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Dysarthria is a common and debilitating symptom of many neurodegenerative diseases, including those resulting in ataxia. Changes to speech lead to significant reductions in quality of life, impacting the speaker in most daily activities. Recognition of its importance as an objective outcome measure in clinical trials for ataxia is growing. Its viability as an endpoint across the disease spectrum (i.e. pre-symptomatic onwards) means that trials can recruit ambulant individuals and later-stage individuals who are often excluded because of difficulty completing lower limb tasks. Here we discuss the key considerations for speech testing in clinical trials including hardware selection, suitability of tasks and their role in protocols for trials and propose a core set of tasks for speech testing in clinical trials. Test batteries could include forms suitable for remote short, sensitive and easy to use, with norms available in several languages. The use of artificial intelligence also could improve accuracy and automaticity of analytical pipelines in clinic and trials.

Introduction: Smart devices are widely available and capable of quickly recording and uploading speech segments for health-related analysis. The switch from laboratory recordings with professional-grade microphone setups to remote, smart device-based recordings offers immense potential for the scalability of voice assessment. Yet, a growing body of literature points to a wide heterogeneity among acoustic metrics for their robustness to variation in recording devices. The addition of consumer-grade plug-and-play microphones has been proposed as a possible solution. The aim of our study was to assess if the addition of consumer-grade plug-and-play microphones increases the acoustic measurement agreement between ultra-portable devices and a reference microphone. Methods: Speech was simultaneously recorded by a reference high-quality microphone commonly used in research and by two configurations with plug-and-play microphones. Twelve speech-acoustic features were calculated using recordings from each microphone to determine the agreement intervals in measurements between microphones. Agreement intervals were then compared to expected deviations in speech in various neurological conditions. Each microphone’s response to speech and to silence was characterized through acoustic analysis to explore possible reasons for differences in acoustic measurements between microphones. The statistical differentiation of two groups, neurotypical and people with multiple sclerosis, using metrics from each tested microphone was compared to that of the reference microphone. Results: The two consumer-grade plug-and-play microphones favored high frequencies (mean center of gravity difference ≥ +175.3 Hz) and recorded more noise (mean difference in signal to noise ≤ −4.2 dB) when compared to the reference microphone. Between consumer-grade microphones, differences in relative noise were closely related to distance between the microphone and the speaker’s mouth. Agreement intervals between t

OBJECTIVES: Speech and language impairments are core features of the neurodevelopmental genetic condition Kleefstra syndrome. Communication has not been systematically examined to guide intervention recommendations. We define the speech, language and cognitive phenotypic spectrum in a large cohort of individuals with Kleefstra syndrome. METHOD: 103 individuals with Kleefstra syndrome (40 males, median age 9.5 years, range 1-43 years) with pathogenic variants (52 9q34.3 deletions, 50 intragenic variants, 1 balanced translocation) were included. Speech, language and non-verbal communication were assessed. Cognitive, health and neurodevelopmental data were obtained. RESULTS: The cognitive spectrum ranged from average intelligence (12/79, 15%) to severe intellectual disability (12/79, 15%). Language ability also ranged from average intelligence (10/90, 11%) to severe intellectual disability (53/90, 59%). Speech disorders occurred in 48/49 (98%) verbal individuals and even occurred alongside average language and cognition. Developmental regression occurred in 11/80 (14%) individuals across motor, language and psychosocial domains. Communication aids, such as sign and speech-generating devices, were crucial for 61/103 (59%) individuals including those who were minimally verbal, had a speech disorder or following regression. CONCLUSIONS: The speech, language and cognitive profile of Kleefstra syndrome is broad, ranging from severe impairment to average ability. Genotype and age do not explain the phenotypic variability. Early access to communication aids may improve communication and quality of life.

BACKGROUND: Verbal fluency tests (VFTs) are widely used to assess cognitive-linguistic performance in neurological diseases. However, the influence of dysarthria on performance in tests requiring oral responses is unclear in ataxia and Parkinson's disease. OBJECTIVES: To determine the impact of dysarthria on VFT performance and evaluate the validity and reliability of alternative methods for analyzing VFT data. METHOD: Trained raters evaluated dysarthria using VFT recordings in people with ataxia (N = 61) or Parkinson's disease (PD; N = 69). Total Correct Items scores and qualitative parameters (intrusions, ambiguous verbalizations, perseverations, and interjections) were compared across semantic, phonemic, and alternating fluency tasks. Disease severity was considered as a covariate in the regression model. RESULTS: VFT dysarthria ratings correlated with the benchmark (ground truth) dysarthria scores derived from a monologue. Ambiguous responses resulting from unclear speech impeded the rater's ability to determine if a response was correct. Regression analysis indicated that more severe dysarthria ratings predicted diminished scores in all three tasks (semantic fluency, phonemic fluency and alternating fluency) in the ataxia group. The contribution of disease severity to semantic, phonemic and alternating fluency was reduced substantially in the ataxia group after accounting for dysarthria severity in the model in both groups. CONCLUSIONS: Dysarthria severity can be estimated based on speech samples derived from VFT. Dysarthria can lead to lower total correct items and is associated with more ambiguous verbalizations in VFT. Dysarthria severity should be considered when interpreting VFT performance in common movement disorders.

Neurodegenerative disease often affects speech. Speech acoustics can be used as objective clinical markers of pathology. Previous investigations of pathological speech have primarily compared controls with one specific condition and excluded comorbidities. We broaden the utility of speech markers by examining how multiple acoustic features can delineate diseases. We used supervised machine learning with gradient boosting (CatBoost) to delineate healthy speech from speech of people with multiple sclerosis or Friedreich ataxia. Participants performed a diadochokinetic task where they repeated alternating syllables. We subjected 74 spectral and temporal prosodic features from the speech recordings to machine learning. Results showed that Friedreich ataxia, multiple sclerosis and healthy controls were all identified with high accuracy (over 82%). Twenty-one acoustic features were strong markers of neurodegenerative diseases, falling under the categories of spectral qualia, spectral power, and speech rate. We demonstrated that speech markers can delineate neurodegenerative diseases and distinguish healthy speech from pathological speech with high accuracy. Findings emphasize the importance of examining speech outcomes when assessing indicators of neurodegenerative disease. We propose large-scale initiatives to broaden the scope for differentiating other neurological diseases and affective disorders.

Mice communicate through audible vocalizations, which are within the human hearing range, and ultrasonic vocalizations (USVs), which are above the upper limit of human hearing. USVs are produced by rodents in social contexts including pup separation, territorial, and courting assays. Like birdsong, an established model for human speech, USVs in mice have been used as a model for understanding human communication. Their utility as a model of social communication is illustrated in neurodevelopmental conditions with a genetic basis, like autism spectrum disorders and Rett syndrome. As mice do not exhibit clear visual cues when they vocalize, the source of vocalization is often assumed. Therefore, there is potential to better discern the true vocal contribution of individual mice if the upper limit of human hearing were to be extended. Currently, there are efforts to increase the precision of sound-localizing technology, which will develop our understanding of communication in mice and other animal models.

