Jervell–Lange-Nielsen syndrome is characterised by congenital deafness and a long QT interval on electrocardiography.

(1) To survey UK national practice regarding electrocardiography screening of deaf children referred to cochlear implant centres, performed to evaluate for prolonged QT interval as recommended by national guidelines, and (2) to review local practice.

Data were collected via a questionnaire sent to all UK cochlear implant centres, and via review of the medical records of a local cochlear implant centre database.

Eight (42 per cent) of the 19 cochlear implant centres surveyed performed electrocardiographic screening. Thirteen cases of long QT syndrome were reported in seven centres, with two related deaths. In our local cochlear implant centre, 14 (7.1 per cent) of 193 children had abnormal electrocardiograms; one definite long QT syndrome case and 13 borderline cases were identified.

Despite clear national guidelines for electrocardiographic screening of deaf children, there is wide variation in practice. Our local practice of performing investigations, including electrocardiography, during magnetic resonance imaging sedation has been very successful. Electrocardiograms should be reviewed by trained clinicians, and corrected QT intervals should be calculated manually.