A case report of Jervell Neilson syndrome in Babol

Abstract

Objective: Jervell Neilson syndrome is a rare congenital disease, which manifests with prolonged QT-interval, deafness and recurrent syncope. This disease is transmitted in autosomal recessive form and in this paper we report a rare case of Jervell Neilson syndrome with recurrent syncope and deafness. Case: A 14-year boy, which had neural deafness since childhood referred with recurrent episodes of syncope. On physical examination, blood pressure, jugular venous pulse, the first and the second heart sounds were normal. Chest X-ray and ECHO-Cardiography were also normal. In ECG, there was prolonged QT interval. He was treated with beta-blocker agents and had no syncope after 3 years on follow up. Conclusion: Jervell Neilson syndrome must be considered in differential diagnosis of childhood syncope in order to prevent cardiac syncope complications.

Keywords

• sensory deafness
• syncope
• cardiac syncope
• prolonged qt-interval