Stuttering is a common speech disorder that interrupts speech fluency and tends to cluster in families. Typically, stuttering is characterized by speech sounds, words or syllables which may be repeated or prolonged and speech that may be further interrupted by hesitations or 'blocks'. Rare variants in a small number of genes encoding lysosomal pathway proteins have been linked to stuttering. We studied a large four-generation family in which persistent stuttering was inherited in an autosomal dominant manner with disruption of the cortico-basal-ganglia-thalamo-cortical network found on imaging. Exome sequencing of three affected family members revealed the PPID c.808C>T (p.Pro270Ser) variant that segregated with stuttering in the family. We generated a Ppid p.Pro270Ser knock-in mouse model and performed ex vivo imaging to assess for brain changes. Diffusion-weighted MRI in the mouse revealed significant microstructural changes in the left corticospinal tract, as previously implicated in stuttering. Quantitative susceptibility mapping also detected changes in cortico-striatal-thalamo-cortical loop tissue composition, consistent with findings in affected family members. This is the first report to implicate a chaperone protein in the pathogenesis of stuttering. The humanized Ppid murine model recapitulates network findings observed in affected family members.

Multiple sclerosis (MS) is a progressive disease that often affects the cerebellum. It is characterised by demyelination, inflammation, and neurodegeneration within the central nervous system. Damage to the cerebellum in MS is associated with increased disability and decreased quality of life. Symptoms include gait and balance problems, motor speech disorder, upper limb dysfunction, and oculomotor difficulties. Monitoring symptoms is crucial for effective management of MS. A combination of clinical, neuroimaging, and task-based measures is generally used to diagnose and monitor MS. This paper reviews the present and new tools used by clinicians and researchers to assess cerebellar impairment in people with MS (pwMS). It also describes recent advances in digital and home-based monitoring for people with MS.

BACKGROUND: A purpose-built outcome measure for assessing communication effectiveness in patients with an artificial airway is needed. OBJECTIVES: The objective of this study was to develop the Communication with an Artificial airway Tool (CAT) and to test the feasibility and to preliminary evaluate the clinical metrics of the tool. METHODS: Eligible patients with an artificial airway in the Intensive Care Unit were enrolled in the pilot study (Crit-CAT). The CAT was administered at least twice before and after the communication intervention. Item correlation analysis was performed. Participant and family member acceptability ratings and feedback were solicited. A qualitative thematic analysis was undertaken. RESULTS: Fifteen patients with a mean age of 53 years (standard deviation [SD]: 19.26) were included. The clinician-reported scale was administered on 50 attempts (100%) with a mean completion time of 4.5 (SD: 0.77) minutes. The patient-reported scale was administered on 46 out of 49 attempts (94%) and took a mean of 1.5 (SD: 0.39) minutes to complete. The CAT was feasible for use in the Intensive Care Unit, with patients with either an endotracheal or tracheostomy tube, whilst receiving invasive mechanical ventilation or not, and while using either verbal or nonverbal modes of communication. Preliminary establishment of responsiveness, validity, and reliability was made. The tool was acceptable to participants and their family members. CONCLUSION: The clinician-reported and patient-reported components of the study were feasible for use. The CAT has the potential to enable quantifiable comparison of communication interventions for patients with an artificial airway. Future research is required to determine external validity and reliability.

OBJECTIVES: To define effective communication and identify its key elements specific to critically ill patients with an artificial airway. DESIGN: A modified Consensus Development Panel methodology. SETTING: International video-conferences. MAIN OUTCOME MEASURES: Definition of effective communication and it's key elements. RESULTS: Eight experts across four international regions and three professions agreed to form the Consensus Development Panel together with a Chair and one person with lived experience who reviewed the outputs prior to finalisation. "Communication for critically ill adult patients with an artificial airway (endotracheal or tracheostomy tube) is defined as the degree in which a patient can initiate, impart, receive, and understand information, and can range from an ineffective to effective exchange of basic to complex information between the patient and the communication partner(s). Effective communication encompasses seven key elements including: comprehension, quantity, rate, effort, duration, independence, and satisfaction. In critically ill adults, communication is impacted by factors including medical, physical and cognitive status, delirium, fatigue, emotional status, the communication partner and the nature of the ICU environment (e.g., staff wearing personal protective equipment, noisy equipment, bright lights)." The panel agreed that communication occurs on a continuum from ineffective to effective for basic and complex communication. CONCLUSION: We developed a definition and list of key elements which constitute effective communication for critically ill patients with an artificial airway. These can be used as the basis of standard terminology to support future research on the development of communication-related outcome measurement tools in this population. IMPLICATIONS FOR CLINICAL PRACTICE: This study provides international multi-professional consensus terminology and a definition of effective communication which can be used in clinica

BACKGROUND: Subtle progressive changes in speech motor function and cognition begin prior to diagnosis of Huntington's disease (HD). OBJECTIVE: To determine the nature of listener-rated speech differences in premanifest and early-stage HD (i.e., PreHD and EarlyHD), compared to neurologically healthy controls. METHODS: We administered a speech battery to 60 adults (16 people with PreHD, 14 with EarlyHD, and 30 neurologically healthy controls), and conducted a cognitive test of processing speed/visual attention, the Symbol Digit Modalities Test (SDMT) on participants with HD. Voice recordings were rated by expert listeners and analyzed for acoustic and perceptual speech features. RESULTS: Listeners perceived subtle differences in the speech of PreHD compared to controls, including abnormal pitch level and speech rate, reduced loudness and loudness inflection, altered voice quality, hypernasality, imprecise articulation, and reduced naturalness of speech. Listeners detected abnormal speech rate in PreHD compared to healthy speakers on a reading task, which correlated with slower speech rate from acoustic analysis and a lower cognitive performance score. In early-stage HD, continuous speech was characterized by longer pauses, a higher proportion of silence, and slower rate. CONCLUSION: Differences in speech and voice acoustic features are detectable in PreHD by expert listeners and align with some acoustically-derived objective speech measures. Slower speech rate in PreHD suggests altered oral motor control and/or subtle cognitive deficits that begin prior to diagnosis. Speakers with EarlyHD exhibited more silences compared to the PreHD and control groups, raising the likelihood of a link between speech and cognition that is not yet well characterized in HD.

This study evaluated the feasibility and outcomes of a telerehabilitation adaptation of the Be Clear speech treatment program for adults with non-progressive dysarthria to determine clinical delivery viability and future research directions. Treatment effects on speech clarity, intelligibility, communication effectiveness, and participation, as well as psychosocial outcomes in 15 participants with non-progressive dysarthria, were explored. Intervention involved daily 1-h online sessions (4 days per week for 4 weeks, totalling 16 sessions) and daily home practice. Outcome measures were obtained at baseline (PRE), post-treatment (POST), and 12 weeks following treatment (FUP). Feasibility measures targeting participant satisfaction, treatment adherence and fidelity, and technical viability were also employed. The programme was feasible concerning technical viability and implementation, treatment adherence and fidelity. High levels of participant satisfaction were reported. Increases in overall ratings of communication participation and effectiveness were identified at POST and FUP. Reductions in speech rate were identified at FUP. Improvements in aspects of lingual and laryngeal function were also noted after treatment. Over time, improvements relating to the negative impact of dysarthria were identified. Naïve listeners perceived negligible changes in speech clarity following treatment. Online delivery of the Be Clear speech treatment program was feasible, and some positive speech benefits were observed. Due to the small sample size included in this research, statistically significant findings related to speech outcomes must be interpreted with caution. An adequately powered randomised controlled trial of Be Clear online is warranted to evaluate treatment efficacy.

