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Objectives: To explore functional neuroanatomical responses to auditory stimulation before and after implantation. Study Design: A prospective study of three cochlear implant candidates (pure-tone averages of 90 dB HL or greater bilaterally and hearing in noise test [HINT] performances of <40%) in which regional cerebral blood flow (rCBF) was assessed using single photon emission computed tomography (SPECT). Methods: Candidates watched a 15-minute videotaped story under four conditions: audio presented monaurally in the right and left ears (aided), audio presented binaurally (aided), and visual-only presentation of the story. Five minutes into each story, 20 to 25 mCi of technetium 99m (99mTc) hexamethyl-propyleneamine-oxime (HMPAO) (Ceratec; Nycomed Amersham, Princeton, NJ, U.SA) was injected over a 30-second period to ensure that subjects were unaware of tracer administration. Subjects were scanned for 20 minutes using a PRISM 3000 gamma camera (Picker International, Cleveland, OH, U.S.A.). Data were normalized and co-registered, and subtraction images were compiled. Subtraction images contrasted activation patterns generated under the visual-only control condition to the auditory activation states acquired monaurally and binaurally. Results: Right and left ear monaural stimulation in normal hearing subjects resulted in significant bilateral activation of Brodmann areas 41, 42, 21, 22, and 38. Although substantial intersubject response variability was noted, subjects generally failed to bilaterally activate these areas under monaural hearing aid presentations; however, bilateral activation of areas 41 and 22 was noted under binaural presentations. Conclusions: Despite relatively similar hearing losses in each ear, significant differences in preoperative auditory cortex activation were observed between ears. These data suggest that functional brain imaging provides a useful tool for exploring the responsiveness of the auditory cortex in cochlear implant candidates. [ABSTRACT FROM AUTHOR]

In this study we examined the role of functional brain imaging of regional cerebral bloodflow (rCBF) with the use of single photon emission computed tomography (SPECT) for the objective measurement of brain performance in adult cochlear implant (CI) users during speech perception. The subjects consisted of nine normal-hearing and eight CI individuals who watched a 15-min videotape under two conditions: (1) a visual-only presentation; and (2) a left-monaural audio and visual presentation. Cortical activations were observed bilaterally in Brodmann areas 41, 42, 21, 22 and 38 for normal-hearing control subjects. Bilateral activations were also observed in CI individuals who demonstrated high performance on open-set speech recognition tasks; however, activations were smaller in both amplitude and extent than those observed for normal-hearing individuals. CI individuals with minimal open-set recognition demonstrated only unilateral activation of auditory cortex in the hemisphere contralateral to the ear of implantation. These data support SPECT as a tool for objectively documenting cortical activations in adult CI users. [ABSTRACT FROM AUTHOR]

The efficacy of cochlear implants in children who are deaf has been firmly established in the literature. However, the effectiveness of cochlear implants varies widely and is influenced by demographic and experiential factors. Several key findings suggest new directions for research on central auditory factors that underlie the effectiveness of cochlear implants. First, enormous individual differences have been observed in both adults and children on a wide range of audiological outcome measures. Some patients show large increases in speech perception scores after implantation, whereas others display only modest gains on standardized tests. Second, age of implantation and length of deafness affect all outcome measures. Children implanted at younger ages do better than children implanted at older ages, and children who have been deaf for shorter periods do better than children who have been deaf for longer periods. Third, communication mode affects outcome measures. Children from 'oral-only' environments do much better on standardized tests that assess phonological processing skills than children who use Total Communication. Fourth, at the present time there are no preimplant predictors of outcome performance in young children. The underlying perceptual, cognitive, and linguistic abilities and skills emerge after implantation and improve over time. Finally, there are no significant differences in audiological outcome measures among current implant devices or processing strategies. This finding suggests that the major source of variance in outcome measures lies in the neural and cognitive information processing operations that the user applies to the signal provided by the implant. Taken together, this overall pattern of results suggests that higher-level central processes such as perception, attention, learning, and memory may play important roles in explaining the large individual differences observed among users of cochlear implants. Investigations of the content and flow of information in the central nervous system and interactions between sensory input and stored knowledge may provide important new insights into the basis of individual differences. Knowledge about the underlying basis of individual differences may also help in developing new intervention strategies to improve the effectiveness of cochlear implants in children who show relatively poor development of oral/aural language skills. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study was to investigate the development of consonant inventory and accuracy in pediatric cochlear implant (CI) recipients and compare their performance to typical hearing (TH) children., Methods: One hundred and twenty nine children with CIs, implanted between 6-38 months of age, and 30 age-matched children with TH participated in this study. Spontaneous speech samples were collected at 3.5 and 4.5 years chronological age and the first 100 different words spoken by each participant were transcribed. Two consonant production measures were subsequently calculated to assess consonant acquisition and mastery. The percentage of Consonants Correct (CC) was used for measuring accuracy and Consonant Diversity (CD), an inventory measure, was used to identify the number of different consonants spoken by each participant. Repeated measures analyses of variance were conducted to examine the differences in consonant production scores based on presence of CI (participants with CI versus typical hearing (TH) participants), and chronological age at data collection (3.5 years versus 4.5 years)., Results: CI recipients displayed lower consonant production scores compared to TH children. Children with the most device experience (32-38 months at 3.5 years) performed on par with their TH peers., Conclusions: The two measures used in this study together appear capable of comprehensively describing the changes in consonant production skills of children. Results from this study indicate that while most CI participants display lower scores compared to TH children, many of the CI users are able to produce speech sounds on par with TH children., Competing Interests: Declaration of Competing Interest None., (Copyright © 2019. Published by Elsevier Inc.)

