scholarly journals Treating Hypernasal Speech in Children with Infantile-Onset Pompe Disease: Effectiveness of Continuous Positive Airway Pressure

Author(s):  
Yin-Ting Zeng ◽  
Wen-Yu Liu ◽  
Pao-Chuan Torng ◽  
Wuh-Liang Hwu ◽  
Ni-Chung Lee ◽  
...  

Abstract Children with infantile-onset Pompe disease (IOPD) demonstrate hypernasality. This study aimed to evaluate whether continuous positive airway pressure (CPAP) training, effective in reducing hypernasality in subjects with velopharyngeal insufficiency, may reduce hypernasality in children with IOPD. Five children with IOPD were enrolled to partake in an 8-week, 6-day-per-week regimen of CPAP training at home, involving 8 week baseline, 8 week CPAP training, and 8 week follow-up outcome assessment combined with regular speech therapy once per week. The outcomes 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 .05 (z ≥ 1.645) and visual analysis were used 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 ≤ .05). At the follow-up phase, both DH and SIS were improved compared with the baseline (p ≤ .05), but the PCC had returned to the baseline level (p ≥ .05). CPAP training demonstrated effectiveness in reducing nasal sounds in IOPD patients. Further studies involving training younger children with normal hearing may help elucidate the persistence of the effects in children with IOPD.

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yin-Ting Zeng ◽  
Wen-Yu Liu ◽  
Pao-Chuan Torng ◽  
Wuh-Liang Hwu ◽  
Ni-Chung Lee ◽  
...  

AbstractChildren 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.


2021 ◽  
Vol 2 (Supplement_1) ◽  
pp. A66-A67
Author(s):  
A Sweetman ◽  
L Lack ◽  
S Smith ◽  
C Chai-Coetzer ◽  
P Catcheside ◽  
...  

Abstract Introduction Co-morbid insomnia and sleep apnoea (COMISA) is a prevalent and debilitating condition that is difficult to treat. COMISA patients have lower average adherence to continuous positive airway pressure (CPAP) therapy compared to patients with sleep apnoea alone. However, a sub-sample of COMISA patients may show adequate CPAP use that improves both the insomnia and sleep apnoea. It is important to identify this group of CPAP-responsive COMISA patients to guide personalised-medicine approaches. Methods Seventy-three COMISA patients (AHI≥15; ICSD-3 insomnia; 55% male, Age M=57y) completed questionnaires, home-based polysomnography, and one-week sleep diaries before and 6-months after commencing CPAP therapy. No patients accessed CBTi. We investigated baseline predictors of CPAP adherence (min/night) and overall change in Insomnia Severity Index (ISI) scores during treatment. Results Average CPAP adherence was 205 minutes/night (SD=153). 56% of patients used CPAP at least 4h/night. Average CPAP adherence was predicted by higher baseline AHI (r=0.39), arousal index (r=0.28), N1 sleep (r=0.32) and age (r=0.26), and lower N3 sleep (r=-0.28). The ISI decreased from baseline (17.9, CI=1.2) to 6-month follow-up (11.6, CI=1.3; p&lt;0.001). There was a significant positive association between ISI reduction and CPAP use (r=0.31). 26% of patients reported an ISI&lt;8 at 6-month follow-up. Conclusion Approximately half of COMISA patients show CPAP adherence of ≥4h/night and one quarter experience insomnia remission with CPAP. CPAP use is positively associated with AHI, light sleep, and age at baseline, and reduction of insomnia severity during treatment. Future randomized controlled trials are required to confirm the results of this small un-controlled study.


2020 ◽  
Vol 16 (3) ◽  
pp. 353-359 ◽  
Author(s):  
Julia A. M. Uniken Venema ◽  
Michiel H. J. Doff ◽  
Dilyana Joffe-Sokolova ◽  
Peter J. Wijkstra ◽  
Johannes H. van der Hoeven ◽  
...  

SLEEP ◽  
2013 ◽  
Vol 36 (9) ◽  
pp. 1289-1296 ◽  
Author(s):  
Michiel H. J. Doff ◽  
Aarnoud Hoekema ◽  
Peter J. Wijkstra ◽  
Johannes H. van der Hoeven ◽  
James J. R. Huddleston Slater ◽  
...  

