BACKGROUND: Congenital hearing loss is the most common birth anomaly, typically influencing speech and language development, with potential for later academic, social and employment impacts. Yet, surprisingly, the nuances of how speech is affected have not been well examined with regards to the subtypes of speech-sound disorder (SSD). Nor have the predictors of speech outcome been investigated within a sizeable population cohort. AIMS: (1) To describe the subtypes and prevalence of SSD in children with hearing loss. (2) To determine which characteristics of hearing loss predict the presence of SSD. METHODS & PROCEDURES: A total of 90 children (5-12 years of age) with permanent hearing loss were recruited from an Australian population cohort. Children completed a standardized speech assessment to determine the presence and subtype of SSD. Logistic regression was used to determine the predictors of speech outcome. Demographic, developmental and hearing-related predictors were examined. OUTCOMES & RESULTS: The prevalence of speech disorder overall was 58%, with the most common subtype being phonological delay in 49% of the sample. Factors most predictive of speech disorder were being male, younger and a bimodal user (i.e., using both a hearing aid and a cochlear implant). CONCLUSIONS & IMPLICATIONS: This is the first study, in a sizeable cohort, to describe the prevalence and predictive factors for SSD associated with hearing loss. Clinically, it could be beneficial to implement earlier targeted phonological interventions for children with hearing loss. What this paper adds What is already known on this subject Speech issues are common in children with hearing loss; however, the breakdown of subtypes of SSD (e.g., articulation versus phonological disorder) have not been previously described in a population cohort. This distinction is relevant, as each subtype calls for specific targeted intervention. Studies examining factors predictive of speech outcomes, across a range of hearing levels, are also lacking in a population cohort. What this paper adds to existing knowledge Data suggest the most common type of SSD in children with hearing loss is phonological delay. Males, younger children, and bimodal users were at greater risk of having a subtype of SSD. What are the potential or actual clinical implications of this work? The results are clinically pertinent as the speech diagnosis determines the targeted treatment. Phonological delay is responsive to treatment, and early targeted intervention may improve prognosis for speech outcomes for children with hearing loss.

BACKGROUND: There is no sufficiently accurate short-language measure that could be used by speech-language pathologists, teachers or paraprofessionals to screen young school-aged children to identify those requiring in-depth language evaluations. This may be due to poor development of the available measures, which have omitted crucial test development steps. Applying more stringent development procedures could result in a measure with sufficient accuracy. AIMS: To create and validate a short-language measure that has acceptable accuracy, validity and reliability, and which can be used to identify children who require further assessment and/or referral to speech-language services. METHODS & PROCEDURES: The study consisted of two phases. In Phase 1 (measure creation), 56 children were assessed with 160 direction-following and sentence-recall test items and a reference measure, the Clinical Evaluation of Language Fundamentals-Fourth Edition (CELF-4). Items were then examined for their individual characteristics (validity, reliability, difficulty and discrimination) via item analysis and the highest quality items were selected to form the Short Language Measure (SLaM). In Phase 2 (measure validation), 126 children were assessed with the SLaM and the reference measure (CELF-4) to determine SLaM's accuracy, validity and reliability. OUTCOMES & RESULTS: A total of 40 test items were selected to form SLaM in Phase 1. Findings from Phase 2 indicated that SLaM had an accuracy of 94% (sensitivity = 94%, specificity = 93%), validity of 0.89 and reliability of 0.93. These values remained relatively consistent across both phases. CONCLUSIONS & IMPLICATIONS: The results indicated that SLaM has excellent psychometric properties. It can be used to identify children who need further evaluation by a speech-language pathologist. What this paper adds What is already known on this subject Prior research suggests that combining a direction-following and a sentence-recall task has sufficient discrimination accuracy and agreement with an omnibus language measure. Trialling a large set of direction-following and sentence-recall test items to select those with the highest individual characteristics could result in an effective short-language measure. What this paper adds to existing knowledge A short-language measure (SLaM) was created and validated on two independent samples of children. Items with the highest validities, reliabilities and discrimination capacities were selected to form SLaM. This procedure resulted in a measure with high validity and reliability that exceeded the criterion for adequate discrimination accuracy. What are the potential or actual clinical implications of this work? SLaM is an effective measure that can accurately identify children who require detailed evaluations by speech-language pathologists.

