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Sensorineural deafness with dilated cardiomyopathy

ORPHA:217622· ICD-10 I42.0

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.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant
Age of onset
Childhood