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Cochlear implantation in children with Jervell, Lange-Nielsen syndrome

Published online by Cambridge University Press:  14 May 2007

A Daneshi ,
M M Ghassemi ,
M Talee  and
S Hassanzadeh
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Abstract

Jervell, Lange-Nielsen syndrome is a condition that causes profound hearing loss and disruption of the normal cardiac rhythm. This disorder is a form of long QT syndrome, a cardiac disorder that causes the cardiac muscle to take longer than usual to recharge between beats. A retrospective case study was performed to document cochlear implantation in three profoundly deaf children (two of them siblings) with Jervell, Lange-Nielsen syndrome. We discuss diagnosis and management of this syndrome and also the long-term performance of cochlear implantation in these Iranian patients, referring especially to the role of the ENT specialist in diagnosis and treatment. The collected data show that cochlear implantation can be relatively safely performed in patients with Jervell, Lange-Nielsen syndrome and that these children received significant benefit from cochlear implantation.

Keywords

Jervell and Lange-Nielsen SyndromeCochlear ImplantsSensorineural Deafness
Type
Research Article
Information
The Journal of Laryngology & Otology , Volume 122 , Issue 3 , March 2008 , pp. 314 - 317
Copyright
Copyright © JLO (1984) Limited 2007

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References

1 Jervell, A, Lange-Nielsen, F. Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval and sudden death. Am Heart J 1957;54:59–68CrossRefGoogle Scholar
2 Moss, AJ, Schwartz, PJ, Crampton, RS, Locati, E, Carleen, E. The long QT syndrome: a prospective international study. Circulation 1985;71:17–21CrossRefGoogle Scholar
3 Garson, A Jr, Dick, M 2nd, Fournier, A, Gillette, PC, Hamilton, R, Kugler, JD et al. The long QT syndrome in children: an international study of 287 patients. Circulation 1993;87:1866–72CrossRefGoogle Scholar
4 Hashiba, K, Hereditary QT. Prolongation syndrome in Japan: genetic analysis and pathological findings of the conducting system. Jpn Circ J 1978;42:1133–9CrossRefGoogle ScholarPubMed
5 Komsuoglu, B, Goldeli, O, Kulan, K, Budak, F, Gedik, Y, Tuncer, C et al. The Jervell and Lange-Nielsen syndrome. Int J Cardiol 1994;47:189–92CrossRefGoogle ScholarPubMed
6 Wang, Q, Curran, ME, Splawski, I, Burn, TC, Millholland, JM, Van Raay, TJ et al. Positional cloning of a novel potassium channel gene: KVLQT1 mutations cause cardiac arrhythmias. Nat Genet 1996;12:17–23CrossRefGoogle ScholarPubMed
7 Li, H, Chen, Q, Moss, AJ, Robinson, J, Goytia, V, Perry, JC et al. New mutations in the KVLQT1 potassium channel that cause long QT syndrome. Circulation 1998;97:1264–9CrossRefGoogle ScholarPubMed
8 Choube, C, Neyroud, N, Guicheney, P, Lazdunski, M, Romey, G, Barhanin, J. Properties of KVLQTI K+  channel mutations in Romano-Ward and Jervel and Lange-Nielsen inherited cardiac arrhythmias. EMBO J 1997;16:5472–9CrossRefGoogle Scholar
9 Wollnik, B, Schreeder, BC, Kubish, C, Esperer, HD, Wieacker, P, Jensch, TJ. Pathophysiological mechanisms of dominant and recessive KVLQTI K+  channel mutations found in inherited cardiac arrhythmias. Hum Mol Genet 1997;6:1943–9CrossRefGoogle Scholar
10 Chiang, CE. Congenital and acquired long QT syndrome. Current concepts and management. Cardiol Rev 2004;12:222–34CrossRefGoogle Scholar
11 Schwartz, PJ, Periti, M, Malliani, A. The long QT syndrome. Am Heart J 1975;89:378–90CrossRefGoogle Scholar
12 Berrettini, S, Forli, F, Ursino, F, Franceschini, SS. Cochlear implant in Jervell and Lange-Nielsen syndrome. Audiological Medicine 2003;1:224–7CrossRefGoogle Scholar
13 Chorbachi, R, Graham, JM, Ford, J, Raine, CH. Cochlear implantation in Jervell and Lange-Nielsen syndrome. Int J Pediatr Otorhinolaryngol 2002;66:213–21CrossRefGoogle ScholarPubMed
14 Green, JD, Schuh, MJ, Maddern, BR, Haymond, J, Helffrich, RA. Cochlear implantation in Jervell and Lange-Nielsen syndrome. Ann Otol Rhinol Laryngol Suppl 2000;185:27–8CrossRefGoogle ScholarPubMed
15 Archbold, S, Lutman, ME, Nikolopoulos, T. Categories of auditory performance: inter-user reliability. Br J Audiol 1998;32:7–12CrossRefGoogle ScholarPubMed
16 Allen, MC, Nikolopoulos, TP, O'Donoghue, GM. Speech intelligibility in children after cochlear implantation. Am J Otol 1998;19:742–6Google ScholarPubMed
17 American Heart Association. Long Q-T syndrome. http://www.americanheart.org/presenter.jhtml?dentifier=993 [16 August 2006]Google Scholar