Cochlear implantation in children with Jervell and Lange-Nielsen syndrome - a cautionary tale

Stephen J. Broomfield, Iain A. Bruce, Lise Henderson, Richard T. Ramsden, Kevin M J Green

    Research output: Contribution to journalArticlepeer-review


    Objective and importance: Jervell and Lange-Nielsen (JLN) syndrome is a rare cause of congenital profound hearing loss associated with a prolonged QT interval on the electrocardiogram. Children presenting for cochlear implantation with this condition may be asymptomatic but are at risk of sudden death. Screening and subsequent careful management is therefore required to ensure a successful outcome. We present our experience of cochlear implantation in children with JLN syndrome, including two who died unexpectedly, and suggest a protocol for management of such cases. Clinical presentation: Four cases of cochlear implantation in JLN syndrome are described. None had any previous cardiological family history. Two were diagnosed pre-operatively but, despite appropriate management under a cardiologist, died from cardiac arrest; the first in the perioperative period following reimplantation for infection, and the second unrelated to his cochlear implant surgery. The other two patients were diagnosed only subsequent to their implantation and continue to use their implants successfully. Conclusion: These cases highlight the variation in presentation of JLN syndrome, and the spectrum of disease severity that exists. Our protocol stresses the importance of careful assessment and counselling of parents by an experienced implant team. © W.S. Maney & Son Ltd 2012.
    Original languageEnglish
    Pages (from-to)168-172
    Number of pages4
    JournalCochlear Implants International
    Issue number3
    Publication statusPublished - Aug 2012


    • Cochlear implants
    • Jervell and Lange-Nielsen syndrome


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