of 16 years. The contribution to QOL of seizure status in the prior year, sex, number of antiepileptic drugs, and mood were evaluated. METHODS: Sixty-nine young adults who had undergone surgery were subdivided into those who were seizure-free in the past year (n = 38) and those who had seizures (n = 31) in that time. A nonsurgical comparison group of young adults (n = 29) with childhood-onset medically intractable epilepsy was also studied. All groups completed measures of QOL and mood. KEY FINDINGS: After accounting for mood, sex, and number of antiepileptic drugs, the seizure-free group reported better cognitive and physical function and overall QOL, experienced less seizure worry, and had better self-perception. Mood was the most consistently predictive covariate, and was independently predictive of many aspects of QOL. SIGNIFICANCE: Seizure freedom associated with surgery in childhood is associated with improved QOL in certain domains. Findings highlight the importance of mood in determining self-perception of QOL.

Self-reported symptoms of psychological well-being in young adults who underwent resective epilepsy surgery in childhood.

PURPOSE: This study investigated the relationship of childhood resective surgery for lesional epilepsy and recent seizure history on self-reported symptoms of mood and psychological distress in young adults (aged 18-30). METHODS: Ninety-eight individuals with epilepsy of childhood onset were divided into three groups: a seizure-free surgical group (n = 39), a surgical group still experiencing seizures (n = 31), and a nonsurgical epilepsy comparison group (n = 28). Participants completed two standardized questionnaires about current mood state and psychological and psychiatric symptoms: the Profile of Mood States (POMS) and the Symptom Checklist-90 Revised (SCL-90R). KEY FINDINGS: Forty-eight percent of all participants reported a history of psychological problems. The percentage of the seizure-free surgical group who met the SCL-90R criteria for current clinically significant distress was statistically less than in the other groups. Those who were seizure free also reported significantly fewer total symptoms on the SCL-90R. The current number of antiepileptic medications was related to scores on a number of the scales. SIGNIFICANCE: These results provide modest support for the contention that seizure freedom after pediatric epilepsy surgery is associated with reduced risk for psychological distress during early adulthood.

Patient-reported outcome of pediatric epilepsy surgery: social inclusion or exclusion as young adults?

PURPOSE: The purpose of this study was to examine the social relationships and participation in educational, vocational, and community life in young adults who had undergone epilepsy surgery during childhood or adolescence. METHODS: This was a retrospective, cross-sectional, case-controlled, and multisite design study. Findings were compared between young adults who had undergone epilepsy surgery and were seizure-free in the previous 12 months (n = 38), those who had undergone epilepsy surgery and were not seizure-free (n = 33), and a group of individuals with epilepsy who had not undergone surgery (n = 31). RESULTS: The surgical seizure-free group had significantly better general social well-being than the other two groups. Specifically, these participants were employed for more months over the past year, were less likely to report that epilepsy had affected their employment, and were more likely to belong to at least one community organization. However, these participants were not any more likely to be involved in a relationship than those in the other two groups. In addition, there were no group differences in the reported number of friends or the frequency of visits with friends, or in what participants described as their principal activity (i.e., student, employed or unemployed). DISCUSSION: There are a number of benefits to social functioning associated with having had epilepsy surgery and being seizure-free in the past year. Further research is recommended to increase our understanding of the mechanisms involved in the social and personal challenges that these individuals face.

Pediatric cochlear implantation: how much hearing is too much?

Audiologic candidacy criteria for determining cochlear implantation candidacy in children are evolving. The objective of the study was to examine clinical practice related to the cochlear implantation of children who typically do not meet audiologic criteria for this technology. Practitioners' perspectives on the process and the factors influencing candidacy decisions were explored through focus group interviews with hospital and school-based practitioners. The interviews were analysed using qualitative techniques to identify key issues. The findings from the interviews informed a questionnaire which was sent to all cochlear implant centers in Canada to further examine clinician views and experiences with this special population. Responses were collected from 11 of the 12 centers and indicated that children with hearing outside typical criteria were receiving implants. The definition of 'borderline' varied across the programs from approximately 70 dB HL to less than 90 dB HL. All centers emphasized the importance of considering factors beyond the child's audiometric thresholds in candidacy decision-making.

When symptoms don't fit: a case series of conversion disorder in the pediatric otolaryngology practice.

