Background: The Jervell and Lange-Nielsen syndrome is characterized by syncope, congenital sensorineural deafness, prolonged QT interval and abnormal T wave morphology in ECG. However, QT interval and T wave morphology in patients with congenital sensorineural deafness without syncope is not well known. We investigated QT interval and T wave morphology in patients with congenital sensorineural deafness. Subjects and Methods: Subjects consisted of 38 patients with congenital sensorineural deafness (Group I) and 42 patients with congenital conductive deafness which has no relation with the Jervell and Lange-Nielsen syndrome (Group II), served as controls, who visited Kyungpook National University hospital from 2004 to 2006. We evaluated corrected QT (QTc) interval and T wave morphology in these patients. The QTc interval was calculated by Fridericia`s formula. Results: Heart rate was higher in Group I and QT interval was longer in Group II. However, QTc interval and T wave morphology were similar between two groups. None of 80 patients with congenital deafness had syncope and a high or intermediate probability of long QT syndrome based on Schwartz`s criteria scores. Conclusions: QTc interval and T wave morphology in patients with congenital sensorineural deafness were similar to those in patients with congenital conductive deafness.