The Lombard effect is an involuntary response speakers experience in the presence of noise during voice communication. This phenomenon is known to cause changes in speech production such as an increase in intensity, pitch structure, formant characteristics, etc., for enhanced audibility in noisy environments. Although well studied for normal hearing listeners, the Lombard effect has received little, if any, attention in the field of cochlear implants (CIs). The objective of this study is to analyze speech production of CI users who are postlingually deafened adults with respect to environmental context. A total of six adult CI users were recruited to produce spontaneous speech in various realistic environments. Acoustic-phonetic analysis was then carried out to characterize their speech production in these environments. The Lombard effect was observed in the speech production of all CI users who participated in this study in adverse listening environments. The results indicate that both suprasegmental (e.g., F0, glottal spectral tilt and vocal intensity) and segmental (e.g., F1 for /i/ and /u/) features were altered in such environments. The analysis from this study suggests that modification of speech production of CI users under the Lombard effect may contribute to some degree an intelligible communication in adverse noisy environments.

Objectives: The goal of this study is to investigate whether prior exposure to reverberant listening environment improves speech intelligibility of adult cochlear implant (CI) users., Methods: Six adult CI users participated in this study. Speech intelligibility was measured in five different simulated reverberant listening environments with two different speech corpuses. Within each listening environment, prior exposure was varied by either having the same environment across all trials (blocked presentation) or having different environment from trial to trial (unblocked)., Results: Speech intelligibility decreased as reverberation time increased. Although substantial individual variability was observed, all CI listeners showed an increase in the blocked presentation condition as compared to the unblocked presentation condition for both speech corpuses., Conclusion: Prior listening exposure to a reverberant listening environment improves speech intelligibility in adult CI listeners. Further research is required to understand the underlying mechanism of adaptation to listening environment.

Children with hearing loss are at risk for lower self-esteem due to differences from hearing peers relative to communication skills, physical appearance, and social maturity. This study examines the influence of generic factors unrelated to hearing loss (e.g., age, gender, temperament) and specific factors associated with hearing loss (e.g., age at identification, communication skills) on how children with hearing loss wearing cochlear implants or hearing aids appraise self-esteem. Fifty children with hearing loss wearing cochlear implants or hearing aids participated (Mean age: 12.88 years; mean duration of device use: 3.43 years). Participants independently completed online questionnaires to assess communication skills, social engagement, self-esteem, and temperament. Children with hearing loss rated global self-esteem significantly more positively than hearing peers, t = 2.38, p = .02. Self-esteem ratings attained significant positive correlations with affiliation (r = .42, p = .002) and attention (r = .45, p = .001) temperaments and a significant negative association with depressive mood (r = - .60, p < .0001). No significant correlations emerged between self-esteem and demographic factors, communication skills, or social engagement. Because successful communication abilities do not always co-occur with excellent quality of life, clinicians and professionals working with children with hearing loss need to understand components contributing to self-esteem to improve identification, counseling, and external referrals for children in this population., (© The Author(s) 2015.)

The aim of this study is to investigate the effect of Adaptive Dynamic Range Optimization (ADRO) on speech identification for cochlear implant (CI) users in adverse listening conditions. In this study, anechoic quiet, noisy, reverberant, noisy reverberant, and reverberant noisy conditions are evaluated. Two scenarios are considered when modeling the combined effects of reverberation and noise: (a) noise is added to the reverberant speech, and (b) noisy speech is reverberated. CI users were tested in different listening environments using IEEE sentences presented at 65 dB sound pressure level. No significant effect of ADRO processing on speech intelligibility was observed.

Purpose: To examine the impact of cochlear implant (CI) intervention on health-related quality of life (HRQOL) assessed by both self- and parent-reported measures., Methods: In this national study of children implanted between ages 6 months and 5 years, HRQOL of 129 children 6-year post-CI was compared to 62 internal study (NH1) and 185 external (NH2) samples of hearing children frequency-matched to the CI group on sociodemographic variables. HRQOL ratings of children and their parents in each group, measured using the Child Health and Illness Profile-Child Edition, were compared, and their associations with the Family Stress Scale were investigated., Results: CI children reported overall and domain-specific HRQOL that was comparable to both NH1 and NH2 peers. CI parents reported worse child scores than NH1 parents in Achievement, Resilience, and Global score (p's < 0.01) but similar or better scores than socioeconomically comparable NH2 parents. Higher family stress was negatively associated with all parent-reported HRQOL outcomes (p's < 0.01). Parent-child correlations in HRQOL global scores trended higher in CI recipients (r = 0.50) than NH1 (r = 0.42) and NH2 (r = 0.35) controls., Conclusions: CI recipients report HRQOL comparable to NH peers. These results, from both child and parent perspective, lend support to the effectiveness of CI intervention in mitigating the impact of early childhood deafness. Family stress was associated with worse HRQOL, underscoring a potential therapeutic target. Parent-child agreement in HRQOL scores was higher for CI families than NH families, which may reflect higher caregiver insight and involvement related to the CI intervention.

