Bilateral cochlear implantation in a young patient with xeroderma pigmentosum (XP-D) and progressive sensorineural hearing loss—How to do it?

Abstract

Abstract Xeroderma pigmentosum (XP), a rare genetic skin condition, causes ultraviolet (UV)-induced neoplasms and possible neurological deficits including sensorineural hearingloss. We present the first case in literature of bilateral cochlear implantation (CI) in a patient with XP-D with neurodegeneration. Multi-disciplinary team members (national XP team, dermatologist, anaesthetist, theatre team, biophysicists) were involved. UV exposure from equipment and areas where the 14-year-old patient would track was measured. Maximum possible surgery was performed under operating headlights to limit higher-UV microscope exposure. Its bulb light intensity was reduced to achieve safe UV level (0–10 μW/cm2). Skin was protected under surgical drapes. Challenges included drilling unpredicted hard thick bone under low-intensity light and requiring bulkier Nucleus®-7 processor due to unanticipated increased scarring. A delayed left facial weakness was resolved with steroids. He is undergoing hearing rehabilitation. This highlights challenges of CI in XP. Its impact in preserving cognition and on neurodegeneration should also be observed.