Cochlear Implantation in a Case of Jervell and Lange-Nielsen Syndrome Presenting with Absence Seizure and Anemia

Here, we describe the case of a pediatric patient who presented with an episode of absence seizures and was later diagnosed to have Jervell and Lange-Nielsen syndrome (JLNS) and underwent cochlear implantation (CI). A 4-year-old male child born out of a third degree consanguineous marriage presented with congenital profound sensorineural hearing loss and an episode of absence seizures. Electrocardiogram showed a prolonged QT interval of 509 ms. The patient subsequently underwent CI. Here, we outline the challenges anticipated and the precautions to be taken when performing implantation on a patient with JLNS. JLNS is a rare autosomal recessive form of congenital long QT syndrome associated with congenital sensorineural deafness and a high incidence of sudden cardiac death in childhood. Patients with JLNS face added intra- and postoperative challenges during CI which require multidisciplinary and intensive care unit care.