Jervell-Lange Nielsen Syndrome: A Case Report

Sogol  Asgari; Seyed Sam Mehdi  Hosseininasab; Faranak  Behnaz

doi:10.18502/aacc.v11i4.19383

Sogol Asgari Anesthesiology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Seyed Sam Mehdi Hosseininasab Anesthesiology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Faranak Behnaz Department of Anesthesiology, School of Medicine, Shohada-e-Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

DOI: https://doi.org/10.18502/aacc.v11i4.19383

Keywords: Cochlear implantation; Sensorineural hearing loss; Jervell and lange-nielsen syndrome; Long QT syndrome

Abstract

There is a rare genetic disorder called Jervell-Lange Nielsen syndrome that leaves people congenitally deaf and with a long QT interval. This can lead to deadly heart rhythm problems and sudden death. For the treatment of hearing loss, cochlear implants, and for the treatment of heart difficulties, beta-blockers, and in certain circumstances, implantable cardioverter defibrillators, arrhythmias, syncope attacks, and sudden death are recommended. We discuss the case of an 8-year-old child who was referred for cochlear implantation after being diagnosed with Jervell-Lange Nielsen syndrome. In this study, we want to deal with patient management preoperatively and during surgery and describe the side effect of this syndrome