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Showing papers on "Dysarthria published in 2015"


Journal ArticleDOI
TL;DR: The articulatory and phonatory predictors are sensitive indicators of early bulbar decline due to ALS, which has implications for predicting disease onset and progression and clinical management of ALS.
Abstract: Purpose. To develop a predictive model of speech loss in persons with amyotrophic lateral sclerosis (ALS) based on measures of respiratory, phonatory, articulatory, and resonatory functions that were selected using a data-mining approach. Method. Physiologic speech subsystem (respiratory, phonatory, articulatory, and resonatory) functions were evaluated longitudinally in 66 individuals with ALS using multiple instrumentation approaches including acoustic, aerodynamic, nasometeric, and kinematic. The instrumental measures of the subsystem functions were subjected to a principal component analysis and linear mixed effects models to derive a set of comprehensive predictors of bulbar dysfunction. These subsystem predictors were subjected to a Kaplan-Meier analysis to estimate the time until speech loss. Results. For a majority of participants, speech subsystem decline was detectible prior to declines in speech intelligibility and speaking rate. Among all subsystems, the articulatory and phonatory predictors were most responsive to early bulbar deterioration; and the resonatory and respiratory predictors were as responsive to bulbar decline as was speaking rate. Conclusions. The articulatory and phonatory predictors are sensitive indicators of early bulbar decline due to ALS, which has implications for predicting disease onset and progression and clinical management of ALS.

111 citations


Journal ArticleDOI
TL;DR: Dysarthria with various combinations of hypokinetic, spastic and ataxic components reflects differing pathophysiology in PD, PSP and MSA and may provide useful information in the evaluation of these diseases with similar manifestations.
Abstract: Although speech disorder is frequently an early and prominent clinical feature of Parkinson's disease (PD) as well as atypical parkinsonian syndromes (APS) such as progressive supranuclear palsy (PSP) and multiple system atrophy (MSA), there is a lack of objective and quantitative evidence to verify whether any specific speech characteristics allow differentiation between PD, PSP and MSA. Speech samples were acquired from 77 subjects including 15 PD, 12 PSP, 13 MSA and 37 healthy controls. The accurate differential diagnosis of dysarthria subtypes was based on the quantitative acoustic analysis of 16 speech dimensions. Dysarthria was uniformly present in all parkinsonian patients but was more severe in PSP and MSA than in PD. Whilst PD speakers manifested pure hypokinetic dysarthria, ataxic components were more affected in MSA whilst PSP subjects demonstrated severe deficits in hypokinetic and spastic elements of dysarthria. Dysarthria in PSP was dominated by increased dysfluency, decreased slow rate, inappropriate silences, deficits in vowel articulation and harsh voice quality whereas MSA by pitch fluctuations, excess intensity variations, prolonged phonemes, vocal tremor and strained-strangled voice quality. Objective speech measurements were able to discriminate between APS and PD with 95% accuracy and between PSP and MSA with 75% accuracy. Dysarthria severity in APS was related to overall disease severity (r = 0.54, p = 0.006). Dysarthria with various combinations of hypokinetic, spastic and ataxic components reflects differing pathophysiology in PD, PSP and MSA. Thus, motor speech examination may provide useful information in the evaluation of these diseases with similar manifestations.

103 citations


Journal ArticleDOI
TL;DR: The association between persistent aphasia at three-months and poor modified Rankin Scale renders this impairment a major therapeutic target for recovery and restitution.
Abstract: BackgroundAphasia and dysarthria have major implications for activities of daily living and social participation following stroke. Few studies describe recovery in the acute stroke setting. We described the evolution of aphasia and dysarthria by three-months poststroke.MethodsWe conducted a retrospective analysis of pooled clinical trial data from the Virtual International Stroke Trials Archive. We defined aphasia and dysarthria at baseline as a score of ≥1 on the Best Language (Item 9) and Dysarthria (Item 10) domains of the National Institutes of Health Stroke Scale, respectively. We described recovery from these impairments by three-months. Covariate adjusted analyses described the associations between aphasia, dysarthria, and functional outcome using the modified Rankin Scale at three-months following stroke.ResultsAt baseline, 4039/8904 (45·4%) people presented with aphasia and 6192 (69·5%) with dysarthria; 2639 (29·6%) had both impairments. By three-months, aphasia and dysarthria had resolved in 129...

73 citations


Journal ArticleDOI
TL;DR: It was showed that intensive therapies in PD are the most effective for hypophonia and can lead to some improvement of voice pitch, and behavioral speech rehabilitation in PD still needs further validation.
Abstract: Parkinson's disease (PD) results from neurodegenerative processes leading to alteration of motor functions. Most motor symptoms respond well to pharmacological and neurosurgical treatments, except some axial symptoms such as speech impairment, so-called dysarthria. However, speech therapy is rarely proposed to PD patients. This review aims at evaluating previous research on the effects of speech behavioral therapies in patients with PD. We also performed two meta-analyses focusing on speech loudness and voice pitch. We showed that intensive therapies in PD are the most effective for hypophonia and can lead to some improvement of voice pitch. Although speech therapy is effective in handling PD dysarthria, behavioral speech rehabilitation in PD still needs further validation.

