Cochlear implantation in children with Jervell and Lange-Nielsen syndrome – a cautionary tale
| Autor: | Lise Henderson, Stephen J. Broomfield, Iain A. Bruce, Kevin Green, Richard T. Ramsden |
|---|---|
| Rok vydání: | 2012 |
| Předmět: |
Male
Reoperation medicine.medical_specialty Prosthesis-Related Infections Infant Premature Diseases Asymptomatic QT interval Sudden death Speech and Hearing Fatal Outcome Postoperative Complications Clinical Protocols Risk Factors Humans Mass Screening Medicine Family history Child Mass screening business.industry Infant Newborn Infant Perioperative medicine.disease Cochlear Implantation Prosthesis Failure Surgery Jervell and Lange-Nielsen syndrome Death Sudden Cardiac Otorhinolaryngology Child Preschool Jervell-Lange Nielsen Syndrome Female Implant medicine.symptom business Follow-Up Studies |
| Zdroj: | Cochlear Implants International. 13:168-172 |
| ISSN: | 1754-7628 1467-0100 |
| DOI: | 10.1179/1754762810y.0000000006 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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