The oral, pharyngeal, and esophageal stages of swallowing were evaluat
ed in 8 patients with recessively or dominantly inherited pure sensory
ataxia. Six patients had swallowing difficulties: solid bolus obstruc
tion, coughs during eating, and choking episodes. One patient had chro
nic bronchitis and another had recurrent pneumonia. The patients under
went a biphasic radiological barium swallow, including videofluoroscop
y. No patient had a completely normal swallow. All had normal oral fun
ction, whereas pharyngeal function was abnormal in 6 patients. Esophag
eal function was abnormal in 6 patients. The swallowing dysfunction di
d not correlate with the severity of motor or sensory dysfunction in t
he limbs, nor with age or duration of ataxia. Our study shows that swa
llowing dysfunction is common in hereditary sensory ataxia. This dysfu
nction is likely to be due to involvement of the nucleus of the solita
ry tract in the brainstem. Despite some of the patients having suffere
d from choking episodes and others from bronchopulmonary complications
, they did not spontaneously admit dysphagia. Swallowing should be eva
luated thoroughly in patients with hereditary sensory ataxia since dys
phagia in these patients might bring serious and potentially fatal com
plications.