Similar Diseases
- WFS2
- Diabetes Insipidus and Mellitus With Optic Atrophy and Deafness
- Cerebrooculofacioskeletal syndrome 2
- Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness Syndrome
- Neu-Laxova syndrome 2
- EARLOBES, THICKENED, WITH CONDUCTIVE DEAFNESS FROM INCUDOSTAPEDIAL ABNORMALITIES
- HYC2
- UFS2
- Cerebrooculofacioskeletal syndrome 3
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Wolfram syndrome – Abstract
Wolfram syndrome, also called DIDMOAD (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.
It was first described in four siblings in 1938 by Dr. Don J. Wolfram, M.D. The disease affects the central nervous system (especially the brainstem).
Wolfram syndrome – Prognosis
The first symptom is typically diabetes mellitus, which is usually diagnosed around the age of 6. The next symptom to appear is often optic atrophy, the wasting of optic nerves, around the age of 11. The first signs of this are loss of colour vision and peripheral vision. The condition worsens over time, and people with optic atrophy are usually blind within 8 years of the first symptoms. Life expectancy of people suffering from this syndrome is about 30 years.