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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.

ORPHA:217622

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: -

  A summary on this disease is available in Deutsch (2014) Español (2014) Français (2014) Nederlands (2014) Polski (2014, pdf) Russian (2014, pdf)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.