Long-term follow-up of children with hearing loss that is minimally progressive.

OBJECTIVES: The purpose of this study was to describe changes in audiometric thresholds over time in children whose hearing loss demonstrated early mild progression. DESIGN: This was a retrospective follow-up study to examine long-term audiologic results in children with progressive loss. STUDY SAMPLE: We examined audiologic data for 69 children, (diagnosed from 2003 to 2013), who had been previously categorised as having "minimal" progressive hearing loss. RESULTS: Children had a median of 10.0 (7.5, 12.1) years of follow-up and a median age of 12.5 (IQR: 11.0, 14.5) years; 92.8%; 64 of 69) of children continued to show progressive hearing loss (defined as a decrease of ≥10 dB at two or more adjacent frequencies between 0.5 and 4 kHz or a decrease in 15 dB at one frequency) in at least one ear since diagnosis. Further examination showed that 82.8% of ears (106 of 128) had deterioration in hearing. Of the 64 children, 29.7% (19/64) showed further deterioration since the first analysis. CONCLUSION: More than 90% of children identified as having minimal progressive hearing loss continued to show deterioration in hearing. Ongoing audiological monitoring of children with hearing loss is indicated to ensure timely intervention and to better counsel families.

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.

Benefits of auditory-verbal intervention for adult cochlear implant users: perspectives of users and their coaches.

OBJECTIVE: Post-implant rehabilitation is limited for adult cochlear implant (CI) recipients. The objective of this research was to capture the perspectives of CI users and their coaches regarding their experiences with auditory-verbal intervention as an example of post-implant rehabilitation and their views on perceived benefits and challenges related to the intervention. DESIGN: This qualitative study involved semi-structured focus group interviews with adult CI users and their coaches who accompanied them in a 24-week auditory-verbal intervention program. STUDY SAMPLE: A total of 17 participants (eight CI users and nine coaches) contributed to the interviews. RESULTS: Three key topic areas emerged from the interviews capturing CI users' and coaches' experiences related to the intervention program: (1) benefits of the intervention, (2) factors affecting experiences, and (3) challenges and barriers. Benefits included increased confidence in hearing, communication, social participation, and new knowledge about technology and hearing. Factors affecting the experience were participants' motivation and the therapist's skills. The primary challenge was the time commitment for weekly therapy. CONCLUSIONS: Both CI users and coaches perceived a focussed auditory-verbal intervention to be beneficial in improving speech understanding, confidence in using hearing, social interaction, and knowledge about technology. Participants recommended reducing the intensity of intervention to facilitate participation.

Paediatric hearing aid use: a systematic review.

CONTEXT: Hearing loss (HL) is one of the most common disorders present at birth. Parents' management of their child's hearing aids (HAs) and regular follow-up with healthcare providers HA are fundamental components of effective intervention. OBJECTIVE: The primary objective of this systematic review was to synthesise the current literature on HA use in the paediatric population, and the secondary objective was to review the factors associated with HA use. METHODS: Electronic databases, including MEDLINE, EMBASE, CINAHL, and LLBA from 2005 to 2019, were searched. Two reviewers individually screened potentially relevant articles over two phases. RESULTS: Fifteen studies met this review criteria. Four studies reported HA use based on data logging records. In nine studies, the amount of HA use was evaluated based on parents' reports, and three studies concluded that parents overestimate their child's HA use. Age, degree of HL and parents' education level were the most frequently reported factors associated with a child's amount of HA use. CONCLUSIONS: The results of this review will provide a foundation for future studies on the importance of monitoring HA use and the impact of consistent HA use on the language development of children with HL.

Parent-Reported Stress and Child Behavior for 4-Year-Old Children with Unilateral or Mild Bilateral Hearing Loss.