67 citations


Proceedings ArticleDOI
14 Oct 2015
TL;DR: EchoWear, a smartwatch-based system, to remotely monitor speech and voice exercises as prescribed by speech-language pathologists is designed and preliminary analysis shows promising outcomes for using EchoWear in speech therapies for people with PD.
Abstract: About 90 percent of people with Parkinson's disease (PD) experience decreased functional communication due to the presence of voice and speech disorders associated with dysarthria that can be characterized by monotony of pitch (or fundamental frequency), reduced loudness, irregular rate of speech, imprecise consonants, and changes in voice quality. Speech-language pathologists (SLPs) work with patients with PD to improve speech intelligibility using various intensive in-clinic speech treatments. SLPs also prescribe home exercises to enhance generalization of speech strategies outside of the treatment room. Even though speech therapies are found to be highly effective in improving vocal loudness and speech quality, patients with PD find it difficult to follow the prescribed exercise regimes outside the clinic and to continue exercises once the treatment is completed. SLPs need techniques to monitor compliance and accuracy of their patients' exercises at home and in ecologically valid communication situations. We have designed EchoWear, a smartwatch-based system, to remotely monitor speech and voice exercises as prescribed by SLPs. We conducted a study of 6 individuals; three with PD and three healthy controls. To assess the performance of EchoWear technology compared with high-quality audio equipment obtained in a speech laboratory. Our preliminary analysis shows promising outcomes for using EchoWear in speech therapies for people with PD.

66 citations


Journal ArticleDOI
TL;DR: Clinical ratings of its attributes and acoustic measures of some of its temporal characteristics can support its diagnosis and help quantify its salient characteristics and progression over time.
Abstract: Purpose This study summarizes 2 illustrative cases of a neurodegenerative speech disorder, primary progressive apraxia of speech (AOS), as a vehicle for providing an overview of the disorder and an...

66 citations


Journal ArticleDOI
TL;DR: Distinctive features of dysarthria and dyspraxia are found in individuals with GRIN2A mutations, often in the setting of epilepsy-aphasia syndromes; Dysarthria has not been previously recognized in these disorders.
Abstract: Objective: To delineate the specific speech deficits in individuals with epilepsy-aphasia syndromes associated with mutations in the glutamate receptor subunit gene GRIN2A . Methods: We analyzed the speech phenotype associated with GRIN2A mutations in 11 individuals, aged 16 to 64 years, from 3 families. Standardized clinical speech assessments and perceptual analyses of conversational samples were conducted. Results: Individuals showed a characteristic phenotype of dysarthria and dyspraxia with lifelong impact on speech intelligibility in some. Speech was typified by imprecise articulation (11/11, 100%), impaired pitch (monopitch 10/11, 91%) and prosody (stress errors 7/11, 64%), and hypernasality (7/11, 64%). Oral motor impairments and poor performance on maximum vowel duration (8/11, 73%) and repetition of monosyllables (10/11, 91%) and trisyllables (7/11, 64%) supported conversational speech findings. The speech phenotype was present in one individual who did not have seizures. Conclusions: Distinctive features of dysarthria and dyspraxia are found in individuals with GRIN2A mutations, often in the setting of epilepsy-aphasia syndromes; dysarthria has not been previously recognized in these disorders. Of note, the speech phenotype may occur in the absence of a seizure disorder, reinforcing an important role for GRIN2A in motor speech function. Our findings highlight the need for precise clinical speech assessment and intervention in this group. By understanding the mechanisms involved in GRIN2A disorders, targeted therapy may be designed to improve chronic lifelong deficits in intelligibility.

62 citations


Journal ArticleDOI
TL;DR: The findings suggest that prosodic characteristics such as monopitch should be explored as adjuncts to this treatment of dysarthria in PD and development of such prosodic treatments may enhance speech naturalness and thus improve quality of life.
Abstract: Purpose Given the potential significance of speech naturalness to functional and social rehabilitation outcomes, the objective of this study was to examine the effect of listener perceptions of monopitch on speech naturalness and intelligibility in individuals with Parkinson's disease (PD). Method Two short utterances were extracted from monologue samples of 16 speakers with PD and 5 age-matched adults without PD. Sixteen listeners evaluated these stimuli for monopitch, speech naturalness and intelligibility using the visual sort and rate method. Results Naive listeners can reliably judge monopitch, speech naturalness, and intelligibility with minimal familiarization. While monopitch and speech intelligibility were only moderately correlated, monopitch and speech naturalness were highly correlated. Conclusions A great deal of attention is currently being paid to improvement of vocal loudness and thus speech intelligibility in PD. Our findings suggest that prosodic characteristics such as monopitch should ...

