DIDMOAD: An acronym that stands for Diabetes Insipidus (inability to concentrate the urine), Diabetes Mellitus , Optic Atrophy (degeneration of the nerve to the eye), and Deafness . These features are characteristic of this genetic disease which is also called the Wolfram syndrome . Patients usually also suffer from severe abnormalities of the nervous system that can be accompanied by behavior problems, psychiatric hospitalizations and, in about a quarter of cases, suicide attempts.

The DIDMOAD or Wolfram syndrome is inherited as an autosomal recessive trait so that brothers and sisters of a child with the syndrome each have a 25 percent chance of receiving the gene from both parents and having the syndrome. The syndrome is caused by a mutation in the gene responsible for the production of a protein called wolframin, resulting in loss of function of this protein. The gene is in chromosome 4p16.1.

Wolfram syndrome is genetically heterogeneous (mixed). There is, for example, a type of Wolfram syndrome with additional atypical features such as profound upper gastrointestinal ulceration and bleeding as well as an absence of diabetes insipidus . The gene for this type of Wolfram syndrome is not in the region of chromosome 4p16.1 but rather is in chromosome 4q22-24.

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