Hearing loss is the most common congenital sensory impairment, rising to a prevalence of >15; of 19-year-olds. Advancements in genetic diagnosis, temporal bone imaging, and increased awareness of the importance of congenital cytomegalovirus (CMV) infection make knowledge of the fundamentals of evaluation and management essential. We present up to date information for the practicing otolaryngologist about the diagnosis of pediatric hearing loss, which can directly affect prognosis and management, including timing of cochlear implantation and cochlear implant outcomes. Over 50 of pediatric hearing loss has a genetic etiology, with genetic therapy on the horizon. Additional causes covered in this presentation include congenital CMV and its current diagnosis and management, and congenital and acquired temporal bone anomalies and related brain findings based on CT and MRI imaging. Unilateral and sudden hearing loss will also be addressed.