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Hypoglossal Nerve Stimulation in Adolescents With Down Syndrome and Obstructive Sleep Apnea
Gillian R Diercks1,2, Carissa Wentland3,4, Donald Keamy1,2,5
1Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston.
Insights
Hypoglossal nerve stimulation is a safe and effective treatment for children with Down syndrome (DS) and obstructive sleep apnea (OSA). This therapy significantly reduced apnea events and improved quality of life in adolescents with DS.
Area of Science:
- Pediatric Otolaryngology
- Sleep Medicine
- Genetics
Background:
- Obstructive sleep apnea (OSA) affects up to 60% of children with Down syndrome (DS).
- Persistent OSA in children with DS often leads to poor tolerance of positive airway pressure devices or tracheotomy.
- Hypoglossal nerve stimulation offers a potential solution for upper airway obstruction.
Purpose of the Study:
- To evaluate the safety and efficacy of hypoglossal nerve stimulation in children with Down syndrome.
Main Methods:
- A case series involving 6 adolescents (12-18 years) with DS and severe OSA post-adenotonsillectomy.
- Implantation of the Inspire hypoglossal nerve stimulator.
- Monitoring for adverse events, adherence, and efficacy via apnea-hypopnea index (AHI) and OSA-18 quality-of-life scores.
Main Results:
- Hypoglossal nerve stimulator therapy was well-tolerated, with device use ranging from 5.6 to 10.0 hours/night.
- Significant reduction in AHI (56%-85%), with 4 children achieving <5 events/h and 2 achieving <10 events/h.
- Clinically significant improvement in quality of life as measured by the OSA-18 instrument.
Conclusions:
- Hypoglossal nerve stimulation is a safe and effective treatment option for children with DS and refractory OSA.
- This therapy is suitable for patients with DS who cannot tolerate positive pressure airway devices.
- It represents a promising therapeutic advancement for managing severe OSA in this population.
Importance:
Obstructive sleep apnea (OSA) affects up to 60% of children with Down syndrome (DS) and may persist in half of patients after adenotonsillectomy. Children with DS who have persistent OSA often do not tolerate treatment with positive pressure airway support devices or tracheotomy for their residual moderate to severe OSA. The hypoglossal nerve stimulator is an implantable device that delivers an electrical impulse to anterior branches of the hypoglossal nerve in response to respiratory variation, resulting in tongue base protrusion that alleviates upper airway obstruction in adults.
Objective:
To determine whether hypoglossal nerve stimulation is safe and effective in children with DS.
Design, Setting, And Participants:
Case series of the first 6 adolescents with DS to undergo hypoglossal nerve stimulator implantation. Participants were 6 children and adolescents (12-18 years) with DS and severe OSA (apnea hypopnea index [AHI] > 10 events/h) despite prior adenotonsillectomy.
Intervention:
Inspire hypoglossal nerve stimulator placement.
Main Outcomes And Measures:
Patients were monitored for adverse events. Adherence to therapy was measured by hours of use recorded by the device. Efficacy was evaluated by comparing AHI and OSA-18, a validated quality-of-life instrument, scores at baseline and follow-up.
Results:
In 6 patients (4 male, 2 female; aged 12-18 years), hypoglossal nerve stimulator therapy was well tolerated (mean use, 5.6-10.0 h/night) and effective, resulting in significant improvement in OSA. At 6- to 12-month follow-up, patients demonstrated a 56% to 85% reduction in AHI, with an overall AHI of less than 5 events/h in 4 children and less than 10 events/h in 2 children. Children also demonstrated a clinically significant improvement (mean [SD] overall change score, 1.5 [0.6]; range, 0.9-2.3) on the OSA-18, a validated quality-of-life instrument.
Conclusions And Relevance:
Hypoglossal nerve stimulation was well tolerated and effective in the study population, representing a potential therapeutic option for patients with DS and refractory OSA after adenotonsillectomy who are unable to tolerate positive pressure airway devices.
Trial Registration:
clinicaltrials.gov Identifier: NCT2344108.
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