54 citations


Journal ArticleDOI
TL;DR: Patients with ALS exhibit multifactorial deficits in sentence expression that are independent of their motor disorder, and these deficits are related to disease in a network of brain regions associated with syntactic processing.
Abstract: Quantitative examinations of speech production in amyotrophic lateral sclerosis (ALS) are rare. To identify language features minimally confounded by a motor disorder, we investigated linguistic and motor sources of impaired sentence expression in ALS, and we related deficits to gray matter (GM) and white matter (WM) MRI abnormalities. We analyzed a semi-structured speech sample in 26 ALS patients and 19 healthy seniors for motor- and language-related deficits. Regression analyses related grammaticality to GM atrophy and reduced WM fractional anisotropy (FA). Results demonstrated that ALS patients were impaired relative to controls on quantity of speech, speech rate, speech articulation errors, and grammaticality. Speech rate and articulation errors were related to the patients’ motor impairment, while grammatical difficulty was independent of motor difficulty. This was confirmed in subgroups without dysarthria and without executive deficits. Regressions related grammatical expression to GM atrophy in left inferior frontal and anterior temporal regions and to reduced FA in superior longitudinal and inferior frontal-occipital fasciculi. In conclusion, patients with ALS exhibit multifactorial deficits in sentence expression. They demonstrate a deficit in grammatical expression that is independent of their motor disorder. Impaired grammatical expression is related to disease in a network of brain regions associated with syntactic processing.

50 citations


Journal ArticleDOI
TL;DR: Results highlight left PM involvement in AOS, begin to differentiate its neural mechanisms from those of other motor impairments following stroke, and help inform us of the neural mechanisms driving differences in speech motor planning and programming impairment following stroke.

49 citations


Journal ArticleDOI
TL;DR: Intelligibility seems to be a useful clinical tool for differentiating between TD children and children with SMI at 5 years of age; however, there is considerable variability within and between listeners, highlighting the need for more than one listener per child to ensure validity of an intelligibility measure.
Abstract: Purpose We examined variability of speech intelligibility scores and how well intelligibility scores predicted group membership among 5-year-old children with speech motor impairment (SMI) secondary to cerebral palsy and an age-matched group of typically developing (TD) children. Method Speech samples varying in length from 1–4 words were elicited from 24 children with cerebral palsy (mean age 60.50 months) and 20 TD children (mean age 60.33 months). Two hundred twenty adult listeners made orthographic transcriptions of speech samples (n = 5 per child). Results Variability associated with listeners made a significant contribution to explaining the variance in intelligibility scores for TD and SMI children, but the magnitude was greater for TD children. Intelligibility scores differentiated very well between children who have SMI and TD children when intelligibility was at or below approximately 75% and above approximately 85%. Conclusions Intelligibility seems to be a useful clinical tool for differentiat...

Journal ArticleDOI
TL;DR: Results showed that vowel contrast increased in F1/F2 acoustic space after administration of the LSVT®, which expanded clinical understanding of the treatment and indicate that loud speech changes C–V coarticulation patterns.
Abstract: The purpose of this study was to investigate the impact of the Lee Silverman Voice Treatment (LSVT®) on vowel articulation and consonant-vowel (C-V) coarticulation in dysarthric speakers with Parkinson's disease (PD). Nine Quebec French speakers diagnosed with idiopathic PD underwent the LSVT®. Speech characteristics were compared before and after treatment. Vowel articulation was measured using acoustic vowel space and calculated with the first (F1) and second formant (F2) of the vowels /i/, /u/ and /a/. C-V coarticulation was measured using locus equations, an acoustic metric based on the F2 transitions within vowels in relation to the preceding consonant. The relationship between these variables, speech loudness and vowel duration was also analysed. Results showed that vowel contrast increased in F1/F2 acoustic space after administration of the LSVT®. This improvement was associated with the gain in speech loudness and longer vowel duration. C-V coarticulation patterns between consonant contexts showed greater distinctiveness after the treatment. This improvement was associated with the gain in speech loudness only. These results support the conclusions of previous studies investigating the relationship between the LSVT®, speech loudness and articulation in PD. These results expand clinical understanding of the treatment and indicate that loud speech changes C-V coarticulation patterns. Clinical applications and theoretical considerations are discussed.

Journal ArticleDOI
TL;DR: Dysarthria and dysphagia were common symptoms in patients with spinal onset of ALS and the swallowing quality of life decrease was directly related to with severity of dysarthria, and the SWAL-QOL outcomes was significant for the same domains.
Abstract: BACKGROUND: Dysarthria and dysphagia are the most common clinical problems encountered in Amyotrophic Lateral Sclerosis and may reduce the quality of life. OBJECTIVE: Evaluate the association of dysarthria and dysphagia and to evaluate the impact of dysphagia on swallowing quality of life in patients with ALS with spinal onset. METHODS: Seventeen patients underwent to speech and swallowing evaluation and filled out self-report assessment of the Swallowing Quality of Life (SWAL-QOL). The dysarthria severity was graded using the Speech Subscale of ALS Severity Scale. The dysphagia severity was graded using a scale proposed by Dziweas et al., and the Functional Oral Intake Scale. RESULTS: Over 70% of the ALS patients with spinal onset had dysarthria and dysphagia. The correlation of dysarthria and dysphagia scales was statistically significant (p < 0.001). The correlation between dysarthria and dysphagia severity and SWAL-QOL outcomes was significant for the same domains: “symptom frequency”, “communication” and “fear of eating”. The SWAL-QOL domains presented a mild to moderate impact on quality of life. Disease duration did not impact on SWAL-QOL. CONCLUSIONS: Dysarthria and dysphagia were common symptoms in patients with spinal onset of ALS and the swallowing quality of life decrease was directly related to with severity of dysarthria and dysphagia.

