The Jervell and Lange-Nielsen syndrome

Abstract

The association of prolongation of the QT interval with recurrent attacks of syncope, sudden death, and malignant ventricular arrhythmias is known as the long QT syndrome. The syndrome may be familial with or without congenital deafness, or idiopatic. The syndrome with deafness (Jervell and Lange-Nielsen syndrome) is inherited through an autosomal recessive mechanism. In this study, we attempted to identify patients with the Jervell Lange-Nielsen syndrome amongst 154 deaf mute school children. Two patients had a corrected QT interval of 0.52 and congenital sensorineural hearing loss with the other electrocardiographic changes characteristic of the syndrome, such as inverted or bifid T wave. There was no evidence of electrocardiographic (ECG) abnormality in family members, except only one case of parental deafness. This is the first and preliminary report that analyzed the incidence of the Jervell and Lange-Nielsen syndrome amongst 154 deaf mute school children in Turkey. Our study was conducted to identify patients with this syndrome amongst children of another deaf mute school in Turkey.