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.

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.

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.

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.

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.

Progressive Hearing Loss in Children With Mild Bilateral Hearing Loss.

Purpose This study investigated progressive hearing loss in a cohort of children who were identified with permanent mild bilateral hearing loss. Method This population-based study included 207 children with permanent mild bilateral hearing loss, diagnosed and followed from 2003 to 2016 in 1 region of Canada. Clinical characteristics and initial audiologic results were collected prospectively at diagnosis, and audiologic information was updated. Changes in hearing levels between the 1st and most recent assessment were analyzed to determine progressive hearing loss. Clinical characteristics were compared between children with progressive and stable hearing loss. The association between risk indicators and progressive hearing loss was explored through logistic regression. Results A total of 47.4% (94 of 207) had progressive hearing loss in at least 1 ear, and 50% (47 of 94) of those experienced more than 20-dB average drop in thresholds. For these 94 children, a total of 147 ears were affected, and 116 (78.9%) ears experienced sufficient change in thresholds to be coded as a worse category of hearing loss. In the subset of 85 children with more than 5 years of audiologic follow-up, 56.4% (53/85) showed a decrease in hearing. Of the total sample of 207 children, 137 (66.2%) continued to have mild hearing loss in the better ear. There was no association between the risk factors examined (family history of hearing loss, admission to the neonatal intensive care unit, or presence of a syndrome) and progressive hearing loss. Conclusion This study found that almost half of children with mild bilateral hearing loss showed a decrease in hearing in at least 1 ear. One third of the children first diagnosed with mild hearing loss in the better ear now have moderate or worse hearing loss in both ears. These findings point to the importance of careful long-term monitoring of children who present with mild hearing loss.

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.

Auditory and language outcomes in children with unilateral hearing loss.

OBJECTIVES: Children with unilateral hearing loss (UHL) are being diagnosed at younger ages because of newborn hearing screening. Historically, they have been considered at risk for difficulties in listening and language development. Little information is available on contemporary cohorts of children identified in the early months of life. We examined auditory and language acquisition outcomes in a contemporary cohort of early-identified children with UHL and compared their outcomes at preschool age with peers with mild bilateral loss and with normal hearing. DESIGN: As part of the Mild and Unilateral Hearing Loss in Children Study, we collected auditory and spoken language outcomes on children with unilateral, bilateral hearing loss and with normal hearing over a four-year period. This report provides a cross-sectional analysis of results at age 48 months. A total of 120 children (38 unilateral and 31 bilateral mild, 51 normal hearing) were enrolled in the study from 2010 to 2015. Children started the study at varying ages between 12 and 36 months of age and were followed until age 36-48 months. The median age of identification of hearing loss was 3.4 months (IQR: 2.0, 5.5) for unilateral and 3.6 months (IQR: 2.7, 5.9) for the mild bilateral group. Families completed an intake form at enrolment to provide baseline child and family-related characteristics. Data on amplification fitting and use were collected via parent questionnaires at each annual assessment interval. This study involved a range of auditory development and language measures. For this report, we focus on the end of follow-up results from two auditory development questionnaires and three standardized speech-language assessments. Assessments included in this report were completed at a median age of 47.8 months (IQR: 38.8, 48.5). Using ANOVA, we examined auditory and language outcomes in children with UHL and compared their scores to children with mild bilateral hearing loss and those with normal hearing. RESULTS: On most measures, children with UHL performed poorer than those in the mild bilateral and normal hearing study groups. All children with hearing loss performed at lower levels compared to the normal hearing control group. However, mean standard scores for the normal hearing group in this study were above normative means for the language measures. In particular, children with UHL showed gaps compared to the normal hearing control group in functional auditory listening and in receptive and expressive language skills (three quarters of one standard deviation below) at age 48 months. Their performance in receptive vocabulary and speech production was not significantly different from that of their hearing peers. CONCLUSIONS: Even when identified in the first months of life, children with UHL show a tendency to lag behind their normal hearing peers in functional auditory listening and in receptive and expressive language development.

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.

Parent Report of Amplification Use in Children with Mild Bilateral or Unilateral Hearing Loss.