Journal ArticleDOI
TL;DR: A structured sparse linear model incorporated with phonological knowledge that simultaneously addresses phonologically structured sparse feature selection and intelligibility prediction is proposed for automatically assessing the speech intelligibility of patients with dysarthria.
Abstract: This paper presents a new method for automatically assessing the speech intelligibility of patients with dysarthria, which is a motor speech disorder impeding the physical production of speech. The proposed method consists of two main steps: feature representation and prediction. In the feature representation step, the speech utterance is converted into a phone sequence using an automatic speech recognition technique and is then aligned with a canonical phone sequence from a pronunciation dictionary using a weighted finite state transducer to capture the pronunciation mappings such as match, substitution, and deletion. The histograms of the pronunciation mappings on a pre-defined word set are used for features. Next, in the prediction step, a structured sparse linear model incorporated with phonological knowledge that simultaneously addresses phonologically structured sparse feature selection and intelligibility prediction is proposed. Evaluation of the proposed method on a database of 109 speakers consisting of 94 dysarthric and 15 control speakers yielded a root mean square error of 8.14 compared to subjectively rated scores in the range of 0 to 100. This is a promising performance in which the system can be successfully applied to help speech therapists in diagnosing the degree of speech disorder.

Journal ArticleDOI
TL;DR: It is shown that intelligibility assessments work best if there is a pre-existing set of words annotated for intelligibility from the speaker to be evaluated, which can be used for training the system.
Abstract: Automated intelligibility assessments can support speech and language therapists in determining the type of dysarthria presented by their clients. Such assessments can also help predict how well a person with dysarthria might cope with a voice interface to assistive technology. Our approach to intelligibility assessment is based on iVectors, a set of measures that capture many aspects of a person’s speech, including intelligibility. The major advantage of iVectors is that they compress all acoustic information contained in an utterance into a reduced number of measures, and they are very suitable to be used with simple predictors. We show that intelligibility assessments work best if there is a pre-existing set of words annotated for intelligibility from the speaker to be evaluated, which can be used for training our system. We discuss the implications of our findings for practice.

Journal ArticleDOI
TL;DR: Comparisons of dysarthria profiles of patients with SCA3 and SCA6 vs. Friedreich ataxia (FRDA) showed regularity of DDK was specifically impaired inSCA3, whereas impairments of speech parameters, i.e., rate and modulation were stronger affected in SCA 6.
Abstract: Patterns of dysarthria in spinocerebellar ataxias (SCAs) and their discriminative features still remain elusive. Here we aimed to compare dysarthria profiles of patients with (SCA3 and SCA6 vs. Friedreich ataxia (FRDA), focussing on three particularly vulnerable speech parameters (speaking rate, prosodic modulation, and intelligibility) in ataxic dysarthria as well as on a specific oral non-speech variable of ataxic impairment, i.e., the irregularity of oral motor diadochokinesis (DDK). 30 Patients with SCA3, SCA6, and FRDA, matched for group size (n = 10 each), disease severity, and disease duration produced various speech samples and DDK tasks. A discriminant analysis was used to differentiate speech and non-speech parameters between groups. Regularity of DDK was specifically impaired in SCA3, whereas impairments of speech parameters, i.e., rate and modulation were stronger affected in SCA6. Speech parameters are particularly vulnerable in SCA6, while non-speech oral motor features are notably impaired in SCA3.

Proceedings ArticleDOI
11 Sep 2015
TL;DR: A statistical analysis performed across various systems and its specific implementation in modelling different dysarthric severity sub-groups showed that, SAT-adapted systems were more applicable to handle disfluencies of more severe speech and SI systems prepared from typical speech were more apt for modelling speech with low level of severity.
Abstract: Dysarthria is a neurological speech disorder, which exhibits multi-fold disturbances in the speech production system of an individual and can have a detrimental effect on the speech output. In addition to the data sparseness problems, dysarthric speech is characterised by inconsistencies in the acoustic space making it extremely challenging to model. This paper investigates a variety of baseline speaker independent (SI) systems and its suitability for adaptation. The study also explores the usefulness of speaker adaptive training (SAT) for implicitly annihilating inter-speaker variations in a dysarthric corpus. The paper implements a hybrid MLLR-MAP based approach to adapt the SI and SAT systems. ALL the results reported uses UASPEECH dysarthric data. Our best adapted systems gave a significant absolute gain of 11.05% (20.42% relative) over the last published best result in the literature. A statistical analysis performed across various systems and its specific implementation in modelling different dysarthric severity sub-groups, showed that, SAT-adapted systems were more applicable to handle disfluencies of more severe speech and SI systems prepared from typical speech were more apt for modelling speech with low level of severity.

