Wolfram Syndrome-Ataxia and Dysphagia

Wolfram syndrome is characterized by progressive neurodegeneration. The most common problem is ataxia. Cerebellar ataxia should be assessed yearly or twice a year by neurologists. Dysarthria and swallowing disorders (dysphagia) are commonly seen. Swallowing treatment by a speech-language pathologist is beneficial for patients to prevent aspiration pneumonia. Surgical procedures, including esophageal dilation and esophagomyotomy, have been shown to be effective in some patients. Oral hygiene and dental care are important because dysphagia may lead to the impaired clearance of organisms and pathogenic colonization.