AIM: To examine the phenomenology of stuttering across the lifespan in the largest prospective cohort to date. METHOD: Participants aged 7 years and older with a history of developmental stuttering were recruited. Self-reported phenotypic data were collected online including stuttering symptomatology, co-occurring phenotypes, genetic predisposition, factors associated with stuttering severity, and impact on anxiety, education, and employment. RESULTS: A total of 987 participants (852 adults: 590 males, 262 females, mean age 49 years [SD = 17 years 10 months; range = 18-93 years] and 135 children: 97 males, 38 females, mean age 11 years 4 months [SD = 3 years; range = 7-17 years]) were recruited. Stuttering onset occurred at age 3 to 6 years in 64.0%. Blocking (73.2%) was the most frequent phenotype; 75.9% had sought stuttering therapy and 15.5% identified as having recovered. Half (49.9%) reported a family history. There was a significant negative correlation with age for both stuttering frequency and severity in adults. Most were anxious due to stuttering (90.4%) and perceived stuttering as a barrier to education and employment outcomes (80.7%). INTERPRETATION: The frequent persistence of stuttering and the high proportion with a family history suggest that stuttering is a complex trait that does not often resolve, even with therapy. These data provide new insights into the phenotype and prognosis of stuttering, information that is critically needed to encourage the development of more effective speech therapies. WHAT THIS PAPER ADDS: Half of the study cohort had a family history of stuttering. While 75.9% of participants had sought stuttering therapy, only 15.5% identified as having recovered. There was a significant negative correlation between age and stuttering frequency and severity in adults.

The growing body of information-seeking and decision-making literature in motor neurone disease (MND) has not yet explored the impact of health literacy. Health literacy relates to the skills people have to access, understand, and use health information and is influenced by motivation to engage with healthcare. We aimed to better understand how people affected by MND engage in healthcare by examining longitudinal interview data using the construct of health literacy. Semi-structured interviews were conducted with 19 persons living with MND and 15 carers recruited from a specialist MND clinic using maximum variation sampling. Transcripts were deductively coded using a framework of health literacy behaviours. The analysis used a matrix-based approach for thematic analysis of longitudinal data. People living with MND and carers sought nuanced information dependent on their priorities and attitudes. Information uptake was influenced by perceived relevancy and changed over time. Time allowed opportunity to reflect on and understand the significance of information provided. The findings indicate that persons living with MND and carers benefit when information and consultations are adapted to meet their communication needs. The results highlight the potential benefits of gaining an early understanding of and accommodating the communication needs, personal preferences, and emotional readiness for information for persons living with MND and their carers.

CAG repeat-expansion spinocerebellar ataxias (CAG-SCAs) are genetically defined multisystemic degenerative diseases, resulting in motor symptoms including dysarthria with a substantial impact on daily living. Whilst speech therapy is widely recommended in ataxia, very limited evidence exists for its use. We evaluated the efficacy of a home-delivered, ataxia-tailored biofeedback-driven speech therapy in CAG-SCA in 16 individuals with SCA1, 2, 3, or 6. Treatment was delivered intensively over 20 days. Efficacy was evaluated by blinded ratings of intelligibility (primary) and acoustic measures (secondary) leveraging an intra-individual control design. Intelligibility improved post-treatment (Z = -3.18, p = 0.004) whilst remaining stable prior to treatment (Z = 0.53, p = 1.00).

BACKGROUND: Communication and cognitive impairments are known barriers to shared decision-making. Most people diagnosed with motor neurone disease (MND) will develop a motor speech impairment over the disease course. Some will develop cognitive, linguistic or behavioural disturbance. Despite this, the impact of communication and cognitive impairment on personal healthcare decision-making in MND is not well known. AIMS: This exploratory, longitudinal study aimed to capture the perspectives of people living with MND (plwMND) and family members on managing their healthcare with, or in anticipation of, a communication impairment. METHODS & PROCEDURES: Semi-structured interviews and functional assessments were conducted with plwMND and family members over one to three time points between December 2017 and January 2020. Participants were recruited from a specialist MND clinic using a maximum variation sampling approach. Interview transcripts were analysed using trajectory data analysis: a matrix-based approach for thematic analysis of longitudinal data. The study was underpinned by interpretive descriptive methodology. OUTCOMES & RESULTS: A total of 19 plwMND with a range of MND phenotypes and 15 family members were recruited. Disease progression and participant withdrawal resulted in attrition, however 12 plwMND and seven family members participated at all three time points. Consistent cognitive screening was not feasible, which limited the opportunity to explore the impact of cognitive change. An overarching theme 'Communicating takes effort' was identified and illustrates the efforts required to compensate for, or circumnavigate, impairments to maintain involvement in healthcare. Assistance from family and accommodation from healthcare professionals (HCPs) was needed for ongoing engagement. Where plwMND were dependent on alternative communication devices, this assistance was essential and primarily carried out by family members. Despite these efforts, the quality, quan

Automatic speech recognition (ASR) could potentially improve communication by providing transcriptions of speech in real time. ASR is particularly useful for people with progressive disorders that lead to reduced speech intelligibility or difficulties performing motor tasks. ASR services are usually trained on healthy speech and may not be optimized for impaired speech, creating a barrier for accessing augmented assistance devices. We tested the performance of three state-of-the-art ASR platforms on two groups of people with neurodegenerative disease and healthy controls. We further examined individual differences that may explain errors in ASR services within groups, such as age and sex. Speakers were recorded while reading a standard text. Speech was elicited from individuals with multiple sclerosis, Friedreich’s ataxia, and healthy controls. Recordings were manually transcribed and compared to ASR transcriptions using Amazon Web Services, Google Cloud, and IBM Watson. Accuracy was measured as the proportion of words that were correctly classified. ASR accuracy was higher for controls than clinical groups, and higher for multiple sclerosis compared to Friedreich’s ataxia for all ASR services. Amazon Web Services and Google Cloud yielded higher accuracy than IBM Watson. ASR accuracy decreased with increased disease duration. Age and sex did not significantly affect ASR accuracy. ASR faces challenges for people with neuromuscular disorders. Until improvements are made in recognizing less intelligible speech, the true value of ASR for people requiring augmented assistance devices and alternative communication remains unrealized. We suggest potential methods to improve ASR for those with impaired speech.

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.

