Audiologic Management and Outcomes of Children with Auditory Neuropathy Spectrum Disorder (ANSD)

Auditory Neuropathy Spectrum Disorder (ANSD) is clinically characterized by abnormal Auditory Brainstem Response (ABR) wave morphology and normal outer hair cell function with present cochlear microphonic and/or otoacoustic emissions (OAEs) (Starr et al, 1996). This combination of features makes electrophysiologic prediction of functional hearing and outcomes of amplification difficult to predict (Berlin et al, 2010). Hearing ranges from normal to profound loss in one or both ears, and with equally variable speech performance that is often disproportionately poor relative to the degree of hearing loss (Rance et al, 1999). There is no current international standard of management for this population. Clinical interventions vary between no intervention, hearing aids, and cochlear implants. The purpose of this systematic review is to evaluate the current literature for management strategies for ANSD internationally and their associated outcomes