Children with unilateral or mild bilateral hearing loss are increasingly identified in early childhood. Relatively little is known about how hearing loss affects their developmental trajectory or whether it contributes to parenting stress for these parents. This study aimed to examine child behavior and parenting stress in parents of children with unilateral/mild bilateral hearing loss compared to children with typical hearing. This prospective study involved 54 children with unilateral/mild bilateral hearing loss identified at a median age of 4.5 months (IQR 2.6, 6.5) and 42 children with typical hearing. At age 48 months, child behavior and parenting stress were measured. Auditory and language results were also analyzed in relation to child behavior and parenting stress. Parents of these children did not report significantly more parenting stress or behavior problems than parents of children with typical hearing. However, both parenting stress and child behavior were related to functional hearing in noise.

Cytomegalovirus-A Risk Factor for Childhood Hearing Loss: A Systematic Review.

OBJECTIVE: Permanent hearing loss is an important public health issue in children with consequences for language, social, and academic functioning. Early hearing detection, intervention, and monitoring are important in mitigating the impact of permanent childhood hearing loss. Congenital cytomegalovirus (CMV) infection is a leading cause of hearing loss. The purpose of this review was to synthesize the evidence on the association between CMV infection and permanent childhood hearing loss. DESIGN: We performed a systematic review and examined scientific literature from the following databases: MEDLINE, Ovid MEDLINE(R) Daily and Ovid MEDLINE(R), Embase, and CINAHL. The primary outcome was permanent bilateral or unilateral hearing loss with congenital onset or onset during childhood (birth to 18 years). The secondary outcome was progressive hearing loss. We included studies reporting data on CMV infection. Randomized controlled trials, quasi-experimental studies, nonrandomized comparative and noncomparative studies, and case series were considered. Data were extracted and the quality of individual studies was assessed with the Qualitative Assessment Tool for Quantitative Studies (McMaster University). The quality and strength of the evidence were graded using the Grading of Recommendations Assessment, Development and Evaluation (GRADE). A narrative synthesis was completed. RESULTS: Sixty-five articles were included in the review. Prevalence of hearing loss at birth was over 33% among symptomatic CMV-infected newborns and less than 15% in asymptomatic infections. This difference in prevalence was maintained during childhood with more than 40% prevalence reported for symptomatic and less than 30% for asymptomatic CMV. Late-onset and progressive hearing loss appear to be characteristic of congenital CMV infections. Definitions of hearing loss, degree of loss, and reporting of laterality varied across studies. All degrees and both bilateral and unilateral loss were reported, regardless of symptomatic and asymptomatic status at birth, and no conclusions about the characteristics of hearing loss could be drawn. Various patterns of hearing loss were reported including stable, progressive, and fluctuating, and improvement in hearing (sometimes to normal hearing) was documented. These changes were reported in children with symptomatic/asymptomatic congenital CMV infection, presenting with congenital/early onset/late-onset hearing loss and in children treated and untreated with antiviral medication. CONCLUSIONS: Symptomatic and asymptomatic congenital CMV infection should be considered a risk factor for hearing loss at birth and during childhood and for progressive hearing loss. Therefore, CMV should be included as a risk factor in screening and surveillance programs and be taken into account in clinical follow-up of children with hearing loss.

Hearing loss prevalence and hearing health among school-aged children in the Canadian Arctic.

OBJECTIVE: Hearing loss is an important health concern in Canada's Arctic. The objective of this research was to provide information on the prevalence of childhood hearing loss in Nunavut. DESIGN: This cross-sectional study involved comprehensive audiologic assessments of school-aged children in six communities to determine overall and community-specific prevalence of hearing loss. Data were collected about hearing aid use and factors affecting use through a parent questionnaire. STUDY SAMPLE: Assessments were completed for 644 children in kindergarten to grade 6. RESULTS: 124 (19.3%) children had hearing loss of ≥ 30 dB HL at one or more frequencies in at least one ear (93.5% conductive loss). Applying a Canadian prevalence study definition, 148 (23.5%) children had hearing loss. Tympanic membrane perforations were present in 36.8% (n = 28) of children with unilateral and 45.8% (n = 22) with bilateral loss. CONCLUSIONS: The prevalence of hearing loss in Canada's North was almost three times that reported for non-indigenous children. One in five school-aged children was found to have hearing loss that is likely to affect classroom learning and social/emotional development. A hearing health strategy tailored to this population is critically needed.

