A MALADY OF THE HEART AND EARS: A RARE CASE OF JERVELL AND LANGE NIELSON SYNDROME - CASE REPORT

Abstract

Jervell and Lange-Nielsen syndrome (JLNS) is a autosomal recessive disease,characterized by profound
congenital sensorineural deafness and a prolonged QTc interval (greater than 500 milliseconds). Sudden cardiac
deaths have been reported in morethan 25% of JLNS patients. JLNS can occur without any cardiac symptoms.
Pharmacological management consists of β-blockers. We are reporting a 5 year old child with JLNS without any
cardiac symptoms and his prolonged QTc
 interval responded to propranolol.