Cochlear implantation in Jervell and Lange-Nielsen syndrome

Jervell and Lange-Nielsen syndrome represents a rare, autosomal recessive cause of congenital deafness. Affected patients have a characteristic prolongation of the QT interval on electrocardiogram, along with cardiac arrhythmias, recurrent syncopal episodes, and a predisposition to sudden death. We present the first reported case of cochlear implantation in a child with Jervell and Lange-Nielsen syndrome. Special perioperative precautions were required, including cardiac monitoring for 48 hours, perioperative beta blockade, and special anesthetic considerations. Because sudden patient death has been associated with exposures to loud sounds in patients with prolonged QT syndrome without hearing loss (Ward-Romano syndrome), initial cochlear implant stimulation was performed with cardiac monitoring. This patient has responded well to cochlear implantation with 11 months of implant use. The patient has achieved limited open-set word comprehension and significantly improved speech, as is expected for her age. Although the patient had no problems with cardiac arrhythmias at surgery, she has since experienced a syncopal episode requiring placement of an automatic pacemaker and defibrillator. The implant has continued to work well despite the pacemaker and defibrillator. Cochlear implantation can be relatively safely performed in patients with Jervell and Lange-Nielsen syndrome, provided proper precautions are followed.