Objectives: This study assessed self-reported quality of life of children with a cochlear implant (CI), comparing results with two published reports from the past decade., Methods: Participants included 33 pediatric CI recipients with a mean age of 10.12 years (SD = 3.59), mean implantation age of 1.36 years (SD = 0.46), and mean CI experience of 6.23 years (SD = 1.75). Children in all three studies completed a self-report quality-of-life questionnaire that included seven benefit and six problem items, rated on a 5-point Likert scale., Results: Outcomes revealed agreement across studies in overall benefit and problem ratings. Environmental awareness and frustration reduction were the most and least positively rated outcomes, respectively. Items contributing to overall ratings differed across studies. Current CI recipients rated speech production, making new friends, and understanding speech more positively and taking extra care of the device more negatively than previous generations of pediatric CI users., Discussion: Overall, benefits outweigh problems of the device, according to children using CI. Differences in issues motivating self-report ratings reflect changes in CI candidacy, technology, and social participation over the past decade. These findings emphasize the need for clinicians to address not only communication needs, but also quality-of-life issues to optimize outcomes in children using CI.

Objectives: Cochlear implantation (CI) has become the mainstay of treatment for children with severe-to-profound sensorineural hearing loss (SNHL). Yet, despite mounting evidence of the clinical benefits of early implantation, little data are available on the long-term societal benefits and comparative effectiveness of this procedure across various ages of implantation-a choice parameter for parents and clinicians with high prognostic value for clinical outcome. As such, the aim of the present study is to evaluate a model of the consequences of the timing of this intervention from a societal economic perspective. Average cost utility of pediatric CI by age at intervention will be analyzed., Design: Prospective, longitudinal assessment of health utility and educational placement outcomes in 175 children recruited from six U.S. centers between November 2002 and December 2004, who had severe-to-profound SNHL onset within 1 year of age, underwent CI before 5 years of age, and had up to 6 years of postimplant follow-up that ended in November 2008 to December 2011. Costs of care were collected retrospectively and stratified by preoperative, operative, and postoperative expenditures. Incremental costs and benefits of implantation were compared among the three age groups and relative to a nonimplantation baseline., Results: Children implanted at <18 months of age gained an average of 10.7 quality-adjusted life years (QALYs) over their projected lifetime as compared with 9.0 and 8.4 QALYs for those implanted between 18 and 36 months and at >36 months of age, respectively. Medical and surgical complication rates were not significantly different among the three age groups. In addition, mean lifetime costs of implantation were similar among the three groups, at approximately $2000/child/year (77.5-year life expectancy), yielding costs of $14,996, $17,849, and $19,173 per QALY for the youngest, middle, and oldest implant age groups, respectively. Full mainstream classroom integration rate was significantly higher in the youngest group at 81% as compared with 57 and 63% for the middle and oldest groups, respectively (p < 0.05) after 6 years of follow-up. After incorporating lifetime educational cost savings, CI led to net societal savings of $31,252, $10,217, and $6,680 for the youngest, middle, and oldest groups at CI, respectively, over the child's projected lifetime., Conclusions: Even without considering improvements in lifetime earnings, the overall cost-utility results indicate highly favorable ratios. Early (<18 months) intervention with CI was associated with greater and longer quality-of-life improvements, similar direct costs of implantation, and economically valuable improved classroom placement, without a greater incidence of medical and surgical complications when compared to CI at older ages.

Objective: This study examined specific spoken language abilities of 160 children with severe-to-profound sensorineural hearing loss followed prospectively 4, 5, or 6 years after cochlear implantation., Study Sample: Ninety-eight children received implants before 2.5 years, and 62 children received implants between 2.5 and 5 years of age., Design: Language was assessed using four subtests of the Comprehensive Assessment of Spoken Language (CASL). Standard scores were evaluated by contrasting age of implantation and follow-up test time., Results: Children implanted under 2.5 years of age achieved higher standard scores than children with older ages of implantation for expressive vocabulary, expressive syntax, and pragmatic judgments. However, in both groups, some children performed more than two standard deviations below the standardization group mean, while some scored at or well above the mean., Conclusions: Younger ages of implantation are associated with higher levels of performance, while later ages of implantation are associated with higher probabilities of continued language delays, particularly within subdomains of grammar and pragmatics. Longitudinal data from this cohort study demonstrate that after 6 years of implant experience, there is large variability in language outcomes associated with modifiers of rates of language learning that differ as children with implants age.