BACKGROUND AND PURPOSE: Objective measurement of speech has shown promising results to monitor disease state in multiple sclerosis. In this study, we characterize the relationship between disease severity and speech metrics through perceptual (listener based) and objective acoustic analysis. We further look at deviations of acoustic metrics in people with no perceivable dysarthria. METHODS: Correlations and regression were calculated between speech measurements and disability scores, brain volume, lesion load and quality of life. Speech measurements were further compared between three subgroups of increasing overall neurological disability: mild (as rated by the Expanded Disability Status Scale ≤2.5), moderate (≥3 and ≤5.5) and severe (≥6). RESULTS: Clinical speech impairment occurred majorly in people with severe disability. An experimental acoustic composite score differentiated mild from moderate (P < 0.001) and moderate from severe subgroups (P = 0.003), and correlated with overall neurological disability (r = 0.6, P < 0.001), quality of life (r = 0.5, P < 0.001), white matter volume (r = 0.3, P = 0.007) and lesion load (r = 0.3, P = 0.008). Acoustic metrics also correlated with disability scores in people with no perceivable dysarthria. CONCLUSIONS: Acoustic analysis offers a valuable insight into the development of speech impairment in multiple sclerosis. These results highlight the potential of automated analysis of speech to assist in monitoring disease progression and treatment response.

AIM: To examine the frequency, characteristics, and factors associated with speech delay and disorder in a community sample of children with cerebral palsy (CP). METHOD: Participants were 84 children (37 females, 47 males; aged between 4y 11mo-6y 6mo) with CP identified through a population-based registry. Speech and oromotor function were systematically evaluated to provide a differential diagnosis of articulation, phonological, and motor speech disorders. RESULTS: In total, 82% (69/84) of participants had delayed or disordered speech production, including minimally verbal presentations (n=20). Verbal participants (n=64) presented with dysarthria (78%), articulation delay or disorder (54%), phonological delay or disorder (43%), features of childhood apraxia of speech (CAS) (17%), or mixed presentations across these conditions. Speech intelligibility was poorest in those with dysarthria and features of CAS. Speech delay or disorder in verbal participants was associated with language impairment (p=0.002) and reduced health-related quality of life (p=0.04) (Fisher's exact test). Poorer speech accuracy (i.e. lower percentage consonants correct) correlated with greater impairments in both language (p<0.001) and oromotor function (p<0.001) (Spearman's test). INTERPRETATION: The speech profile of children with CP is characterized by impairment at multiple levels of speech production (phonetic, cognitive-linguistic, neuromuscular execution, and high-level planning/programming), highlighting the importance of a personalized differential diagnosis informing targeted treatment.

The impact of protein fouling on platinum electrodes was assessed by electrochemical methods. Protein fouling affected the electrode potential and charge transfer through the electrode-solution interface. Adsorbed proteins partially blocked the electrode, with no charge passing through blocked regions. The electrochemical theory and methodology for investigating partially blocked electrodes is fully presented, applied to protein adsorption, and the implications for bionics applications are discussed. The partially blocked electrode had a reduced admittance, and increased impedance and polarization resistance consistent with a smaller effective electrode area. The charge storage capacity and charge injection capacity decreased after protein adsorption. The effective electrode area was assessed by impedance and cyclic voltammetry. The diffusion profile towards the partially blocked electrode was mixed between linear and radial diffusion.

Developmental language disorder (DLD) and developmental speech disorder (DSD) are highly prevalent childhood conditions. An impaired ability to repeat nonsense words ("nonword repetition"), is claimed to be a robust behavioural marker for these conditions. Yet how brain function is altered during this task remains poorly understood. Previous research suggests that DLD or DSD may be associated with reduced brain activation in the inferior frontal and posterior temporal regions when compared to controls. However, this research is limited by within and between group variability in age, speech/language phenotype, and comorbidities. Here, we used functional MRI to examine brain activation during nonword repetition. As anticipated, behavioural findings confirmed that the DLD and DSD groups had poorer nonword repetition performance compared to typical controls. In contrast, fMRI revealed no statistically significant differences in brain activation, despite the groups appearing to engage slightly different regions when compared at identical thresholds. Therefore, whilst nonword repetition is a sensitive clinical marker for DLD and DSD, the findings from this study suggest that this task is not a sensitive brain MRI marker for children with these disorders, unlike for individuals with single gene mutations like FOXP2 mutations.

