Project ASPIRE: Spoken Language Intervention Curriculum for Parents of Low-socioeconomic Status and Their Deaf and Hard-of-Hearing Children.

OBJECTIVE: To investigate the impact of a spoken language intervention curriculum aiming to improve the language environments caregivers of low socioeconomic status (SES) provide for their D/HH children with CI & HA to support children's spoken language development. STUDY DESIGN: Quasiexperimental. SETTING: Tertiary. PATIENTS: Thirty-two caregiver-child dyads of low-SES (as defined by caregiver education ≤ MA/MS and the income proxies = Medicaid or WIC/LINK) and children aged < 4.5 years, hearing loss of ≥ 30 dB, between 500 and 4000 Hz, using at least one amplification device with adequate amplification (hearing aid, cochlear implant, osseo-integrated device). INTERVENTION: Behavioral. Caregiver-directed educational intervention curriculum designed to improve D/HH children's early language environments. MAIN OUTCOME MEASURES: Changes in caregiver knowledge of child language development (questionnaire scores) and language behavior (word types, word tokens, utterances, mean length of utterance [MLU], LENA Adult Word Count (AWC), Conversational Turn Count (CTC)). RESULTS: Significant increases in caregiver questionnaire scores as well as utterances, word types, word tokens, and MLU in the treatment but not the control group. No significant changes in LENA outcomes. CONCLUSION: Results partially support the notion that caregiver-directed language enrichment interventions can change home language environments of D/HH children from low-SES backgrounds. Further longitudinal studies are necessary.

Safety and efficacy of alternative alglucosidase alfa regimens in Pompe disease.

Emerging phenotypes in long-term survivors with Pompe disease on standard enzyme replacement therapy (ERT) (alglucosidase alfa 20 mg/kg/2 weeks) can include patients with worsening motor function. Whether higher doses of ERT improve skeletal function in these patients has not been systematically studied. This exploratory, randomized, open-label, 52-week study examined the safety and efficacy of 2 ERT regimens of alglucosidase alfa (20 mg/kg/week or 40 mg/kg/2 weeks) in 13 patients with Pompe disease and clinical decline or a lack of improvement on standard ERT: late-onset (n = 4), infantile-onset (n = 9). Cross-reactive immunologic material assay-negative patients were excluded. Eleven of 13 patients completed the study. Trends for improvement were seen in total gross motor function, but not mobility; however, 6 (late-onset, 2; infantile-onset, 4) of 11 patients (55%) who met the entry criteria of motor decline (late-onset, 4; infantile-onset, 7) showed improvement in motor and/or mobility skills. No between-regimen differences in efficacy emerged. Two case studies highlight the benefits of increased ERT dose in patients with Pompe disease experiencing clinical decline. Both alternative regimens were generally well tolerated. This study was limited by the small sample size, which is not uncommon for small clinical studies of rare diseases. Additionally, the study did not include direct assessment of muscle pathology, which may have identified potential causes of decreased response to ERT. Results were inconclusive but suggest that increased ERT dose may be beneficial in some patients with Pompe disease experiencing motor decline. Controlled studies are needed to clarify the benefits and risks of this strategy.

Child-centered collaborative conversations that maximize listening and spoken language development for children with hearing loss.

In the period that begins with early intervention enrollment and ends with the termination of formal education, speech-language pathologists (SLPs) will have numerous opportunities to form professional relationships that can enhance any child's listening and spoken language accomplishments. SLPs who initiate and/or nurture these relationships are urged to place the needs of the child as the core value that drives decision making. Addressing this priority will allow for the collaborative conversations necessary to develop an effective intervention plan at any level. For the SLP, the purpose of these collaborative conversations will be twofold: identifying the functional communication needs of the child with hearing loss across settings and sharing practical strategies to encourage listening and spoken language skill development. Auditory first, wait time, sabotage, and thinking turns are offered as four techniques easily implemented by all service providers to support the child with hearing loss in all educational settings.

Health disparities in pediatric cochlear implantation: an audiologic perspective.

OBJECTIVES: A national survey of pediatric cochlear implantation (PCI) audiologists was conducted with three aims: (1) to determine if PCI audiologists perceive within their clinical practice a negative effect of low socioeconomic status (SES) on postimplant speech and language outcomes; (2) to understand their perceptions of the underlying factors leading to outcome disparities; and (3) to elicit suggestions for improving outcomes in disadvantaged populations. We hypothesized that audiologists would perceive reduced speech and language outcomes within their lower SES patient population, and that this noted disparity would be related to parental adherence (compliance) and access to habilitation. DESIGN: A survey containing 22 quantitative and open-ended questions was electronically mailed to a data base of 234 PCI audiologists. Forty-four percent (N = 103 of 234) responded to the survey, with the majority (98 of 103) answering all questions. Quantitative responses were analyzed using the Stata 9 statistical package with significance at p < 0.05. Qualitative responses were analyzed using standardized codebook and content analysis. Transcripts were read and coded for the main ideas expressed in each response. The codes were then analyzed for patterns and organized into subthemes that were then grouped into themes. RESULTS: Seventy-eight percent (N = 76 of 98) of respondents perceived an effect of SES on postimplant speech and language outcomes. Qualitative responses uniformly demonstrated audiologists' perception that lower SES patient populations were more likely to experience reduced speech and language outcomes. Two major themes emerged in audiologists' explanations of SES-related disparities: internal factors of parental influence (i.e., parental self-efficacy, adherence, and habilitation carryover), and external factors (i.e., inadequate therapy and lack of available resources). Three primary suggestions were offered for reducing the disparity: improvement in cochlear implant services (92%), increased emphasis on parental education and intervention (87%), and the development of stricter candidacy requirements (15%). CONCLUSIONS: This study offers evidence to show that PCI audiologists note an SES-related disparity in the field of PCI. Respondents suggest the need for a broad and culturally sensitive effort to both increase access to qualified healthcare professionals and develop approaches that will aid parents in the at-home habilitation process.