title: Congenital hearing loss in Jervell and Lange-Nielsen syndrome.
abstract: 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.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of Texas Health Science Center, Houston 77030.
authors.forename: J
authors.initials: J
authors.lastname: Jacobson
authors.forename: C
authors.initials: C
authors.lastname: Jacobson
authors.forename: P
authors.initials: P
authors.lastname: Francis
mesh_terms: D002675:Child, Preschool; D002658:Developmental Disabilities / Q000503:physiopathology; D004200:Diseases in Twins; D004562:Electrocardiography; D016057:Evoked Potentials, Auditory, Brain Stem; D006311:Hearing Disorders / Q000151:congenital* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008133:Long QT Syndrome / Q000235:genetics* / Q000503:physiopathology; D008297:Male