Audiology and Speech Pathology - Research Publications

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Articulatory Kinematics in the Dysarthria Associated with Friedreich's Ataxia

Folker, JE ; Murdoch, BE ; Cahill, LM ; Rosen, KM ; Delatycki, MB ; Corben, LA ; Vogel, AP (HUMAN KINETICS PUBL INC, 2011-07)

Electromagnetic articulography (EMA) was used to investigate the tongue kinematics in the dysarthria associated with Friedreich's ataxia (FRDA). The subject group consisted of four individuals diagnosed with FRDA. Five nonneurologically impaired individuals, matched for age and gender, served as controls. Each participant was assessed using the AG-200 EMA system during six repetitions of the tongue tip sentence Tess told Dan to stay fit and the tongue back sentence Karl got a croaking frog. Results revealed reduced speed measures (i.e., maximum acceleration / deceleration / velocity), greater movement durations and increased articulatory distances for the approach phases of consonant productions. The approach phase, involving movement up to the palate, was more affected than the release phase. It is suggested that deviant lingual kinematics could be the outcome of disturbances to cerebellar function, or possibly in combination with disturbances to upper motor neuron systems.
Differentiating Impairment Levels in Temporal Versus Spatial Aspects of Linguopalatal Contacts in Friedreich's Ataxia

Folker, JE ; Murdoch, BE ; Cahill, LM ; Rosen, KM ; Delatycki, MB ; Corben, LA ; Vogel, AP (HUMAN KINETICS PUBL INC, 2010-10)

Electropalatography (EPG) was used to describe the pattern of linguopalatal contact and the consonant phase durations exhibited by a group of seven individuals with dysarthria associated with Friedreich's ataxia (FRDA). A group of 14 non-neurologically impaired individuals served as controls. The Reading Electropalatograph (EPG3) system was used to record linguopalatal contact during production of the target consonants (/t/, /l/, /s/, /k/) elicited in five words of CV and CVC construction, with the target consonants in word initial position. These words were embedded into short sentences and repeated five times by each participant. The FRDA group exhibited significantly increased consonant durations compared with the controls while maintaining normal linguopalatal contact patterns. These findings suggest that the articulatory impairment in FRDA manifests as a temporal rather than spatial disturbance.
Differentiating profiles of speech impairments in Friedreich's ataxia: a perceptual and instrumental approach

Folker, JE ; Murdoch, BE ; Rosen, KM ; Cahill, LM ; Delatycki, MB ; Corben, LA ; Vogel, AP (WILEY, 2012)

BACKGROUND: The speech disorder associated with Friedreich's ataxia (FRDA) is classically described as ataxic dysarthria. However, variable neuropathology beyond the cerebellum, which may include the corticospinal and corticobulbar tracts, means that the dysarthria can be mixed rather than a pure ataxic dysarthria. AIMS: To characterize physiological features of the dysarthria associated with FRDA and identify differential patterns of deviation that may occur across the subsystems of the speech-production mechanism in a series of seven case studies. METHODS & PROCEDURES: The assessment battery included a perceptual analysis of a speech sample using an interval rating scale, and a range of instrumental measures to investigate the respiratory, laryngeal, velopharyngeal and articulatory systems. OUTCOMES & RESULTS: The results demonstrated the variability that exists in the dysarthria associated with FRDA, highlighting the existence of differential profiles of speech impairment. A particular distinction was observed between the presence of hypernasality and phonatory dysfunction, as evidenced by the instrumental results. CONCLUSIONS & IMPLICATIONS: The distinct profiles of dysarthria associated with FRDA indicate that approaches that address multiple subsystems are necessary for the accurate characterization and quantification of the motor speech disorder. Further research is required to investigate the decline in speech function as the disease progresses, as changes in speech function over time may be a good indicator of neurological decline in FRDA.
Dysphagia and swallowing-related quality of life in Friedreich ataxia

Vogel, AP ; Brown, SE ; Folker, JE ; Corben, LA ; Delatycki, MB (SPRINGER HEIDELBERG, 2014-02)

Dysphagia in Friedreich ataxia (FRDA) and its impact on quality of life is not adequately understood. The objective of this study was to characterise dysphagia in FRDA and to determine the impact of swallowing dysfunction on activities, participation, and sense of well-being. Thirty-six individuals with a confirmed diagnosis of FRDA were assessed via a clinical bedside examination (CBE), the Royal Brisbane Hospital outcome measure for swallowing, an oral-motor examination and the Australian therapy outcome measures for speech and swallowing (AusTOMS). Data on swallowing function, diet modification and swallowing strategies were collated. Thirty-three (91.67 %) participants exhibited clinical signs of dysphagia according to the CBE, and all participants received ratings indicating swallowing difficulties on at least one other measure. Dysphagia in FRDA is characterised by oral and pharyngeal stage impairment relating to incoordination, weakness and spasticity. A significant positive correlation was found between the severity of impairment, activity, participation and distress/well-being on the AusTOMS, suggesting that swallowing function decreases with overall reductions in quality of life. A significant correlation was found between activity on the AusTOMS and disease duration (r = -0.283, p = 0.012). No significant correlations were found between dysphagia severity and GAA repeat length, age of onset or disease severity. Participants employing diet modification and swallowing strategies demonstrated higher dysphagia severity, activity limitations and participation restrictions. These data advocate a holistic approach to dysphagia management in FRDA. Early detection of swallowing impairment and consideration of the potential impact dysphagia has on quality of life should be key aspects in disease management.
Nasality in Friedreich ataxia

