Intravenous N-Acetylcysteine to Prevent Cisplatin-Induced Hearing Loss in Children: A Nonrandomized Controlled Phase I Trial.

PURPOSE: Cisplatin-induced hearing loss (CIHL) is common and permanent. As compared with earlier otoprotectants, we hypothesized N-acetylcysteine (NAC) offers potential for stronger otoprotection through stimulation of glutathione (GSH) production. This study tested the optimal dose, safety, and efficacy of NAC to prevent CIHL. PATIENTS AND METHODS: In this nonrandomized, controlled phase Ia/Ib trial, children and adolescents newly diagnosed with nonmetastatic, cisplatin-treated tumors received NAC intravenously 4 hours post-cisplatin. The trial performed dose-escalation across three dose levels to establish a safe dose that exceeded the targeted peak serum NAC concentration of 1.5 mmol/L (as identified from preclinical models). Patients with metastatic disease or who were otherwise ineligible were enrolled in an observation-only/control arm. To evaluate efficacy, serial age-appropriate audiology assessments were performed. Integrated biology examined genes involved in GSH metabolism and post-NAC GSH concentrations. RESULTS: Of 52 patients enrolled, 24 received NAC and 28 were in the control arm. The maximum tolerated dose was not reached; analysis of peak NAC concentration identified 450 mg/kg as the recommended phase II dose (RP2D). Infusion-related reactions were common. No severe adverse events occurred. Compared with the control arm, NAC decreased likelihood of CIHL at the end of cisplatin therapy [OR, 0.13; 95% confidence interval (CI), 0.021-0.847; P = 0.033] and recommendations for hearing intervention at end of study (OR, 0.082; 95% CI, 0.011-0.60; P = 0.014). NAC increased GSH; GSTP1 influenced risk for CIHL and NAC otoprotection. CONCLUSIONS: NAC was safe at the RP2D, with strong evidence for efficacy to prevent CIHL, warranting further development as a next-generation otoprotectant.

Home Literacy Experiences and Shared Reading Practices: Preschoolers With Hearing Loss.

Home literacy experiences and observed parent and child behaviors during shared book reading were investigated in preschool-age children with hearing loss and with typical hearing to examine the relationships between those factors and children's language skills. The methods involved parent-reported home literacy experiences and videotaped parent-child dyads during shared book reading. Children's language skills were tested using the Preschool Language Scale-4. The results indicated significant differences between groups for home literacy experiences and observed parent and child behaviors. Parents of children with hearing loss were found to read more frequently to their children than parents of children with typical hearing, yet scored lower for literacy strategies and teaching techniques compared to parents of children with typical hearing. Children with hearing loss scored lower in interactive reading behaviors compared to children with typical hearing. For children with hearing loss, frequency of book reading and child interactive reading behaviors were strong predictive factors for children's language skills. These results suggest that families of children with hearing loss would benefit from professional support as they read storybooks to their children. Similarly, children with hearing loss should be encouraged to be more interactive during shared book reading.

Parenting Stress, Self-Efficacy, and Involvement: Effects on Spoken Language Ability Three Years After Cochlear Implantation.

OBJECTIVE: This study evaluated associations among parenting stress, self-efficacy, and involvement in relation to spoken language outcomes in young children 3 years following cochlear implantation. STUDY DESIGN: Cross-sectional. SETTING: Six university tertiary medical centers. PATIENTS: One hundred sixty-four young children with bilateral, severe-to-profound sensorineural hearing loss who had 3 years of experience with a CI; children with substantial cognitive impairments were excluded from the study. MAIN OUTCOME MEASURESS: Family Stress Scale (FSS), Scale of Parental Involvement and Self-Efficacy (SPISE), Oral and Written Language Scales (OWLS). RESULTS: Correlations were of moderate strength between FSS scores and SPISE scores (Parental Self-Efficacy, r = -0.45, p < 0.01, Parental Involvement r = -0.32, p < 0.01). As hypothesized, parents reporting higher levels of stress reported lower perceptions of self-efficacy and involvement. In addition, results showed that family stress had a direct, negative effect on spoken language (-4.43 [95% confidence interval: -6.97; -1.89]). After controlling for maternal education and activation age, parental self-efficacy mediated the negative effect between family stress and spoken language (indirect effect = -1.91 [3.45; -0.69]; proportion mediated = 0.43). No mediating effects were found for parental involvement. CONCLUSIONS: These findings highlight the need for parenting interventions that focus on reducing stressors and increasing parents' perceptions of self-efficacy in families of children using cochlear implants. Integration of mental health screening and tailored parenting interventions in CI clinics may increase parental self-efficacy and involvement, with measurable benefits in the child's use of spoken language.