This report provides an overview of many research projects conducted by the Dallas Cochlear Implant Program, a joint enterprise between the University of Texas at Dallas, the University of Texas Southwestern Medical Center, and Children's Medical Center. The studies extend our knowledge of factors influencing communication outcomes in users of cochlear implants. Multiple designs and statistical techniques are used in the studies described including both cross sectional and longitudinal analyses. Sample sizes vary across the studies, and many of the samples represent large populations of children from North America. Multiple statistical techniques are used by the team to analyze outcomes. The team has provided critical information regarding electrode placement, signal processing, and communication outcomes in users of cochlear implants., (American Academy of Audiology.)

Growing evidence supports the notion that dynamic gene expression, subject to epigenetic control, organizes multiple influences to enable a child to learn to listen and to talk. Here, we review neurobiological and genetic influences on spoken language development in the context of results of a longitudinal trial of cochlear implantation of young children with severe to profound sensorineural hearing loss in the Childhood Development after Cochlear Implantation study. We specifically examine the results of cochlear implantation in participants who were congenitally deaf (N = 116). Prior to intervention, these participants were subject to naturally imposed constraints in sensory (acoustic-phonologic) inputs during critical phases of development when spoken language skills are typically achieved rapidly. Their candidacy for a cochlear implant was prompted by delays (n = 20) or an essential absence of spoken language acquisition (n = 96). Observations thus present an opportunity to evaluate the impact of factors that influence the emergence of spoken language, particularly in the context of hearing restoration in sensitive periods for language acquisition. Outcomes demonstrate considerable variation in spoken language learning, although significant advantages exist for the congenitally deaf children implanted prior to 18 months of age. While age at implantation carries high predictive value in forecasting performance on measures of spoken language, several factors show significant association, particularly those related to parent-child interactions. Importantly, the significance of environmental variables in their predictive value for language development varies with age at implantation. These observations are considered in the context of an epigenetic model in which dynamic genomic expression can modulate aspects of auditory learning, offering insights into factors that can influence a child's acquisition of spoken language after cochlear implantation. Increased understanding of these interactions could lead to targeted interventions that interact with the epigenome to influence language outcomes with intervention, particularly in periods in which development is subject to time-sensitive experience.

Objectives: This article describes participants in a follow-up study of a nationwide sample of children who had used a cochlear implant (CI) since preschool. The children were originally tested when they were in early elementary grades, and results were published in a monograph supplement of Ear and Hearing. Recently, many of these children returned for follow-up testing when they were in high school with >10 yrs experience with a CI. This introductory article will (1) discuss the extent to which the sample tested is representative of typical populations and (2) describe how sample characteristics changed over time for the 112 students tested in both elementary grades and high school., Design: Over a 4-yr period, 112 teenagers from across North America, accompanied by a parent, attended a research camp that was similar to one in which they had participated 8 yrs earlier. A battery of auditory, speech, language, and reading tests was administered, and responses to questionnaires and written language samples were obtained and are described in the following articles in this issue. This article summarizes child, family, and educational characteristics that were quantified so that their role in outcome levels achieved could be examined statistically. For example, metrics were devised to reflect the extent to which a student's language improved when sign language was added to spoken language (i.e., sign enhancement) based on test results obtained in elementary grades and in high school., Results: Comparisons of early characteristics of the 112 students who returned for follow-up testing with the 72 who did not return indicated comparable Performance Intelligence Quotients, communication mode ratings, family education/income, and age at implant. However, follow-up participants had better speech perception, speech intelligibility, and language skills at 8 or 9 yrs of age. Seventy-five percent of returning teenagers were fully mainstreamed in high school (compared with 63% in elementary grades). Only 5% of adolescents were in full-time special education. Grade placement of the teenagers was appropriate to their chronologic age. Estimates of sign enhancement, family characteristics, and Performance Intelligence Quotient were consistent over the two test sessions., Conclusions: A large proportion of the original sample returned for follow-up testing in adolescence, but they were a more selective group than nonreturning subjects, and their scores may overestimate long-term CI outcomes for the general population. On the other hand, CI-HS students who participated in this study received their device >10 yrs ago and did not have some of the advantages available to more recently implanted children, so their results may underestimate those outcomes.

