Cochlear Implantation in a Case of Relapsing Polychondritis With Profound Hearing Loss and Labyrinthine Ossification.

ABSTRACT

Relapsing polychondritis is an autoimmune disorder causing inflammation of cartilaginous structures, sensory epithelium, and cardiovascular system. Hearing loss is a rare and dreadful complication of this pathology. We report a case of relapsing polychondritis in a 38-year-old female who developed gradually progressive bilateral profound hearing loss. She did not have any improvement with medical management. Cochlear implantation was performed to rehabilitate her hearing. As the scala tympani was obliterated, a scala vestibuli insertion was performed. A complete insertion was possible with a compressed electrode, and she had good evoked compound action potential scores. Her categories of auditory performance scores were 6 at the end of one year. Patients with relapsing polychondritis can progress to profound hearing loss in rare cases and should be carefully followed up to identify early labyrinthine ossification. A scala vestibuli insertion can be performed with good outcomes in cases with ossification involving scala tympani. The surgeon should be ready for a middle-turn cochleostomy or a drill-out procedure in patients with advanced ossification.