Cochlear implantation in Cockayne syndrome: our experience of two cases with different outcomes.

Abstract

Cockayne syndrome is a rare autosomal recessive defect in DNA repair resulting in a classic facies with potential visual and auditory impairment. The hearing loss begins peripherally and may become central as the condition progresses. Coexisting sensory deprivation from visual impairment and the possibility of progressive deterioration in mental function conspire with a lack of published experience to produce many challenges for the cochlear implant team. To the best of our knowledge, we present the first case reports with documented follow-up of cochlear implantation in two patients with different manifestations of Cockayne syndrome.