The cause of (pediatric) sensorineural hearing loss is diverse, comprising genetic, acquired, and idiopathic conditions. Identifying the specific cause requires otolaryngologic and audiometric evaluations. These generally include laboratory tests and temporal bone imaging studies. A sequential diagnostic approach has been shown to be both prudent and cost-effective. Although a definitive cause is being established, amplification and a comprehensive treatment strategy should be initiated to ensure that developmental, auditory, and speech and language delays are minimized.