Developmental Outcomes in Early-Identified Children Who Are Hard of Hearing at 2 to 3 Years of Age.

OBJECTIVES: This study examined the psychosocial, adaptive behavior, and language outcomes of young children who are hard of hearing (HH) without additional disabilities or neurocognitive impairments. Relations between early developmental outcomes and child and parent demographic variables, and parenting stress and self-efficacy were also explored. DESIGN: Participants were 39 children with early identified, permanent mild to severe hearing loss, between the ages of 2 and 3 years, and a comparison group of 47 children with normal hearing (NH). Developmental outcomes were measured using clinician-administered standardized tests and parent-completed behavior rating instruments specific to language, psychosocial functioning, and adaptive behavior. Mothers completed self-report measures that assess parenting stress and maternal self-efficacy. RESULTS: The children who are HH were similar to the children with NH in terms of their psychosocial functioning and adaptive behavior, with the exception of their socialization skills. As a group, the children who are HH performed significantly worse than their peers with NH on all measures of language ability. Among the children who are HH, maternal self-efficacy showed a strong positive correlation with adaptive behavior outcomes; however, it failed to contribute unique variance above that explained by language ability and gender. Maternal self-efficacy was also significantly correlated with better psychosocial outcomes, but only parenting stress proved to be a significant predictor of child behavioral problems once other variables considered were in the model. CONCLUSIONS: Early-identified young children who are HH can demonstrate age-appropriate development in multiple domains, including language, psychosocial, and adaptive behavior. However, mild to severe hearing loss places young children with no additional disabilities or neurocognitive impairments at risk for language delays. Although the children who are HH demonstrated no more emotional or behavioral problems than their same-age peers with NH, results suggest that language delays increase their vulnerability for delays in various aspects of social competence.

Children With Normal Hearing Are Efficient Users of Fundamental Frequency and Vocal Tract Length Cues for Voice Discrimination.