Coaching Caregivers of Children who are Deaf or Hard of Hearing: A Scoping Review.

Caregiver coaching is an expected practice in early intervention. However, little is known about coaching with caregivers of children who are deaf or hard of hearing, receiving services for listening and spoken language (LSL). A systematic review of 7 databases, the gray literature, and consultation with 7 expert LSL practitioners yielded 506 records for full-text review, 22 of which were ultimately included in the review. Our findings are presented as 3 themes: coaching practices, training for coaching, and effectiveness of coaching. Eight models of coaching were identified in the literature, from which we identified commonalities to propose a consolidated model that illustrates the recommendations and process of caregiver coaching found in the LSL literature.

Auditory, Social, and Behavioral Skills of Children With Unilateral/Mild Hearing Loss.

This study explored the impact of mild bilateral or unilateral hearing loss on auditory, social, and behavior skills in early school-aged children. Thirty-two children (aged 5-9 years) were evaluated with parent and teacher questionnaires. Most outcomes were within the range of expected scores. However, functional auditory skills were below published results for children with typical hearing. On the social skills scale, about 21.4% (parent-reported) and 20.0% (teacher-reported) of children were below one standard deviation (SD) of the normative mean (i.e., a standard score below 85). On the parent-reported behavior test, over a quarter of children scored beyond 1 SD on some subscales. Laterality of hearing loss had no effect on outcomes (p > .05). Agreement between parents and teachers varied from poor (intraclass correlation coefficient [ICC]: .162) to moderate (ICC: .448). Results indicate that these children are functioning in most areas like their peers with typical hearing. Additional research on this population of children who may benefit from early identification and amplification is warranted.

A Qualitative Study on Factors Modulating Tinnitus Experience.

OBJECTIVES: It is well recognized that tinnitus can severely interfere with important aspects of life such as sleep, concentration abilities, social activities, and mood. However, the impact of tinnitus may vary greatly from one individual to another, with some being severely affected and others only experiencing a slight handicap. Information from participants' experiences with tinnitus could help explain such variability. The full experience of living with tinnitus from a patient's perspective has been under-investigated, especially in comparison with the large and extensive body of qualitative research on hearing loss. This study aims to contribute to fill this gap and assess participants' experience of living with tinnitus. DESIGN: As part of a larger mixed design study, this inquiry used qualitative analyses to investigate participant's experiences with tinnitus. Twenty-seven tinnitus participants of different ages, sexes, audiometric profiles, and tinnitus severities were invited to discuss their tinnitus through semistructured interviews and journal entries. The interviews took place 1 month after participants completed a 3-week trial period with tinnitus maskers. RESULTS: The findings were grouped into three main themes: participants' appraisal of their tinnitus, factors modulating tinnitus experience, and consequences of tinnitus. Regarding participants' appraisal of their tinnitus, a dissociation between the tinnitus sensation (pitch, loudness) and tinnitus experience (annoyance/distress) was reported. Factors modulating tinnitus experience were classified as environmental factors and individual-specific factors, which were further divided into soundscape and other environmental factors for the former and attentional focus, stress level, physical state, fatigue, and auditory effort for the latter. Consequences of tinnitus included distraction, disrupted mental health and mood, headaches, fatigue, sleep disturbances, hearing difficulties, disrupted social life, and frustration. CONCLUSIONS: Two of the main themes (participants' appraisal of their tinnitus and consequences of tinnitus) were consistent with findings from a few previous qualitative studies. Factors modulating tinnitus experience also emerged as a prominent theme. Indeed, participants described a plethora of factors that affected their experiences of tinnitus. On the basis of these findings, an attentional model is proposed to account for how these factors might modulate the effects of tinnitus.

Service Preferences of Parents of Children With Mild Bilateral or Unilateral Hearing Loss: A Conjoint Analysis Study.