INTRODUCTION: Parkinson's disease can be associated with speech deterioration and low communication confidence which in turn compromises social interaction. Therapeutic singing is an engaging method for combatting speech decline; however, face-to-face delivery can limit access to group singing. The aim of this study is to test the feasibility and acceptability of an online mode of delivery for a Parkinson's singing intervention (ParkinSong) as well as remote data collection procedures. METHODS AND ANALYSIS: This ParkinSong Online feasibility trial is a single-arm, pre-post study of online singing delivery and remote data collection for 30 people living with Parkinson's. The primary outcome measure is feasibility: recruitment, retention, attendance, safety, intervention fidelity, acceptability and associated costs. Secondary outcomes are speech (loudness, intelligibility, quality, communication-related quality of life) and wellbeing (apathy, depression, anxiety, stress, health-related quality of life). This mode of delivery aims to increase the accessibility of singing interventions. ETHICS AND DISSEMINATION: Ethics approval was obtained from The University of Melbourne Human Research Ethics Committee (2021-14465-16053-3) and the trial has been prospectively registered. Results will be presented at national and international conferences, published in a peer-reviewed journal, and disseminated to the Parkinson's community, researchers and policymakers. TRIAL REGISTRATION NUMBER: ACTRN12621000940875.

OBJECTIVE: To determine whether objective and quantitative assessment of dysarthria and dysphagia in spinocerebellar ataxia type 2 (SCA2), specifically at pre-ataxic and early disease phases, can act as sensitive disease markers. METHODS: Forty-six individuals (16 with pre-ataxic SCA2, 14 with early-stage ataxic SCA2, and 16 healthy controls) were recruited in Holguin, Cuba. All participants underwent a comprehensive battery of assessments including objective acoustic analysis, clinician-derived ratings of speech function and swallowing, and quality of life assessments of swallowing. RESULTS: Reduced speech agility manifest at the pre-ataxic stage was observed during diadochokinetic tasks, with the magnitude of speech deficit augmented in the early ataxic stage. Speech rate was slower in early-stage ataxic SCA2 compared with pre-ataxic SCA2 and healthy controls. Reduced speech agility and speech rate correlated with disease severity and time to ataxia onset, verifying that speech deficits occur prior to ataxia onset and increase in severity as the disease progresses. Whereas dysphagia was observed in both pre-ataxic and ataxic SCA2, it was not associated with swallowing-related quality of life, disease severity, or time to ataxia onset. CONCLUSIONS: Speech and swallowing deficits appear sensitive to disease progression in early-stage SCA2, with syllabic rate a viable marker. Findings provide insight into mechanisms of disease progression in early-stage SCA2, signaling an opportunity for stratifying early-stage SCA2 and identifying salient markers of disease onset as well as outcome measures in future early-stage therapeutic studies.

Wearing face masks (alongside physical distancing) provides some protection against infection from COVID-19. Face masks can also change how people communicate and subsequently affect speech signal quality. This study investigated how three common face mask types (N95, surgical, and cloth) affected acoustic analysis of speech and perceived intelligibility in healthy subjects. Acoustic measures of timing, frequency, perturbation, and power spectral density were measured. Speech intelligibility and word and sentence accuracy were also examined using the Assessment of Intelligibility of Dysarthric Speech. Mask type impacted the power distribution in frequencies above 3 kHz for the N95 mask, and above 5 kHz in surgical and cloth masks. Measures of timing and spectral tilt mainly differed with N95 mask use. Cepstral and harmonics to noise ratios remained unchanged across mask type. No differences were observed across conditions for word or sentence intelligibility measures; however, accuracy of word and sentence translations were affected by all masks. Data presented in this study show that face masks change the speech signal, but some specific acoustic features remain largely unaffected (e.g., measures of voice quality) irrespective of mask type. Outcomes have bearing on how future speech studies are run when personal protective equipment is worn.

OBJECTIVES: Cognitive and other biases can influence the quality of healthcare decision making. While substantial research has explored how biases can lead to diagnostic or other errors in medicine, fewer studies have examined how they impact the decision making of other healthcare professionals. This scoping review aimed to identify and synthesise a broad range of research investigating whether decisions made by allied health professionals are influenced by cognitive, affective or other biases. MATERIALS AND METHODS: A systematic literature search was conducted in five electronic databases. Title, abstract and full text screening was undertaken in duplicate, using prespecified eligibility criteria designed to identify studies attempting to demonstrate the presence of bias when allied healthcare professionals make decisions. A narrative synthesis was undertaken, focussing on the type of allied health profession, type of decision, and type of bias reported within the included studies. RESULTS: The search strategy identified 149 studies. Of these, 119 studies came from the field of psychology, with substantially fewer from social work, physical and occupational therapy, speech pathology, audiology and genetic counselling. Diagnostic and assessment decisions were the most common decision types, with fewer studies assessing treatment, prognostic or other clinical decisions. Studies investigated the presence of over 30 cognitive, affective and other decision making biases, including stereotyping biases, anchoring, and confirmation bias. Overall, 77% of the studies reported at least one outcome that represented the presence of a bias. CONCLUSION: This scoping review provides an overview of studies investigating whether decisions made by allied health professionals are influenced by cognitive, affective or other biases. Biases have the potential to seriously impact the quality, consistency and accuracy of decision making in allied health practice. The findings highlight a

Background: Parkinson’s disease (PD) frequently causes progressive deterioration in speech, voice and cognitive aspects of communication. These affect wellbeing and quality of life and are associated with caregiver strain and burden. Therapeutic singing groups can ameliorate PD-related communication disorders and increase social interaction and wellbeing for caregivers and care recipients. Objective: To analyse the effects of ParkinSong group singing sessions on Parkinson’s communication and wellbeing outcomes for people with PD and caregivers over 12 months. Methods: A 4-armed controlled clinical trial compared ParkinSong with active non-singing control conditions over 12 months. Two dosage levels (weekly versus monthly) were available for each condition. ParkinSong comprised high-effort vocal, respiratory and speech exercises, group singing, and social interaction. PD-specific outcomes included vocal loudness, speech intelligibility, maximum phonation time, respiratory muscle strength, and voice related quality of life (QoL). Wellbeing outcomes were also measured for caregivers and care recipients. Results: We recruited 75 people with PD and 44 caregivers who attended weekly ParkinSong, monthly ParkinSong, weekly control or monthly control groups. We found significant improvements in the primary outcome of vocal loudness (p = 0.032), with weekly singers 5.13 dB louder (p = 0.044) and monthly singers 5.69 dB louder (p = 0.015) than monthly controls at 12 months. ParkinSong participants also showed greater improvements in voice-related QoL and anxiety. Caregivers who attended ParkinSong showed greater reductions in depression and stress scores. Conclusions: This 12-month controlled clinical trial of ParkinSong demonstrated improvements in speech loudness and voice-related QoL for participants with PD, and enhanced wellbeing for both caregivers and care recipients. No adverse effects were reported over 12 months and improvements were sustained.

Purpose Many industrialised nations have systems of injury compensation and rehabilitation that are designed to support injury recovery and return to work. Despite their intention, there is now substantial evidence that injured people, employers and healthcare providers can experience those systems as difficult to navigate, and that this can affect injury recovery. This study sought to characterise the relationships and interactions occurring between actors in three Australian injury compensation systems, to identify the range of factors that impact on injury recovery, and the interactions and inter-relationships between these factors. Methods This study uses data collected directly from injured workers and their family members via qualitative interviews, analysed for major themes and interactions between themes, and then mapped to a system level model. Results Multiple factors across multiple system levels were reported by participants as influencing injury recovery. Factors at the level of the injured person's immediate environment, the organisations and personnel involved in rehabilitation and compensation processes were more commonly cited than governmental or societal factors as influencing physical function, psychological function and work participation. Conclusions The study demonstrates that injury recovery is a complex process influenced by the decisions and actions of organisations and individuals operating across multiple levels of the compensation system. Changes occurring 'upstream', for instance at the level of governmental or organisational policy, can impact injury recovery through both direct and diffuse pathways.