Proceedings ArticleDOI
01 Sep 2015
TL;DR: Different types of input features used by deep neural networks (DNNs) to automatically detect repetition stuttering and non-speech dysfluencies within dysarthric speech are investigated.
Abstract: Dysarthria is a speech disorder caused by difficulties in controlling muscles, such as the tongue and lips, that are needed to produce speech. These differences in motor skills cause speech to be slurred, mumbled, and spoken relatively slowly, and can also increase the likelihood of dysfluency. This includes nonspeech sounds, and ‘stuttering’, defined here as a disruption in the fluency of speech manifested by prolongations, stop-gaps, and repetitions. This paper investigates different types of input features used by deep neural networks (DNNs) to automatically detect repetition stuttering and non-speech dysfluencies within dysarthric speech. The experiments test the effects of dimensionality within Mel-frequency cepstral coefficients (MFCCs) and linear predictive cepstral coefficients (LPCCs), and explore the detection capabilities in dyarthric versus non-dysarthric speech. The results obtained using MFCC and LPCC features produced similar recognition accuracies; repetition stuttering in dysarthric speech was identified correctly at approximately 86% and 84% for non-dysarthric speech. Non-speech sounds were recognized with approximately 75% accuracy in dysarthric speakers.

24 Sep 2015
TL;DR: It is recommended that each individual be managed by a multidisciplinary team of relevant specialists such as neurologists, occupational therapists, physical therapists, physiatrists, orthopedists, nutritionists, speech therapists, social workers, and psychologists.
Abstract: Clinical characteristics Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is characterized by progressive cerebellar ataxia and variable findings including pyramidal signs, a dystonic-rigid extrapyramidal syndrome, significant peripheral amyotrophy and generalized areflexia, progressive external ophthalmoplegia, action-induced facial and lingual fasciculations, and bulging eyes. Neurologic findings tend to evolve as the disorder progresses. Diagnosis/testing The diagnosis of SCA3 is established in a proband with suggestive findings and a heterozygous abnormal CAG trinucleotide repeat expansion in ATXN3 identified by molecular genetic testing. Management Treatment of manifestations: Management is supportive as no medication slows the course of disease. The goals of treatment are to maximize function and reduce complications. It is recommended that each individual be managed by a multidisciplinary team of relevant specialists such as neurologists, occupational therapists, physical therapists, physiatrists, orthopedists, nutritionists, speech therapists, social workers, and psychologists. Various manifestations may respond to pharmacologic agents. Regular physical activity is recommended, including combined physical and occupational therapy focused on gait and coordination. Canes and walkers help prevent falling; motorized scooters, weighted eating utensils, and dressing hooks help to maintain independence. Speech therapy and communication devices may benefit those with dysarthria, and dietary modification those with dysphagia. Other recommendations include home adaptations to prevent falls and improve mobility, dietary supplements if caloric intake is reduced, weight control to facilitate ambulation and mobility, and caution with general anesthesia. Surveillance: Annual assessments (or more frequently as needed) of neurologic findings (e.g., dysarthria, dysphagia, bladder dysfunction, neuropathic pain, cognitive and psychiatric manifestations), weight and nutritional status, and social support. Genetic counseling SCA3 is inherited in an autosomal dominant manner. Each child of an affected individual has a 50% chance of inheriting the ATXN3 CAG repeat expansion. Once the CAG repeat expansion has been identified in an affected family member, prenatal testing for a pregnancy at increased risk and preimplantation genetic testing are possible. Note: The prenatal finding of an ATXN3 CAG repeat expansion cannot be used to accurately predict onset, severity, type of symptoms, or rate of progression of SCA3.

Journal ArticleDOI
TL;DR: This paper proposes two convex cost functions for learning the weighting functions and an adaptation strategy to customize the approach to a particular speaker using minimal training, and shows that the proposed methods outperform three competing methods on both healthy and dysarthric speech.
Abstract: Speaking rate estimation directly from the speech waveform is a long-standing problem in speech signal processing. In this paper, we pose the speaking rate estimation problem as that of estimating a temporal density function whose integral over a given interval yields the speaking rate within that interval. In contrast to many existing methods, we avoid the more difficult task of detecting individual phonemes within the speech signal and we avoid heuristics such as thresholding the temporal envelope to estimate the number of vowels. Rather, the proposed method aims to learn an optimal weighting function that can be directly applied to time-frequency features in a speech signal to yield a temporal density function. We propose two convex cost functions for learning the weighting functions and an adaptation strategy to customize the approach to a particular speaker using minimal training. The algorithms are evaluated on the TIMIT corpus, on a dysarthric speech corpus, and on the ICSI Switchboard spontaneous speech corpus. Results show that the proposed methods outperform three competing methods on both healthy and dysarthric speech. In addition, for spontaneous speech rate estimation, the result show a high correlation between the estimated speaking rate and ground truth values.

Journal ArticleDOI
TL;DR: All individuals with Kabuki syndrome have some level of communication deficit, warranting speech pathology involvement in all cases, and variability fits with the multisystem nature of the disorder.
Abstract: Speech and language deficits are commonly associated with Kabuki syndrome. Yet little is known regarding the specific symptomatology of these disorders, preventing use of targeted treatment programs. Here we detail speech and language in 16 individuals with Kabuki syndrome (thirteen with KMT2D mutations, one with a KDM6A mutation, and two mutation-negative cases), aged 4-21 years. The most striking speech deficit was dysarthria, characterised by imprecise consonants, harsh vocal quality, hypernasality, reduced rate and stress, and distorted pitch. Oromotor functioning was also impaired. Delayed, rather than disordered, articulation and phonology was common. Both receptive and expressive language abilities were reduced in the majority and deficits were noted across all language sub-domains (i.e., semantics, syntax, morphology, and pragmatics) with no clear differentiation or specific language profile. Individuals with Kabuki syndrome present with a heterogenous pattern of oromotor, speech, and language deficits. This variability fits with the multisystem nature of the disorder, which may encompass neurological, orofacial structural, hearing, and cognitive deficits, any or all of which may contribute to speech or language impairment. Our results suggest that all individuals with Kabuki syndrome have some level of communication deficit, warranting speech pathology involvement in all cases.

