Related Experiment Video

Updated: Aug 26, 2025

Minimally Invasive Murine Laryngoscopy for Close-Up Imaging of Laryngeal Motion During Breathing and Swallowing
07:22

Minimally Invasive Murine Laryngoscopy for Close-Up Imaging of Laryngeal Motion During Breathing and Swallowing

Published on: December 1, 2023

627

Flexible endoscopic evaluation of swallowing in children with type 1 spinal muscular atrophy

Jana Zang1, Jessika Johannsen2, Jonas Denecke2

  • 1Department of Voice, Speech and Hearing Disorders, University Dysphagia Center, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, 20246, Hamburg, Germany. j.zang@uke.de.

European Archives of Oto-Rhino-Laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
|October 8, 2022
PubMed

Insights

Flexible endoscopic evaluation of swallowing (FEES) in infants with spinal muscular atrophy (SMA) revealed significant swallowing issues. Clinical scores did not correlate with swallowing function, indicating a need for better assessment tools.

Area of Science:

  • Pediatric Neurology
  • Gastroenterology
  • Speech-Language Pathology

Background:

  • Spinal muscular atrophy (SMA) type 1 significantly impacts motor function and can lead to severe swallowing difficulties (dysphagia).
  • Early diagnosis and intervention are crucial for managing SMA type 1, but assessing swallowing function in infants and toddlers presents challenges.

Purpose of the Study:

  • To implement and evaluate flexible endoscopic evaluation of swallowing (FEES) in infants and toddlers diagnosed with SMA type 1.
  • To compare FEES findings with established clinical swallowing and motor function scores.

Main Methods:

  • A prospective pilot study involving ten infants with SMA type 1 (two SMN2 copies) receiving approved SMA therapies.
  • FEES was conducted using the Penetration-Aspiration Scale (PAS) and Murray Secretion Scale. Motor function and other swallowing scales (CHOP-INTEND, NdSSS, OrSAT) were also assessed.

Main Results:

  • Eight out of ten infants presented with distinct swallowing disorders, including pharyngeal secretion pooling, penetration, aspiration, and delayed swallowing initiation.
  • Despite improvements in motor function (CHOP-INTEND), no corresponding improvement in swallowing function was observed.
  • Clinical swallowing scores did not show significant correlation with FEES-based penetration-aspiration severity.

Conclusions:

  • FEES provides valuable insights into dysphagia in infants with SMA type 1, especially regarding secretion management and limitations in oral intake.
  • Current clinical tools may reflect nutritional status changes but are insufficient for accurately assessing swallowing ability in this population.
  • Further development of sensitive and reliable clinical swallowing assessment tools for infants with SMA type 1 is necessary.
Abstract

Keywords:
DeglutitionFlexible endoscopic evaluation of swallowingNusinersenOnasemnogene abeparvovecRisdiplamSpinal muscular atrophyDysphagia

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