Examination of rodent vocalizations in experimental conditions can yield valuable insights into how disease manifests and progresses over time. It can also be used as an index of social interest, motivation, emotional development or motor function depending on the animal model under investigation. Most mouse communication is produced in ultrasonic frequencies beyond human hearing. These ultrasonic vocalizations (USV) are typically described and evaluated using expert defined classification of the spectrographic appearance or simplistic acoustic metrics resulting in nine call types. In this study, we aimed to replicate the standard expert-defined call types of communicative vocal behavior in mice by using acoustic analysis to characterize USVs and a principled supervised learning setup. We used four feature selection algorithms to select parsimonious subsets with maximum predictive accuracy, which are then presented into support vector machines (SVM) and random forests (RF). We assessed the resulting models using 10-fold cross-validation with 100 repetitions for statistical confidence and found that a parsimonious subset of 8 acoustic measures presented to RF led to 85% correct out-of-sample classification, replicating the experts' labels. Acoustic measures can be used by labs to describe USVs and compare data between groups, and provide insight into vocal-behavioral patterns of mice by automating the process on matching the experts' call types.

BACKGROUND: Tremor is a debilitating symptom of Multiple Sclerosis (MS). Little is known about its pathophysiology and treatments are limited. Clinical trials investigating new interventions often rely on subjective clinical rating scales to provide supporting evidence of efficacy. NEW METHOD: We present a novel instrument (TREMBAL) which uses electromagnetic motion capture technology to quantify MS tremor. We aim to validate TREMBAL by comparison to clinical ratings using regression modelling with 310 samples of tremor captured from 13 MS participants who performed five different hand exercises during several follow-up visits. Minimum detectable change (MDC) and test-retest reliability were calculated and comparisons were made between MS tremor and data from 12 healthy volunteers. RESULTS: Velocity of the index finger was most congruent with clinical observation. Regression modelling combining different features, sensor configurations, and labelling exercises did not improve results. TREMBAL MDC was 84% of its initial measurement compared to 91% for the clinical rating. Intra-class correlations for test-retest reliability were 0.781 for TREMBAL and 0.703 for clinical ratings. Tremor was lower (p = 0.002) in healthy subjects. COMPARISON WITH EXISTING METHODS: Subjective scales have low sensitivity, suffer from ceiling effects, and mitigation against inter-rater variability is challenging. Inertial sensors are ubiquitous, however, their output is nonlinearly related to tremor frequency, compensation is required for gravitational artefacts, and their raw data cannot be intuitively comprehended. CONCLUSIONS: TREMBAL, compared with clinical ratings, gave measures in agreement with clinical observation, had marginally lower MDC, and similar test-retest reliability.

Introduction: Idiopathic Parkinson's Disease (PD) results in a range of motor and non-motor impairments. Clinical diagnosis commonly occurs after substantial neurophysiological damage limiting the opportunity for neuroprotective treatments. Uncovering sensitive objective markers with the capacity to detect pre-symptomatic disease and track disease progression is therefore a priority. Speech may provide an ideal proxy marker for PD; a quantifiable biometric that displays salient changes in early disease and appears to evolve with disease progression.Areas covered: This review describes the endophenotype of speech, voice, cognition and language modalities in PD and investigates the speech as a 'proxy marker' of PD disease state.Expert opinion: Detailed characterization at different disease stages are needed and must incorporate longitudinal assessment to capture small but significant changes in speech, voice, cognition and language modalities within patient changes over time. Advances in technology are leading to new opportunities for acquiring data remotely and more frequently, offering more ecologically valid testing environments. Combined with automated signal processing and analysis, symptoms may also be tracked in-home readily. Features extracted may provide a 'proxy marker' for early identification of PD and objective monitoring of disease progression.

RATIONALE: Motor neurone disease (MND) is a neurodegenerative disease presenting with progressive weakness of voluntary muscles. For any condition, person-centred health care relies on the sharing of information and a mutual understanding of the person's needs and preferences. Decision making in MND becomes more complex as there is no cure and a high prevalence of co-morbid communication and/or cognitive difficulties. OBJECTIVE: To identify the reported impact of communication and/or cognitive impairment on patient and carer involvement in health care decision making in MND. METHODS: A review and synthesis of studies addressing issues of communication impairment and/or cognitive impairment in relation to decision making focussed on MND was conducted. Articles were excluded if they were reviews, case studies, conference papers, or commentaries. To be included studies needed to address issues of communication impairment or cognitive impairment specifically in relation to decision making. Relevant data were extracted verbatim and subjected to content analysis to support the narrative summary. RESULTS: Seventy-six articles were identified, and 35 articles screened. Six articles met inclusion criteria each describing examples of decision making in MND. There was limited data related to communication and/or cognitive impairment, and the impact these impairments may have on decision making despite recognition that many people with MND may lose verbal communication or develop subtle cognitive impairments. The literature is primarily from the perspective of others. CONCLUSION: This review highlights that the current body of literature exploring decision making within the MND population presents us with extremely limited insights into the impact of communication and/or cognitive impairments on health care decision making. Extant literature focuses on interventions (namely, ventilation and gastrostomy), the broad process of decision making, or cognitive assessment of decision

Background. Communication impairment is one of the most common symptoms of Parkinson's disease (PD), significantly affecting quality of life. Singing shares many of the neural networks and structural mechanisms used during speech and, thus, has potential for therapeutic application to address speech disorders. Objective. To explore the effects of an interdisciplinary singing-based therapeutic intervention (ParkinSong) on voice and communication in people with PD. Methods. A controlled trial compared the effects of the ParkinSong intervention with an active control condition at 2 dosage levels (weekly vs monthly) over 3 months, on voice, speech, respiratory strength, and voice-related quality-of-life outcomes for 75 people living with PD. The interdisciplinary ParkinSong model comprised high-effort vocal and respiratory tasks, speech exercises, group singing, and social communication opportunities. Results. ParkinSong intervention participants demonstrated significant improvements in vocal intensity (P = .018), maximum expiratory pressure (P = .032), and voice-related quality of life (P = .043) in comparison to controls. Weekly ParkinSong participants increased vocal intensity more than monthly participants (P = .011). Vocal intensity declined in nontreatment control groups. No statistical differences between groups on maximum phonation length or maximum inspiratory pressure were observed at 3 months. Conclusions. ParkinSong is an engaging intervention with the potential to increase loudness and respiratory function in people with mild to moderately severe PD.