OBJECTIVE: Universal newborn hearing screening results in substantially more children with mild bilateral and unilateral hearing loss identified in the early years of life. While intervention services for children with moderate loss and greater are generally well-established, considerable uncertainty and variation surrounds the need for intervention services for children with milder losses. This study was undertaken with parents of young children with permanent mild bilateral and unilateral hearing loss to examine their preferences for characteristics associated with intervention services. DESIGN: Conjoint analysis, a preference-based technique, was employed to study parents' strength of preferences. Using a cross-sectional survey that consisted of eight hypothetical clinic scenarios, we invited parents to make a discrete choice (to select one of two or more different services) between available services with different characteristics. The survey was informed by qualitative interviews conducted for this purpose. The questionnaire was administered to parents receiving intervention services in the province of Ontario, Canada, who were enrolled in a mixed-methods longitudinal study examining outcomes in early-identified children with mild bilateral/unilateral hearing loss. Data were analyzed using a generalized linear model (probit link) to identify attributes of interest for the respondents. Characteristics of the children were entered into the model to control for differences in age of diagnosis, sex, laterality of hearing loss, and hearing aid use. RESULTS: A total of 51 of 62 invited parents completed the questionnaire. All four attributes of care that were included in the survey were found to be statistically significant, that is, parents valued support for amplification, support for speech-language development, emotional support, and communication from professionals. Analysis showed greater preference for enhanced levels relating to support for speech-language development than for support for amplification. Preference for attributes relating to emotional support and communication were also greater than for support for amplification use. CONCLUSIONS: Conjoint analysis was used to quantify parents' preferences for service attributes. Parents' values provide insights into the aspects of a service model that should receive consideration in the development of intervention programs for young children with mild bilateral or unilateral hearing loss and their families. Although parents of young children with mild bilateral or unilateral hearing loss valued several components of care, they indicated a clear preference for speech-language support compared with support for amplification use.

Development and validation of the FrBio, an international French adaptation of the AzBio sentence lists.

Objectives: The goal of this work was to develop and normalise an international French version of the AzBio sentence test. Design: A corpus of 1000 sentences was generated. These sentences were recorded with four talkers and processed through a four-channel cochlear implant simulation. The mean intelligibility for each sentence achieved by 16 normal-hearing listeners was computed. The consecutively ordered 165 sentences from each talker rendering an average score of 85% were sequentially assigned to 33 lists of 20 sentences. All lists were presented to 30 normal-hearing and 25 hearing-impaired listeners in order to verify their equivalency. Thirty normal-hearing adults were also recruited to assess the test's psychometrics and define norms. Results: The results of the list equivalency validation study showed no significant differences in percent correct scores for 30 sentence lists. A binomial distribution model was used to estimate the 95% critical differences for each potential percentage score. Normalization data showed an average performance between 96% and 99% with a very low standard deviation. Conclusions: With a set of 30 lists, researchers and clinicians can use the FrBio to evaluate a large number of experimental conditions; changes in performance over time or across conditions can then be tracked.

School-aged Children with Mild Bilateral and Unilateral Hearing Loss: Parents' Reflections on Services, Experiences, and Outcomes.

Following the establishment of newborn hearing screening programs, age of identification and length of time before receiving interventions has been reduced for children, including those with milder degrees of hearing loss who were previously not identified until school age. This population of early-identified children requires new support programs for parents. Although literature is emerging on how parents experience the initial years, there is limited information on support needs during early school years. The objectives were to gain insights into parents' experiences with services during the early period of identification until early school years, as well as their perceptions of the consequences of hearing loss on their child's overall development. A qualitative research design informed by Interpretive Description was employed. Individual semi-structured interviews were conducted with 12 parents of children identified with mild hearing loss. Transcripts were analyzed using a constant comparative method. Four themes emerged from the data: early experiences with services and hearing technology, effects of hearing loss on social functioning, effects of hearing loss on language and academics, and experiences in early school years. From parents' perspectives, more support during the early school years is needed to help ensure academic success.

Characteristics of children with unilateral hearing loss.

