Speech reception after cochlear implantation for Cogan's syndrome: Case series following CARE guidelines

Cogan's syndrome is a rare form of vasculitis mainly affecting young subjects of whatever gender, associating cochleovestibular and ophthalmological damage. Despite medical treatment, auditory prognosis is uncertain, with 50-60% of patients showing irreversible severe to profound hearing loss, thus being candidates for cochlear implantation. Following CARE guidelines, we report 10 cases of cochlear implantation in Cogan's syndrome, with assessment of speech reception threshold and maximum intelligibility after a minimum 1 year's experience with the implant.

Ten patients from 4 centers received cochlear implants (14 implants) for Cogan's syndrome between 2005 and 2020. After 1 year's experience, there was significant improvement in speech reception threshold (P=0.0002) and maximum intelligibility (P=0.0002).

Audiovestibular signs associated with ophthalmological manifestations should suggest Cogan's syndrome. Hearing impairment is usually irreversible. Cochlear implantation may be necessary and improves hearing performance.