Pompe Disease
Conditions
Brief summary
Patients with infantile onset Pompe disease will be trained with continuous positive airway pressure to see if hypernasality can be improved.
Interventions
CPAP training for muscle strength
Sponsors
Study design
Eligibility
Inclusion criteria
1. confirm as Pompe disease 2. Age 4 and can speak 2-words sentence by order 3. mild to severe hypernasality 4. have regular speech therapy for 8 weeks or longer
Exclusion criteria
(1)hearing loss (2)congenital pharyngeal or laryngeal structure anomalies including cleft palate (3)cerebral palsy (4)recent otitis media effusion (5)very severe hypernasality (6)use non-invasive ventilator
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| decrease in hypernasality | 6 months | score hypernasality degree and compare |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| increase in speech intelligibility | 6 months | score speech intelligibility degree and compare |
Countries
Taiwan