Training using continuous positive airway pressure (CPAP) improved speech in a small cohort of children with infantile-onset Pompe disease (IOPD). The study, published in Scientific Reports, showed that a speech training protocol that included increasing pressure and time wearing a CPAP improved the degree of hypernasality and speech intelligibility scores in 3 patients.

The authors highlighted the fact that while some other studies had used CPAP to improve speech, this was the first study to use CPAP speech training in patients with progressive neuromuscular disorders.

The authors summarized, “This study suggests that speech disorders in children with IOPD improved following CPAP training, especially in terms of hypernasality and speech intelligibility. Even with the cessation of CPAP training, the benefit was largely maintained during an 8-week follow-up period.”

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According to the authors, the increased pressure from the CPAP provides extra resistance during speech training and can improve the strength of velopharyngeal closure muscles including the levator palatini muscles, palatoglossus muscles, and palatopharyngeus muscles.

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All participants underwent an 8-week speech therapy phase (baseline), followed by an 8-week phase where CPAP was added to the speech therapy, and a final 8-week speech therapy phase without CPAP (maintenance).

Hypernasality improved in all 3 participants during the CPAP phase, and this improvement continued or was stationary during the maintenance phase in 2 of these patients (Patients 1 and 3). The hypernasality of Patient 2 worsened during the maintenance phase but was still improved compared to levels during the baseline phase.

All 3 patients also showed improved speech intelligibility scores during the CPAP phase. There was variability in the score changes during the maintenance phase, however, with Patient 1 continuing to improve, Patient 3 maintaining their speech intelligibility scores from the CPAP phase, and Patient 2 maintaining their improved scores for only 3 sessions before returning to baseline levels.

The study also investigated changes to the percentage of consonants correct and observed improvements from baseline levels. Due to high degrees of variability within the patients, however, these measurements were not statistically different during the different phases.

Five children with IOPD who had received enzyme replacement therapy at an early age were initially recruited for the study. Only 3 of the children completed the program, however, and were used in the analysis. The participants received speech therapy and were assessed once per week for 24 weeks.

During the CPAP portion of the study, participants also received caregiver-administered CPAP speech training 6 days per week, where the patients had to produce a set of 75 speech samples and read 10 short sentences while wearing a CPAP. The amount of time the CPAP was worn and the pressure that was applied was gradually increased over the 8-week period.


Zeng YT, Liu WY, Torng PC, et al. A pilot study shows the positive effects of continuous airway pressure for treating hypernasal speech in children with infantile-onset Pompe disease. Sci Rep. 2021;11(1):18826. doi:10.1038/s41598-021-97877-1