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Purpose: Best practices recommend promoting the use of the home language and allowing caregivers to choose the language(s) that they want to use with their child who is deaf or hard of hearing (DHH). We examined whether Spanish-speaking caregivers of children who are DHH receive professional recommendations on oral bilingualism that follow best practices. We also assessed whether professional recommendations, caregiver beliefs, and language practices had an impact on child language(s) proficiency. Method: Sixty caregivers completed a questionnaire on demographic questions, language(s) use and recommendations, beliefs on bilingualism, and child language proficiency measures in English, Spanish, and American Sign Language (ASL). Professional recommendations on oral bilingualism were reported descriptively, and linear regression was used to identify the predictors of child language(s) proficiency. Results: We found that only 23.3% of the caregivers were actively encouraged to raise their child orally bilingual. Language practices predicted child proficiency in each language (English, Spanish, and ASL), but professional recommendations and caregiver beliefs did not. Conclusions: Our results revealed that most caregivers received recommendations that do not follow current best practices. Professional training is still needed to promote bilingualism and increase cultural competence when providing services to caregivers who speak languages different from English. Supplemental Material: https://doi.org/10.23641/asha.21644846

Introduction. Mycobacterium abscessus is an emerging pulmonary pathogen with limited treatment options. Nitric oxide (NO) demonstrates antibacterial activity against various bacterial species, including mycobacteria. In this study, we evaluated the effect of adjunctive inhaled NO therapy, using a novel NO generator, in a CF patient with pulmonary M. abscessus disease, and examined heterogeneity of response to NO in vitro. Methods. In the compassionate-use treatment, a 24-year-old CF patient with pulmonary M. abscessus was treated with two courses of adjunctive intermittent NO, first at 160 p.p.m. for 21 days and subsequently by escalating the dose up to 240 p.p.m. for 8 days. Methemoglobin, pulmonary function, 6 min walk distance (6MWD), qualify of life and sputum microbiology were assessed. In vitro susceptibility tests were performed against patient’s isolate and comparison clinical isolates and quantified by Hill’s slopes calculated from time–kill curves. Results. M. abscessus lung infection eradication was not achieved, but improvements in selected qualify of life domains, lung function and 6MWD were observed during the study. Inhaled NO was well tolerated at 160 p.p.m. Dosing at 240 p.p.m. was stopped due to adverse symptoms, although methemoglobin levels remained within safety thresholds. In vitro susceptibility tests showed a dose-dependent NO effect on M. abscessus susceptibility and significant heterogeneity in response between M. abscessus clinical isolates. The patient’s isolate was found to be the least susceptible strain in vitro. Conclusion. These results demonstrate heterogeneity in M. abscessus susceptibility to NO and suggest that longer treatment regimens could be required to see the reduction or eradication of more resistant pulmonary strains.

Introduction Mycobacterium abscessus is an emerging pulmonary pathogen with limited treatment options. Nitric oxide (NO) demonstrates antibacterial activity against various bacterial species, including mycobacteria. In this study, we evaluated the effect of adjunctive inhaled NO therapy, using a novel NO generator, in a CF patient with pulmonary M. abscessus disease, and examined heterogeneity of response to NO in vitro. Methods In the compassionate-use treatment, a 24-year-old CF patient with pulmonary M. abscessus was treated with two courses of adjunctive intermittent NO, first at 160 p.p.m. for 21 days and subsequently by escalating the dose up to 240 p.p.m. for 8 days. Methemoglobin, pulmonary function, 6 min walk distance (6MWD), qualify of life and sputum microbiology were assessed. In vitro susceptibility tests were performed against patient’s isolate and comparison clinical isolates and quantified by Hill’s slopes calculated from time–kill curves. Results M. abscessus lung infection eradication was not achieved, but improvements in selected qualify of life domains, lung function and 6MWD were observed during the study. Inhaled NO was well tolerated at 160 p.p.m. Dosing at 240 p.p.m. was stopped due to adverse symptoms, although methemoglobin levels remained within safety thresholds. In vitro susceptibility tests showed a dose-dependent NO effect on M. abscessus susceptibility and significant heterogeneity in response between M. abscessus clinical isolates. The patient’s isolate was found to be the least susceptible strain in vitro. Conclusion These results demonstrate heterogeneity in M. abscessus susceptibility to NO and suggest that longer treatment regimens could be required to see the reduction or eradication of more resistant pulmonary strains.

Mycobacterium abscessus (M. abscessus) is an emerging multi-drug resistant non-tuberculous mycobacterium (NTM) with increasing prevalence in patients with underlying lung disease. We have demonstrated that high-dose nitric oxide (NO) shows potent antibacterial activity against M. abscessus in vitro. Arikayce® (amikacin liposome inhalation) was recently approved by FDA for the treatment of refractory Mycobacterium avium complex lung infection. Clofazimine, used in treatment of leprosy and tuberculosis, has been increasingly utilized in the treatment of M. abscessus lung infection. In this study, we investigated the synergistic effect of high-dose NO combined with amikacin and clofazimine against M. abscessus. We have used a NO Exposure Chamber to test the susceptibility of three M. abscessus clinical strains (MAB-062600, MAB-030804, and MAB-010708) and ATCC 19977 to NO and its combination with clofazimine and amikacin. Disc Diffusion or E-test methods was used to determine the minimum inhibitory concentrations (MICs) for each strain followed by Agar Dilution method to determine synergistic effects. Treatment with 250ppm NO alone for 10hr demonstrated a 5- to 6-log reductions in M. abscessus clinical strains and ATCC 19977. Preliminary results indicate that NO in combination with clofazimine (2X MIC) results in an additional 1-log reduction in MAB-062600 strain compared to NO and CLO alone. Tests on the effects of clofazimine and amikacin in combination with 250ppm NO in remaining M. abscessus strains are ongoing. Our findings suggests that 250ppm NO is highly efficacious against M. abscessus and combination with clofazimine may further improve its activity.

The precise spatial and temporal expression of genes is essential for proper organismal development. Despite their importance, however, many developmental genes have yet to be identified. We have determined that Fer1l6, a member of the ferlin family of genes, is a novel factor in zebrafish development. We find that Fer1l6 is expressed broadly in the trunk and head of zebrafish larvae and is more restricted to gills and female gonads in adult zebrafish. Using both genetic mutant and morpholino knockdown models, we found that loss of Fer1l6 led to deformation of striated muscle tissues, delayed development of the heart, and high morbidity. Further, expression of genes associated with muscle cell proliferation and differentiation were affected. Fer1l6 was also detected in the C2C12 cell line, and unlike other ferlin homologues, we found Fer1l6 expression was independent of the myoblast-to-myotube transition. Finally, analysis of cell and recombinant protein–based assays indicate that Fer1l6 colocalizes with syntaxin 4 and vinculin, and that the putative C2 domains interact with lipid membranes. We conclude that Fer1l6 has diverged from other vertebrate ferlins to play an essential role in zebrafish skeletal and cardiac muscle development.