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Cochlear Implant Outcomes for Children With Auditory Neuropathy Spectrum Disorder

Cochlear Implant Clinic, Royal Victorian Eye and Ear Hospital and Department of Otolaryngology, University of Melbourne
Melbourne, Australia

Gary Rance

Department of Otolaryngology, University of Melbourne
Melbourne, Australia

Shani Dettman

Department of Otolaryngology, University of Melbourne
Melbourne, Australia

Richard Dowell

Cochlear Implant Clinic, Royal Victorian Eye and Ear Hospital and Department of Otolaryngology, University of Melbourne
Melbourne, Australia

Cochlear implantation is currently the intervention option of choice for many children with auditory neuropathy spectrum disorder (ANSD) who are unable to obtain benefit from conventional amplification. The aim of this study was to review the speech perception and language outcomes for children with ANSD who had received a cochlear implant and highlight specific clinical considerations for working with this population of children with hearing impairment who are being considered for implantation. Finds for the group of 17 children with ANSD using cochlear implants were compared to previously reported outcomes for children with sensori-neural (SN) type hearing loss using cochlear implants.

Two children, identified with cochlear nerve deficiency pre-operatively, received no useful auditory percepts from their cochlear implant and discontinued device use. The remaining children demonstrated speech perception and language outcomes comparable to those observed for SN hearing loss peers using cochlear implants.

This paper highlights a number of considerations for clinicians to be aware of and the importance of careful counseling pre-operatively regarding the potential for less-than-optimal outcomes, particularly for those children identified with cochlear nerve deficiency.