Introduction: Auditory Neuropathy Spectrum Disorder (ANSD) is characterized by normal cochlear function and abnormal neural function. Diagnosis must be performed early, so that rehabilitation is adequate and minimizes the negative consequences. Objectives: exhibit the procedures used for the early diagnosis of ANSD in children by developing a protocol, highlighting the importance of conducting studies on the incidence of ANSD and raising awareness among health professionals. Methods: systematic literature review. The electronic databases PubMed and Web of Science were used, applying the boolean operators "AND" and "NOT", with the terms, "Auditory Neuropathy", "diagnosis", "children", "hearing aids" and "cochlear implantation”. Results: 41 studies were analyzed after applying the inclusion/exclusion criteria, dating from 2015-2021. The USA is the country with the highest number of articles published. Discussion: The diagnosis of ANSD is made through otoacoustic emissions, cochlear microphonics and auditory brainstem response. This must be carried out in the first six months of life, and it is necessary to identify the possible etiology. Conclusion: the proposed objectives were achieved, namely the elaboration of the protocol. However it’s necessary to carry out studies on the incidence of ANSD in Portugal, as well as raising awareness among health professionals to this pathology.