Journal ArticleDOI
TL;DR: Evidence that speech impairment is not a necessary side-effect of GPi-DBS in primary dystonia is provided, as both, recurring of stuttering and a worsening of dysarthria may be seen in individual patients.

25 Nov 2015
TL;DR: The 7q11.23 duplication syndrome is characterized by distinctive facial features; cardiovascular disease, neurologic abnormalities, and speech sound disorders including motor speech disorders and phonologic disorders; behavior problems including anxiety disorders (especially social anxiety disorder [social phobia]), selective mutism, attention deficit hyperactivity disorder (ADHD), oppositional disorders, physical aggression, and autism spectrum disorders (ASD).
Abstract: Clinical characteristics 7q11.23 duplication syndrome is characterized by distinctive facial features; cardiovascular disease (dilation of the ascending aorta in 46%); neurologic abnormalities (hypotonia, adventitious movements, and abnormal gait and station); speech sound disorders including motor speech disorders (childhood apraxia of speech and/or dysarthria) and phonologic disorders; behavior problems including anxiety disorders (especially social anxiety disorder [social phobia]), selective mutism, attention deficit hyperactivity disorder (ADHD), oppositional disorders, physical aggression, and autism spectrum disorders (ASD); delayed motor, speech, and social skills in early childhood; and intellectual ability ranging from intellectual disability (~18%) to borderline intellectual ability (~20%) to low average to high average (the remainder). Approximately 30% of individuals with the 7q11.23 duplication have one or more congenital anomalies. Diagnosis/testing The diagnosis of the 7q11.23 duplication syndrome is established by detection of a recurrent 1.5- to 1.8-Mb heterozygous duplication of the Williams-Beuren syndrome critical region (WBSCR). Management Treatment of manifestations: Aortic dilation is treated with beta blocker therapy and/or surgery as needed. Constipation should be aggressively managed at all ages to prevent encopresis and impaction. Address developmental disabilities, including ASD, through early intervention programs (including speech/language therapy, physical therapy, and occupational therapy), special education programs, and vocational training. Address ASD with applied behavior analytic interventions and other empirically supported psychosocial approaches. Address childhood apraxia of speech (CAS) or manifestations of this disorder with intensive speech/language therapy to maximize effective oral communication and prevent or limit later language impairment and/or reading disorder. Address emotional and behavioral disorders (aggression, social anxiety, selective mutism) with cognitive-behavioral therapy, applied behavior analysis behavior modification intervention, and psychotropic medications as needed. Surveillance: Routine monitoring of head circumference in infancy. Annual monitoring of aortic diameter (including Z scores in children) and behavior. Annual assessment by occupational and physical therapists and speech and language pathologists until age six years. Genetic counseling The 7q11.23 duplication syndrome is transmitted in an autosomal dominant manner. About 27% of probands have an affected parent. When a parent has the 7q11.23 duplication, the risk to each sib of inheriting the duplication is 50%. If the 7q11.23 duplication identified in the proband is not identified in the parents, the risk to sibs is low (

Journal ArticleDOI
TL;DR: The present study designs a robust signal processing technique that allows the automatic detection of spectrally distinctive nuclei of syllable vocalizations and to determine speech features that represent rhythm instability (RI) and rhythm acceleration (RA).
Abstract: Speech rhythm abnormalities are commonly present in patients with different neurodegenerative disorders These alterations are hypothesized to be a consequence of disruption to the basal ganglia circuitry involving dysfunction of motor planning, programing, and execution, which can be detected by a syllable repetition paradigm Therefore, the aim of the present study was to design a robust signal processing technique that allows the automatic detection of spectrally distinctive nuclei of syllable vocalizations and to determine speech features that represent rhythm instability (RI) and rhythm acceleration (RA) A further aim was to elucidate specific patterns of dysrhythmia across various neurodegenerative disorders that share disruption of basal ganglia function Speech samples based on repetition of the syllable /pa/ at a self-determined steady pace were acquired from 109 subjects, including 22 with Parkinson's disease (PD), 11 progressive supranuclear palsy (PSP), 9 multiple system atrophy (MSA), 24 ephedrone-induced parkinsonism (EP), 20 Huntington's disease (HD), and 23 healthy controls Subsequently, an algorithm for the automatic detection of syllables as well as features representing RI and RA were designed The proposed detection algorithm was able to correctly identify syllables and remove erroneous detections due to excessive inspiration and non-speech sounds with a very high accuracy of 996% Instability of vocal pace performance was observed in PSP, MSA, EP, and HD groups Significantly increased pace acceleration was observed only in the PD group Although not significant, a tendency for pace acceleration was observed also in the PSP and MSA groups Our findings underline the crucial role of the basal ganglia in the execution and maintenance of automatic speech motor sequences We envisage the current approach to become the first step toward the development of acoustic technologies allowing automated assessment of rhythm in dysarthrias