BACKGROUND: Conversion disorder refers to functional bodily impairments that can be precipitated by high stress situations including trauma and surgery. Symptoms of conversion disorder may mimic or complicate otolaryngology diseases in the pediatric population. CASE PRESENTATION: In this report, the authors describe 3 cases of conversion disorder that presented to a pediatric otolaryngology-head and neck surgery practice. This report highlights a unique population of patients who have not previously been investigated. The clinical presentation and management of these cases are discussed in detail. Non-organic otolaryngology symptoms of conversion disorder in the pediatric population are reviewed. In addition, we discuss the challenges faced by clinicians in appropriately identifying and treating these patients and present an approach to management of their care. CONCLUSION: In this report, the authors highlight the importance of considering psychogenic illnesses in patients with atypical clinical presentations of otolaryngology disorders.

The impact of newborn hearing screening on communication development.

OBJECTIVE: Universal newborn hearing screening has become standard practice in many countries. The primary goal of this study was to assess the impact of early identification of permanent childhood hearing loss on oral communication development. SETTING: Participants were recruited from three clinical programmes in two cities in the province of Ontario, Canada. The study sample was born during two consecutive periods of newborn hearing screening. The first period, prior to 2002, was targeted on high-risk infants only, and the second, from 2002, included both high- and standard-risk infants (universal newborn hearing screening - UNHS). All children were enrolled in rehabilitation programmes focused on oral language development. METHODS: In this multicentre observational study, 65 children under the age of five years with onset of hearing loss before six months of age, 26 identified through systematic newborn screening (14 through targeted screening and 12 through UNHS) and 39 without screening, were assessed with an extensive battery of child- and parent-administered speech and language measures. The degree of hearing loss ranged from mild to profound with 22 children in the mild, moderate and moderately severe categories and 43 in the severe and profound categories. Data are reported for the three-year study period. RESULTS: The screened group of children was identified at a median age of 6.6 (interquartile range, 3.0-8.2) months and children referred from sources other than newborn screening were diagnosed at a median age of 16.5 (interquartile range, 10.2-29.0) months. Assessment of oral communication development showed no significant difference between the screened and unscreened groups. The communication outcomes for children identified before 12 months of age did not differ from those of later identified children. CONCLUSIONS: Systematic screening of newborn hearing results in earlier identification and intervention for children with permanent hearing loss. Superior language outcome following newborn screening was not demonstrable in the setting of this study.

Practitioners' perspectives on the functioning of school-age children with cochlear implants.

OBJECTIVE: The availability of cochlear implants has increased the number of children with profound deafness educated in classrooms alongside peers with normal hearing. The purpose of this research was to better understand the functioning of these children from the perspective of their service providers. METHODS: Semi-structured focus group interviews were conducted with 28 practitioners to elicit their perceptions of children's abilities in oral communication, academic, and social functioning. Data were coded inductively and examined through content analysis. RESULTS: The central theme was that cochlear implantation has improved school functioning for children both in hearing and related spoken language abilities and beyond hearing in academic and social development. While these benefits were a consistent theme, a wide range of performance was identified across all areas of functioning. In particular, areas of concern included full participation in classroom activities and social interaction with peers. CONCLUSIONS: The findings provide insights into functioning for children with cochlear implants from the perspective of those who interact with them in everyday settings. These findings underscore the challenges for children in achieving full participation despite improvements in communication skills. Practitioners identified areas where intervention is required to facilitate the inclusion of children in school programs.

Functional MRI study of verbal fluency in a patient with subcortical laminar heterotopia.

RATIONALE: Double cortex syndrome is a malformation in which there is a band of subcortical heterotopic grey matter separated from the cortex by white matter. The functional activity of the heterotopic neurons is unclear. PATIENT: A 13-year-old female was evaluated for seizures. The EEG showed bifrontal spike wave disturbance. Band heterotopia, in association with mild reduction of sulcation of the cerebral hemispheres, was found on MRI. Psychological assessment indicated the presence of variable cognitive abilities, with verbal IQ [82] generally better than nonverbal IQ [59], and specific difficulties in language comprehension and mathematics. METHOD: Functional MRI was used to localize the areas of language and motor activation. The language activation paradigm was a visual verb generation task with a visual fixation baseline. The motor paradigm consisted of alternating blocks of sequential finger tapping and rest. Coronal functional and anatomical images were obtained. RESULTS: The motor paradigm produced activation of the primary motor cortex, the band heterotopia and the supplementary motor cortex. The language paradigm produced activation of the left inferior frontal gyrus and left supplementary motor area, but not of the band heterotopia. CONCLUSIONS: The activation of heterotopic grey matter during a motor task demonstrates a hemodynamic association with motor activity and suggests that this tissue may be functional. Such association was not seen with the language task. We speculate that later maturing functions such as language are restricted in their development to the normal situated superficial cortex in our patient.

