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Harrison N. Jones

Associate Professor of Head and Neck Surgery & Communication Sciences
Communication Sciences
Box 3887 Med Ctr, Durham, NC 27710
155 Baker House, DUMC 3887, Durham, NC 27710

Selected Publications


Preoperative dysphagia and adverse postoperative outcomes in middle aged and older adults.

Journal Article J Clin Anesth · January 2025 BACKGROUND: Dysphagia is a swallowing impairment with adverse health consequences. The impact of preoperative dysphagia on postoperative outcomes is not known. This study will examine the association between preoperative dysphagia and postoperative outcome ... Full text Link to item Cite

Remote respiratory resistance exercise training improves respiratory function in individuals with VCP multisystem proteinopathy.

Journal Article Neuromuscul Disord · January 2024 Valosin-containing protein (VCP) disease is an autosomal dominant multisystem proteinopathy associated with hereditary inclusion body myopathy, Paget disease of bone, and frontotemporal dementia. Myopathy frequently results in respiratory muscle weakness, ... Full text Link to item Cite

Self-Reported Dysphagia and Psychosocial Health Among Community-Dwelling Older Adults: Results of a National Study.

Journal Article J Gen Intern Med · November 2023 BACKGROUND: The risk of dysphagia increases with age, affecting up to 33% of adults over the age of 65. Older adults with dysphagia are at increased risk for negative physical health outcomes such as aspiration pneumonia and death. However, the relationshi ... Full text Link to item Cite

Dysphagia in Older Adults is Associated With Food Insecurity and Being Homebound.

Journal Article J Appl Gerontol · September 2023 Objective: Our aim was to evaluate relationships between swallowing difficulty (dysphagia) and social determinants of health (SDOH) in older adults ≥65 years. Method: Cross-sectional analyses were performed in community-dwelling Medicare beneficiaries from ... Full text Link to item Cite

Validation of the Observer-Reported Communication Ability (ORCA) measure for individuals with Rett syndrome.

Journal Article Eur J Paediatr Neurol · September 2023 PURPOSE: The study goal was to validate the Observer-Reported Communication Ability (ORCA) measure for use with females with Rett Syndrome (RTT). METHODS: Qualitative interviews, including concept elicitation and cognitive interviewing methods, were conduc ... Full text Link to item Cite

Speech Disorders in Children With Pompe Disease: Articulation, Resonance, and Voice Measures.

Journal Article Am J Speech Lang Pathol · July 10, 2023 PURPOSE: Children with Pompe disease, a rare genetic metabolic myopathy, often have speech impairments. In this study, we provide a comprehensive description of articulation, resonance, and voice in children with Pompe disease. METHOD: Fifteen children wit ... Full text Link to item Cite

Validation of the Observer-Reported Communication Ability (ORCA) Measure for Individuals With Angelman Syndrome.

Journal Article Am J Intellect Dev Disabil · May 1, 2023 There is a critical need for high-quality clinical outcome assessments to capture the important aspects of communication ability of individuals with Angelman syndrome (AS). To center the perspective of caregivers, our team developed the novel Observer-Repo ... Full text Open Access Link to item Cite

Exploring Communication Ability in Individuals With Angelman Syndrome: Findings From Qualitative Interviews With Caregivers.

Journal Article Am J Intellect Dev Disabil · May 1, 2023 Communication deficits have a substantial impact on quality of life for individuals with Angelman syndrome (AS) and their families, but limited qualitative work exists to support the necessary content of measures aiming to assess communication for these in ... Full text Open Access Link to item Cite

Preoperative dysphagia risk in community-dwelling adults aged ≥50 years: Prevalence and risk factors.

Journal Article Nutr Clin Pract · February 2023 BACKGROUND: Preoperative dysphagia screening is rare. The purpose of this study was to assess the prevalence and potential risk factors of preoperative dysphagia risk in adults preparing for surgery. METHODS: The Eating Assessment Tool (EAT-10), Patient-Ge ... Full text Open Access Link to item Cite

Early clinical phenotype of late onset Pompe disease: Lessons learned from newborn screening.

Journal Article Mol Genet Metab · March 2022 PURPOSE: Thoroughly phenotype children with late-onset Pompe disease (LOPD) diagnosed via newborn screening (NBS) to provide guidance for long-term follow up. METHODS: Twenty infants ages 6-21 months with LOPD diagnosed by NBS underwent systematic clinical ... Full text Open Access Link to item Cite

Inspiratory Muscle Rehabilitation Training in Pediatrics: What Is the Evidence?

