Background: Intranasal dexmedetomidine (IN DEX) is a relatively new sedative agent with supporting evidence on its efficacy and safety, which can be used for procedural sedation in children, and could have a major role in auditory brainstem response testing, especially in the case of non-cooperative children. The goal of this systematic review is to assess the role of IN DEX in ABR testing, evaluating the reported protocol, potential, and limits. Methods: We performed a comprehensive search strategy on PubMed, Scopus, and Google Scholar, including studies in English on the pediatric population, without time restrictions. Results: Six articles, published between 2016 and 2021, were included in the systematic review. Sedation effectiveness was high across the studies, except for one study; 3 μg/kg was the dosing most often used. A comparison group was present in three studies, with oral chloral hydrate as the drug of comparison. Adverse effects were rarely reported. Conclusion: This systematic review showed how IN DEX can represent an adequate sedative for children undergoing ABR testing; larger and more rigorous trials are warranted in order to recommend its systematic utilization.