Objectives: The objectives of this report are to (1) describe the speech perception abilities of long-term pediatric cochlear implant (CI) recipients by comparing scores obtained at elementary school (CI-E, 8 to 9 yrs) with scores obtained at high school (CI-HS, 15 to 18 yrs); (2) evaluate speech perception abilities in demanding listening conditions (i.e., noise and lower intensity levels) at adolescence; and (3) examine the relation of speech perception scores to speech and language development over this longitudinal timeframe., Design: All 112 teenagers were part of a previous nationwide study of 8- and 9-yr-olds (N = 181) who received a CI between 2 and 5 yrs of age. The test battery included (1) the Lexical Neighborhood Test (LNT; hard and easy word lists); (2) the Bamford Kowal Bench sentence test; (3) the Children's Auditory-Visual Enhancement Test; (4) the Test of Auditory Comprehension of Language at CI-E; (5) the Peabody Picture Vocabulary Test at CI-HS; and (6) the McGarr sentences (consonants correct) at CI-E and CI-HS. CI-HS speech perception was measured in both optimal and demanding listening conditions (i.e., background noise and low-intensity level). Speech perception scores were compared based on age at test, lexical difficulty of stimuli, listening environment (optimal and demanding), input mode (visual and auditory-visual), and language age., Results: All group mean scores significantly increased with age across the two test sessions. Scores of adolescents significantly decreased in demanding listening conditions. The effect of lexical difficulty on the LNT scores, as evidenced by the difference in performance between easy versus hard lists, increased with age and decreased for adolescents in challenging listening conditions. Calculated curves for percent correct speech perception scores (LNT and Bamford Kowal Bench) and consonants correct on the McGarr sentences plotted against age-equivalent language scores on the Test of Auditory Comprehension of Language and Peabody Picture Vocabulary Test achieved asymptote at similar ages, around 10 to 11 yrs., Conclusions: On average, children receiving CIs between 2 and 5 yrs of age exhibited significant improvement on tests of speech perception, lipreading, speech production, and language skills measured between primary grades and adolescence. Evidence suggests that improvement in speech perception scores with age reflects increased spoken language level up to a language age of about 10 yrs. Speech perception performance significantly decreased with softer stimulus intensity level and with introduction of background noise. Upgrades to newer speech processing strategies and greater use of frequency-modulated systems may be beneficial for ameliorating performance under these demanding listening conditions.

Objective: A great amount of variability is observed in speech perception outcomes with cochlear implants. The mechanisms behind the observed variability need to be elucidated. One possible mechanism contributing to the observed variability is the development of cross-modal plasticity. This study examines the association between visual/auditory cross-modal plasticity and speech perception with a cochlear implant in individuals with pre- and postlingual onset of severe to profound hearing loss., Design: The N1 visual evoked potential (VEP) in response to peripheral visual motion stimuli was recorded in individuals with pre- (N = 10) and postlingual (N = 12) onset of severe to profound hearing loss who use a cochlear implant. The association between the amplitude of the N1 VEP response over the right temporal lobe and sentence and word perception scores obtained with the cochlear implant was examined through linear regression analyses. In addition, the association between the duration of auditory deprivation and the amplitude of the N1 VEP response was examined., Results: As the amplitude of the N1 VEP recorded over the right temporal lobe increased, speech perception scores in individuals with prelingual onset of severe to profound hearing loss decreased. However, a clear association between the amplitude of the N1 VEP over the right temporal lobe and speech perception scores was not observed for individuals with postlingual onset of severe to profound hearing loss. Neither group demonstrated an association between the amplitude of the VEP over the right temporal lobe and the duration of auditory deprivation before cochlear implantation., Conclusion: The results suggest that cross-modal plasticity accounts for a significant amount of the variability observed in speech perception performance with a cochlear implant in individuals with prelingual onset of severe to profound hearing loss but not in individuals who acquire severe to profound hearing loss later in life. Furthermore, the results suggest that the influence of cross-modal plasticity on speech perception ability is more greatly influenced by when (pre- or postlingually) a person acquires a severe to profound hearing impairment rather than the duration of auditory deprivation before receipt of a cochlear implant.

Objectives: This report focuses on how speech perception, speech production, language, and literacy performance in adolescence are influenced by a common set of predictor variables obtained during elementary school in a large group of teenagers using cochlear implants (CIs)., Design: Time-lag analyses incorporating seven common predictor variables associated with the elementary school test period were evaluated. The elementary school-age variables included five contributors across the performance domains: gender, performance intelligence quotient, family size, socioeconomic status, and duration of deafness (operationally defined as the time period between the age of implantation and the onset of deafness). Regression analyses then examined how communication mode in early elementary grades influenced skills exhibited in high school and how this influence was mediated by information capacity of immediate memory., Results: High correlations occurred between outcome measures collected at CI-E session and similar measures collected at CI-HS (values ranging from 0.75 to 0.83), indicating that the relative standing of individuals on these outcomes is highly stable over time. The best performers in elementary grades exhibit the best outcomes in high school, and early difficulties tend to persist throughout the elementary and high school years. The most highly related outcome areas were language and reading/literacy (values ranging from 0.74 to 0.88). These skills seem closely linked, and CI children who demonstrate the best vocabulary and syntax skills in elementary grades achieved the highest literacy performance in high school. Speech perception and speech production skills are also highly correlated with one another (r = 0.69 to 0.87), suggesting that the most direct result of improved auditory input from a CI is the child's ability to produce intelligible speech. The lowest correlations are observed between reading/literacy and speech perception (r = 0.30 to 0.54) or speech production (values ranging from 0.31 to 0.58). CI-E verbal rehearsal speed is an independent and powerful predictor of each early performance outcome, accounting for between 13% and 30% of the variance in early outcomes above and beyond that accounted for by gender, family size, socioeconomic status, performance intelligence quotient, duration of deafness, and the CI-E sign enhancement ratio. Group mean scores for language, reading, and social adjustment were generally within an SD of normative samples of typically developing age-mates with normal hearing., Conclusions: Use of sign to enhance spoken communication negatively influenced verbal rehearsal speed, which was a strong predictor of all early outcomes, which in turn strongly influenced later outcomes. These analyses suggest that early communication mode exerts a powerful influence on early outcomes that persist into later years. Speech perception, speech intelligibility, language, literacy, and psychosocial adjustment far exceeded that reported for similar groups before the advent of CI technology.