BACKGROUND: The ability to discriminate between talkers assists listeners in understanding speech in a multitalker environment. This ability has been shown to be influenced by sensory processing of vocal acoustic cues, such as fundamental frequency (F0) and formant frequencies that reflect the listener's vocal tract length (VTL), and by cognitive processes, such as attention and memory. It is, therefore, suggested that children who exhibit immature sensory and/or cognitive processing will demonstrate poor voice discrimination (VD) compared with young adults. Moreover, greater difficulties in VD may be associated with spectral degradation as in children with cochlear implants. OBJECTIVES: The aim of this study was as follows: (1) to assess the use of F0 cues, VTL cues, and the combination of both cues for VD in normal-hearing (NH) school-age children and to compare their performance with that of NH adults; (2) to assess the influence of spectral degradation by means of vocoded speech on the use of F0 and VTL cues for VD in NH children; and (3) to assess the contribution of attention, working memory, and nonverbal reasoning to performance. DESIGN: Forty-one children, 8 to 11 years of age, were tested with nonvocoded stimuli. Twenty-one of them were also tested with eight-channel, noise-vocoded stimuli. Twenty-one young adults (18 to 35 years) were tested for comparison. A three-interval, three-alternative forced-choice paradigm with an adaptive tracking procedure was used to estimate the difference limens (DLs) for VD when F0, VTL, and F0 + VTL were manipulated separately. Auditory memory, visual attention, and nonverbal reasoning were assessed for all participants. RESULTS: (a) Children' F0 and VTL discrimination abilities were comparable to those of adults, suggesting that most school-age children utilize both cues effectively for VD. (b) Children's VD was associated with trail making test scores that assessed visual attention abilities and speed of processing, possibly reflecting their need to recruit cognitive resources for the task. (c) Best DLs were achieved for the combined (F0 + VTL) manipulation for both children and adults, suggesting that children at this age are already capable of integrating spectral and temporal cues. (d) Both children and adults found the VTL manipulations more beneficial for VD compared with the F0 manipulations, suggesting that formant frequencies are more reliable for identifying a specific speaker than F0. (e) Poorer DLs were achieved with the vocoded stimuli, though the children maintained similar thresholds and pattern of performance among manipulations as the adults. CONCLUSIONS: The present study is the first to assess the contribution of F0, VTL, and the combined F0 + VTL to the discrimination of speakers in school-age children. The findings support the notion that many NH school-age children have effective spectral and temporal coding mechanisms that allow sufficient VD, even in the presence of spectrally degraded information. These results may challenge the notion that immature sensory processing underlies poor listening abilities in children, further implying that other processing mechanisms contribute to their difficulties to understand speech in a multitalker environment. These outcomes may also provide insight into VD processes of children under listening conditions that are similar to cochlear implant users.

Temporal Modulation Detection in Children and Adults With Cochlear Implants: Initial Results.

OBJECTIVES: The auditory experience of early deafened pediatric cochlear implant (CI) users is different from that of postlingually deafened adult CI users due to disparities in the developing auditory system. It is therefore expected that the auditory psychophysical capabilities between these two groups would differ. In this study, temporal resolving ability was investigated using a temporal modulation detection task to compare the performance outcomes between these two groups. DESIGN: The minimum detectable modulation depth of amplitude modulated broadband noise at 100 Hz was measured for 11 early deafened children with a CI and 16 postlingually deafened adult CI users. RESULTS: Amplitude modulation detection thresholds were significantly lower (i.e., better) for the pediatric CI users than for the adult CI users. Within each group, modulation detection thresholds were not significantly associated with chronologic age, age at implantation, or years of CI experience. CONCLUSIONS: Early implanted children whose auditory systems develop in response to electric stimulation demonstrate better temporal resolving abilities than postlingually deafened adult CI users. This finding provides evidence to suggest that early implanted children might benefit from sound coding strategies emphasizing temporal information.

Assessing the Benefit-Risk Profile for Pediatric Implantable Auditory Prostheses.

BACKGROUND/AIMS: Children with congenital cochleovestibular abnormalities associated with profound hearing loss have few treatment options if cochlear implantation does not yield benefit. An alternative is the auditory brainstem implant (ABI). Regulatory authority device approvals currently include a structured benefit-risk assessment. Such an assessment, for regulatory purposes or to guide clinical decision making, has not been published, to our knowledge, for the ABI and may lead to the design of a research program that incorporates regulatory authority, family, and professional input. METHODS: Much structured benefit-risk research has been conducted in the context of drug trials; here we apply this approach to device studies. A qualitative framework organized benefit (speech recognition, parent self-report measures) and risk (surgery- and device-related) information to guide the selection of candidates thought to have potential benefit from ABI. RESULTS: Children with cochleovestibular anatomical abnormalities are challenging for appropriate assessment of candidacy for a cochlear implant or an ABI. While the research is still preliminary, children with an ABI appear to slowly obtain benefit over time. A team of professionals, including audiological, occupational, and educational therapy, affords maximum opportunity for benefit. CONCLUSIONS: Pediatric patients who have abnormal anatomy and are candidates for an implantable auditory prosthetic require an individualized, multisystems review. The qualitative benefit-risk assessment used here to characterize the condition, the medical need, potential benefits, risks, and risk management strategies has revealed the complex factors involved. After implantation, continued team support for the family during extensive postimplant therapy is needed to develop maximum auditory skill benefit.

