Details of Disease

General Information of Disease (ID: DISI95DW)

• Disease Name: Wolfram-like syndrome
• Synonyms: hearing loss, progressive, with optic atrophy and/or impaired glucose Regulation; Wolfram-like syndrome, autosomal dominant; WFSL; Wolfram-like syndrome
• Definition: Wolfram-like syndrome is a rare endocrine disease characterized by the triad of adult-onset diabetes mellitus, progressive hearing loss (usually presenting in the first decade of life and principally of low to moderate frequencies), and/or juvenile-onset optic atrophy. Psychiatric (i.e. anxiety, depression, hallucinations) and sleep disorders, the only neurologic abnormalities observed in this disease, have been reported in rare cases. Unlike Wolfram syndrome, patients with Wolfram-like syndrome do not report endocrine or cardiac findings.
• Disease Hierarchy:
  DISRGY2N: Endocrine disease
  DISYKSRF: Genetic disease
  DISI95DW: Wolfram-like syndrome
• Disease Identifiers:
  MONDO ID: MONDO_0013673
  MESH ID: C565631
  UMLS CUI: C3280358
  OMIM ID: 614296
  MedGen ID: 481988
  Orphanet ID: 411590

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 1 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
WFS1	OTYF2UYI	Definitive	Autosomal dominant	[1]

References

• [1] Technical standards for the interpretation and reporting of constitutional copy-number variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics (ACMG) and the Clinical Genome Resource (ClinGen). Genet Med. 2020 Feb;22(2):245-257. doi: 10.1038/s41436-019-0686-8. Epub 2019 Nov 6.