Neuroimaging features in Wolfram syndrome type 1

Neurol Sci. 2024 Jun;45(6):2943-2944. doi: 10.1007/s10072-024-07352-8. Epub 2024 Jan 31.


Wolfram syndrome type 1 is a rare autosomal recessive genetic disorder which is characterized by the co-existence of diabetes insipidus, diabetes mellitus, optic atrophy, and deafness, and hence is also referred to as the acronym DIDMOAD. In this neuroimage, the typical neuroimaging features of a genetically confirmed case of Wolfram syndrome type 1 are presented. The presence of left-sided vestibulocochlear dysplasia is a novel finding in our case which has not been reported previously.

Keywords: Acronym DIDMOAD; Vestibulocochlear dysplasia; Wolfram syndrome type 1.

Publication types

  • Case Reports

MeSH terms

  • Brain / diagnostic imaging
  • Humans
  • Magnetic Resonance Imaging
  • Neuroimaging* / methods
  • Wolfram Syndrome* / complications
  • Wolfram Syndrome* / diagnostic imaging
  • Wolfram Syndrome* / genetics