Early Communication Development of Children with Auditory Brainstem Implants.

The auditory brainstem implant (ABI) is an auditory sensory device that is surgically placed on the cochlear nucleus of the brainstem for individuals who are deaf but unable to benefit from a cochlear implant (CI) due to anatomical abnormalities of the cochlea and/or eighth nerve, specific disease processes, or temporal bone fractures. In the United States, the Food and Drug Administration has authorized a Phase I clinical trial to determine safety and feasibility of the ABI in up to 10 eligible young children who are deaf and either derived no benefit from the CI or were anatomically unable to receive a CI. In this paper, we describe the study protocol and the children who have enrolled in the study thus far. In addition, we report the scores on speech perception, speech production, and language (spoken and signed) for five children with 1-3 years of assessment post-ABI activation. To date, the results indicate that spoken communication skills are slow to develop and that visual communication remains essential for post-ABI intervention.

Voice Discrimination by Adults with Cochlear Implants: the Benefits of Early Implantation for Vocal-Tract Length Perception.

Cochlear implant (CI) users find it extremely difficult to discriminate between talkers, which may partially explain why they struggle to understand speech in a multi-talker environment. Recent studies, based on findings with postlingually deafened CI users, suggest that these difficulties may stem from their limited use of vocal-tract length (VTL) cues due to the degraded spectral resolution transmitted by the CI device. The aim of the present study was to assess the ability of adult CI users who had no prior acoustic experience, i.e., prelingually deafened adults, to discriminate between resynthesized "talkers" based on either fundamental frequency (F0) cues, VTL cues, or both. Performance was compared to individuals with normal hearing (NH), listening either to degraded stimuli, using a noise-excited channel vocoder, or non-degraded stimuli. Results show that (a) age of implantation was associated with VTL but not F0 cues in discriminating between talkers, with improved discrimination for those subjects who were implanted at earlier age; (b) there was a positive relationship for the CI users between VTL discrimination and speech recognition score in quiet and in noise, but not with frequency discrimination or cognitive abilities; (c) early-implanted CI users showed similar voice discrimination ability as the NH adults who listened to vocoded stimuli. These data support the notion that voice discrimination is limited by the speech processing of the CI device. However, they also suggest that early implantation may facilitate sensory-driven tonotopicity and/or improve higher-order auditory functions, enabling better perception of VTL spectral cues for voice discrimination.

Spectral-Temporal Modulated Ripple Discrimination by Children With Cochlear Implants.

OBJECTIVES: A postlingually implanted adult typically develops hearing with an intact auditory system, followed by periods of deafness (or near deafness) and adaptation to the implant. For an early implanted child whose brain is highly plastic, the auditory system matures with consistent input from a cochlear implant. It is likely that the auditory system of early implanted cochlear implant users is fundamentally different than postlingually implanted adults. The purpose of this study is to compare the basic psychophysical capabilities and limitations of these two populations on a spectral resolution task to determine potential effects of early deprivation and plasticity. DESIGN: Performance on a spectral resolution task (Spectral-temporally Modulated Ripple Test [SMRT]) was measured for 20 bilaterally implanted, prelingually deafened children (between 5 and 13 years of age) and 20 hearing children within the same age range. Additionally, 15 bilaterally implanted, postlingually deafened adults, and 10 hearing adults were tested on the same task. Cochlear implant users (adults and children) were tested bilaterally, and with each ear alone. Hearing listeners (adults and children) were tested with the unprocessed SMRT and with a vocoded version that simulates an 8-channel cochlear implant. RESULTS: For children with normal hearing, a positive correlation was found between age and SMRT score for both the unprocessed and vocoded versions. Older hearing children performed similarly to hearing adults in both the unprocessed and vocoded test conditions. However, for children with cochlear implants, no significant relationship was found between SMRT score and chronological age, age at implantation, or years of implant experience. Performance by children with cochlear implants was poorer than performance by cochlear implanted adults. It was also found that children implanted sequentially tended to have better scores with the first implant compared with the second implant. This difference was not observed for adults. An additional finding was that SMRT score was negatively correlated with age for adults with implants. CONCLUSIONS: Results from this study suggest that basic psychophysical capabilities of early implanted children and postlingually implanted adults differ when assessed in the sound field using their personal implant processors. Because spectral resolution does not improve with age for early implanted children, it seems likely that the sparse representation of the signal provided by a cochlear implant limits spectral resolution development. These results are supported by the finding that postlingually implanted adults, whose auditory systems matured before the onset of hearing loss, perform significantly better than early implanted children on the spectral resolution test.

