title: Jervell and Lange-Nielsen syndrome in cochlear implanted patients: our experience and a review of literature.
abstract: OBJECTIVE
  To share our experience in cochlear implanted patients with Jervell and Lange-Nielsen syndrome (JLNS), to review the literature results and to disclose precautions which have to be taken dealing with these patients.
  
  
  METHODS
  Electrocardiograms (ECG) of 503 children with congenital bilateral profound hearing loss which were cochlear implanted in cochlear implant center of a tertiary hospital were evaluated for long QT syndrome. Clinical reports of the patients with JLNS were evaluated and a review of literature performed.
  
  
  RESULTS
  The prevalence of disease was 0.79% (four cases) in our center which is in the range of literature reports (0-2.6%). None of our patients had a history of syncopal attack. Two patients (50%) were born from parents with consanguineous marriage. Considering all precautions their cochlear implant surgeries were done uneventfully. A review of the literature has identified sixteen reports on cochlear implantation in a total of 38 children with JLNS. Similar to our cases none of the authors reported cardiac events during device switch-on. Nine available reports about the outcome of cochlear implantation in these patients indicated good auditory outcome.
  
  
  CONCLUSIONS
  It is recommended that all congenitally deaf patients have an ECG taken as a part of the evaluation. As auditory stimuli is reported to be a specific trigger, it is prudent to activate the processor with continuous heart monitoring even though there is no reported cardiac event during device switch-on. Cochlear implantation can be performed relatively safely in these patients if necessary precautions have been taken appropriately and their auditory outcome is good. Triggers of the cardiac events should be avoided throughout their life.
authors.affiliation: Hearing Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Electronic address: alishko@gmail.com.
authors.forename: Ali
authors.initials: A
authors.lastname: Eftekharian
authors.affiliation: Hearing Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Mozhgan Hosseinerezai
authors.initials: MH
authors.lastname: Mahani
mesh_terms: D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation / Q000009:adverse effects* / Q000379:methods; D003054:Cochlear Implants; D004562:Electrocardiography; D005260:Female; D006801:Humans; D007223:Infant; D029593:Jervell-Lange Nielsen Syndrome / Q000150:complications / Q000601:surgery*; D008297:Male
keywords: Cochlear implantation; Congenital hearing loss; Jervell and Lange-Nielsen syndrome; Long QT syndrome