Wolfram syndrome

Wolfram syndrome, also called DIDMOAD for diabetes insipidus, diabetes mellitus, optic atrophy and deafness, is a rare autosomal recessive genetic disorder that causes childhood-onset diabetes mellitus, optic atrophy and deafness as well as various other possible disorders including neurodegeneration. It is caused by mutations in the WFS1 or CISD2 genes, with WFS1 mutations being more common, and was first described in 1938 by Dr. Don J. Wolfram, who documented the condition in four siblings. Symptoms typically begin between ages 2 and 65, with diabetes mellitus usually appearing around age 6, and the condition affects fewer than 5,000 people in the United States.