Early Sign Language Exposure and Cochlear Implantation Benefits.

BACKGROUND: Most children with hearing loss who receive cochlear implants (CI) learn spoken language, and parents must choose early on whether to use sign language to accompany speech at home. We address whether parents' use of sign language before and after CI positively influences auditory-only speech recognition, speech intelligibility, spoken language, and reading outcomes. METHODS: Three groups of children with CIs from a nationwide database who differed in the duration of early sign language exposure provided in their homes were compared in their progress through elementary grades. The groups did not differ in demographic, auditory, or linguistic characteristics before implantation. RESULTS: Children without early sign language exposure achieved better speech recognition skills over the first 3 years postimplant and exhibited a statistically significant advantage in spoken language and reading near the end of elementary grades over children exposed to sign language. Over 70% of children without sign language exposure achieved age-appropriate spoken language compared with only 39% of those exposed for 3 or more years. Early speech perception predicted speech intelligibility in middle elementary grades. Children without sign language exposure produced speech that was more intelligible (mean = 70%) than those exposed to sign language (mean = 51%). CONCLUSIONS: This study provides the most compelling support yet available in CI literature for the benefits of spoken language input for promoting verbal development in children implanted by 3 years of age. Contrary to earlier published assertions, there was no advantage to parents' use of sign language either before or after CI.

A Longitudinal Investigation of the Home Literacy Environment and Shared Book Reading in Young Children With Hearing Loss.

OBJECTIVES: The principle goal of this longitudinal study was to examine parent perceptions of home literacy environment (e.g., frequency of book reading, ease of book reading with child) and observed behaviors during shared book reading (SBR) interactions between parents and their children with hearing loss (HL) as compared with parents and their children with normal hearing (NH) across 3 time points (12, 24, and 36 months old). Relationships were also explored among home literacy environment factors and SBR behaviors and later language outcomes, across all three time points for parents of children with and without HL. DESIGN: Participants were a group of parents and their children with HL (N = 17) and typically developing children with NH (N = 34). Parent perceptions about the home literacy environment were captured through a questionnaire. Observed parent behaviors and their use of facilitative language techniques were coded during videotaped SBR interactions. Children's oral language skills were assessed using a standardized language measure at each time point. RESULTS: No significant differences emerged between groups of parents (HL and NH) in terms of perceived home literacy environment at 12 and 36 months. However, significant group differences were evident for parent perceived ease of reading to their child at 24 months. Group differences also emerged for parental SBR behaviors for literacy strategies and interactive reading at 12 months and for engagement and interactive reading at 36 months, with parents of children with HL scoring lower in all factors. No significant relationships emerged between early home literacy factors and SBR behaviors at 12 months and oral language skills at 36 months for parents of children with NH. However, significant positive relationships were evident between early home literacy environment factors at 12 months and oral language skills at 36 months for parents and their children with HL. CONCLUSIONS: Although both groups of parents increased their frequency of SBR behaviors over time, parents of children with HL may need additional support to optimize SBR experiences to better guide their toddlers' and preschoolers' language skills. Early intervention efforts that focus on SBR interactions that are mutually enjoyed and incorporate specific ways to encourage parent-child conversations will be essential as children with HL acquire language.

