Familial hypertrophic cardiomyopathy (HCM) occurs as an autosomal dominant mendelian-inherited disease in about 50 percent of cases. It has been associated with different disorders. HCM is a genetically heterogeneous disease, however the genetic heterogenity does not appear to explain the clinical variability. In some patients with an abnormal gene and no echocardiographic evidence of HCM, the electrocardiogram is abnormal. Therefore, otherwise unexplained abnormalities of the electrocardiogram in first degree relatives of patients with HCM, may be indicative of a carrier or preclinical state. We report a family with HCM and neurosensorial hearing loss.