2014 ◽  
Vol 44 (5) ◽  
pp. 1262-1274 ◽  
Author(s):  
Izolde Bouloukaki ◽  
Katerina Giannadaki ◽  
Charalampos Mermigkis ◽  
Nikolaos Tzanakis ◽  
Eleni Mauroudi ◽  
...  

We aimed to compare the effect of intensiveversusstandard interventions on continuous positive airway pressure (CPAP) adherence 2 years after CPAP initiation, as well as on sleepiness, quality of life, depression, hospitalisation and death rate due to cardiovascular disease (CVD).3100 patients with newly diagnosed sleep apnoea were randomised into the standard group, with usual follow-up care, or the intensive group, with additional visits, telephone calls and education. Subjective daytime sleepiness (Epworth Sleepiness Scale; ESS), quality of life (36-item Short Form Health Survey; SF-36) and the patient’s level of depression (Beck Depression Inventory; BDI) were recorded before and 2 years after CPAP initiation, together with CVD hospitalisations and death rate.2 years after CPAP initiation, the intensive group used CPAP significantly more than the standard group (6.9versus5.2 h per night; p<0.001). ESS, SF-36 and BDI scores were also significantly better in the intensive group. Furthermore, the standard group had significantly more deaths and hospitalisations due to CVD.CPAP usage can be improved by both intensive and standard patient support. However, the patients who received intensive CPAP support had significantly better ESS, BDI and SF-36 scores, and lower cardiovascular morbidity and mortality, suggesting that an intensive programme could be worthwhile.


Hypertension ◽  
2021 ◽  
Vol 78 (Suppl_1) ◽  
Author(s):  
Oksana Rekovets ◽  
Yuriy Sirenko ◽  
Nina Krushynska ◽  
Olena Torbas ◽  
Svitlana Kushnir ◽  
...  

The aim was to assess the arterial stiffness changes in patients with resistant arterial hypertension (AH) and obstructive sleep apnea (OSA) and possibilities of its correction by continuous positive airway pressure (CPAP)-therapy. Design: In 10 month follow-up study were included 46 patients with RAH, who were divided into groups: 1-st - patients with RAH and moderate to severe OSA on CPAP (n=21); 2-nd - patients with RAH and moderate to severe OSA without CPAP (n=25). They underwent somnography by dual-channel portable monitor device, office and ambulatory blood pressure monitoring, echocardiography and applanation tonometry. All patients received similar antihypertensive therapy according to 2013 ESH Guidelines for the management of arterial hypertension. Results: Patients with RAH and OSA (mean apnea-hypopnea index (AHI) 36.5±2.7 event h-1) in comparison with patients with RAH without OSA (mean AHI 3.4±0.2 event h-1) had significantly higher body mass index (34.2±0.7 vs 31.6±0.7 kg m-2, P<0.05), uric acid level (6.7±0.1 vs 5.6±0.4 mg dl-1, P<0,05)). Patients with RAH and OSA in comparison with patients with RAH without OSA had higher carotid-femoral pulse wave velocity (PWVcf) (12.1±0.5 vs 10.2 m s-1, P<0,05) and central systolic blood pressure (CSBP) (143.8±2.7 vs 136.2±3.4 mm Hg, P<0,05). During 10 months follow-up in patients with RAH and moderate and sever OSA on CPAP-therapy there were significantly decrease of PWVcf (from 12.1±0.5 to 10.5±0.5 m s-1, P<0,05), decrease office systolic blood pressure (from 147.8±3.7 to 136.7±2.8 mm Hg; P<0,05) and diastolic blood pressure (from 96.8±3.5 to 87.0±3.3 mm Hg; P<0,05) with achievement of target levels in 67,2% patients. Central systolic BP decreased (from 143.8±2.7 to 137.7±2.8 mm Hg; P<0,05). Conclusion: The combination of therapy continuous positive airway pressure with antihypertensive treatment in patients with resistant arterial hypertension and moderate to severe obstructive sleep apnea improved achievement of target blood pressure, decreased arterial stiffness and decreased central blood pressure.


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