BACKGROUND: Amplification is considered to be one of the most important interventions for children with hearing loss. However, achieving consistent use of hearing technology in young children is an important problem, particularly when hearing loss is of mild degree. Little information is available about amplification use specifically for children with mild bilateral or unilateral hearing loss when such losses are targeted and identified early because of the availability of newborn hearing screening. PURPOSE: We examined amplification use in a contemporary cohort of early-identified children with mild bilateral and unilateral hearing loss. RESEARCH DESIGN: As part of the Mild and Unilateral Hearing Loss in Children Study, we collected parent reports on their child's use of amplification during the preschool years. STUDY SAMPLE: A total of 69 children (38 unilateral and 31 bilateral mild) enrolled in the study from 2010 to 2015. Children entered the study at various ages between 12 and 36 mo of age and were followed up to age 48 mo. The median age of the children at enrollment was 16.5 mo (interquartile range [IQR] = 9.5, 26.8). Hearing loss was confirmed in these children at a median age of 3.6 mo (IQR = 2.4, 5.7). DATA COLLECTION AND ANALYSIS: Baseline characteristics related to the child and family were collected through an intake form at study enrollment. Data on amplification fitting and use were collected via parent questionnaires at each assessment interval. Information from parent questionnaires was summarized descriptively and amplification use was grouped into categories. Through logistic regression, we examined the relationship between amplification use and laterality of hearing loss, sex, and maternal education. RESULTS: Amplification was recommended for 59 (85.5%) children at a median age of 6.5 mo (IQR = 3.6, 21.2) and children were fitted at a median age of 10.9 mo (IQR = 6.0, 22.1). Based on parent report, hearing aid use was consistent for 39 (66.1%) of 59 children who had amplification recommended. Parent questionnaires showed very little change in use for most of the children over the study period. More children with bilateral hearing loss used their amplification consistently than those with unilateral hearing loss. After adjusting for maternal education and sex of the child, the odds for consistent use in children with mild bilateral loss was almost seven times higher (odds ratio = 6.75; 95% confidence interval = 1.84, 24.8) than for those with unilateral loss. CONCLUSIONS: Although 85.5% of children with mild bilateral or unilateral hearing loss received amplification recommendations, only two-thirds achieved consistent use by age 3-4 yr based on parent report. Children with mild bilateral loss were more likely to use amplification during the preschool years than those with unilateral loss.

Exploring reasons for late identification of children with early-onset hearing loss.

INTRODUCTION: Several studies have shown that early identification of childhood hearing loss leads to better language outcomes. However, delays in the confirmation of hearing loss persist even in the presence of well-established universal newborn hearing screening programs (UNHS). The objective of this population-based study was to document the proportion of children who experienced delayed confirmation of congenital and early onset hearing loss in a UNHS program in one region of Canada. The study also sought to determine the reasons for delayed confirmation of hearing loss in children. METHODS: Population level data related to age of first assessment, age of identification and clinical characteristics were collected prospectively for all children identified through the UNHS program. We documented the number of children who experienced delay (defined as more than 3 months) from initial audiologic assessment to confirmation of hearing loss. A detailed chart review was subsequently performed to examine the reasons for delay to confirmation. RESULTS: Of 418 children identified from 2003 to 2013, 182 (43.5%) presented with congenital or early onset hearing loss, of whom 30 (16.5%) experienced more than 3 months delay from initial audiologic assessment to confirmation of their hearing disorder. The median age of first assessment and confirmation of hearing loss for these 30 children was 3.7 months (IQR: 2.0, 7.6) and 13.8 months (IQR: 9.7, 26.1) respectively. Close examination of the factors related to delay to confirmation revealed that for the overwhelming majority of children, a constellation of factors contributed to late diagnosis. Several children (n = 22; 73.3%) presented with developmental/medical issues, 15 of whom also had middle ear dysfunction at assessment, and 9 of whom had documented family follow-up concerns. For the remaining eight children, additional reasons included ongoing middle ear dysfunction for five children, complicated by family follow-up concerns (n = 3) and mild hearing loss (n = 1) and the remaining three children had isolated reasons related to family follow-up (n = 1) or mild hearing loss (n = 2). CONCLUSION: Despite the progress made in the early detection of pediatric hearing loss since UNHS, a substantial number of children referred for early assessment can experience late confirmation and intervention. In particular, infants with developmental and/or medical issues including middle ear disorders are at particular risk for longer time to confirmation of hearing loss.

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.

Progressive Hearing Loss in Early Childhood.