OBJECTIVE: The purpose of this study was to describe the clinical characteristics of children with unilateral hearing loss (UHL), examine deterioration in hearing, and explore amplification decisions. DESIGN: Population-based data were collected prospectively from time of diagnosis. Serial audiograms and amplification details were retrospectively extracted from clinical charts to document the trajectory and management of hearing loss. SAMPLE: The study included all children identified with UHL in one region of Canada over a 13-year period (2003-2015) after implementation of universal newborn hearing screening. RESULTS: Of 537 children with permanent hearing loss, 20.1% (108) presented with UHL at diagnosis. They were identified at a median age of 13.9 months (IQR: 2.8, 49.0). Children with congenital loss were identified at 2.8 months (IQR: 2.0, 3.6) and made up 47.2% (n = 51), reflecting that a substantial portion had late-onset, acquired or late-identified loss. A total of 42.4% (n = 39) showed deterioration in hearing, including 16 (17.4%) who developed bilateral loss. By study end, 73.1% (79/108) of children had received amplification recommendations. CONCLUSIONS: Up to 20% of children with permanent HL are first diagnosed with UHL. About 40% are at risk for deterioration in hearing either in the impaired ear and/or in the normal hearing ear.

Pediatric Cochlear Implantation: Why Do Children Receive Implants Late?

OBJECTIVES: Early cochlear implantation has been widely promoted for children who derive inadequate benefit from conventional acoustic amplification. Universal newborn hearing screening has led to earlier identification and intervention, including cochlear implantation in much of the world. The purpose of this study was to examine age and time to cochlear implantation and to understand the factors that affected late cochlear implantation in children who received cochlear implants. DESIGN: In this population-based study, data were examined for all children who underwent cochlear implant surgery in one region of Canada from 2002 to 2013. Clinical characteristics were collected prospectively as part of a larger project examining outcomes from newborn hearing screening. For this study, audiologic details including age and severity of hearing loss at diagnosis, age at cochlear implant candidacy, and age at cochlear implantation were documented. Additional detailed medical chart information was extracted to identify the factors associated with late implantation for children who received cochlear implants more than 12 months after confirmation of hearing loss. RESULTS: The median age of diagnosis of permanent hearing loss for 187 children was 12.6 (interquartile range: 5.5, 21.7) months, and the age of cochlear implantation over the 12-year period was highly variable with a median age of 36.2 (interquartile range: 21.4, 71.3) months. A total of 118 (63.1%) received their first implant more than 12 months after confirmation of hearing loss. Detailed analysis of clinical profiles for these 118 children revealed that late implantation could be accounted for primarily by progressive hearing loss (52.5%), complex medical conditions (16.9%), family indecision (9.3%), geographical location (5.9%), and other miscellaneous known (6.8%) and unknown factors (8.5%). CONCLUSIONS: This study confirms that despite the trend toward earlier implantation, a substantial number of children can be expected to receive their first cochlear implant well beyond their first birthday because they do not meet audiologic criteria of severe to profound hearing loss for cochlear implantation at the time of identification of permanent hearing loss. This study underscores the importance of carefully monitoring all children with permanent hearing loss to ensure that optimal intervention including cochlear implantation occurs in a timely manner.

Mild bilateral and unilateral hearing loss in childhood: a 20-year view of hearing characteristics, and audiologic practices before and after newborn hearing screening.