Objective: Examination of health-related quality of life (HRQoL) in children and adolescents who wear a cochlear implant (CI) primarily has depended on parent proxy report of the child's HRQoL rather than child self-report and generic domains rather than CI-specific issues. This study simultaneously assessed self-report ratings on a generic HRQoL instrument and a preliminary CI module in pediatric CI users. The impact of demographic factors (chronologic age, age at CI, and CI experience) on HRQoL also was explored., Methods: This cross-sectional study included 138 children grouped by chronologic age: 4-7, 8-11 and 12-16 years. The KINDL(R) questionnaire for measuring HRQoL in children and adolescents (generic) and a preliminary CI module (specific) were completed as a researcher-administered interview (4-7 years) or self-administered questionnaire (8-16 years) at CI summer camp or home. Scores were transformed to a 100-point scale with 100 representing the most positive response. The impact of chronologic age group on HRQoL ratings was evaluated using Analysis of Variance. Spearman rank-order correlations and point-biserial correlations tested associations between demographic factors and HRQoL scores. Principal factor analysis was used to discover the factor structure and internal consistency of the preliminary CI module., Results: The youngest group (M=82.8) rated generic HRQoL significantly more positively than older children (8-11 years: M=75.3; 12-16 years: M=70.4). Similar significant results emerged on the overall CI module (4-7 years: M=79.8; 8-11 years: M=77.8; 12-16 years: M=71.3). The youngest group rated CI-specific items on friends and self-image more positively than older groups, but reported greater difficulties hearing teachers at school. The oldest group provided more consistent responses than younger groups on the CI module (Cronbach α=0.72). Generic and CI module scores correlated positively (r=0.19, p=.03) but this association reflects the strong correlation in the oldest group (r=0.49, p=0.0033) and camouflages non-significant results in younger groups., Conclusion: Chronologic age impacts self-report of HRQoL for pediatric CI users such that younger children rate HRQoL more positively than older children and adolescents on a generic instrument and preliminary CI module. Older children provide more consistent responses on the CI module. Results support the need for further development of a CI-specific self-report HRQoL instrument., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Context: Cochlear implantation is a surgical alternative to traditional amplification (hearing aids) that can facilitate spoken language development in young children with severe to profound sensorineural hearing loss (SNHL)., Objective: To prospectively assess spoken language acquisition following cochlear implantation in young children., Design, Setting, and Participants: Prospective, longitudinal, and multidimensional assessment of spoken language development over a 3-year period in children who underwent cochlear implantation before 5 years of age (n = 188) from 6 US centers and hearing children of similar ages (n = 97) from 2 preschools recruited between November 2002 and December 2004. Follow-up completed between November 2005 and May 2008., Main Outcome Measures: Performance on measures of spoken language comprehension and expression (Reynell Developmental Language Scales)., Results: Children undergoing cochlear implantation showed greater improvement in spoken language performance (10.4; 95% confidence interval [CI], 9.6-11.2 points per year in comprehension; 8.4; 95% CI, 7.8-9.0 in expression) than would be predicted by their preimplantation baseline scores (5.4; 95% CI, 4.1-6.7, comprehension; 5.8; 95% CI, 4.6-7.0, expression), although mean scores were not restored to age-appropriate levels after 3 years. Younger age at cochlear implantation was associated with significantly steeper rate increases in comprehension (1.1; 95% CI, 0.5-1.7 points per year younger) and expression (1.0; 95% CI, 0.6-1.5 points per year younger). Similarly, each 1-year shorter history of hearing deficit was associated with steeper rate increases in comprehension (0.8; 95% CI, 0.2-1.2 points per year shorter) and expression (0.6; 95% CI, 0.2-1.0 points per year shorter). In multivariable analyses, greater residual hearing prior to cochlear implantation, higher ratings of parent-child interactions, and higher socioeconomic status were associated with greater rates of improvement in comprehension and expression., Conclusion: The use of cochlear implants in young children was associated with better spoken language learning than would be predicted from their preimplantation scores.

Objective: To examine the results of health-related quality-of-life questionnaire scores from profoundly deaf children fitted with at least one cochlear implant (CI) and to compare their responses with those of normal-hearing mates of similar age and their parents., Study Design: Cross-sectional study utilizing a generic quality-of-life questionnaire designed to be completed by both parents and children independently of each other., Setting: Questionnaires completed at various summer camps designed for children with CIs in Texas and Colorado., Subjects and Methods: Eighty-eight families from 16 states were divided into two subgroups by age of cochlear implantation: an eight- to 11-year-old group and a 12- to 16-year-old group. The KINDL-R Questionnaire for Measuring Health-Related Quality of Life in Children and Adolescents was distributed, and CI participants completed the questionnaire independently of their participating family member., Results: CI users in both age groups scored similarly to their normal-hearing peers and their parents. Younger CI users scored their family domain lower compared with their normal-hearing peers. Teen CI users scored the school domain lower compared with their parents. Among CI participants, earlier implantation and longer CI use resulted in higher quality-of-life scores., Conclusion: Children with CIs experience quality of life similar to that of normal-hearing peers. Parents are reliable reporters on the status of their child's overall quality of life., (Copyright 2010 American Academy of Otolaryngology-Head and Neck Surgery Foundation. Published by Mosby, Inc. All rights reserved.)

