This syndrome dates from the 1938 report by Wolfram and Wagener of a constellation of diabetes insipidus, diabetes mellitus, optic atrophy and sensorineural deafness, which gave rise to the acronym DIDMOAD.
Wolfram syndrome is inherited in an autosomal-recessive manner. The prevalence is believed to be approximately 1 in 500,000 children. A carrier of mutations causing Wolfram syndrome have a significantly higher chance of developing disturbances and may have a higher chance of developing diabetes and hearing impairment.
Wolfram syndrome is a heterogeneous condition, with two forms identified to date. These are designated Wolfram syndrome 1 and Wolfram syndrome 2 or WFS1 and WFS2 respectively.
Progressive optic atrophy is usually diagnosed before 12 years of age. Other clinical signs may appear at various ages before 25 years.
Wolfram syndrome