Journal Article Can Respir J · 2022 Pulmonary rehabilitation is typically used for reducing respiratory symptoms and improving fitness and quality of life for patients with chronic lung disease. However, it is rarely prescribed and may be underused in pediatric conditions. Pulmonary rehabili ... Full text Open Access Link to item Cite

Hypoventilation syndrome in neuromuscular disorders.

Journal Article Curr Opin Neurol · October 1, 2021 PURPOSE OF REVIEW: Hypoventilation syndrome in neuromuscular disorders (NMDs) is primarily due to respiratory muscle weakness and results in increased morbidity and mortality. This article highlights current aspects of neuromuscular hypoventilation syndrom ... Full text Open Access Link to item Cite

Assessment of Dysphonia in Children with Pompe Disease Using Auditory-Perceptual and Acoustic/Physiologic Methods.

Journal Article J Clin Med · August 16, 2021 Bulbar and respiratory weakness occur commonly in children with Pompe disease and frequently lead to dysarthria. However, changes in vocal quality associated with this motor speech disorder are poorly described. The goal of this study was to characterize t ... Full text Open Access Link to item Cite

Tongue weakness and atrophy differentiates late-onset Pompe disease from other forms of acquired/hereditary myopathy.

Journal Article Mol Genet Metab · July 2021 Late-onset Pompe disease (LOPD) is an inherited autosomal recessive progressive metabolic myopathy that presents in the first year of life to adulthood. Clinical presentation is heterogeneous, differential diagnosis is challenging, and diagnostic delay is ... Full text Open Access Link to item Cite

Association between Dysphagia and Surgical Outcomes across the Continuum of Frailty.

Journal Article J Nutr Gerontol Geriatr · 2021 This study examined the relationship between dysphagia and adverse outcomes across frailty conditions among surgical patients ≥50 years of age. A retrospective cohort analysis of surgical hospitalizations in the Healthcare Cost and Utilization Project's Na ... Full text Open Access Link to item Cite

Respiratory muscle training in late-onset Pompe disease: Results of a sham-controlled clinical trial.

Journal Article Neuromuscul Disord · November 2020 To address progressive respiratory muscle weakness in late-onset Pompe disease (LOPD), we developed a 12-week respiratory muscle training (RMT) program. In this exploratory, double-blind, randomized control trial, 22 adults with LOPD were randomized to RMT ... Full text Open Access Link to item Cite

Novel approaches to quantify CNS involvement in children with Pompe disease.

Journal Article Neurology · August 11, 2020 OBJECTIVE: To characterize the extent of CNS involvement in children with Pompe disease using brain MRI and developmental assessments. METHODS: The study included 14 children (ages 6-18 years) with infantile Pompe disease (IPD) (n = 12) or late-onset Pompe ... Full text Open Access Link to item Cite

Higher dosing of alglucosidase alfa improves outcomes in children with Pompe disease: a clinical study and review of the literature.

Journal Article Genet Med · May 2020 PURPOSE: Enzyme replacement therapy (ERT) with recombinant human acid-α glucosidase (rhGAA) at standard dose of 20 mg/kg every other week is insufficient to halt the long-term progression of myopathy in Pompe disease. METHODS: We conducted a retrospective ... Full text Open Access Link to item Cite

Training, detraining, and retraining: Two 12-week respiratory muscle training regimens in a child with infantile-onset Pompe disease.

Journal Article J Pediatr Rehabil Med · 2020 BACKGROUND: Respiratory muscle weakness is a primary cause of morbidity and mortality in patients with Pompe disease. We previously described the effects of our 12-week respiratory muscle training (RMT) regimen in 8 adults with late-onset Pompe disease [1] ... Full text Open Access Link to item Cite

Vocal Fold Paralysis/Paresis as a Marker for Poor Swallowing Outcomes After Thoracic Surgery Procedures.

Journal Article Dysphagia · December 2019 (1) To examine the association between vocal fold paresis/paralysis (VFP) and poor swallowing outcomes in a thoracic surgery cohort at the population level, and (2) to assess utilization of ENT/speech-language pathology intervention in these cases. The Nat ... Full text Open Access Link to item Cite

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Journal Article Mol Genet Metab · August 2019 INTRODUCTION:: Morbidity and mortality in adults with late-onset Pompe disease (LOPD) results primarily from persistent progressive respiratory muscle weakness despite treatment with enzyme replacement therapy (ERT). To address this need, we have developed ... Full text Open Access Link to item Cite

Auditory-Perceptual Speech Features in Children With Down Syndrome.