OBJECTIVES: There is some evidence that the presence of even mild degrees of hearing loss and unilateral loss in early childhood can negatively affect typical development. Consequently, the identification of all children with permanent hearing loss including those with milder degrees of hearing loss is currently targeted through some newborn hearing screening initiatives. The objectives of this study were to document the proportion of children identified with mild bilateral or unilateral hearing loss (MBUHL) before and after the implementation of a universal newborn hearing screening program (UNHS). A second objective was to examine the clinical characteristics and audiologic management of this population of children. DESIGN: This population-based study involved an analysis of data on children with hearing loss; these data had been collected prospectively over a 20-year period from 1990 to 2010 in a publicly funded pediatric teaching hospital in Canada, which provided diagnostic audiology services for all children in the region. The proportion of all children with hearing loss identified with MBUHL before and after implementation of UNHS and their clinical profiles were documented. Data related to amplification use were also retrospectively extracted from detailed medical chart reviews to investigate clinical recommendations regarding amplification and subsequent use of hearing devices. Factors affecting amplification decisions and amplification use were examined. RESULTS: A total of 381 of 823 children (46.3%) aged 0 to 18 years who were identified with permanent nonacquired hearing loss presented with MBUHL. The proportions identified were similar in the pre- and post-UNHS periods (p = 0.207). Before UNHS children were identified at a median age of 5.0 years (interquartile range: 3.6 to 7.0) compared with a median of 0.8 years, (interquartile range: 0.3 to 2.3) post-UNHS. Progression of hearing loss was documented for 22% of all children, and for the group identified since UNHS, 32.4% experienced deterioration in hearing thresholds. Although 87.2 % of children received recommendations for amplification, more than 50% experienced considerable delay from identification to amplification. Age at identification and severity of hearing loss in the better ear were associated with amplification decisions for bilateral but not unilateral loss. CONCLUSIONS: MBUHL constitutes almost half of childhood hearing loss. UNHS has lowered age of confirmation of MBUHL by 4 to 5 years. Delay to amplification suggests uncertainty of the benefit of intervening early for these losses. Further research is required to determine the effects of early-identified MBUHL on children's development and to document the benefits of early amplification.

Examination of characteristics and management of children with hearing loss and autism spectrum disorders.

OBJECTIVE: Up to 40% of children with hearing loss present with other developmental disabilities. The purpose of this study was to document the prevalence of autism spectrum disorders (ASD) in children with permanent hearing loss, to describe the audiologic characteristics, and to examine clinical management. DESIGN: Prospective data related to clinical characteristics of children identified with hearing loss and ASD were examined. A retrospective chart review was also conducted to explore clinical management and uptake of amplification. STUDY SAMPLE: The study included all children in one Canadian region identified with permanent hearing loss and followed from 2002-2010. RESULTS: Of a total of 785 children with permanent hearing loss, 2.2% (n = 17) also received a diagnosis of ASD. The 13 boys and 4 girls presented with a range of audiologic profiles from unilateral to profound bilateral hearing loss. Four of five children with unilateral hearing loss experienced progression to bilateral loss. Amplification was recommended for all but one child and 9 of 16 children continued to use their hearing devices. CONCLUSIONS: The higher prevalence rate of ASD in this clinical population is consistent with previous reports. Our findings suggest that some children with autism can derive benefits from the use of amplification.

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.

Predictors of cochlear implant outcomes in pediatric auditory neuropathy: A matched case-control study.

OBJECTIVES: Current evidence supports the benefits of cochlear implants (CIs) in children with hearing loss, including those with auditory neuropathy spectrum disorder (ANSD). However, there is limited evidence regarding factors that hold predictive value for intervention outcomes. DESIGN: This retrospective case-control study consisted of 66 children with CIs, including 22 with ANSD and 44 with sensorineural hearing loss (SNHL) matched on sex, age, age at CI activation, and the length of follow-up with CIs (1:2 ratio). The case and control groups were compared in the results of five open-set speech perception tests, and a Forward Linear Regression Model was used to identify factors that can predict the post-CI outcomes. RESULTS: There was no significant difference in average scores between the two groups across five outcome measures, ranging from 88.40% to 95.65%. The correlation matrix revealed that younger ages at hearing aid fitting and CI activation positively influenced improvements in speech perception test scores. Furthermore, among the variables incorporated in the regression model, the duration of follow-up with CIs, age at CI activation, and the utilization of two CIs demonstrated prognostic significance for improved post-CI speech perception outcomes. CONCLUSIONS: Children with ANSD can achieve similar open-set speech perception outcomes as children with SNHL. A longer CI follow-up, a lower age at CI activation, and the use of two CIs are predictive for optimal CI outcome.