Jervell and Lange-Nielsen syndrome is an autosomal recessive hereditary 
condition that presents with cardiac abnormalities characterized by a prolonged 
Q-T electrocardiographic pattern and congenital severe-to-profound auditory 
deficits. This paper describes the auditory history of twin boys born out of 
consanguinity and diagnosed with this syndrome. Both infants were products of 
the neonatal intensive care unit (NICU) and failed initial ABR screening. 
Diagnostic evaluation demonstrated profound hearing loss and developmental 
delays for each infant. Because sudden death is a consequence, audiologists are 
advised to recognize signs and symptoms associated with this syndrome.