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VOLUME 5 , ISSUE 4 ( October-December, 2019 ) > List of Articles


Cochlear Implantation Surgery in Jervell and Lange-Nielsen Syndrome Patients: Our Experience

Joyshankar Jana, Prithviraj Gauri, Amol B Mhetre

DOI: 10.5005/jp-journals-10045-00133

License: CC BY-NC 4.0

Published Online: 21-12-2020

Copyright Statement:  Copyright © 2019; The Author(s).


Jervell and Lange-Nielsen syndrome represents a rare autosomal recessive cause of congenital deafness. The affected patients have prolongation of QT interval on electrocardiogram. These patients may present with cardiac arrhythmias, syncopal episodes, and predisposition to sudden death. These patients are predisposed to polymorphic ventricular tachycardia–torsades de pointes leading to syncope and sudden death. Surgical stress in the perioperative period, anxiety, loud sounds, and fear may trigger fatal arrhythmias. We are reporting anesthetic management of two pediatric patients posted for cochlear implantation surgery.

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