Congenital hearing loss in Jervell and Lange-Nielsen syndrome
Jacobson, J.; Jacobson, C.; Francis, P.
Journal of the American Academy of Audiology 1(3): 171-173
1990
ISSN/ISBN: 1050-0545 PMID: 1721555 Document Number: 367128
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.