Introduction: Tremor of the upper limbs is a disabling symptom that is present during several neurological disorders and is currently without treatment. Functional MRI (fMRI) is an essential tool to investigate the pathophysiology of tremor and aid the development of treatment options. However, no adequately or standardized protocols for fMRI exists at present. Here we present a novel, online available fMRI task that could be used to assess the in vivo pathology of tremor. Objective: This study aims to validate the tremor-evoking potential of the fMRI task in a small group of tremor patients outside the scanner and assess the reproducibility of the fMRI task related activation in healthy controls. Methods: Twelve HCs were scanned at two time points (baseline and after 6-weeks). There were two runs of multi-band fMRI and the tasks included a "brick-breaker" joystick game. The game consisted of three conditions designed to control for most of the activation related to performing the task by contrasting the conditions: WATCH (look at the game without moving joystick), MOVE (rhythmic left/right movement of joystick without game), and PLAY (playing the game). Task fMRI was analyzed using FSL FEAT to determine clusters of activation during the different conditions. Maximum activation within the clusters was used to assess the ability to control for task related activation and reproducibility. Four tremor patients have been included to test ecological and construct validity of the joystick task by assessing tremor frequencies captured by the joystick. Results: In HCs the game activated areas corresponding to motor, attention and visual areas. Most areas of activation by our game showed moderate to good reproducibility (intraclass correlation coefficient (ICC) 0.531-0.906) with only inferior parietal lobe activation showing poor reproducibility (ICC 0.446). Furthermore, the joystick captured significantly more tremulous movement in tremor patients compared to HCs (p = 0.01)

BACKGROUND: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a rare early onset neurodegenerative disease that typically results in ataxia, upper motor neuron dysfunction and sensorimotor peripheral neuropathy. Dysarthria and dysphagia are anecdotally described as key features of ARSACS but the nature, severity and impact of these deficits in ARSACS are not known. A comprehensive quantitative and qualitative characterization of speech and swallowing function will support diagnostics, provide insights into the underlying pathology, and guide day-to-day clinical management. METHODS: 11 consecutive non-Quebec ARSACS patients were recruited, and compared to healthy participants from several published and unpublished cohorts. A comprehensive behavioural assessment including objective acoustic analysis and expert perceptual ratings of motor speech, the Clinical Assessment of Dysphagia in Neurodegeneration (CADN), videofluoroscopy and standardized tests of dysarthria and swallowing related quality of life was conducted. RESULTS: Speech in this ARSACS cohort is characterized by pitch breaks, prosodic deficits including reduced rate and prolonged intervals, and articulatory deficits. The swallowing profile was characterized by delayed initiation of the swallowing reflex and late epiglottic closure. Four out of ten patients were observed aspirating thin liquids on videofluoroscopy. Patients report that they regularly cough or choke on thin liquids and solids during mealtimes. Swallowing and speech-related quality of life was worse than healthy controls on all domains except sleep. CONCLUSIONS: The dysphagia and dysarthria profile of this ARSACS cohort reflects impaired coordination and timing. Dysphagia contributes to a significant impairment in functional quality of life in ARSACS, and appears to manifest distinctly from other ARSACS dysfunctions such as ataxia or spasticity.

IMPORTANCE: Multiple sclerosis produces neurological impairments that are variable in duration, severity and quality. Speech is frequently impaired, resulting in decreased communication skills and quality of life. Advancements in technology now makes it possible to use quantitative acoustic assessment of speech as biomarkers of disease progression. OBSERVATIONS: Four domains of speech have been identified: articulation (slow articulation and imprecise consonants), voice (pitch and loudness instability), respiration (decreased phonatory time and expiratory pressure) and prosody (longer and frequent pauses, deficient loudness control). Studies also explored I) predictive models for diagnosis of MS and of ataxia using speech variables, II) the relationship of dysarthria with cognition and III) very few studies correlated neuroimaging with dysarthria. We could not identify longitudinal studies of speech or dysarthria in Multiple Sclerosis. CONCLUSION AND RELEVANCE: Refinement of objective measures of speech has enhanced our understanding of Multiple Sclerosis-related deficits in cross-sectional analysis while both integrative and longitudinal studies are identified as major gaps. This review highlights the potential for using quantitative acoustic assessments as clinical endpoints for diagnosing, monitoring progression and treatment in disease modifying trials.

PURPOSE: The purpose of this study is to assess Cantonese word recognition and the discrimination of Cantonese tones with manipulated contours by child and adolescent cochlear implant (CI) users and a group of peers with normal hearing (NH). It was hypothesized that the CI users would perform more poorly than their counterparts with NH in both tasks and that CI users implanted before 2 years of age would perform better than those implanted after 2 years. METHOD: Forty-one participants were recruited from hospitals, schools, and kindergartens in Hong Kong: Ten CI users implanted at or before 2 years of age ("early" CI group), 13 CI users implanted after 2 years of age ("late" CI group), and 18 individuals with NH. The mean age at implantation of the early CI group was 1.5 years (SD = 0.3), and for the late CI group, it was 4.3 years (SD = 2.1). Participants were a mean of 13.3 years of age (SD = 3.7) at time of testing. Participants completed a Cantonese word recognition test and a discrimination task using Cantonese tones with modified fundamental frequency trajectories. RESULTS: Both CI user groups obtained significantly lower scores than the group with NH on the word recognition test. Mean percent correct scores for the word recognition test were 79% for the early CI group, 69% for the late CI group, and 97% for the group with NH. The group with NH consistently achieved higher scores than the CI user groups when discriminating manipulated Cantonese tones. Increasing the acoustic difference between tones improved discrimination performance for CI users for level tone contrasts only. CI users implanted at or before 2 years of age obtained higher scores than those implanted later. CONCLUSIONS: The results of this study add further evidence that children using CIs do not perform as well as peers in perceiving Cantonese tones. Modification of tones to increase pitch range did not consistently improve the ability of children with implants to perceive the difference betw

BACKGROUND: Frontotemporal lobar degeneration (FTLD) can result in a decline in behavior, language, and motor function. Mealtime disturbances are a common and significant outcome of FTLD. Disturbances during mealtimes can arise from dysphagia or may occur secondary to behavioral changes such as rapid eating, mealtime rigidity, and altered diet preferences. SUMMARY: Few studies have comprehensively evaluated eating behavior or dysphagia in individuals presenting with FTLD pathology despite the potential impact on medical safety and individual quality of life. Dysphagia is reported in the late stages of frontotemporal dementia and early in the motor subtypes of FTLD. The identification of dysphagia can alert individuals and medical teams to disease progression and provide insight into the nature and spread of the underlying neuropathology. Improved understanding of eating behaviors can improve individual care and may enhance diagnostic accuracy. Key Message: Aberrant eating behavior and swallowing difficulties are reported in the conditions associated with FTLD neuropathology. The consequences of mealtime disturbances include health risks associated with an increased BMI and aspiration, reduction of an individual's independence, and an increase in caregiver stress and burden. Here we review and summarize the literature on eating behavior and swallow impairments (dysphagia) in each of the syndromes caused by FTLD.

