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Year : 2022  |  Volume : 28  |  Issue : 1  |  Page : 94-97

Cochlear implantation in a case of jervell and lange-nielsen syndrome presenting with absence seizure and anemia

1 Department of ENT and Head and Neck Surgery, JSS Medical College, JSS Academy of Higher Education and Research, Mysore, India
2 Medikeri's Super Speciality ENT Center, Bengaluru, Karnataka, India

Correspondence Address:
Dr. Murundi Basavarajaiah Bharathi
Department of ENT and Head and Neck Surgery, JSS Hospital, Agrahara Circle, Mysore - 570 004, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/indianjotol.indianjotol_167_21

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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.

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