The most complex interactions between human beings occur through speech, and often in the presence of background noise. Understanding speech in noisy environments requires the integrity of highly integrated and widespread auditory networks likely to be impacted by multiple sclerosis (MS) related neurogenic injury. Despite the impact auditory communication has on a person's ability to navigate the world, build relationships, and maintain employability; studies of speech-in-noise (SiN) perception in people with MS (pwMS) have been minimal to date. Thus, this paper presents a dataset related to the acquisition of pure-tone thresholds, SiN performance and questionnaire responses in age-matched controls and pwMS. Bilateral pure-tone hearing thresholds were obtained at frequencies of 250 hertz (Hz), 500 Hz, 750 Hz, 1000 Hz, 1500 Hz, 2000 Hz, 4000 Hz, 6000 Hz and 8000 Hz, and hearing thresholds were defined as the lowest level at which the tone was perceived 50% of the time. Thresholds at 500 Hz, 1000 Hz, 2000 Hz and 4000 Hz were used to calculate the four-tone average for each participant, and only those with a bilateral four tone average of ≤ 25 dB HL were included in the analysis. To investigate SiN performance in pwMS, pre-recorded Bamford-Kowal-Bench (BKB) sentences were presented binaurally through headphones at five signal-to-noise ratios (SNR) in two noise conditions: speech-weighted noise and multi-talker babble. Participants were required to verbally repeat each sentence they had just heard; or indicate their inability to do so. A 33-item questionnaire, based on validated inventories for specific adult clinical populations with abnormal auditory processing, was used to evaluate auditory processing in daily life for pwMS. For analysis, pwMS were grouped according to their Expanded Disability Status Scale (EDSS) score as rated by a neurologist. PwMS with EDSS scores ≤ 1.5 were classified as 'mild' (n = 20); between 2 and 4.5 as 'moderate' (n = 16) and between 5 and 7 as 'advanced' (n = 10) and were compared to neurologically healthy controls (n = 38). The outcomes of the SiN task conducted in pwMS can be found in Iva et al., (2021). The present data has important implications for the timing and delivery of preparatory education to patients, family, and caregivers about communication abilities in pwMS. This dataset will also be valuable for the reuse/reanalysis required for future investigations into the clinical utility of SiN tasks to monitor disease progression.

Very preterm birth is associated with altered white matter microstructure and language difficulties, which may compromise communication, social function and academic achievement, but the relationship between these two factors is unclear. The aim of this study was to explore associations between white matter microstructure and language domains of semantics, grammar and phonological awareness at 7-years of age on a whole-brain level and within the arcuate fasciculus, an important language pathway, in very preterm and term-born children. Language was assessed in 145 very preterm-born (<30 weeks' gestation and/or <1250 g birth weight) and 33 term-born children aged 7 years. Fractional anisotropy (FA), axial diffusivity (AD), radial diffusivity (RD), mean diffusivity (MD), axon orientation dispersion and axon density were estimated from diffusion magnetic resonance images also obtained at 7 years. The correlation between diffusion values and language was assessed using Tract-Based Spatial Statistics (TBSS). The arcuate fasciculus was delineated using constrained spherical deconvolution tractography and diffusion parameters from this tract were related to language measures using linear regression. While there was evidence for widespread associations between white matter microstructure and language, there was little evidence of differences in these associations between very preterm and term-born groups. TBSS analyses revealed that higher FA and lower AD, RD, and MD in major fibre tracts, including those subserving language, were associated with better semantic, grammar and phonological awareness performance. Higher axon density in widespread fibre tracts was also associated with better semantic performance. The tractography analyses of the arcuate fasciculus showed some evidence for associations between white matter microstructure and language outcomes. White matter microstructural organisation in widespread fibre tracts, including language-relevant pathways, was associated with language performance in whole-brain and tract-based analyses. The associations were similar for very preterm and term-born groups, despite very preterm children performing more poorly across language domains.

