Paediatrics (RCH) - Research Publications

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    Comparability of Modern Recording Devices for Speech Analysis: Smartphone, Landline, Laptop, and Hard Disc Recorder
    Vogel, AP ; Rosen, KM ; Morgan, AT ; Reilly, S (KARGER, 2014)
    BACKGROUND: Large-scale multi-site experimental and clinical speech protocols require high-fidelity, easy-to-use speech recording technologies. However, little is known about the reliability and comparability of affordable, portable and commonly used technologies with traditional well-validated devices (e.g., a hard disc recorder with a high-quality microphone). OBJECTIVE: To examine the comparability of speech and voice samples acquired from protocols involving high- and low-quality devices. METHODS: Speech samples were acquired simultaneously from 15 healthy adults using four devices and analyzed acoustically for measures of timing and voice quality. For the purpose of making initial comparisons, methods were deemed comparable if the resultant acoustic data yielded root mean squared error values ≤10% and statistically significant Spearman's correlation coefficients. RESULTS: The data suggest that there is significant and widespread variability in the quality and reliability between different acquisition methods for voice and speech recording. Not one method provided statistically similar data to the protocol using the benchmark device (i.e., a high-quality recorder coupled with a condenser microphone). Acoustic analysis cannot be assumed to be comparable if different recording methods are used to record speech. CONCLUSIONS: Findings have implications for researchers and clinicians hoping to make comparisons between labs or, where lower-quality devices are suggested, to offer equal fidelity.
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    Feasibility of automated speech sample collection with stuttering children using interactive voice response (IVR) technology
    Vogel, AP ; Block, S ; Kefalianos, E ; Onslow, M ; Eadie, P ; Barth, B ; Conway, L ; Mundt, JC ; Reilly, S (TAYLOR & FRANCIS LTD, 2015-04)
    PURPOSE: To investigate the feasibility of adopting automated interactive voice response (IVR) technology for remotely capturing standardized speech samples from stuttering children. METHOD: Participants were 10 6-year-old stuttering children. Their parents called a toll-free number from their homes and were prompted to elicit speech from their children using a standard protocol involving conversation, picture description and games. The automated IVR system was implemented using an off-the-shelf telephony software program and delivered by a standard desktop computer. The software infrastructure utilizes voice over internet protocol. Speech samples were automatically recorded during the calls. Video recordings were simultaneously acquired in the home at the time of the call to evaluate the fidelity of the telephone collected samples. Key outcome measures included syllables spoken, percentage of syllables stuttered and an overall rating of stuttering severity using a 10-point scale. RESULT: Data revealed a high level of relative reliability in terms of intra-class correlation between the video and telephone acquired samples on all outcome measures during the conversation task. Findings were less consistent for speech samples during picture description and games. CONCLUSION: Results suggest that IVR technology can be used successfully to automate remote capture of child speech samples.
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    An open-label trial in Friedreich ataxia suggests clinical benefit with high-dose resveratrol, without effect on frataxin levels
    Yiu, EM ; Tai, G ; Peverill, RE ; Lee, KJ ; Croft, KD ; Mori, TA ; Scheiber-Mojdehkar, B ; Sturm, B ; Praschberger, M ; Vogel, AP ; Rance, G ; Stephenson, SEM ; Sarsero, JP ; Stockley, C ; Lee, C-YJ ; Churchyard, A ; Evans-Galea, MV ; Ryan, MM ; Lockhart, PJ ; Corben, LA ; Delatycki, MB (SPRINGER HEIDELBERG, 2015-05)
    Friedreich ataxia (FRDA) is due to a triplet repeat expansion in FXN, resulting in deficiency of the mitochondrial protein frataxin. Resveratrol is a naturally occurring polyphenol, identified to increase frataxin expression in cellular and mouse models of FRDA and has anti-oxidant properties. This open-label, non-randomized trial evaluated the effect of two different doses of resveratrol on peripheral blood mononuclear cell (PBMC) frataxin levels over a 12-week period in individuals with FRDA. Secondary outcome measures included PMBC FXN mRNA, oxidative stress markers, and clinical measures of disease severity. Safety and tolerability were studied. Twenty-four participants completed the study; 12 received low-dose resveratrol (1 g daily) and 12 high-dose resveratrol (5 g daily). PBMC frataxin levels did not change in either dosage group [low-dose group change: 0.08 pg/μg protein (95% CI -0.05, 0.21, p = 0.21); high-dose group change: 0.03 pg/μg protein (95% CI -0.10, 0.15, p = 0.62)]. Improvement in neurologic function was evident in the high-dose group [change in Friedreich Ataxia Rating Scale -3.4 points, 95% CI (-6.6, -0.3), p = 0.036], but not the low-dose group. Significant improvements in audiologic and speech measures, and in the oxidative stress marker plasma F2-isoprostane were demonstrated in the high-dose group only. There were no improvements in cardiac measures or patient-reported outcome measures. No serious adverse events were recorded. Gastrointestinal side-effects were a common, dose-related adverse event. This open-label study shows no effect of resveratrol on frataxin levels in FRDA, but suggests that independent positive clinical and biologic effects of high-dose resveratrol may exist. Further assessment of efficacy is warranted in a randomized placebo-controlled trial.
