Congenital hearing loss (CHL) refers to hearing loss present at birth and has significant implications for a child’s development and quality of life. In the United States, CHL affects approximately 2–3 out of every 1000 children. The majority of cases involve sensorineural hearing loss, with causes categorized as genetic, nongenetic/acquired factors, or idiopathic in nature. Genetic causes account for around half of congenital or childhood hearing loss cases and can be further classified as syndromic or non-syndromic. Nongenetic CHL is commonly associated with infectious diseases such as cytomegalovirus (CMV), toxoplasmosis, rubella, herpes, syphilis, as well as factors like ototoxicity, prematurity, and asphyxiation. Early diagnosis is crucial for minimizing the impact of CHL. Full-term newborns typically undergo hearing screening using methods such as otoacoustic emissions (OAEs). If an infant does not pass the initial screening, a follow-up screening is recommended. If the second screening results in a referral, diagnostic auditory brainstem response (ABR) testing is advised. If the ABR results indicate hearing loss, comprehensive audiological and etiological assessments are necessary. These assessments should include a review of the family history, imaging studies such as magnetic resonance imaging (MRI) or computed tomography (CT) scans, virological examinations, and genetic tests based on the suspected cause. Management and treatment of CHL depend on the severity and underlying cause. Options for hearing restoration include implantable or non-implantable devices such as conventional hearing aids, cochlear implants, and bone-anchored hearing aids.