Oropharyngeal dysphagia and aspiration in patients with ataxia-telangiectasia

J Pediatr. 2000 Feb;136(2):225-31. doi: 10.1016/s0022-3476(00)70106-5.


Objectives: To determine whether patients with ataxia-telangiectasia exhibit oropharyngeal dysphagia with concomitant aspiration and to examine the relationships among swallowing function, age, and nutritional status.

Study design: Seventy patients (mean age, 10.7 years; range, 1.8 to 30 years) had feeding/swallowing and nutritional evaluations. Fifty-one patients, in whom there were concerns about swallowing safety, were examined with a standardized videofluoroscopic swallow study.

Results: Fourteen of the 51 patients (27%) with histories suggestive of dysphagia demonstrated aspiration. Of these, silent aspiration (aspiration without a cough) occurred in 10 (71%) patients. Aspirators were significantly older than non-aspirators (mean age, 16.9 vs 10.8 years; P =.002). Advancing age was the strongest factor associated with aspiration during continuous drinking (P =.01). In patients with ataxia-telangiectasia, weight and weight/height were abnormally low at all ages and most compromised in older patients. Patients who aspirated had significantly lower mean weight (P <.002) and weight/height z scores (P <.001) than did patients who did not aspirate.

Conclusions: Oropharyngeal dysphagia is common and appears to be progressive in patients with ataxia-telangiectasia. Older patients also have a higher incidence of poorer nutritional status. The relationship between dysphagia and nutritional status deserves further investigation.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Age Factors
  • Ataxia Telangiectasia / complications*
  • Ataxia Telangiectasia / physiopathology
  • Child
  • Cough / etiology
  • Deglutition / physiology
  • Deglutition Disorders / etiology*
  • Deglutition Disorders / physiopathology
  • Female
  • Humans
  • Male
  • Nutritional Status
  • Pneumonia, Aspiration / etiology*
  • Videotape Recording