Objective Recent advances in artificial intelligence (AI) are driving innovative new health care solutions. We aim to review the state of the art of AI in otology and provide a discussion of work underway, current limitations, and future directions. Data Sources Two comprehensive databases, MEDLINE and EMBASE, were mined using a directed search strategy to identify all articles that applied AI to otology. Review Methods An initial abstract and title screening was completed. Exclusion criteria included nonavailable abstract and full text, language, and nonrelevance. References of included studies and relevant review articles were cross-checked to identify additional studies. Conclusion The database search identified 1374 articles. Abstract and title screening resulted in full-text retrieval of 96 articles. A total of N = 38 articles were retained. Applications of AI technologies involved the optimization of hearing aid technology (n = 5; 13% of all articles), speech enhancement technologies (n = 4; 11%), diagnosis and management of vestibular disorders (n = 11; 29%), prediction of sensorineural hearing loss outcomes (n = 9; 24%), interpretation of automatic brainstem responses (n = 5; 13%), and imaging modalities and image-processing techniques (n = 4; 10%). Publication counts of the included articles from each decade demonstrated a marked increase in interest in AI in recent years. Implications for Practice This review highlights several applications of AI that otologists and otolaryngologists alike should be aware of given the possibility of implementation in mainstream clinical practice. Although there remain significant ethical and regulatory challenges, AI powered systems offer great potential to shape how healthcare systems of the future operate and clinicians are key stakeholders in this process.