Adult-onset hearing loss is insidious and typically diagnosed and managed several years after onset. Often, this is after the loss having led to multiple negative consequences including effects on employment, depressive symptoms, and increased risk of mortality. In contrast, the use of hearing aids is associated with reduced depression, longer life expectancy, and retention in the workplace. Despite this, several studies indicate high levels of unmet need for hearing health services in older adults and poor use of prescribed hearing aids, often leading to their abandonment. In Australia, the largest component of financial cost of hearing loss (excluding the loss of well-being) is due to lost workplace productivity. Nonetheless, the Australian public health system does not have an effective and sustainable hearing screening strategy to tackle the problem of poor detection of adult-onset hearing loss. Given the increasing prevalence and disease burden of hearing impairment in adults, two key areas are not adequately met in the Australian healthcare system: (1) early identification of persons with chronic hearing impairment; (2) appropriate and targeted referral of these patients to hearing health service providers. This paper reviews the current literature, including population-based data from the Blue Mountains Hearing Study, and suggests different models for early detection of adult-onset hearing loss.

BACKGROUND: Clinicians require brief outcome measures in their busy daily practice to document global client outcomes. Based on the UK Therapy Outcome Measure, the Australian Therapy Outcome Measures were designed to capture global therapy outcomes of occupational therapy, physiotherapy and speech pathology in the Australian clinical context. The aim of this study was to investigate the construct (convergent) validity of the Australian Therapy Outcome Measures (AusTOMs) by comparing it with the EuroQuol-5D (EQ-5D). METHODS: The research was a prospective, longitudinal cohort study, with data collected over a seven month time period. The study was conducted at a total of 13 metropolitan and rural health-care sites including acute, sub-acute and community facilities. Two-hundred and five clients were asked to score themselves on the EQ-5D, and the same clients were scored by approximately 115 therapists (physiotherapists, speech pathologists and occupational therapists) using the AusTOMs at admission and discharge. Clients were consecutive admissions who agreed to participate in the study. Clients of all diagnoses, aged 18 years and over (a criteria of the EQ-5D), and able to give informed consent were scored on the measures. Spearman rank order correlation coefficients were used to analyze the relationships between scores from the two tools. The clients were scored on the AusTOMs and EQ-5D. RESULTS: There were many health care areas where correlations were expected and found between scores on the AusTOMs and the EQ-5D. CONCLUSION: In the quest to measure the effectiveness of therapy services, managers, health care founders and clinicians are urgently seeking to undertake the first step by identifying tools that can measure therapy outcome. AusTOMs is one tool that can measure global client outcomes following therapy. In this study, it was found that on the whole, the AusTOMs and the EQ-5D measure similar constructs. Hence, although the validity of a tool is never 'proven', this study offers preliminary support for the construct validity of AusTOMs.

PURPOSE: The purpose of this study was to expand the genetic architecture of neurodevelopmental disorders, and to characterize the clinical features of a novel cohort of affected individuals with variants in ZNF142, a C2H2 domain-containing transcription factor. METHODS: Four independent research centers used exome sequencing to elucidate the genetic basis of neurodevelopmental phenotypes in four unrelated families. Following bioinformatic filtering, query of control data sets, and secondary variant confirmation, we aggregated findings using an online data sharing platform. We performed in-depth clinical phenotyping in all affected individuals. RESULTS: We identified seven affected females in four pedigrees with likely pathogenic variants in ZNF142 that segregate with recessive disease. Affected cases in three families harbor either nonsense or frameshifting likely pathogenic variants predicted to undergo nonsense mediated decay. One additional trio bears ultrarare missense variants in conserved regions of ZNF142 that are predicted to be damaging to protein function. We performed clinical comparisons across our cohort and noted consistent presence of intellectual disability and speech impairment, with variable manifestation of seizures, tremor, and dystonia. CONCLUSION: Our aggregate data support a role for ZNF142 in nervous system development and add to the emergent list of zinc finger proteins that contribute to neurocognitive disorders.