Outcome of cochlear implantation in a case of cochlear aplasia with cochlear nerve deficiency

Cochlear nerve deficiency in cochlear aplasia is a contraindication for cochlear implantation (CI) anticipating poor auditory response. Few authors have reported auditory outcomes even without nerves following CI. This study outlines the audiological outcomes of a patient in early childhood with cochlear aplasia and cochlear nerve deficiency who underwent CI. Auditory and speech-language development were assessed using the Categories of Auditory Performance (CAP) scale, Speech Intelligibility Rating (SIR) scale and Ling-6 sound test; at the time of switch-on, after 6 and 12 months of auditory verbal therapy. Significant differences across CAP, SIR and Ling-6 sound detection scores were noted, with the highest mean scores at the 12th-month postimplantation, indicating substantial improvement in auditory and speech-language skills. In cochlear aplasia cases, residual cochlear elements and nerve fibres cannot be ruled out. Our report emphasises the need for research, as this has the potential to impact the existing guidelines for CI candidacy.