Initial Results of a Safety and Feasibility Study of Auditory Brainstem Implantation in Congenitally Deaf Children.

OBJECTIVE: To determine the safety and feasibility of the auditory brainstem implant (ABI) in congenitally deaf children with cochlear aplasia and/or cochlear nerve deficiency. STUDY DESIGN: Phase I feasibility clinical trial of surgery in 10 children, ages 2 to 5 years, over a 3-year period. SETTING: Tertiary children's hospital and university-based pediatric speech/language/hearing center. INTERVENTION(S): ABI implantation and postsurgical programming. MAIN OUTCOME MEASURE(S): The primary outcome measure is the number and type of adverse events during ABI surgery and postsurgical follow-up, including behavioral mapping of the device. The secondary outcome measure is access to and early integration of sound. RESULTS: To date, nine children are enrolled. Five children have successfully undergone ABI surgery and postoperative behavioral programming. Three children were screen failures, and one child is currently undergoing candidacy evaluation. Expected adverse events have been documented in three of the five children who received the ABI. One child experienced a cerebral spinal fluid leak, which resolved with lumbar drainage. One child demonstrated vestibular side effects during device programming, which resolved by deactivating one electrode. One child experienced postoperative vomiting resulting in an abdominal radiograph. Four children have completed their 1-year follow-up and have speech detection thresholds of 30 to 35 dB HL. Scores on the IT-MAIS/MAIS range from 8 to 31 (out of a total of 40), and the children are demonstrating some ability to discriminate between closed-sets words that differ by number of syllables (pattern perception). CONCLUSION: ABI surgery and device activation seem to be safe and feasible in this preliminary cohort.

Sentence Recognition in Quiet and Noise by Pediatric Cochlear Implant Users: Relationships to Spoken Language.

OBJECTIVE: We investigated associations between sentence recognition and spoken language for children with cochlear implants (CI) enrolled in the Childhood Development after Cochlear Implantation (CDaCI) study. STUDY DESIGN: In a prospective longitudinal study, sentence recognition percent-correct scores and language standard scores were correlated at 48-, 60-, and 72-months post-CI activation. SETTING: Six tertiary CI centers in the United States. PATIENTS: Children with CIs participating in the CDaCI study. INTERVENTION (S): Cochlear implantation. MAIN OUTCOME MEASURE (S): Sentence recognition was assessed using the Hearing In Noise Test for Children (HINT-C) in quiet and at +10, +5, and 0 dB signal-to-noise ratio (S/N). Spoken language was assessed using the Clinical Assessment of Spoken Language (CASL) core composite and the antonyms, paragraph comprehension (syntax comprehension), syntax construction (expression), and pragmatic judgment tests. RESULTS: Positive linear relationships were found between CASL scores and HINT-C sentence scores when the sentences were delivered in quiet and at +10 and +5 dB S/N, but not at 0 dB S/N. At 48 months post-CI, sentence scores at +10 and +5 dB S/N were most strongly associated with CASL antonyms. At 60 and 72 months, sentence recognition in noise was most strongly associated with paragraph comprehension and syntax construction. CONCLUSIONS: Children with CIs learn spoken language in a variety of acoustic environments. Despite the observed inconsistent performance in different listening situations and noise-challenged environments, many children with CIs are able to build lexicons and learn the rules of grammar that enable recognition of sentences.

A Prospective Longitudinal Study of U.S. Children Unable to Achieve Open-Set Speech Recognition 5 Years After Cochlear Implantation.