OBJECTIVES: Deterioration in hearing thresholds in children is of concern due to the effect on language development. Before universal newborn hearing screening (UNHS), accurate information on the progression of hearing loss was difficult to obtain due to limited information on hearing loss onset. The objective of this population-based study was to document the proportion of children who experienced progressive loss in a cohort followed through a UNHS program in one region of Canada. We explored risk factors for progression including risk indicators, audiologic, and clinical characteristics of children. We also investigated deterioration in hearing as a function of age. For this study, two working definitions of progressive hearing loss were adopted: (1) a change of ≥20 dB in the 3 frequencies (500, 1000, and 2000 Hz) pure-tone average, and (2) a decrease of ≥10 dB at two or more adjacent frequencies between 500 and 4000 Hz or a decrease in 15 dB at one octave frequency in the same frequency range. DESIGN: Population-based data were collected prospectively on a cohort of children identified from 2003 to 2013 after the implementation of UNHS. Clinical characteristics including risk indicators (as per Joint Committee on Infant Hearing), age at diagnosis, type and severity of hearing loss, and initial audiologic information were recorded when children were first identified with hearing loss. Serial audiometric results were extracted from the medical charts for this study. Differences between children with progressive and stable hearing loss were explored using χ tests. Association between risk indicators and progressive hearing loss was assessed through logistic regression. The cumulative amount of deterioration in hearing from 1 to 4 years of age was also examined. RESULTS: Our analysis of 330 children (251 exposed to screening) with detailed audiologic records showed that 158 (47.9%) children had some deterioration (at least ≥10 dB and) in hearing thresholds in at least one ear. The 158 children included 76 (48.1%) with ≥20 dB loss in pure-tone average in at least one ear and 82 (51.9%) with less deterioration in hearing levels (≥10 but <20 dB). In the children with progressive hearing loss, of 131 children initially diagnosed with bilateral loss, 75 (57.3%) experienced deterioration in 1 ear and 56 (112 ears; 42.7%) in both ears (total of 187 ears). Of 27 children with an initial diagnosis of unilateral loss, 25 experienced deterioration in the impaired ear and 5 in the normal-hearing ear, progressing to bilateral hearing loss. Within 4 years after diagnosis, the mean decrease in hearing for children with progressive loss was 25.9 dB (SD: 16.4) in the right ear and 28.3 dB (SD: 12.9) in the left ear. We explored the risk factors for hearing loss identified by Joint Committee on Infant Hearing where there were sufficient numbers in our sample. On multivariate analysis, there was no statistically significant relationship between most risk indicators examined (neonatal intensive care unit admission, family history, syndromes, and postnatal infections) and the likelihood of progressive loss. However, the presence of craniofacial anomalies was inversely associated with risk of progressive hearing loss (odds ratio = 0.27; 95% confidence interval: 0.10, 0.71; p = 0.01), that is, these children were more likely to have stable hearing. CONCLUSIONS: Given that almost half of the children in this cohort experienced deterioration in hearing, close postneonatal monitoring of hearing following early hearing loss identification is essential to ensure optimal amplification and therapy.

Communication Development in Early-Identified Children With Mild Bilateral and Unilateral Hearing Loss.

PURPOSE: This clinical focus article provides preliminary findings from a multicenter longitudinal study investigating auditory and communication development in children with mild bilateral or unilateral hearing loss of any degree. METHOD: A total of 100 children (55 with mild bilateral or unilateral hearing loss and 45 with normal hearing) underwent 1 or more assessments from a battery of auditory and language measures. RESULTS: Children up to age 4 years demonstrated auditory and language skills similar to those of children with normal hearing. CONCLUSIONS: Factors that affect outcomes in communication development will be explored as additional data are collected in this ongoing study.

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.

Factors constraining the benefit to speech understanding of combining information from low-frequency hearing and a cochlear implant.

Many studies have documented the benefits to speech understanding when cochlear implant (CI) patients can access low-frequency acoustic information from the ear opposite the implant. In this study we assessed the role of three factors in determining the magnitude of bimodal benefit - (i) the level of CI-only performance, (ii) the magnitude of the hearing loss in the ear with low-frequency acoustic hearing and (iii) the type of test material. The patients had low-frequency PTAs (average of 125, 250 and 500 Hz) varying over a large range (<30 dB HL to >70 dB HL) in the ear contralateral to the implant. The patients were tested with (i) CNC words presented in quiet (n = 105) (ii) AzBio sentences presented in quiet (n = 102), (iii) AzBio sentences in noise at +10 dB signal-to-noise ratio (SNR) (n = 69), and (iv) AzBio sentences at +5 dB SNR (n = 64). We find maximum bimodal benefit when (i) CI scores are less than 60 percent correct, (ii) hearing loss is less than 60 dB HL in low-frequencies and (iii) the test material is sentences presented against a noise background. When these criteria are met, some bimodal patients can gain 40-60 percentage points in performance relative to performance with a CI. This article is part of a Special Issue entitled .

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.

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.