Congenital Deafness with Cardiac Arrhythmias: The Jervell and Lange-Nielsen Syndrome

The Jervell and Lange-Nielsen syndrome, affecting 0.3% of congenitally deaf persons, consists of severe cardiac arrhythmias and sensori-neural hearing loss. The disorder, presenting as syncope or loss of consciousness, is associated with a prolonged Q-T interval on the electrocardiogram. The loss of consciousness is due to ventricular tachycardia or fibrillation, which in turn has been related to a decrease in the autonomic sympathetic activity to the heart from the right stellate ganglion. It is this decrease in autonomic synpathetic activity, manifesting as the prolonged Q-T interval, which renders the patient more vulnerable to dangerous arrhythmias. Although recovery is usually spontaneous, sudden death can ensue.

The syndrome is often erroneously diagnosed as a seizure disorder and improperly treated. Therapy with the beta blocker, propranolol, has been effective in reducing the frequency of spells and preventing death. Surgical removal of the left stellate ganglion is under investigation and appears promising. We recommend that every congenitally deaf child with suspicious symptoms receive a electrocardiogram and that professionals who work with deaf children not only inform themselves about the syndrome but also receive training in cardiopulmonary resuscitation.