GTR Home > Conditions/Phenotypes > Wolfram-like syndrome, autosomal dominant


Autosomal dominant Wolfram-like syndrome is characterized by the clinical triad of congenital progressive hearing impairment, diabetes mellitus, and optic atrophy. The hearing impairment, which is usually diagnosed in the first decade of life, is relatively constant and alters mainly low- and middle-frequency ranges (summary by Valero et al., 2008). Wolfram syndrome (WFS1; 222300) is an autosomal recessive allelic disorder characterized by optic atrophy, diabetes mellitus, hearing loss, and diabetes insipidus, and is caused by homozygous or compound heterozygous mutation in the WFS1 gene. An autosomal dominant syndrome involving optic atrophy with or without deafness, ophthalmoplegia, myopathy, ataxia, and neuropathy (125250), is caused by heterozygous mutation in the OPA1 gene (605290). [from OMIM]

Available tests

29 tests are in the database for this condition.

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Genes See tests for all associated and related genes

  • Also known as: CTRCT41, WFRS, WFS, WFSL, WFS1
    Summary: wolframin ER transmembrane glycoprotein

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