Perspectives of young people and their parents in the transition of cochlear implant services: implications for improved service delivery.

OBJECTIVES: Youth and young adults with cochlear implants are now transitioning from pediatric to adult services in increasing numbers. Research in other areas of health care has indicated that there is a gap in the transition from pediatric services for the young adult, and that it is important to obtain their perspectives to reduce disruption and improve care. Previous research has documented issues from the perspective of cochlear implant professionals. The objectives of this study were to examine current practices from the perspective of young adults and their families and to make recommendations for future practice. METHODS: Interviews were conducted with 11 individuals, including cochlear implant recipients and their parents. All patients were within 4 years of transition between pediatric and adult hospital services: four youths were pediatric patients, and two had been discharged to adult services. Qualitative research methodology was used to identify key themes. RESULTS: All participants indicated that they had not anticipated a change to an adult hospital as part of their plan of care. Key themes from interviews were differences between pediatric and adult hospitals, challenges in establishing new relationships with professionals, specific concerns about new health care settings and procedures, and the need for youth to develop independent health-related skills in the context of parental involvement. DISCUSSION: Themes identified through interviews with young people with cochlear implants and their parents were similar to research in other areas of health care, as well as to themes identified in focus groups with professionals providing cochlear implant services. There were some differences which highlight both needs in the provision of health care and opportunities for providers and patients to collaborate to provide improved service delivery.

Comparison of outcomes in children with hearing aids and cochlear implants.

OBJECTIVES: The purpose of this study was to document the performance of a group of children with moderately severe to severe hearing loss who use hearing aids on a range of speech recognition, speech-language, and literacy measures and to compare these results to children with severe to profound hearing loss, who have learned language through cochlear implants. METHODS: This study involved 41 children with bilateral sensorineural hearing impairment, aged 6-18 years. Twenty children had moderately severe/severe hearing loss and used hearing aids, and 21 had severe to profound hearing loss and used cochlear implants. Communication and academic skills were assessed using speech recognition tests and standardized measures of speech production, language, phonology, and literacy. RESULTS: The two groups did not differ in their open-set speech recognition abilities or speech production skills. However, children with hearing aids obtained higher scores than their peers with cochlear implants in the domains of receptive vocabulary, language, phonological memory, and reading comprehension. The findings also indicate that children with moderately severe or severe hearing loss can develop spoken language skills that are within the range expected for normal hearing children. CONCLUSIONS: School-aged children with moderately severe and severe hearing loss performed better in several domains than their peers with profound hearing loss who received cochlear implants between age 2 and 5 years. Further research is required to evaluate the benefits of hearing aids and cochlear implants in children with hearing loss who are diagnosed and receive intervention within the first year of life.

Facilitating the transition from the pediatric to adult cochlear implant setting: perspectives of CI professionals.

OBJECTIVES: Over the last 20 years, the availability of cochlear implantation has resulted in a pediatric population with different health needs than adults who receive cochlear implants (CIs). These pediatric patients are now transitioning to adult hospital settings in significant numbers. This issue of transition is not unique to cochlear implant services: research in other chronic health conditions has documented a variety of challenges for youth and health care providers. The objectives of this study were to identify factors important in the transition from pediatric to adult CI services from the perspective of service providers and to make recommendations to improve transition practices in the future. METHODS: Focus groups were conducted with professionals providing specialized CI services in pediatric and adult hospitals, and specialized educators in the school setting. Qualitative research methodology was used to identify key themes. Data extracted from patient files allowed comparison of pediatric and adult CI recipients. RESULTS: Youth who had received CIs in the pediatric setting differed from other adult patients in the incidence of prelingual hearing losses, and age at CI surgery. Key focus group themes were related to service delivery models, communication between settings, and skills needed by the patients to effectively meet their own health needs. DISCUSSION: Factors identified by CI professionals were very similar to those identified in research for other health conditions. From the focus groups, as well as other literature, a number of recommendations are proposed to facilitate a positive transition of young people to adult health care.