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Int J Pediatr Otorhinolaryngol. 2008 Nov;72(11):1723-9. doi: 10.1016/j.ijporl.2008.07.013. Epub 2008 Sep 20.

Cochlear implantation in patients with Jervell and Lange-Nielsen syndrome, and a review of literature.

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  • 1Department of Otolaryngology, The Sixth People's Hospital, Shanghai JiaoTong University, Institute of Otolaryngology, Medicine School of Shanghai JiaoTong University, Shanghai 200233, China.


Jervell and Lange-Nielsen syndrome is characterized by a long QT interval in electrocardiography, syncopal attacks and congenital sensorineural hearing loss. Upon diagnosis, beta-blockers is recommended as the first choice of medication, which is considered to reduce the occurrence rate of syncopal attack and sudden death during life. Cochlear implantation is indicated for hearing rehabilitation in this syndrome with profound deafness. The auditory and language outcome after cochlear implantation in this syndrome is not worse than those in patients with non-syndromic sensorineural deafness. The life quality in this syndrome could be improved greatly with cochlear implantation only if the cardiologic event has been controlled effectively.

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