Jervell, Lange-Nielsen syndrome is a condition that causes profound hearing loss and disruption of the normal cardiac rhythm. This disorder is a form of long QT syndrome, a cardiac disorder that causes the cardiac muscle to take longer than usual to recharge between beats. A retrospective case study was performed to document cochlear implantation in three profoundly deaf children (two of them siblings) with Jervell, Lange-Nielsen syndrome. We discuss diagnosis and management of this syndrome and also the long-term performance of cochlear implantation in these Iranian patients, referring especially to the role of the ENT specialist in diagnosis and treatment. The collected data show that cochlear implantation can be relatively safely performed in patients with Jervell, Lange-Nielsen syndrome and that these children received significant benefit from cochlear implantation.