Jervell and Lange-Neilsen syndrome is a condition where sensorineural deafness coincides with inherited abnormalities of the heart, resulting in prolonged ventricular repolarisation, frequently shown on an ECG with a prolonged QT interval. These children can present at implant centres for assessment but are at an increased risk from the surgery as medication may lead to a prolongation of the QT interval, triggering arrhythmias and even death. This raises an ethical question of whether the risk involved in elective cochlear implant surgery outweighs the benefits that are achieved and whether anything can be done to make the surgery safer by taking a proactive approach. The cochlear implant programme in Manchester previously assessed the risks of implantation to be too high in these patients following a perioperative death of a child with long QT and ceased to implant these patients. However, demand from these patients and their families caused re-evaluation of this decision. This led to the creation of an evidence-based multi-disciplinary pathway with alteration to the normal CI protocols and medications. These include a base-line preoperative ECG, assessment in anaesthetic-led clinics, extra equipment and a cardiology professional being alerted at surgery and HDU monitoring for 24 hours postop. The article details four patients that were implanted successfully and without adverse event. This illustrates that the new protocol thus far has been successful in minimising the risk to these patients. Furthermore, an interesting finding of the case studies is that two out of the four children were identified with long QT as part of the baseline ECG screening, thus showing the importance of this initial step.

Cochlear implantation in children with congenital long QT syndrome: Introduction of an evidence-based pathway of care.
Scott-Warren V, Bendon A, Bruce IA, et al.
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Kerri Millward

Manchester Paediatric Cochlear Implant Programme, Manchester, UK.

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Morag Lockley

Manchester Auditory Implant Centre, Manchester, UK.

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