Bilateral Cochlear Implantation in Pediatric Neurobrucellosis Complicated by Profound Sensorineural Hearing Loss and Complete Vision Loss.

This case report presents successful bilateral cochlear implantation (CI) in a pediatric patient with neurobrucellosis complicated by bilateral profound sensorineural hearing loss (SNHL) and irreversible bilateral vision loss. The report highlights the positive impact of CI on auditory perception and speech development in this complex clinical context. A 14-year-old girl developed acute bilateral blindness and subsequently experienced rapidly progressive bilateral SNHL despite appropriate antibiotic therapy for neurobrucellosis. In the 12th month following the onset of neurobrucellosis symptoms, she developed bilateral profound SNHL. Comprehensive preoperative audiological evaluation-including pure-tone audiometry, otoacoustic emissions, and auditory brainstem response testing-revealed absent otoacoustic emissions and cochlear microphonics, with no wave V responses at 100 dB nHL bilaterally. These findings functionally excluded auditory neuropathy spectrum disorder and indicated cochlear hair cell involvement rather than retrocochlear or central auditory pathway dysfunction, supporting the potential for favorable CI outcomes. Bilateral CI was performed via the transmastoid facial recess approach within one month after the diagnosis of bilateral profound SNHL. Postoperative outcomes were excellent, with a Categories of Auditory Performance score of 7 and 100% sentence recognition at 22 months postoperatively. Vision loss remained unchanged. This case underscores that, although neurobrucellosis-related SNHL may theoretically affect any part of the auditory pathway, timely diagnosis and appropriate patient selection can provide satisfactory hearing and speech outcomes through CI in pediatric neurobrucellosis.