Cochlear implant in Jervell and Lange‐Nielsen syndrome

Abstract

The Jervell and Lange‐Nielsen syndrome is one of the best known and most extensively studied deafness syndromes inherited as an autosomal recessive trait. Its hallmarks are congenital profound bilateral sensorineural hearing loss associated with electrocardiograph anomalies. In this case study we report on a child affected with the Jervell and Lange‐Nielsen syndrome and suffering from congenital profound bilateral sensorineural hearing loss who had a cochlear implantation at age three. No surgical or cardiological complications were encountered and excellent post‐operative functional hearing and communicative outcomes were achieved. It is concluded that cochlear implantation could be safely performed in these patients, with the achievement of a very good outcome in functional hearing and communication.

• cochlear implantation
• Jervell and Lange‐Nielsen syndrome
• profound sensorineural hearing loss
• Q‐T interval prolongation