Deafness and electrocardiographic changes (prolongation of the Q-T interval and 
inversion of the T wave) with a clinical picture of syncopal attacks and sudden 
death, were described as a distinct syndrome by Jervell and Lange-Nielsen in 
1957. The syndrome is inherited as an autosomal recessive trait. In this study, 
all the cases reported since 1957 and their proposed prevalence are reviewed. 
The authors describe the 4 cases they have studied, all of which presented 
congenital sensorineural hearing loss and electrocardiographic changes 
characteristic of the syndrome. The relatively high number of cases they have 
encountered casts doubt on literature that states that the syndrome occurs more 
frequently in Northern-European populations. Consequently, it is advisable to 
perform an electrocardiogram in all children affected by congenital deafness.