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Sensorineural deafness with dilated cardiomyopathy

Disease definition

Sensorineural deafness with dilated cardiomyopathy is an extremely rare autosomal dominant syndrome described in two families to date and characterized by moderate to severe sensorineural hearing loss manifesting during childhood, and associated with late-onset dilated cardiomyopathy that generally progresses to heart failure.


Classification level: Disorder
  • Synonym(s):
    • Neurosensory deafness with dilated cardiomyopathy
    • Neurosensory hearing loss with dilated cardiomyopathy
    • Sensorineural hearing loss with dilated cardiomyopathy
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood
  • ICD-10: I42.0
  • OMIM: 605362
  • UMLS: C5679819
  • MeSH: -
  • GARD: -
  • MedDRA: -
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