PURPOSE This review summarizes current evidence related to the audiologic management of children with auditory neuropathy spectrum disorder (ANSD). METHOD A systematic search of the literature was conducted in 25 electronic databases (e.g., PubMed, CINAHL, and ERIC) using key words such as auditory neuropathy, auditory neuropathy spectrum disorder, auditory neuropathy/dyssynchrony, and hearing loss. Eighteen studies met the inclusion criteria by addressing 1 or more of 8 clinical questions. Studies were evaluated for methodological quality, and data regarding participant, intervention, and outcome variables are reported. RESULTS Fifteen of the 18 studies addressed the use of cochlear implantation, and 4 addressed conventional acoustic amplification. All participants demonstrated improved auditory performance; however, all 18 studies were considered exploratory, and many had methodological limitations. CONCLUSION The clinical evidence related to intervention for ANSD is at a very preliminary stage. Additional research is needed to address the efficacy of acoustic amplification and cochlear implantation in children with ANSD and the impact of this disorder on developmental outcomes.