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Year : 2015  |  Volume : 21  |  Issue : 1  |  Page : 78-79

Cardiac precautions in patient with Jervell and Lange-Nielson syndrome undergoing cochlear implantation

Department of Otorhinolaryngology and Head and Neck Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India

Date of Web Publication10-Mar-2015

Correspondence Address:
Dr. Kapil Sikka
Department of Otorhinolaryngology and Head and Neck Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-7749.150602

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Jervell and Lange-Nielsen syndrome is a syndrome affecting hearing organ and heart conduction system since birth. It is easy to overlook heart defect as cardiac conduction defect is often asymptomatic, but can lead to disastrous complications if left undiagnosed. Hence, we are presenting a case report to discuss briefly the diagnosis and management of this syndrome to prevent cardiac complications

Keywords: Cochlear implantation, Jervell Lange Nielson Syndrome, Sensorineural hearing loss

How to cite this article:
Yadav V, Sikka K. Cardiac precautions in patient with Jervell and Lange-Nielson syndrome undergoing cochlear implantation. Indian J Otol 2015;21:78-9

How to cite this URL:
Yadav V, Sikka K. Cardiac precautions in patient with Jervell and Lange-Nielson syndrome undergoing cochlear implantation. Indian J Otol [serial online] 2015 [cited 2022 Sep 26];21:78-9. Available from: https://www.indianjotol.org/text.asp?2015/21/1/78/150602

  Introduction Top

Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive disorder, characterized by congenital profound bilateral sensorineural hearing loss and prolonged QTc on electrocardiography (ECG) (repolarization defect), usually > 500 ms. Prolongation of the QTc interval is significant as it is associated with arrhythmias, syncope or sudden death. This case is reported to highlight the importance of careful cardiac evaluation as a standard workup for cochlear implant. Preoperative and perioperative management of such cases is briefly discussed.

  Case Report Top

A 3-year-old female child, with bilateral profound hearing loss, was diagnosed to have long QTc interval on routine cardiac evaluation [Figure 1]. Echocardiogram was normal. Child was started on the tablet propranolol (beta blocker) (1 mg/kg) and underwent pacemaker implantation prior to cochlear implantation. Pacemaker was converted into asynchronous mode on the morning of surgery. Continuous ECG monitoring was performed in the intraoperative period until 24 h in the postoperative period and pacemaker mode was changed to synchronous mode in the evening. Anti-arrhythmic medications including magnesium sulfate were kept ready during surgery with all precautions applicable to handle serious cardiac arrhythmias. Cochlear implantation surgery was uneventful, and child is under regular follow-up.
Figure 1: Electrocardiography (Lead II) shows QTc prolongation (519 ms)

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  Discussion Top

Jervell and Lange-Nielsen syndrome is also known as cardioauditory syndrome of JLNS and surdo-cardiac syndrome. The incidence of JLNS, which appears to account for <10% of total long QT syndrome (LQTS) cases, has been estimated to be approximately 6/1000 among congenitally deaf individuals. [1] Loss-of-function mutations in the KCNQ1 gene cause autosomal recessive JLNS. [2] Among JLNS patients, 15% become symptomatic within the first 23 months of life; the median time (50%) of survival free from cardiac events is 33 months, and by 18 years of age, 90% of JLNS patients had a first cardiac event. [3] LQTS is a cardiac conduction disorder characterized by prolonged ventricular repolarization. The PR interval and QRS complexes are within normal limits; QT interval is strikingly abnormal. While generally greatly prolonged, duration may be greatly variable. The variations can be exacerbated fright or excitement. [4] Patients with this defect are prone to arrhythmia like ventricular fibrillation and also sudden cardiac death. Relatives should be screened by ECG screening. Children presenting for cochlear implantation with this condition may be asymptomatic but are at risk of serious cardiac complications. Broomfield et al. mentioned the serious complication (two deaths) in patients despite appropriate management. [5] Therefore, it is mandatory for cardiac evaluation of all patients with sensorineural hearing loss to diagnose this rare entity and manage it appropriately to avoid complications.

  References Top

Ocal B, Imamoglu A, Atalay S, Ercan Tutar H. Prevalence of idiopathic long QT syndrome in children with congenital deafness. Pediatr Cardiol 1997;18:401-5.  Back to cited text no. 1
Shimizu W, Horie M. Phenotypic manifestations of mutations in genes encoding subunits of cardiac potassium channels. Circ Res 2011;109:97-109.  Back to cited text no. 2
Schwartz PJ, Spazzolini C, Crotti L, Bathen J, Amlie JP, Timothy K, et al. The Jervell and Lange-Nielsen syndrome: Natural history, molecular basis, and clinical outcome. Circulation 2006;113:783-90.  Back to cited text no. 3
Olley PM, Fowler RS. The surdo-cardiac syndrome and therapeutic observations. Br Heart J 1970;32:467-71.  Back to cited text no. 4
Broomfield SJ, Bruce IA, Henderson L, Ramsden RT, Green KM. Cochlear implantation in children with Jervell and Lange-Nielsen syndrome - A cautionary tale. Cochlear Implants Int 2012;13:168-72.  Back to cited text no. 5


  [Figure 1]

This article has been cited by
1 Jervell and Lange-Nielsen syndrome in cochlear implanted patients: Our experience and a review of literature
Ali Eftekharian,Mozhgan Hosseinerezai Mahani
International Journal of Pediatric Otorhinolaryngology. 2015; 79(9): 1544
[Pubmed] | [DOI]


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