Article
Cochlear implantation in Jervell and Lange-Nielsen syndrome.
International journal of pediatric otorhinolaryngology - 2 Dec 2002
Chorbachi R, Graham J M, Ford J, Raine C H
Abstract excerpt
A case of familial prolonged QT interval and congenital sensorineural hearing loss is described emphasising the diagnostic and management implications. Jervell and Lange-Nielsen syndrome is important because of its potential association with sudden death in children with congenital sensorineural deafness. It is known to be associated with mutations of the genes KCNQ1 (KVQTI) and KCNE1 (Isk). The underlying...
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