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  Contents > Previous page > Article detail print Order
o Issue N# 1 - 2017 o

OTOLOGY

Cochlear implantation in patients with Jervell and Lange-Nielsen syndrome: Our experience, outcomes, challenges and review of literature


Authors : Clarós P, Nyada F, Pujol C, Clarós A, Clarós-Pujol A, Carmona A, Calvo MA. (Barcelona)

Ref. : Rev Laryngol Otol Rhinol. 2017;138,1:3-7.

Article published in french
Downloadable PDF document french



Summary : Introduction: Jervell and Lange-Nielsen syndrome is a rare inherited disorder that associates cardiac abnormality with a profound bilateral deafness. Objectives: Our aim is to conduct a retrospective evaluation of the management of the patients in our cochlear implantation center and to review it together with the literature. Materials and methods: We have conducted a retrospective and analytic study. We have studied the epidemiological characteristics, pre/per and post-operative care and the evolution of these patients. Results: A 0.33% (3/900) of the all patients who has been implanted in our centre had the Jervel and Lange-Nielsen Syndrome. All patients had pre­­operative ECG before undergoing surgery. No cardiac inci­dents have been described during surgery and after. All patients were under b-blockers, and only those 3 implanted are well integrated in the normal school system twelve years after. They present mutations on the KCNQ1 and KCEN1 genes. Conclu­sion: Cochlear implantation is well performed in patients with Jervell and Lange-Nielsen syndrome. Early diag­nosis and an appropriate medical treatment have changed mortality. Our experience shows that when cardiac problem is under control, cochlear implantation in patients with Jervell and Lange-Nielsen syndrome is same to a child not suffering this syndrome and improves quality of life.

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