Browsing by Author "Jones, HN"

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    Fundamental frequency and intensity mean and variability before and after two behavioral treatments for aprosodia
    (Journal of Medical Speech-Language Pathology, 2009-03-01) Jones, HN; Shrivastav, R; Wu, SS; Plowman-Prine, EK; Rosenbek, JC
    Emerging data suggest that aprosodia may be amenable to behavioral treatment. This study investigated the use of acoustic analysis of speech to quantify response to two speech treatments previously judged to have an effect based on perceptual assessment in three participants with primarily expressive aprosodia. The mean and variability of fundamental frequency (F0) and intensity (INT) during production of sentences requiring use of four different emotional tones of speech (i.e., happy, angry, sad, or neutral) was calculated before and after two mechanism-based treatments for aprosodia (i.e., TX1 and TX2). Statistical differences in F0 mean and variability were primarily observed following TX1, whereas differences in INT mean and variability were principally revealed following TX2. Additionally, significant differences in these acoustic values were noted across almost all pairwise comparisons of emotional sentence types (i.e., angry vs. sad, happy vs. sad, neutral vs. sad, angry vs. neutral, and happy vs. neutral). These preliminary data suggest that perceptual improvements in aprosodia can be measured quantitatively using acoustic analysis of speech and provide additional support for previously described behavioral treatments for this disorder. These findings also support previous reports that suggest that different emotional tones of speech are associated with differences in the acoustic speech signal. Copyright © 2009 Delmar Cengage Learning.
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    Language and speech function in children with infantile Pompe disease
    (Journal of Pediatric Neurology, 2009-01-01) Muller, CW; Jones, HN; O'Grady, G; Suárez, HA; Heller, JH; Kishnani, PS
    Pompe disease, also known as glycogen storage disease type II and acid maltase deficiency, is a rare autosomal recessive progressive neuromuscular disorder. The natural course of the infantile form of this condition has resulted in mortality for patients prior to 1 year of age, making investigations into language and speech function in this population impossible. However, with the advent of treatment with enzyme replacement therapy (ERT) using alglucosidase alfa (Myozyme®), the lifespan of children with this condition has been extended. A retrospective study of the language and speech skills of 12 children enrolled in clinical trials for treatment with ERT at a tertiary care center was completed. Standardized language assessment instruments were administered to all participants, and six of the 12 were assessed twice. At initial assessment, overall language function was found to be age appropriate in 58% of participants, while, in those who received reassessment, overall normal language function was seen in 83%. Speech assessments were completed during all visits in which subjects were 24 months or older. Articulatory disorders and/or hypernasality were commonly encountered and were exhibited in 82% of speech assessments. Disorders in language and/or speech were found in all participants at some point in the course of the study. Overall, language delays tended to improve with time. Speech disorders were encountered more commonly, were often severe, and appeared to be motoric in nature. Children with infantile Pompe disease treated with ERT appear to be at high risk for speech disorders in particular. Further systematic investigations are needed. © 2009 IOS Press. All rights reserved.
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    Oropharyngeal Dysphagia in Infants and Children with Infantile Pompe Disease.
    (Dysphagia, 2009-09) Jones, HN; Muller, CW; Lin, M; Banugaria, SG; Case, LE; Li, JS; O'Grady, G; Heller, JH; Kishnani, PS
    Pompe disease is a rare genetic progressive neuromuscular disorder. The most severe form, infantile Pompe disease, has historically resulted in early mortality, most commonly due to cardiorespiratory failure. Treatment with enzyme replacement therapy (ERT) using alglucosidase alfa (Myozyme((R))) has extended the lifespan of individuals with this disease. With the introduction of ERT and the resultant improved survival, dysphagia is being encountered clinically with increasing regularity though systematic data remain unavailable. We retrospectively studied the oropharyngeal swallowing of 13 infants and children with Pompe disease using videofluoroscopy before initiation of ERT, allowing for baseline swallow function to be established in an untreated cohort. Dysphagia was present in all 13 subjects, even in a participant only 15 days old. Oral stage signs were present in 77%, most frequently a weak suck in 69%. Pharyngeal stage signs were present in 100%, including a pharyngeal swallow delay in 92% and pharyngeal residue in 77%. Airway invasion was present in 76.9% of subjects, including penetration in five (38.46%) and silent aspiration in an additional five (38.46%). No relationship in the relative involvement of swallowing, gross motor function, and cardiac disease appeared to be present.