Neurologic practice is deeply rooted in the ideal of localization. Some would argue that neurology arose in the mid-18th century from the study of language localization, regarded as a uniquely human function. The classic aphasias, those described by Broca and Wernicke, were believed to represent exemplars of lesion-symptom mapping, but many features of language-onset dementias went unexplained by conventional aphasiology.² The description of aphasia syndromes in neurodegenerative diseases has largely overturned the static conceptualization of a brain language postcode.³ While incremental and cumulative, these neural network–based conceptual changes have proven critical for elucidating the neural basis of communication, both in healthy speakers and those with neurologic disease. Greater understanding of the interconnectedness and functionality of language regions remains particularly important clinically, such as for decision-making around epilepsy or oncology surgery, and can assist in providing prognostic information for affected individuals and their families.

BACKGROUND: Mutations in the nuclear-encoded mitochondrial DNA polymerase gamma (POLG) can result in a wide spectrum of neurological deficits. A common presentation is progressive ataxia (POLG-A) which includes impaired speech and swallowing. The nature, severity and impact of these deficits in POLG-A is not known. A comprehensive quantitative and qualitative characterization of dysarthria and dysphagia in this recurrent ataxia disorder will assist in diagnostics, provide insights into the underlying pathology, and establish the foundation for future therapy trials. METHODS: 14 consecutive patients with POLG (9 females, mean age=50.1y, SD=11.2) and 34 healthy controls were enrolled. Comprehensive assessments of motor speech and swallowing function, acoustic analysis of speech, videofluoroscopy and measures of quality of life were conducted. RESULTS: The speech profile of individuals with POLG-A was characterized by poor control of pitch and strain-strangled voice quality, reduced rate of speech and longer variable silences between words, and articulatory breakdown including imprecise consonants and vowel distortions. Swallowing deficits included slower initiation of the swallow reflex, poor control of bolus and late epiglottic closure. Speech and swallowing related quality of life was worse than healthy controls. CONCLUSIONS: The dysarthria and dysphagia profiles in POLG-A are largely symptomatic of impaired timing, indicating a mainly spinocerebellar deficit. Dysarthria and dysphagia contribute to a significant impairment in functional quality of life, and progress distinctly from other POLG-A dysfunctions like ataxia or cognitive impairment. Our assessments establish meaningful patient focused outcome measures that will be suitable for use in natural history studies and clinical trials.

Background: Dysarthria is highly prevalent in Multiple Sclerosis. The relationship between dysarthria, MS disease severity and other cerebellar manifestations (such as tremor) is poorly understood. Objective: To examine the relationship between objective markers of speech, disease severity and upper limb tremor in relapsing-remitting and secondary progressive Multiple Sclerosis. Design Methods: An experienced neurologist determined A) the presence of upper limb tremor, B) the Expanded Disability Status Scale (EDSS) score and C) the degree of dysarthria (from 0, no disturbance to 4, unintelligible). Through acoustic analysis of speech, we investigated: 1) stability of vocal pitch, in sustained utterance of the vowel /a/; 2) stability of loudness, in the same sustained vowel; 3) diadochokinetic speed, in fast repetition of the meaningless word /pa/ta/ka/ and 4) maximum speed of vocal tract movement (i.e. change in pharynx and mouth cavity shape), measured in the word “always” (from a standard reading passage). After adjustment for multiple comparisons, p<0.0125 was considered for statistical significance. Results: We assessed 24 participants with Multiple Sclerosis and upper limb tremor (47.2±12.3years, 75% female, EDSS=3.7±1.6) and 24 matched patients with Multiple Sclerosis without tremor (51.2±10.7years, 75% female, EDSS=3.6±1.7). Clinical dysarthria scores (median=0, mean=0.375±0.76) correlated with all acoustic variables measured: diadochokinetic speed Spearman’s rho=.561 (p<.001); pitch stability rho=.37 (p=.011); loudness stability rho=.37 (p=.01); and maximum speed of vocal tract movement rho=.363 (p=.012). Diadochokinetic speed strongly correlated with EDSS (rho=.529, p<.001). Speed of vocal tract movement correlated with tremor and differentiated tremor and non-tremor groups (2-tailed t-test p=0.002, rho=.418). Conclusions: In a typically non-to-mildly dysarthric cohort, acoustic speech measurements correlate with disease severity and can differentiate overt

The acoustic properties of speech are a reliable marker of changes in the central nervous system. Measures of speech timing and frequency have been used to assess impairment in clinical patient groups, including stroke, Huntington’s disease, and depression. Associations between speech properties and impaired performance have also been described in healthy individuals under periods of sleep deprivation. In the current study, we aimed to compare the time course of speech outcomes to other objective measures of alertness during 40 hours of sleep deprivation. Methods Twenty-three healthy volunteers (18 males; mean age=25.41 ± 5.73 years) underwent 40 hours of acute sleep deprivation under constant routine conditions. Speech tasks were administered every four hours, beginning three hours after scheduled waketime. The Psychomotor Vigilance Test (PVT), Karolinska Sleepiness Scale (KSS), and EEG collected during the Karolinksa Drowsiness Test (KDT) were measured bi-hourly. Objective measures of speech were derived using spectral, cepstral and timing analyses. Results Acoustic parameters displayed impairment across 40-h of sustained wakefulness. Measures of speech timing (e.g. speech rate) and frequency deteriorated significantly following 23 hours awake compared to baseline (first 16 hours awake). Impairment peaked around 31 hours of wakefulness before showing slight improvements. These findings were consistent with laboratory standard assessments of sleepiness and vigilant attention. Conclusion The time course of changes in the acoustic properties of speech are comparable to those changes observed in alertness and performance impairment across 40 h of sleep deprivation. These findings suggest the acoustic properties of speech may be a reliable indicator of alertness/drowsiness, with utility for development of objective and non-invasive systems to identify and manage sleep-related impairment in workplace settings. Support (If Any) The study was supported by the Cooperative

PURPOSE: Our purpose was to create a comprehensive review of speech impairment in frontotemporal dementia (FTD), primary progressive aphasia (PPA), and progressive apraxia of speech in order to identify the most effective measures for diagnosis and monitoring, and to elucidate associations between speech and neuroimaging. METHOD: Speech and neuroimaging data described in studies of FTD and PPA were systematically reviewed. A meta-analysis was conducted for speech measures that were used consistently in multiple studies. RESULTS: The methods and nomenclature used to describe speech in these disorders varied between studies. Our meta-analysis identified 3 speech measures which differentiate variants or healthy control-group participants (e.g., nonfluent and logopenic variants of PPA from all other groups, behavioral-variant FTD from a control group). Deficits within the frontal-lobe speech networks are linked to motor speech profiles of the nonfluent variant of PPA and progressive apraxia of speech. Motor speech impairment is rarely reported in semantic and logopenic variants of PPA. Limited data are available on motor speech impairment in the behavioral variant of FTD. CONCLUSIONS: Our review identified several measures of speech which may assist with diagnosis and classification, and consolidated the brain-behavior associations relating to speech in FTD, PPA, and progressive apraxia of speech.

