Validation of the Maltese Adaptive Auditory Speech Test (AAST).

The Adaptive Auditory Speech Test (AAST) was developed to record the Speech Recognition Threshold (SRT) in children in quiet or with background noise. AAST is an interlingually valid and reliable standardised tool with speech material developed in several languages. The Maltese version of the Adaptive Auditory Speech Test (AAST) was developed to examine the speech recognition skills of 208 children and 40 Maltese-speaking adults in quiet, noise and high frequency. The aims were to determine the norms in these three settings in adults and children aged 4 years and older. The Maltese version of AAST confirms an age dependent norm threshold with a significant improvement in threshold being observed as children grow older, similar to other AAST versions. This was evident across the three test settings. An approximate difference of 10 dB was also noted between 4-year-old and 10-year-old children in AAST in quiet. Thresholds of 10-year-olds and adults were similar in both the quiet and high frequency versions. Implications for post Universal Newborn Hearing Screening using these tools are addressed.

Validation of the LittlEARS® Questionnaire in Hearing Maltese-Speaking Children.

OBJECTIVES: To adapt the LittlEARS® Auditory Questionnaire into the Maltese language and evaluate the psychometric properties of the Maltese version of the questionnaire for hearing children. METHODS: The English version of LittlEARS® Auditory Questionnaire was adapted into Maltese using a translation/back translation procedure. In this cross-sectional study, a total of 398 parents of normal hearing children aged between 5 days and 36 months completed the Maltese version of LittlEARS®. Psychometric validation was performed through scale analysis, item analysis, and analysis of reliability and validity. A non-linear regression model was derived to obtain normative data for expected and minimum values of total scores from the questionnaire according to age. RESULTS: Predictive accuracy (Guttman's lambda) was 0.921, the Cronbach's alpha coefficient value was 0.921, and the split-half reliability coefficient was 0.949. The Pearson correlation coefficient between scores and age was 0.903. The regression analysis showed that 82% of the variance in the total scores can be explained by age. Norm curves were comparable to the original German data. CONCLUSION: This study confirmed that the Maltese version of LittlEARS® is a valid and reliable tool to evaluate auditory development in children less than two years of age.

What lies beneath: Hydra provides cnidarian perspectives into the evolution of FGFR docking proteins.

Across the Bilateria, FGF/FGFR signaling is critical for normal development, and in both Drosophila and vertebrates, docking proteins are required to connect activated FGFRs with downstream pathways. While vertebrates use Frs2 to dock FGFR to the RAS/MAPK or PI3K pathways, the unrelated protein, downstream of FGFR (Dof/stumps/heartbroken), fulfills the corresponding function in Drosophila. To better understand the evolution of the signaling pathway downstream of FGFR, the available sequence databases were screened to identify Frs2, Dof, and other key pathway components in phyla that diverged early in animal evolution. While Frs2 homologues were detected only in members of the Bilateria, canonical Dof sequences (containing Dof, ankyrin, and SH2/SH3 domains) were present in cnidarians as well as bilaterians (but not in other animals or holozoans), correlating with the appearance of FGFR. Although these data suggested that Dof coupling might be ancestral, gene expression analysis in the cnidarian Hydra revealed that Dof is not upregulated in the zone of strong FGFRa and FGFRb expression at the bud base, where FGFR signaling controls detachment. In contrast, transcripts encoding other, known elements of FGFR signaling in Bilateria, namely the FGFR adaptors Grb2 and Crkl, which are acting downstream of Dof (and Frs2), as well as the guanyl nucleotide exchange factor Sos, and the tyrosine phosphatase Csw/Shp2, were strongly upregulated at the bud base. Our expression analysis, thus, identified transcriptional upregulation of known elements of FGFR signaling at the Hydra bud base indicating a highly conserved toolkit. Lack of transcriptional Dof upregulation raises the interesting question, whether Hydra FGFR signaling requires either of the docking proteins known from Bilateria.

LittlEARS auditory questionnaire as an infant hearing screening in Germany after the newborn hearing screening.

Objective: To investigate the feasibility of using the LittlEARS® Auditory Questionnaire (LEAQ®) as part of the infant hearing screening programme in Germany. Design: LEAQ®s were distributed to 47 paediatric practices and were completed by the parents/guardians of the infants (aged between 9-14 months) involved in the study (= LEAQ® screening). The infants who failed the LEAQ® screening were invited to a LEAQ rescreening. Infants who failed the LEAQ® rescreening were sent to a paediatric ENT specialist. After 3 years, a follow-up was performed on two groups: the first group comprised infants who failed the LEAQ screening; the second group (control group) comprised 200 infants who passed the LEAQ screening. Study Sample: 5316 questionnaires were returned. Results: Six infants with permanent hearing loss were identified using the LEAQ® as a screening tool. Conclusions: An infant hearing screening using the LEAQ® is easily implementable in paediatric practices and may be a good alternative in countries where no objective screening instruments are available. The LEAQ® was suitable for monitoring hearing development in infants in general and could help to identify a late-onset or progressive hearing loss in infants.