Jervell And Lange-Nielsen Syndrome In A Deaf Children Population

The Jervell and Lange-Nielsen syndrome is an infrequent form of long QT syndromes in which prolonged QT interval and congenital deafness exist together. Affected patients are prone to syncopal attacks and sudden death due to fatal ventricular arrhythmias. In this study, we attempted to identify patients with Jervell and Lange-Nielsen syndrome amongst children with congenital hearing loss. The corrected QT interval was measured from the 12 lead electrocardiogram using Bazett's formula, and was considered prolonged when it exceeded the normal upper limit of 440 ms. Nine out of 116 patients (11,8±2,7 years, range 7 to 18 years) with congenital deafness had a corrected QT interval longer than 440 ms. Although they did not met the definite diagnostic criteria according to Schwartz criteria, all could be defined as having intermediate probability of LQTS according to revised criteria. Family history for deafness was positive in 59 (50.8 %) patients, and consanguinity marriage was present in parents of 50 children (43.1 %). In conclusion, this recessively inherited syndrome should be screened in children with congenital deafness especially in areas where consanguineous marriages are frequent.