Cochlear implantation in a patient with Perisylvian syndrome.

Perisylvian syndrome is a rare neurological disorder characterised by the partial paralysis of muscles, epilepsy and mild to severe mental retardation. It is associated with hearing loss and delay in language and speech development. This presents additional challenges in the assessment of whether a child is suitable for cochlea implantation. The method to determine whether the hearing loss is of cochlear or central origin and the progress of a child with Perisylvian syndrome who received a cochlear implant is discussed.