Objective: Children with hearing loss who use cochlear implants have lower quality of life (QoL) in social situations and lower self-esteem than hearing peers. The child's QoL has been assessed primarily by asking the parent rather than asking the child. This poses a problem because parents have difficulty judging less observable aspects like self-esteem and socio-emotional functioning, the domains most affected by hearing loss., Methods: This case-control study evaluated QoL in 50 preschoolers using a cochlear implant and their parents with the Kiddy KINDL(®), an established QoL measure. Children's responses were compared to a hearing control group and correlated with demographic variables. We used a questionnaire for parents and a face-to-face interview with children. T-tests were used to compare (a) paired parent-child ratings and (b) children with cochlear implants versus normal hearing. Pearson rank correlations were used to compare QoL with demographic variables., Results: Children using cochlear implants rated overall QoL significantly more positively than their parents (M(Difference)=4.22, p=.03). Child rating of QoL did not differ significantly by auditory status (cochlear implant (82.8) vs. hearing (80.8), p=.42). Overall QoL correlated inversely with cochlear implant experience and chronologic age, but did not correlate with implantation age., Conclusions: Preschool children using cochlear implants can assess adequately their own QoL, but parents afford valuable complementary perspective on the child's socio-emotional and physical well-being. Preschool children using cochlear implants rate overall QoL measures similar to hearing peers. A constellation of QoL measures should be collected to yield a better understanding of general QoL as well as specific domains centered on hearing loss.

The development of language and communication may play an important role in the emergence of behavioral problems in young children, but they are rarely included in predictive models of behavioral development. In this study, cross-sectional relationships between language, attention, and behavior problems were examined using parent report, videotaped observations, and performance measures in a sample of 116 severely and profoundly deaf and 69 normally hearing children ages 1.5 to 5 years. Secondary analyses were performed on data collected as part of the Childhood Development After Cochlear Implantation Study, funded by the National Institutes of Health. Hearing-impaired children showed more language, attention, and behavioral difficulties, and spent less time communicating with their parents than normally hearing children. Structural equation modeling indicated there were significant relationships between language, attention, and child behavior problems. Language was associated with behavior problems both directly and indirectly through effects on attention. Amount of parent-child communication was not related to behavior problems.

Children with severe to profound sensorineural hearing loss face communication challenges that influence language, psychosocial and scholastic performance. Clinical studies over the past 20 years have supported wider application of cochlear implants in children. The Childhood Development after Cochlear Implantation (CDaCI) study is the first longitudinal multicentre, national cohort study to evaluate systematically early cochlear implant (CI) outcomes in children. The objective of the study was to compare children who have undergone cochlear implantation, with similarly aged hearing peers across multiple domains, including oral language development, auditory performance, psychosocial and behavioural functioning, and quality of life. The CDaCI study is a multicentre national cohort study of CI children and normal hearing (NH) peers. Eligibility criteria include informed consent, age less than 5 years, pre- or post-lingually deaf, developmental criteria met, commitment to educate the child in English and bilateral cochlear implants. All children had a standardised baseline assessment that included demographics, hearing and medical history, communication history, language measures, cognitive tests, speech recognition, an audiological exam, psychosocial assessment including parent-child videotapes and parent reported quality of life. Follow-up visits are scheduled at six-month intervals and include a standardised assessment of the full battery of measures. Quality assurance activities were incorporated into the design of the study. A total of 188 CI children and 97 NH peers were enrolled between November 2002 and December 2004. The mean age, gender and race of the CI and NH children are comparable. With regard to parental demographics, the CI and NH children's families are statistically different. The parents of CI children are younger, and not as well educated, with 49% of CI parents reporting college graduation vs. 84% of the NH parents. The income of the CI parents is also lower than the NH parents. Assessments of cognition suggest that there may be baseline differences between the CI and NH children; however the scores were high enough to suggest language learning potential. The observed group differences identified these baseline characteristics as potential confounders which may require adjustment in analyses of outcomes. This longitudinal cohort study addresses questions related to high variability in language outcomes. Identifying sources of that variance requires research designs that: characterise potential predictors with accuracy, use samples that adequately power a study, and employ controls and approaches to analysis that limit bias and error. The CDaCI study was designed to generate a more complete picture of the interactive processes of language learning after implantation., (2007 John Wiley & Sons, Ltd.)

