scholarly journals Dysarthria and broader motor speech deficits in Dravet syndrome

Neurology ◽  
2017 ◽  
Vol 88 (8) ◽  
pp. 743-749 ◽  
Author(s):  
Samantha J. Turner ◽  
Amy Brown ◽  
Marta Arpone ◽  
Vicki Anderson ◽  
Angela T. Morgan ◽  
...  

Objective:To analyze the oral motor, speech, and language phenotype in 20 children and adults with Dravet syndrome (DS) associated with mutations in SCN1A.Methods:Fifteen verbal and 5 minimally verbal DS patients with SCN1A mutations (aged 15 months-28 years) underwent a tailored assessment battery.Results:Speech was characterized by imprecise articulation, abnormal nasal resonance, voice, and pitch, and prosody errors. Half of verbal patients had moderate to severely impaired conversational speech intelligibility. Oral motor impairment, motor planning/programming difficulties, and poor postural control were typical. Nonverbal individuals had intentional communication. Cognitive skills varied markedly, with intellectual functioning ranging from the low average range to severe intellectual disability. Language impairment was congruent with cognition.Conclusions:We describe a distinctive speech, language, and oral motor phenotype in children and adults with DS associated with mutations in SCN1A. Recognizing this phenotype will guide therapeutic intervention in patients with DS.

Stroke ◽  
2021 ◽  
Author(s):  
Victoria Sherman ◽  
Rosemary Martino ◽  
Ishvinder Bhathal ◽  
MN, Gabrielle DeVeber ◽  
Nomazulu Dlamini ◽  
...  

Background and Purpose: Following adult stroke, dysphagia, dysarthria, and aphasia are common sequelae. Little is known about these impairments in pediatric stroke. We assessed frequencies, co-occurrence and associations of dysphagia, oral motor, motor speech, language impairment, and caregiver burden in pediatric stroke. Methods: Consecutive acute patients from term birth-18 years, hospitalized for arterial ischemic stroke (AIS), and cerebral sinovenous thrombosis, from January 2013 to November 2018 were included. Two raters reviewed patient charts to detect documentation of in-hospital dysphagia, oral motor dysfunction, motor speech and language impairment, and caregiver burden, using a priori operational definitions for notation and assessment findings. Other variables abstracted included demographics, pre-existing conditions, stroke characteristics, and discharge disposition. Impairment frequencies were obtained by univariate and bivariate analysis and associations by simple logistic regression. Results: A total of 173 patients were stratified into neonates (N=67, mean age 2.9 days, 54 AIS, 15 cerebral sinovenous thrombosis) and children (N=106, mean age 6.5 years, 73 AIS, 35 cerebral sinovenous thrombosis). Derived frequencies of impairments included dysphagia (39% neonates, 41% children); oral motor (6% neonates, 41% children); motor speech (37% children); and language (31% children). Common overlapping impairments included oral motor and motor speech (24%) and dysphagia and motor speech (23%) in children. Associations were found only in children between stroke type (AIS over cerebral sinovenous thrombosis) and AIS severity (more severe deficit at presentation) for all impairments except feeding impairment alone. Caregiver burden was present in 58% patients. Conclusions: For the first time, we systematically report the frequencies and associations of dysphagia, oral motor, motor speech, and language impairment during acute presentation of pediatric stroke, ranging from 30% to 40% for each impairment. Further research is needed to determine long-term effects of these impairments and to design standardized age-specific assessment protocols for early recognition following stroke.


2019 ◽  
Vol 62 (5) ◽  
pp. 1517-1531 ◽  
Author(s):  
Sungmin Lee ◽  
Lisa Lucks Mendel ◽  
Gavin M. Bidelman

Purpose Although the speech intelligibility index (SII) has been widely applied in the field of audiology and other related areas, application of this metric to cochlear implants (CIs) has yet to be investigated. In this study, SIIs for CI users were calculated to investigate whether the SII could be an effective tool for predicting speech perception performance in a population with CI. Method Fifteen pre- and postlingually deafened adults with CI participated. Speech recognition scores were measured using the AzBio sentence lists. CI users also completed questionnaires and performed psychoacoustic (spectral and temporal resolution) and cognitive function (digit span) tests. Obtained SIIs were compared with predicted SIIs using a transfer function curve. Correlation and regression analyses were conducted on perceptual and demographic predictor variables to investigate the association between these factors and speech perception performance. Result Because of the considerably poor hearing and large individual variability in performance, the SII did not predict speech performance for this CI group using the traditional calculation. However, new SII models were developed incorporating predictive factors, which improved the accuracy of SII predictions in listeners with CI. Conclusion Conventional SII models are not appropriate for predicting speech perception scores for CI users. Demographic variables (aided audibility and duration of deafness) and perceptual–cognitive skills (gap detection and auditory digit span outcomes) are needed to improve the use of the SII for listeners with CI. Future studies are needed to improve our CI-corrected SII model by considering additional predictive factors. Supplemental Material https://doi.org/10.23641/asha.8057003


2018 ◽  
Vol 34 (3) ◽  
pp. 269-287 ◽  
Author(s):  
Jenni Heikkilä ◽  
Eila Lonka ◽  
Auli Meronen ◽  
Sisko Tuovinen ◽  
Raija Eronen ◽  
...  

We developed a computerized audiovisual training programme for school-aged children with specific language impairment (SLI) to improve their phonological skills. The programme included various tasks requiring phonological decisions. Spoken words, pictures, letters and written syllables were used as training material. Spoken words were presented either as audiovisual speech (together with the talking face), or as auditory speech (voice alone). Two groups (10 children/group) trained for six weeks, five days per week: the audiovisual group trained with audiovisual speech, and the other group received analogically the same training but with auditory speech. Before and after training, language skills and other cognitive skills were assessed. The audiovisual group improved in a non-word-repetition test. Such improvement was not observed with auditory training. This result suggests that audiovisual speech may be helpful in the rehabilitation of children with SLI.


