This article reviews the role of the otolaryngologist-head and neck surgeon-in the diagnosis and treatment of hearing loss in infants and young children. The otolaryngologist is well-versed in the anatomy, physiology, and pathophysiology of the auditory system, as well as the craniofacial syndromes that can involve the head and neck in combination with deafness. In this paper, the various causes of congenital hearing loss are described, as well as the steps required for proper diagnosis. Finally, surgeries used by otolaryngologists to treat childhood hearing loss, their indications, and outcomes, are discussed.