BACKGROUND: Despite evidence that cannabinoid receptors are located in movement-related brain regions (e.g., basal ganglia, cerebral cortex, and cerebellum), and that chronic cannabis use is associated with structural and functional brain changes, little is known about the long-term effect of cannabis use on human movement. The aim of the current study was to investigate balance and walking gait in adults with a history of cannabis use. We hypothesised that cannabis use is associated with subtle changes in gait and balance that are insufficient in magnitude for detection in a clinical setting. METHODS: Cannabis users (n=22, 24±6years) and non-drug using controls (n=22, 25±8years) completed screening tests, a gait and balance test (with a motion capture system and in-built force platforms), and a clinical neurological examination of movement. RESULTS: Compared to controls, cannabis users exhibited significantly greater peak angular velocity of the knee (396±30 versus 426±50°/second, P=0.039), greater peak elbow flexion (53±12 versus 57±7°, P=0.038) and elbow range of motion (33±13 versus 36±10°, P=0.044), and reduced shoulder flexion (41±19 versus 26±16°, P=0.007) during walking gait. However, balance and neurological parameters did not significantly differ between the groups. CONCLUSIONS: The results suggest that history of cannabis use is associated with long-lasting changes in open-chain elements of walking gait, but the magnitude of change is not clinically detectable. Further research is required to investigate if the subtle gait changes observed in this population become more apparent with aging and increased cannabis use.

OBJECTIVES: The ability to recognize tones is vital for speech perception in tonal languages. Cantonese has six tones, which are differentiated almost exclusively by pitch cues (tones 1 to 6). The differences in pitch contours among the tones are subtle, making Cantonese a challenging language for cochlear implant users. The addition of a hearing aid has been shown to improve speech perception in nontonal languages and in Mandarin Chinese. This study (1) investigates the Cantonese tone perception ability of children who use a cochlear implant and a hearing aid in opposite ears; (2) evaluates the effect of varying pitch height and pitch contour cues on Cantonese tone perception for these children; and (3) compares the Cantonese tone perception ability for using a hearing aid and a cochlear implant together versus an implant alone. DESIGN: Eight native Cantonese speaking children using a cochlear implant and a hearing aid in opposite ears were assessed for tone perception and word identification. The tone perception test involved discriminating and ranking tone pairs from natural and artificially manipulated Cantonese tones with various pitch heights and/or pitch contours. The word identification test involved identifying Cantonese words in a four-alternative forced-choice task. All tests were performed in two device conditions: (1) cochlear implant and hearing aid together and (2) implant alone. RESULTS: Seven of the 8 subjects performed significantly above chance in both tests using the cochlear implant alone. Results showed that both pitch height and/or pitch direction were important perceptual cues for implant users. Perception for some tones was improved by increasing the pitch height differences between the tones. The ability to discriminate and rank the tone 2/tone 5 contrast and the tone 4/tone 6 contrast was poor, as the tones in these contrasts are similar in pitch contours and onset frequencies. No significant improvement was observed after artificially inc

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.

Screening assessments for dysphagia are essential in neurodegenerative disease. Yet there are no purpose-built tools to quantify swallowing deficits at bedside or in clinical trials. A quantifiable, brief, easy to administer assessment that measures the impact of dysphagia and predicts the presence or absence of aspiration is needed. The Clinical Assessment of Dysphagia in Neurodegeneration (CADN) was designed by a multidisciplinary team (neurology, neuropsychology, speech pathology) validated against strict methodological criteria in two neurodegenerative diseases, Parkinson's disease (PD) and degenerative ataxia (DA). CADN comprises two parts, an anamnesis (part one) and consumption (part two). Two-thirds of patients were assessed using reference tests, the SWAL-QOL symptoms subscale (part one) and videofluoroscopic assessment of swallowing (part two). CADN has 11 items and can be administered and scored in an average of 7 min. Test-retest reliability was established using correlation and Bland-Altman plots. 125 patients with a neurodegenerative disease were recruited; 60 PD and 65 DA. Validity was established using ROC graphs and correlations. CADN has sensitivity of 79 and 84% and specificity 71 and 69% for parts one and two, respectively. Significant correlations with disease severity were also observed (p < 0.001) for PD with small to large associations between disease severity and CADN scores for DA. Cutoff scores were identified that signal the presence of clinically meaningful dysphagia symptomatology and risk of aspiration. The CADN is a reliable, valid, brief, quantifiable, and easily deployed assessment of swallowing in neurodegenerative disease. It is thus ideally suited for both clinical bedside assessment and future multicentre clinical trials in neurodegenerative disease.

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.

OBJECTIVE: To provide a comprehensive description of motor speech function in behavioral variant frontotemporal dementia (bvFTD). METHODS: Forty-eight individuals (24 bvFTD and 24 age- and sex-matched healthy controls) provided speech samples. These varied in complexity and thus cognitive demand. Their language was assessed using the Progressive Aphasia Language Scale and verbal fluency tasks. Speech was analyzed perceptually to describe the nature of deficits and acoustically to quantify differences between patients with bvFTD and healthy controls. Cortical thickness and subcortical volume derived from MRI scans were correlated with speech outcomes in patients with bvFTD. RESULTS: Speech of affected individuals was significantly different from that of healthy controls. The speech signature of patients with bvFTD is characterized by a reduced rate (75%) and accuracy (65%) on alternating syllable production tasks, and prosodic deficits including reduced speech rate (45%), prolonged intervals (54%), and use of short phrases (41%). Groups differed on acoustic measures derived from the reading, unprepared monologue, and diadochokinetic tasks but not the days of the week or sustained vowel tasks. Variability of silence length was associated with cortical thickness of the inferior frontal gyrus and insula and speech rate with the precentral gyrus. CONCLUSIONS: One in 8 patients presented with moderate speech timing deficits with a further two-thirds rated as mild or subclinical. Subtle but measurable deficits in prosody are common in bvFTD and should be considered during disease management. Language function correlated with speech timing measures derived from the unprepared monologue only.

Diagnosis of the speech motor planning/programming disorder, apraxia of speech (AOS), has proven challenging, largely due to its common co-occurrence with the language-based impairment of aphasia. Currently, diagnosis is based on perceptually identifying and rating the severity of several speech features. It is not known whether all, or a subset of the features, are required for a positive diagnosis. The purpose of this study was to assess predictor variables for the presence of AOS after left-hemisphere stroke, with the goal of increasing diagnostic objectivity and efficiency. This population-based case-control study involved a sample of 72 cases, using the outcome measure of expert judgment on presence of AOS and including a large number of independently collected candidate predictors representing behavioral measures of linguistic, cognitive, nonspeech oral motor, and speech motor ability. We constructed a predictive model using multiple imputation to deal with missing data; the Least Absolute Shrinkage and Selection Operator (Lasso) technique for variable selection to define the most relevant predictors, and bootstrapping to check the model stability and quantify the optimism of the developed model. Two measures were sufficient to distinguish between participants with AOS plus aphasia and those with aphasia alone, (1) a measure of speech errors with words of increasing length and (2) a measure of relative vowel duration in three-syllable words with weak-strong stress pattern (e.g., banana, potato). The model has high discriminative ability to distinguish between cases with and without AOS (c-index=0.93) and good agreement between observed and predicted probabilities (calibration slope=0.94). Some caution is warranted, given the relatively small sample specific to left-hemisphere stroke, and the limitations of imputing missing data. These two speech measures are straightforward to collect and analyse, facilitating use in research and clinical settings.