Poole, ML ; Wee, JS ; Folker, JE ; Corben, LA ; Delatycki, MB ; Vogel, AP (INFORMA HEALTHCARE, 2015-01)

Perceptual speech research in Friedreich ataxia (FRDA) has identified altered nasality as a key component of the dysarthria profile, however the incidence and severity of abnormal nasality remains unknown. Utilizing objective and perceptual methods, data on the relationship between resonance, disease duration, severity, age of onset and genetic profiles were collated. Thirty-seven participants with FRDA and 24 healthy controls provided contemporaneous speech samples for perceptual analysis, and single word samples for acoustic analysis. A subset of participants (eight participants with FRDA and eight controls) underwent nasometry assessment. Twenty-seven participants with FRDA presented with hypernasality and five with hyponasality on perceptual assessment. Acoustic analysis revealed participants with FRDA had greater nasality than controls (p < 0.05). Perceptual ratings of hypernasality correlated with GAA2 repeat length (ρ = 0.37, p = 0.03). Findings highlight the variability of nasality in FRDA, potentially reflecting variation in the neuropathological profile. Data also suggest the influence of genetic profiles on nasality.
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.
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.
Automatic method of pause measurement for normal and dysarthric speech

Rosen, K ; Murdoch, B ; Folker, J ; Vogel, A ; Cahill, L ; Delatycki, M ; Corben, L (TAYLOR & FRANCIS INC, 2010-02)

This study proposes an automatic method for the detection of pauses and identification of pause types in conversational speech for the purpose of measuring the effects of Friedreich's Ataxia (FRDA) on speech. Speech samples of approximately 3 minutes were recorded from 13 speakers with FRDA and 18 healthy controls. Pauses were measured from the intensity contour and fit with bimodal lognormal distributions using the Expectation-Maximization algorithm in Matlab. In the speakers with FRDA, both modes in the pause distributions had significantly larger means, with disproportionately fewer pauses associated with the first mode. From this preliminary study, it is concluded that distributional analysis of pause duration holds promise as a useful method of measuring the effects of FRDA on functional speech.
Dysarthria in Friedreich's Ataxia: A Perceptual Analysis

Folker, J ; Murdoch, B ; Cahill, L ; Delatycki, M ; Corben, L ; Vogel, A (KARGER, 2010)

The aims of this study were to: (1) evaluate the perceptual speech dimensions, speech intelligibility and dysarthria severity of a group of individuals diagnosed with Friedreich's ataxia (FRDA); (2) determine the presence of subgroups within FRDA dysarthria; (3) investigate the relationship between the speech outcome and the clinical factors of disease progression. The study included 38 individuals (21 female, 17 male) with a confirmed diagnosis of FRDA. A group of 20 non-neurologically impaired individuals served as controls. Perceptual analysis, investigating 30 different dimensions of speech, was conducted on a speech sample obtained from each participant. In addition, the Assessment of Intelligibility of Dysarthria Speech was administered. All FRDA participants presented with dysarthria with severities ranging from mild to moderate. Cluster analysis revealed 3 subgroups, the first presenting with mild dysarthric symptoms, the second with increased velopharyngeal involvement and the third characterized by increased laryngeal dysfunction. Dysarthria severity showed a significant correlation to disease duration but to no other clinical measure. The findings support the notion of subgroups in FRDA dysarthria, representing distinct impairments of the speech mechanism and perhaps reflective of differing evolutions beyond the cerebellum.
Kinematic analysis of lingual movements during consonant productions in dysarthric speakers with Friedreich's ataxia: A case-by-case analysis

Folker, JE ; Murdoch, BE ; Cahill, LM ; Delatycki, MB ; Corben, LA ; Vogel, AP (TAYLOR & FRANCIS INC, 2011-01)

Articulatory kinematics were investigated using electromagnetic articulography (EMA) in four dysarthric speakers with Friedreich's ataxia (FRDA). Specifically, tongue-tip and tongue-back movements were recorded by the AG-200 EMA system during production of the consonants /t/ and /k/ as produced within a sentence utterance and during a rapid syllable repetition task. The results obtained for each of the participants with FRDA were individually compared to those obtained by a control group (n = 10). Results revealed significantly greater movement durations and increased articulatory distances, most predominantly during the approach phase of consonant production. A task difference was observed with lingual kinematics more disturbed during the syllable repetition task than during the sentence utterance. Despite expectations of slowed articulatory movements in FRDA dysarthria, the EMA data indicated that the observed prolongation of consonant phase durations was generally associated with greater articulatory distances, rather than slowed movement execution.

Audiology and Speech Pathology - Research Publications

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