|JCDR. 2021; 12(3): 649-650
A MALADY OF THE HEART AND EARS: A RARE CASE OF JERVELL AND LANGE NIELSON SYNDROME - CASE REPORTDR. K. JAGADISH KUMAR,DR. SANGEETHA BALAJI. DR.MANJUNATH.V.G.,
DR. BHARATHI.M.B., DR. KUMAR SHANKAR DE..
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.
Key words: Jervell and Lange-Nielsen syndrome (JLNS)., Prolonged QTc interval, Propranolol