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    Voice in Friedreich Ataxia
    Vogel, AP ; Wardrop, MI ; Folker, JE ; Synofzik, M ; Corben, LA ; Delatycki, MB ; Awan, SN (MOSBY-ELSEVIER, 2017-03)
    BACKGROUND: Friedreich Ataxia (FRDA) is the most common hereditary ataxia, with dysarthria as one of its key clinical signs. OBJECTIVE: To describe the voice profile of individuals with FRDA to inform outcome marker development and goals of speech therapy. METHODS: Thirty-six individuals with FRDA and 30 age-matched controls provided sustained vowel and connected speech samples. Speech and voice samples were analyzed acoustically using the Analysis of Dysphonia in Speech and Voice program and perceptually using the Consensus Auditory-Perceptual Evaluation of Voice form. Correlations between dysphonia and overall dysarthria severity, demographic, clinical, and genetic information were explored. RESULTS: Individuals with FRDA presented with mild dysphonia characterized by hoarseness (combined roughness and breathiness), increased strain, and altered pitch variability (increased in vowel productions; slightly decreased on reading samples). Acoustically, individuals with FRDA had significantly higher scores on the Cepstral Spectral Index of Dysphonia during vowel production. A combination of perceptual and acoustic measures of dysphonia used in this study was quite effective in categorizing the FRDA versus control participants, with >80% overall accuracy. CONCLUSIONS: Although dysphonia severity in FRDA did not correlate significantly with overall disease severity, speaking rate and syllabic duration significantly correlated with age at disease onset and disease duration, and also have an effect on listener perception of dysphonia. The relationship between dysphonia and dysarthria in FRDA suggests that reducing overall dysphonia severity via therapeutic techniques that improve phonatory stability and increase speaking rate is a viable target for speech therapy.
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    The History of Stuttering by 7 Years of Age: Follow-Up of a Prospective Community Cohort
    Kefalianos, E ; Onslow, M ; Packman, A ; Vogel, A ; Pezic, A ; Mensah, F ; Conway, L ; Bavin, E ; Block, S ; Reilly, S (AMER SPEECH-LANGUAGE-HEARING ASSOC, 2017-10)
    PURPOSE: For a community cohort of children confirmed to have stuttered by the age of 4 years, we report (a) the recovery rate from stuttering, (b) predictors of recovery, and (c) comorbidities at the age of 7 years. METHOD: This study was nested in the Early Language in Victoria Study. Predictors of stuttering recovery included child, family, and environmental measures and first-degree relative history of stuttering. Comorbidities examined at 7 years included temperament, language, nonverbal cognition, and health-related quality of life. RESULTS: The recovery rate by the age of 7 years was 65%. Girls with stronger communication skills at the age of 2 years had higher odds of recovery (adjusted OR = 7.1, 95% CI [1.3, 37.9], p = .02), but similar effects were not evident for boys (adjusted OR = 0.5, 95% CI [0.3, 1.1], p = .10). At the age of 7 years, children who had recovered from stuttering were more likely to have stronger language skills than children whose stuttering persisted (p = .05). No evident differences were identified on other outcomes including nonverbal cognition, temperament, and parent-reported quality of life. CONCLUSION: Overall, findings suggested that there may be associations between language ability and recovery from stuttering. Subsequent research is needed to explore the directionality of this relationship.
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    Dysphagia in Friedreich Ataxia
    Keage, MJ ; Delatycki, MB ; Gupta, I ; Corben, LA ; Vogel, AP (SPRINGER, 2017-10)
    The objective of the study was to comprehensively characterise dysphagia in Friedreich ataxia (FRDA) and identify predictors of penetration/aspiration during swallowing. We also investigated the psychosocial impact of dysphagia on individuals with FRDA. Sixty participants with FRDA were screened for dysphagia using a swallowing quality of life questionnaire (Swal-QOL) and case history. Individuals reporting dysphagia underwent a standardised oromotor assessment (Frenchay Dysarthria Assessment, 2, FDA-2) and videofluoroscopic study of swallowing (VFSS). Data were correlated with disease parameters (age at symptom onset, age at assessment, disease duration, FXN intron 1 GAA repeat sizes, and Friedreich Ataxia Rating Scale (FARS) score). Predictors of airway penetration/aspiration were explored using logistic regression analysis. Ninety-eight percent (59/60) of participants reported dysphagia, of whom 35 (58.3%) underwent FDA-2 assessment, and 38 (63.3%) underwent VFSS. Laryngeal, respiratory, and tongue dysfunction was observed on the FDA-2. A Penetration-Aspiration Scale score above 3 (deemed significant airway compromise based on non-clinical groups) was observed on at least one consistency in 13/38 (34.2%) participants. All of those who aspirated (10/38, 26.3%) did so silently, with no overt signs of airway entry such as reflexive cough. Significant correlations were observed between dysphagic symptoms and disease duration and severity. No reliable predictors of penetration or aspiration were identified. Oropharyngeal dysphagia is commonly present in individuals with FRDA and worsens with disease duration and severity. Individuals with FRDA are at risk of aspiration at any stage of the disease and should be reviewed regularly. Instrumental analysis remains the only reliable method to detect aspiration in this population. Dysphagia significantly affects the quality of life of individuals with FRDA.