OBJECTIVE: To identify characteristics associated with the inability to progress to open-set speech recognition in children 5 years after cochlear implantation. STUDY DESIGN: Prospective, longitudinal, and multidimensional assessment of auditory development for 5 years. SETTING: Six tertiary cochlear implant (CI) referral centers in the United States. PATIENTS: Children with severe-to-profound hearing loss who underwent implantation before age 5 years enrolled in the Childhood Development after Cochlear Implantation study, categorized by level of speech recognition ability. INTERVENTION(S): Cochlear implantation before 5 years of age and annual assessment of emergent speech recognition skills. MAIN OUTCOME MEASURE(S): Progression to open-set speech recognition by 5 years after implantation. RESULTS: Less functional hearing before implantation, older age at onset of amplification, lower maternal sensitivity to communication needs, minority status, and complicated perinatal history were associated with the inability to obtain open-set speech recognition by 5 years. CONCLUSION: Characteristics of a subpopulation of children with CIs associated with an inability to achieve open-set speech recognition after 5 years of CI experience were investigated. These data distinguish pediatric CI recipients at risk for poor auditory development and highlight areas for future interventions to enhance support of early implantation.

The contributions of William F. House to the field of implantable auditory devices.

William F. House was a pioneer in the evolving field of cochlear implants and auditory brainstem implants. Because of his vision, innovation and perseverance, the way was paved for future clinicians and researchers to carry on the work and advance a field that has been dedicated to serving adults and children with severe to profound hearing loss. Several of William House's contributions are highlighted in this prestigious volume to honor the recipients of the 2013 Lasker-Debakey Clinical Medical Research Award. Discussed are the early inventive years, clinical trials with the single-channel cochlear implant, the team approach, pediatric cochlear implantation, and the auditory brainstem implant. Readers may be surprised to learn that those early contributions continue to have relevance today. This article is part of a Special Issue entitled .

Developmental outcomes of early-identified children who are hard of hearing at 12 to 18 months of age.

AIMS: To examine the developmental outcomes of early-identified children who are hard of hearing, at 12 to 18 months of age, compared to those for children of similar age with normal hearing; and to investigate parent and child factors that are associated with these developmental outcomes. METHODS: As part of a prospective study, 28 children with mild to severe hearing loss between the ages of 12 and 18 months and 42 children with normal hearing of similar age completed a comprehensive assessment battery. All children with hearing loss were identified by newborn hearing screening and amplified, on average, by 5 months of age. Outcome measures included: Mullen Scales of Early Learning; Preschool Language Scale-4th Ed; MacArthur-Bates Communicative Development Inventory; Infant-Toddler Social and Emotional Assessment; Vineland Adaptive Behavior Scales, Second Edition; Parenting Stress Index-Short Form; and Maternal Self-Efficacy Scale. RESULTS: Children with hearing loss scored comparably to children with normal hearing on select outcome measures, with mean scores for both groups falling within normal limits. Greater maternal self-efficacy was associated with children's better language skills, adaptive behavior, social-emotional competence, and fewer problem behaviors. CONCLUSION: Very young children with mild to severe hearing loss, who are identified early and provided prompt intervention that includes amplification, can demonstrate age appropriate development in multiple domains. Results also underscore the significance of parenting factors, especially perceived maternal self-efficacy, in relation to positive developmental outcomes for these children early in life.

Parental Support for Language Development During Joint Book Reading for Young Children With Hearing Loss.

Parent and child joint book reading (JBR) characteristics and parent facilitative language techniques (FLTs) were investigated in two groups of parents and their young children; children with normal hearing (NH; n = 60) and children with hearing loss (HL; n = 45). Parent-child dyads were videotaped during JBR interactions, and parent and child behaviors were coded for specific JBR behaviors using a scale developed for this study. Children's oral language skills were assessed using the Preschool Language Scale-4 (PLS-4). Parents of children with HL scored higher on two of the four subscales of JBR: Literacy Strategies and Teacher Techniques. Parents of children with NH utilized higher level FLTs with their children who had higher language skills. Higher level FLTs were positively related to children's oral language abilities. Implications are discussed for professionals who work with families of very young children with HL.