Journal Article Am J Intellect Dev Disabil · July 2019 Speech disorders occur commonly in individuals with Down syndrome (DS), although data regarding the auditory-perceptual speech features are limited. This descriptive study assessed 47 perceptual speech features during connected speech samples in 26 childre ... Full text Open Access Link to item Cite

Prevalence and resource utilization for vocal fold paralysis/paresis after esophagectomy.

Journal Article Laryngoscope · December 2018 OBJECTIVES/HYPOTHESIS: Vocal fold paralysis/paresis (VFP) is an uncommon but serious complication following esophagectomy. The objectives of this study were to: 1) identify the prevalence of VFP and associated complications after esophagectomy in the Unite ... Full text Open Access Link to item Cite

Challenges in measuring the effects of pharmacological interventions on cognitive and adaptive functioning in individuals with Down syndrome: A systematic review.

Journal Article Am J Med Genet A · November 2017 We systematically reviewed the measures used in pharmaceutical trials in children/adults with Down syndrome without dementia. Our purpose was to identify developmentally appropriate outcome measures capable of detecting changes in cognitive and adaptive fu ... Full text Open Access Link to item Cite

The emerging phenotype of late-onset Pompe disease: A systematic literature review.

Journal Article Mol Genet Metab · March 2017 BACKGROUND: Pompe disease is an autosomal recessive disorder caused by deficiency of the lysosomal glycogen-hydrolyzing enzyme acid α-glucosidase (GAA). The adult-onset form, late-onset Pompe disease (LOPD), has been characterized by glycogen accumulation ... Full text Open Access Link to item Cite

Respiratory muscle training in late-onset Pompe disease

Conference Molecular Genetics and Metabolism · January 2017 Full text Cite

Practical Recommendations for Diagnosis and Management of Respiratory Muscle Weakness in Late-Onset Pompe Disease.

Journal Article Int J Mol Sci · October 17, 2016 Pompe disease is an autosomal-recessive lysosomal storage disorder characterized by progressive myopathy with proximal muscle weakness, respiratory muscle dysfunction, and cardiomyopathy (in infants only). In patients with juvenile or adult disease onset, ... Full text Open Access Link to item Cite

Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): Effects of training and detraining.

Journal Article Mol Genet Metab · February 2016 BACKGROUND: Determine the effects of a 12-week respiratory muscle training (RMT) program in late-onset Pompe disease (LOPD). METHODS: We investigated the effects of 12-weeks of RMT followed by 3-months detraining using a single-subject A-B-A experimental d ... Full text Open Access Link to item Cite

Correlation between quantitative whole-body muscle magnetic resonance imaging and clinical muscle weakness in Pompe disease.

Journal Article Muscle Nerve · May 2015 INTRODUCTION: Previous examination of whole-body muscle involvement in Pompe disease has been limited to physical examination and/or qualitative magnetic resonance imaging (MRI). In this study we assess the feasibility of quantitative proton-density fat-fr ... Full text Open Access Link to item Cite

Quantitative assessment of lingual strength in late-onset Pompe disease.

Journal Article Muscle Nerve · May 2015 INTRODUCTION: Skeletal muscle is common in late-onset Pompe disease (LOPD). Recent data implicate common bulbar muscle involvement (i.e., the tongue). METHODS: We used quantitative assessment of lingual strength to retrospectively determine the frequency a ... Full text Open Access Link to item Cite

Effects of respiratory muscle training (RMT) in children with infantile-onset Pompe disease and respiratory muscle weakness.

Journal Article J Pediatr Rehabil Med · 2014 PURPOSE: Respiratory muscle weakness is a primary therapeutic challenge for patients with infantile Pompe disease. We previously described the clinical implementation of a respiratory muscle training (RMT) regimen in two adults with late-onset Pompe diseas ... Full text Open Access Link to item Cite

Oropharyngeal dysphagia may occur in late-onset Pompe disease, implicating bulbar muscle involvement.

Journal Article Neuromuscul Disord · April 2013 Late-onset Pompe disease (presenting after 12 months of age) often presents with limb-girdle and respiratory weakness, but oropharyngeal dysphagia has not been reported previously. A retrospective review of all late-onset Pompe disease patients evaluated i ... Full text Open Access Link to item Cite

Bulbar muscle weakness and fatty lingual infiltration in glycogen storage disorder type IIIa.

