طولاني و كري حسي - عصبي ) QT( كاشت حلزون شنوايي در سندروم ژرول و لانگ - نيلسون

Cochlear implantation in Jervell and Lange-Neilson syndrome

Jervell and Langc - Neilson syndrome (JLNS) is a rare, autosomal recessive disorder, characterized by profound congenital sensor neural deafness and prolongation of the QT interval on electrocardiogram, that clinically presented with cardiac arrhythmias, recurrent sync opal episodes and occasionally sudden death. in this article we present a case of cochlear implantation in a child with JLNS. At now she is well and uses speech therapy. This patient is the first reported successfully cochlear implantation in Jervell and Lange -Neilson syndrome at IRAN. Early diagnosis, special preoperative precautions and the use of beta blockers is life saving.