Long QT syndrome is characterized by prolongation of the QT interval associated with a high risk for syncope and sudden death. The Romano-Ward syndrome is of autosomal dominant inheritance, and the Jervell and Lange- Nielsen syndrome, with associated deafness, of autosomal recessive inheritance. If a wrong diagnosis of epilepsy is made, a chance may be missed to avoid sudden death. In this article, we report a patient with congenital sensorineural hearing loss and syncopes mimicking seizures.

Keywords: Uzun QT sendromu, konjenital sağırlık, ani ölüm, long QT syndrome, congenital deafness, sudden death