Book
21 Sep 2015
TL;DR: Shelley Velleman's Speech Language Disorders as mentioned in this paper offers authoritative coverage of the key concepts of normal speech sound development and production, as well as the related articulation and phonological disorders.
Abstract: Filled with examples of typically developing children and those with speech sound disorders, Shelley Velleman's Speech Language Disorders offers authoritative coverage of the key concepts of normal speech sound development and production, as well as the related articulation and phonological disorders. A practical resource for patient assessment and treatment, this up-to-date book integrates the latest research in the field with actual speech data examples. Rather than covering treatment options at the end of the text or as an "add on," the book integrates these vital components directly into the appropriate chapters to help Speech-Language Pathology students understand the connection between the science of the disorders and real-life practice. Using highly readable prose enriched by clear definitions, examples, and exercises, the author covers all key concepts, including important topics often not offered in similar books, including evaluating and treating speech sound disorders in young children, those with neurodevelopmental syndromes, and those with prosody issues. In addition, the book helps students see the connection between speech sound disorders and the related motor speech disorders that overlap and complicate assessment and treatment. *Compelling online videos of children with speech sound disorders (supplemented with assignable exercises) bring concepts to life and connect classroom learning with real-life practice.* Considerations for assessment and treatment of children who speak languages other than English are interwoven throughout the text.*Numerous data sets and examples of children with speech disorders provide a real-world context.*Case studies for practice show readers how to apply the concepts and techniques covered.*Assessment and treatment of motor speech disorders in children are covered, including differential diagnosis of dysarthria and Childhood Apraxia of Speech, an area of expertise of the author.* Quick and easy assessment forms for identifying children's speech strengths and weaknesses are included.*Application to practice elements include integrated coverage of the assessment and treatment options for each subcomponent of speech sound development disorder.* Feature boxes point out "common misconceptions" and "common confusion" areas and explain particularly challenging concepts and innovative approaches.* Author-developed exercises for applying concepts, practicing evaluation techniques, scoring tests, and developing treatment plans give students hands-on practice in applying concepts.*End-of-chapter review questions contain multiple-choice quizzes that allow students to assess their understanding of chapter content.

Journal ArticleDOI
TL;DR: It can be concluded that intelligibility and severity have significant influence on the recognition accuracy of ASR system, and methods and techniques that reduces the influence of these specific factors should be identified.
Abstract: Reviewed existing literature on the performance of ASR system for dysarthria.Analysed influence of speech/speaker mode, vocabulary size & speaking style on WER.Identified specific factors of Dysarthric speech on WER.Analysed influence of specific factor on WER.Measured the correlation of general and specific factors on WER. Automatic speech recognition (ASR) is becoming an important assistive tool among the speech impaired individuals such as dysarthria. Currently, the existing ASR systems were unable to recognise dysarthric speech at an acceptable degree. Little research was carried out to identify factors that influence the performance of ASR system in recognising dysarthric speech. This article aims to identify factors that potentially influence the recognition accuracy of ASR system in recognising dysarthric speech. Some of the factors that influence the recognition accuracy of ASR, which have been confirmed in existing researches for ASR system are speech mode, speaker mode, vocabulary size and speaking styles. We have also focused at factors that are more specific to dysarthric speech such as speech intelligibility, severity and intra-speaker variability that potentially influence the recognition accuracy. We have evaluated the influence of these factors on recognition accuracy using data published in existing researches. It was found that general factors considered in this review have little influence on the recognition accuracy. However, factors more specific to dysarthric speech were found to have a significant influence on the recognition accuracy of the ASR system. From the findings, it can be concluded that intelligibility and severity have significant influence on the recognition accuracy. To improve the recognition accuracy of ASR system, methods and techniques that reduces the influence of these specific factors should be identified.

19 Mar 2015
TL;DR: All affected individuals have generalized areflexia followed by a peripheral neuropathy and quadriplegia with loss of ambulation about seven to ten years after onset, and EMG reveals axonal neuropathy in 100% of individuals with AOA1.
Abstract: Clinical characteristics Ataxia with oculomotor apraxia type 1 (AOA1) is characterized by childhood onset of slowly progressive cerebellar ataxia, followed by oculomotor apraxia and a severe primary motor peripheral axonal motor neuropathy The first manifestation is progressive gait imbalance (mean age of onset: 43 years; range: 2-10 years), followed by dysarthria, then upper-limb dysmetria with mild intention tremor Oculomotor apraxia, usually noticed a few years after the onset of ataxia, progresses to external ophthalmoplegia All affected individuals have generalized areflexia followed by a peripheral neuropathy and quadriplegia with loss of ambulation about seven to ten years after onset Hands and feet are short and atrophic Chorea and upper-limb dystonia are common Intellect remains normal in some individuals; in others, different degrees of cognitive impairment have been observed Diagnosis/testing The diagnosis of AOA1 is based on clinical findings (including family history) and exclusion of the diagnosis of ataxia-telangiectasia Cerebellar atrophy is visible on MRI in all affected individuals EMG reveals axonal neuropathy in 100% of individuals with AOA1 APTX is the only gene known to be associated with AOA1 Management Treatment of manifestations: May include physical therapy, particularly for disabilities resulting from peripheral neuropathy; a wheelchair for mobility, usually by age 15-20 years; educational support for difficulties with speaking, reading, and writing Prevention of secondary complications: High-protein diet to prevent edema by restoring serum albumin concentration; low-cholesterol diet Surveillance: Routine follow up with a neurologist Genetic counseling AOA1 is inherited in an autosomal recessive manner At conception, each sib of an affected individual has a 25% chance of being affected, a 50% chance of being an asymptomatic carrier, and a 25% chance of being neither affected nor a carrier Carrier testing for at-risk family members and prenatal testing for pregnancies at increased risk are possible if both pathogenic variants in a family have been identified

