(PP805) Cochlear implant in Jervell and Lange Nielsen Syndrome: A Case Series Study

Abstract: Jervell and Lange-Nielsen (JLN) syndrome is an autosomalrecessive condition presenting with bilateral severe to profound sensorineural hearing loss (SNHL)  and prolonged QT interval in ECG.
Recently many of the children with JLN receive cochlear implant as a management of their hearing loss problem with risk of intra or post operative death due to the heart disorder if not managed properly.
This presentation discuss the experience of the author with number of cases of JLN syndrome underwent cochlear implant surgery describing the preoperative assessment, intra operative precautions and the special consideration in device switch On, programming and follow  up.

Summary: Jervell and Lange-Nielsen (JLN) syndrome is an autosomalrecessive condition presenting with bilateral severe to profound sensorineural hearing loss (SNHL), prolonged QT interval in ECG, and sudden death in severe cases. . Long QT syndrome causes the cardiac muscle to take longer time than usual to recharge between beats.
Jervell and Lange-Nielsen syndrome is usually presented with attacks of syncope or brief loss of conscious without clear cause. The other red flag in the presence of history of sudden death of one or more of the child’s brother or sisters and had the same condition of SNHL. JLN usually diagnosed through electrocardiogram (ECG) which show prolonged QT interval.
Cochlear implantation provides the single most effective form of hearing rehabilitation in patients with bilateral severe to profound sensorineural hearing loss that is no longer responsive to conventional amplification. Cochlear implantation plays a critical role in hearing restoration for those patients who are either born with sensorineural hearing loss (congenital) or in those who develop a significant sensorineural hearing loss throughout childhood
Recently many of the children with JLN receive cochlear implant as a management of their hearing loss problem with risk of intra or post operative death due to the heart disorder if not managed properly. This clinical poster discuss the experience of the author with series of cases of JLN syndrome underwent cochlear implant surgery at Sohag University hospital. It will demonstrate the preoperative assessment, intra operative precautions and the special consideration in device switch On, programming and follow  up.
The author presenting evidence based protocol for management of such cases including investigation, recommendation for anesthesia, post operative care and cochlear implant external device mapping.