Journal Article Mol Genet Metab · November 2012 Glycogen storage disorder type III (GSD III) is a rare autosomal recessive disorder resulting from a deficiency of glycogen debranching enzyme, critical in cytosolic glycogen degradation. GSD IIIa, the most common form of GSD III, primarily affects the liv ... Full text Open Access Link to item Cite

The emerging phenotype of long-term survivors with infantile Pompe disease.

Journal Article Genet Med · September 2012 PURPOSE: Enzyme replacement therapy with alglucosidase alfa for infantile Pompe disease has improved survival creating new management challenges. We describe an emerging phenotype in a retrospective review of long-term survivors. METHODS: Inclusion criteri ... Full text Open Access Link to item Cite

Auditory-perceptual analysis of dysarthria in bilateral striatopallidodentate calcinosis (fahr's disease)

Journal Article Journal of Medical Speech-Language Pathology · March 1, 2012 A 53-year-old woman with bilateral striatopallidodentate calcinosis (BSPDC) was referred for motor speech examination, and high-quality acoustic recordings were obtained. Three speech-language pathologists experienced in auditory-perceptual assessment of s ... Open Access Cite

Early cognitive development in children with infantile Pompe disease.

Journal Article Mol Genet Metab · March 2012 This report describes the cognitive development of 17 children with infantile Pompe disease who participated in a 52-week clinical trial of enzyme replacement therapy (ERT) via biweekly infusion of Myozyme® (alglucosidase alfa). Subjects were six months of ... Full text Open Access Link to item Cite

Intensive treatment of dysarthria in two adults with Down syndrome.

Journal Article Dev Neurorehabil · 2012 OBJECTIVE: This study investigated the impact of an established behavioural dysarthria treatment on acoustic and perceptual measures of speech in two adults with Down syndrome (DS) and dysarthria to obtain preliminary measures of treatment effect, effect s ... Full text Open Access Link to item Cite

Expanding the phenotype of late-onset Pompe disease: tongue weakness: a new clinical observation.

Journal Article Muscle Nerve · December 2011 INTRODUCTION: Following the clinical observation of lingual weakness in 2 patients with late-onset Pompe disease (LOPD), tongue strength was assessed in 19 consecutive patients to determine the frequency and severity of this neurological sign. METHODS: Lin ... Full text Open Access Link to item Cite

Communication impairments in parkinson's disease

Journal Article Parkinson's Disease · December 1, 2011 Full text Cite

Increased inspiratory and expiratory muscle strength following respiratory muscle strength training (RMST) in two patients with late-onset Pompe disease.

Journal Article Mol Genet Metab · November 2011 Respiratory muscle strength training (RMST) is an exercise-based intervention which targets respiratory muscle weakness. We implemented RMST in two patients with late-onset Pompe disease (LOPD), both who had received long-term enzyme replacement therapy an ... Full text Open Access Link to item Cite

Multidisciplinary assessment and diagnosis of conversion disorder in a patient with foreign accent syndrome.

Journal Article Behav Neurol · 2011 Multiple reports have described patients with disordered articulation and prosody, often following acute aphasia, dysarthria, or apraxia of speech, which results in the perception by listeners of a foreign-like accent. These features led to the term foreig ... Full text Open Access Link to item Cite

Oropharyngeal dysphagia in infants and children with infantile Pompe disease.

Journal Article Dysphagia · December 2010 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) ... Full text Open Access Link to item Cite

Speech motor program maintenance, but not switching, is enhanced by left-hemispheric deep brain stimulation in Parkinson's disease.

Journal Article Int J Speech Lang Pathol · October 2010 Speech reaction time (SRT) was measured in a response priming protocol in 12 participants with Parkinson's disease (PD) and hypokinetic dysarthria "on" and "off" left-hemispheric deep brain stimulation (DBS). Speech preparation was measured during speech m ... Full text Open Access Link to item Cite

Fundamental frequency and intensity mean and variability before and after two behavioral treatments for aprosodia

Journal Article Journal of Medical Speech-Language Pathology · March 1, 2009 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 ... Open Access Cite

Language and speech function in children with infantile Pompe disease

Journal Article Journal of Pediatric Neurology · January 1, 2009 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 p ... Full text Open Access Cite

Expiratory muscle strength training in the treatment of mixed dysarthria in a patient with Lance-Adams syndrome

Journal Article Journal of Medical Speech-Language Pathology · September 1, 2006 The results of an expiratory muscle strength training (EMST) program is described in the rehabilitation of mixed dysarthria in a patient with Lance-Adams syndrome secondary to a motor vehicle accident (MVA) with associated traumatic brain injury (TBI). A p ... Cite