Abstract
Children with infantile-onset Pompe disease (IOPD) demonstrate hypernasality. This study aimed to evaluate whether continuous positive airway pressure (CPAP) training may reduce hypernasality in children with IOPD. Five children with IOPD were enrolled in a single-subject experimental design of type A-B-A′. The intervention comprised an 8-week, 6-day-per-week regimen of CPAP training at home. Participants continued traditional speech therapy once per week throughout the 24-week study duration. The outcome measurements included the degree of hypernasality (DH), the percentage of consonants correct (PCC), and the speech intelligibility score (SIS). C-statistic analysis with an α of 0.05 was used along with visual analysis to assess speech changes. Three patients completed the study. During the CPAP training phase, the DH, PCC, and SIS were significantly improved compared with the baseline (p < 0.05). At the follow-up phase, both DH and SIS were improved compared with the baseline (p < 0.05), but the PCC had returned to the baseline level. CPAP training demonstrated effectiveness in reducing nasal sounds in IOPD patients. Further studies training younger children with normal hearing may help elucidate the persistence of the effects in children with IOPD.
Highlights
Children with infantile-onset Pompe disease (IOPD) demonstrate hypernasality
Each movement results from the synergistic activity of several muscles; for example, the levator veli palatini serves as a sling to pull the velum up and back toward the posterior pharyngeal wall; the palatoglossus brings the velum down for nasal consonants; the palatopharyngeus narrows the pharynx by pulling the lateral pharyngeal walls upward and medially
We aimed to train the muscles involved in velopharyngeal closure in children with IOPD using continuous positive airway pressure (CPAP) training to determine whether hypernasality, articulation disorders, and speech intelligibility could be improved further with this method than with traditional speech therapy alone
Summary
Children with infantile-onset Pompe disease (IOPD) demonstrate hypernasality. This study aimed to evaluate whether continuous positive airway pressure (CPAP) training may reduce hypernasality in children with IOPD. Abbreviations IOPD Infantile-onset Pompe disease ERT Enzyme replacement therapy CPAP Continuous positive airway pressure DH Degree of hypernasality PCC Percentage of consonants correct SIS Speech intelligibility score. Significantly disabling speech disorders, including hypernasality, articulation disorder, and impaired speech intelligibility, frequently occur in children with infantile-onset Pompe disease (IOPD), even with early treatment or high-dosage E RT3–6. Previous research demonstrated that CPAP training effectively reduces hypernasality[11,12,13,14,15] and strengthens the muscles involved in velopharyngeal c losure[15,16]. There have been no studies using CPAP as a treatment for hypernasality in children with progressive deterioration in muscle/neuronal function, such as patients with IOPD or other neuromuscular disorders
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