Purpose: This study explores the relationship between age and resting-state regional cerebral blood flow (rCBF) in regions associated with higher order language skills using a population of normal children, adolescents, and young adults., Method: rCBF was measured in 33 normal participants between the ages of 7 and 19 years using single photon emission computed tomography. Participants' ages were regressed on rCBF values (normalized to whole-brain CBF) in 2 ways: (a) within anatomically defined, language-related regions of interest (ROIs) including Wernicke's area, Broca's area, angular gyrus, planum temporale, and Heschl's gyrus and (b) within clusters of voxels found to be significantly related to age in voxel-wise analyses., Results: rCBF in all anatomically defined ROIs except Heschl's gyrus declined as a function of age. Additionally, voxel-wise analyses revealed clusters where rCBF declined with age in left inferior parietal, left superior temporal, and right middle temporal regions-areas often implicated in higher order language functions., Conclusions: These data suggest that ongoing maturation (e.g., dendritic pruning) in higher order cognitive areas (e.g., angular gyrus) continues into adolescence, as reflected by declining rCBF, while the primary auditory area (Heschl's gyrus) has become a stable neuronal population by age 7 years.

Acoustic analyses and perception experiments were conducted to determine the effects of brief deprivation of auditory feedback on fricatives produced by cochlear implant users. The words /si/ and /Si/ were recorded by four children and four adults with their cochlear implant speech processor turned on or off. In the processor-off condition, word durations increased significantly for a majority of talkers. These increases were greater for children compared to adults, suggesting that children may rely on auditory feedback to a greater extent than adults. Significant differences in spectral measures of /S/ were found between processor-on and processor-off conditions for two of the four children and for one of the four adults. These talkers also demonstrated a larger /s/-/S/ contrast in centroid values compared to the other talkers within their respective groups. This finding may indicate that talkers who produce fine spectral distinctions are able to perceive these distinctions through their implants and to use this feedback to fine tune their speech. Two listening experiments provided evidence that some of the acoustic changes were perceptible to normal-hearing listeners. Taken together, these experiments indicate that for certain cochlear-implant users the brief absence of auditory feedback may lead to perceptible modifications in fricative consonants.

The Childhood Development after Cochlear Implantation (CDaCI) study is a longitudinal multicenter investigation designed to identify factors influencing spoken language in young deaf children with cochlear implants. Normal-hearing peers serve as controls. As part of a comprehensive evaluation battery, a speech recognition hierarchy was designed to assess how well these children recognize speech stimuli across developmental stages. Data were analyzed for the earliest measures in 42 pairs of children reaching 1 year of follow-up. A number of children in the cochlear implant group who met criteria for testing approached levels of performance similar to the normal-hearing controls, and some could identify sentences in competition. These results demonstrate the responsiveness of the speech recognition hierarchy in tracking emergent skills from a sample of the CDaCI cohort., (Copyright 2006 S. Karger AG, Basel.)

Objective: To examine the impact of classroom placement and mode of communication on speech intelligibility scores in children aged 8 to 9 years using multichannel cochlear implants., Design: Classroom placement (special education, partial mainstream, and full mainstream) and mode of communication (total communication and auditory-oral) reported via parental rating scales before and 4 times after implantation were the independent variables. Speech intelligibility scores obtained at 8 to 9 years of age were the dependent variables., Participants: The study included 131 congenitally deafened children between the ages of 8 and 9 years who received a multichannel cochlear implant before the age of 5 years., Results: Higher speech intelligibility scores at 8 to 9 years of age were significantly associated with enrollment in auditory-oral programs rather than enrollment in total communication programs, regardless of when the mode of communication was used (before or after implantation). Speech intelligibility at 8 to 9 years of age was not significantly influenced by classroom placement before implantation, regardless of mode of communication. After implantation, however, there were significant associations between classroom placement and speech intelligibility scores at 8 to 9 years of age. Higher speech intelligibility scores at 8 to 9 years of age were associated with classroom exposure to normal-hearing peers in full or partial mainstream placements than in self-contained, special education placements., Conclusions: Higher speech intelligibility scores in 8- to 9-year-old congenitally deafened cochlear implant recipients were associated with educational settings that emphasize oral communication development. Educational environments that incorporate exposure to normal-hearing peers were also associated with higher speech intelligibility scores at 8 to 9 years of age.

Purpose: The speech-perception abilities of 36 French children, whose onset of deafness occurred before 2 years of age, was longitudinally examined after they received a Nucleus multichannel cochlear implant., Methods: Speech perception was assessed in four areas: phoneme detection, closed-set word and sentence recognition; and modified open-set recognition., Results: All children achieved phoneme detection by 3 months after implantation. Closed-set word and sentence recognition appeared initially less accurate in children with congenital deafness than in the prelingually deafened children; however, these differences disappeared by 18 months after implantation. Some modified open-set recognition was evident by 12 months after implantation and continued to improve 3 years after implantation., Conclusions: Data demonstrate significant improvements in speech perception with implant experience: 15 (60%) of 25 of the children identified sentences in closed sets, 22 (88%) of 25 identified words in closed sets after 12 months' experience, and 13 (81%) of 16 demonstrated open-set recognition after 24 months of implant use.