Journal ArticleDOI
01 Jun 2015
TL;DR: The degree of modification of speech and voice of patients with PD resembles those seen in normal aging process, with the exception of prosody and the habitual frequency, which are related to the greatest negative impact on the QoL of patientswith PD.
Abstract: PURPOSE: To compare the speech and voice of Parkinson's disease (PD) patients and neurologically healthy elderly adults (control group, CG), to find out whether these features are related to the disease or the normal aging process, and investigate the impact that dysarthria has on the Quality of Life (QoL) of these individuals METHODS: This is a cross-sectional study involving 25 individuals, 13 patients with PD and 12 CG All the participants underwent vocal assessment, perceptual and acoustic analysis, based on "Dysarthria Assessment Protocol" and analysis of QoL using a questionnaire, "Living with Dysarthria" The data underwent statistical analysis to compare the groups in each parameter RESULTS: In the assessment of dysarthria, patients with PD showed differences in prosody parameter (p=0012), at the habitual frequency for females (p=0025) and males (p=0028), and the extent of intensity (p=0039) when compared to CG In QoL questionnaire, it was observed that patients with PD showed more negative impact on the QoL compared to CG, as indicated by the total score (p=0005) with various aspects influencing this result CONCLUSION: The degree of modification of speech and voice of patients with PD resembles those seen in normal aging process, with the exception of prosody and the habitual frequency, which are related to the greatest negative impact on the QoL of patients with PD

Journal ArticleDOI
TL;DR: RTMS can have a synergistic effect with speech therapy in treating dysarthria after stroke and showed greater improvement in articulation than did patients in the sham rTMS group, which revealed significant improvements on the Dysarthria scales in both groups.
Abstract: Objective To evaluate whether repetitive transcranial magnetic stimulation (rTMS) could improve dysarthria in stroke patients at the subacute stage. Methods This study was a prospective, randomized, double-blind controlled trial. Patients who had unilateral middle cerebral artery infarction were enrolled. In patients in the rTMS group, we found hot spots by searching for the evoked motor potential of the orbicularis oris on the non-affected side. We performed rTMS at a low frequency (1 Hz), 1,500 stimulations/day, 5 days a week for 2 weeks on the hotspots. We used the same protocol in the sham stimulation group patients as that in the rTMS group, except that the angle of the coil was perpendicular to the skull rather than tangential to it. The patients in both groups received speech therapy for 30 minutes, 5 days a week from a skilled speech therapist. The speech therapist measured the Urimal Test of Articulation and Phonology, alternative motion rates, sequential motion rates, and maximal phonation time before and after intervention sessions. Results Forty-two patients were enrolled in this study and 20 completed the study. Statistical analysis revealed significant improvements on the dysarthria scales in both groups. The sequential motion rate (SMR)-PǝTǝKǝ showed significantly greater improvement in the rTMS group patients than in the sham stimulation group. Conclusion Patients in the rTMS group showed greater improvement in articulation than did patients in the sham rTMS group. Therefore, rTMS can have a synergistic effect with speech therapy in treating dysarthria after stroke.

Journal ArticleDOI
TL;DR: This article investigates automatic speech processing approaches dedicated to the detection and localization of abnormal acoustic phenomena in speech signal produced by people with speech disorders and proposes two different approaches that obtain very encouraging results.
Abstract: Perceptual evaluation is still the most common method in clinical practice for diagnosing and following the progression of the condition of people with speech disorders. Although a number of studies have addressed the acoustic analysis of speech productions exhibiting impairments, additional descriptive analysis is required to manage interperson variability, considering speakers with the same condition or across different conditions. In this context, this article investigates automatic speech processing approaches dedicated to the detection and localization of abnormal acoustic phenomena in speech signal produced by people with speech disorders. This automatic process aims at enhancing the manual investigation of human experts while at the same time reducing the extent of their intervention by calling their attention to specific parts of the speech considered as atypical from an acoustical point of view.Two different approaches are proposed in this article. The first approach models only the normal speech, whereas the second models both normal and dysarthric speech. Both approaches are evaluated following two strategies: one consists of a strict phone comparison between a human annotation of abnormal phones and the automatic output, while the other uses a “one-phone delay” for the comparison.The experimental evaluation of both approaches for the task of detecting acoustic anomalies was conducted on two different corpora composed of French dysarthric speakers and control speakers. These approaches obtain very encouraging results and their potential for clinical uses with different types of dysarthria and neurological diseases is quite promising.