Jervell and Lange-Nielsen Syndrome in deaf school children population

ABSTRACT

To identify the patients of Jervell and Lange-Nielsen syndrome [JLNS] amongst congenitally deaf children. This was a cross-sectional study, conducted at Hamza Foundation Academy for the Deaf, and Combined Military Hospital Lahore over a period of 4 months from February to May 2012. A total of 379 children with congenital sensorineural hearing loss were included in this study. Echocardiographs of all children [ages 4-18 years] were obtained. The corrected QT [QTc] intervals of all 379 ECGs were calculated using the Bazett's formula. Using the Schwartz's criteria, patients with long QTc intervals were further evaluated for Jervell and Lange-Nielsen Syndrome. Out of 379 children, 84 [22.1%] were found to have QTc intervals equal to or longer than 0.44 seconds. As per Schwartz's criteria, 31 [36.9%] out of 84 children with Long QTc [8.17% in sample population], scored high points [4.0 to 6.0], proving presence of JLNS. A sizable proportion of congenitally deaf children had Jervell and Lange-Nielsen Syndrome in our study