Out of these KCNQ1 which is a potassium channel gene, its heterozygous mutation causes RWS while its homozygous mutation causes a much rarer condition Jervell Lange-Nielsen
syndrome in which along with long QT interval deafness also occurs.
Consideration can also be given to obtaining an EKG on all children with sensorineural hearing loss, given the morbidity and mortality associated with the prolonged QT interval in Jervell and Lange-Nielsen
Objective: To identif y the patients of Jervell and Lange-Nielsen syndrome (JLNS) amongst congenitally deaf children.
Using the Schwartz's criteria, patients with long QTc intervals were further evaluated for Jervell and Lange-Nielsen Syndrome.
Conclusion: A sizable proportion of congenitally deaf children had Jervell and Lange-Nielsen Syndrome in our study.
Key Words: Long QT interval, Jervell and Lange-Nielsen Syndrome (JLNS), Congenital deafness, Electrocardiographs.
Jervell and Lange-Nielsen Syndrome (JLNS) is a rare condition that causes bilateral hearing loss from birth and a disruption of the normal heart's rhythm (arrhythmia).
According to the Norwegian newspaper Aftenposten, the lawyer and retired judge Trygve Lange-Nielsen
has said that this is the most serious legal failure seen in the country since the witch processes of the 17th century.
Two inherited forms of LQTS with differing patterns of transmission have been described and include Romano-Ward syndrome (RWS)[5,6] and Jervell and Lange-Nielsen syndrome (JLNS).
Jervell and Lange-Nielsen syndrome is an uncommon inherited form of LQTS.
Jervell A, Lange-Nielsen E Congenital deaf-tourism, function heart disease with prolongation of the Q-T interval and sudden death.
channel mutations in Romano-Ward and Jervell and Lange-Nielsen inherited cardiac arrhythmias.