Author(s):  
Kristine Galek ◽  
Ed M. Bice ◽  
Katie Allen

Introduction Spastic dysarthria is a motor speech disorder produced by bilateral damage to the activation pathways of the central nervous system. Its speech characteristics reflect the effects of hypertonicity and weakness of the bulbar musculature in a way that slows movement and reduces range of motion and force. Perceptually, speech has a high-pitched, strained, hypernasal vocal quality with decreased intelligibility. Purpose The purpose is to present a case illustration describing the use of a novel treatment protocol to improve speech intelligibility in the presence of spastic dysarthria. Method An underlying framework, including principles of exercise, neuroplasticity, and motor learning with adjunctive biofeedback, is described. The protocol consisted of four sessions per week for 4 weeks with daily homework. Results The participant exhibited improvements in intelligibility, patient satisfaction, lingual, and jaw range of motion, nasality, and tongue strength. Conclusion The use of a novel protocol using biofeedback and incorporating principles of exercise science, neuroplasticity, and motor learning for the treatment of spastic dysarthria demonstrated positive outcomes.


2019 ◽  
Vol 36 (8) ◽  
pp. 456-458 ◽  
Author(s):  
Jan Schumacher ◽  
James Arlidge ◽  
Declan Dudley ◽  
Jennifer Van Ross ◽  
Francesca Garnham ◽  
...  

IntroductionRecent terror attacks and assassinations involving highly toxic chemical weapons have stressed the importance of sufficient respiratory protection of medical first responders and receivers. As full-face respirators cause perceptual-motor impairment, they not only impair vision but also significantly reduce speech intelligibility. The recent introduction of electronic voice projection units (VPUs), attached to a respirator, may improve communication while wearing personal respiratory protection.ObjectiveTo determine the influence of currently used respirators and VPUs on medical communication and speech intelligibility.Methods37 trauma anaesthetists carried out an evaluation exercise of six different respirators and VPUs including one control. Participants had to listen to audio clips of a variety of sentences dealing with scenarios of emergency triage and medical history taking.ResultsIn the questionnaire, operators stated that speech intelligibility of the Avon C50 respirator scored the highest (mean 3.9, ±SD 1.0) and that the Respirex Powered Respiratory Protective Suit (PRPS) NHS-suit scored lowest (1.6, 0.9). Regarding loudness the C50 plus the Avon VPU scored highest (4.1, 0.7), followed by the Draeger FPS-7000-com-plus (3.4, 1.0) and the Respirex PRPS NHS-suit scored lowest (2.3, 0.8).ConclusionsWe found that the Avon C50 is the preferred model among the tested respirators. In our model, electronic voice projection modules improved loudness but not speech intelligibility. The Respirex PRPS NHS-suit was rated significantly less favourably in respect of medical communication and speech intelligibility.


2019 ◽  
Vol 40 (04) ◽  
pp. 256-271
Author(s):  
Klara Marton ◽  
Thorfun Gehebe ◽  
Lia Pazuelo

AbstractCognitive control refers to the ability to perform goal-directed behaviors in the presence of other compelling actions or in the face of habitual practices. Cognitive control functions play a critical role in children's language processing and literacy development. In recent years, many clinicians have expanded their assessment and treatment to target specific cognitive skills. Our goal is to provide a review of recent findings on cognitive control functions in children with different language status (i.e., monolingual and bilingual children with and without language impairment). While children with language impairment show performance deficits in specific cognitive functions (e.g., working memory updating and interference control), typically developing bilingual children often outperform their monolingual peers in cognitive control tasks. However, the relationship between bilingualism and cognitive control has been controversial. Several factors that influence these variations are discussed. Given the findings on the joint impact of bilingualism and language impairment on cognitive control functions, we identify conditions in which bilingualism attenuates the negative effects of the language deficit and conditions in which language impairment has a stronger effect than bilingualism. Critical issues of bilingual assessment, suggestions, and future directions are discussed.


2020 ◽  
Vol 51 (3) ◽  
pp. 882-896 ◽  
Author(s):  
Phoebe Natzke ◽  
Ashley Sakash ◽  
Tristan Mahr ◽  
Katherine C. Hustad

Purpose Accurate measurement of speech intelligibility is essential for children with speech production deficits, but wide variability exists in the measures and protocols used. The current study sought to examine relationships among measures of speech intelligibility and the capacity of different measures to capture change over time. Method Forty-five children with cerebral palsy (CP) with and without speech motor impairment were observed at ages 6, 7, and 8 years. The speech performance of each child was rated using four measures at each time point: standardized articulation test scores, multiword intelligibility scores obtained from naïve listeners, parent ratings of intelligibility, and percent intelligible utterances obtained from language transcripts. We analyzed the correlations of measures within each age and within three different severity groups, and we analyzed how these measures changed year over year in each severity group. Results For children with CP who have mild and moderate speech deficits, different measures of speech production were weakly associated, and for children with CP with severe speech impairment, these measures showed stronger associations. The four measures also differed in their ability to capture change over time. Finally, results from standardized assessments of articulation were not found to inform overall speech intelligibility for children with mild and moderate speech deficits. Conclusions Results suggest that speech production is not fully described by any single clinical measure. In order to adequately describe functional speaking abilities and to capture change over time, multiple levels of measurement are required.


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