Prevalence of Long QT Syndrome in Children with Congenital Sensory-Neural Deafness

Background & objectives: Long QT syndrome (LQTS), congenital or acquired disorder, is characterized by a prolonged QT interval associated with syncope attacks and sudden death. Jervell and Lange-Nielsen first described LQTS in conjunction with sensory-neural congenital deafness. In long QT syndrome, the modified QT interval is greater than 440 ms and 460 ms in men and in women respectively. Acquired forms of prolonged QT are the consequence of metabolic disorders and various medications.