Abstract

A report is presented concerning a family with heritable electrocardiographic QT-prolongation attacks of syncope and possible sudden death. In 23 family members investigated, nine living cases were found to have the anomaly. Of these nine patients at least two had had syncopes in early childhood. Hearing loss was found in three of the nine patients, but in one of them this could have been due to noise trauma and in another hearing loss was unilateral. The inheritance follows an autosomal dominant pattern. As far as we know this is the first report of this disease from The Netherlands.