Cochlear implantation for sensorineural hearing loss caused by neonatal hyperbilirubinemia.

BACKGROUND: Hyperbilirubinemia is a leading cause of sensorineural hearing loss (SNHL) and auditory neuropathy spectrum disorder (ANSD) in neonates. This review synthesizes current evidence to describe key aspects of cochlear implantation (CI) in neonates with hyperbilirubinemia. OBJECTIVE: To educate a wide audience on the pathophysiology, audiometric challenges, hearing aid trial considerations, optimal timing, and expected outcomes of CI in neonates with bilirubin-induced hearing loss. METHODS: A comprehensive review was conducted on PubMed and Cochrane databases to explore current clinical practice guidance for evaluating neonates with hyperbilirubinemia for CI. RESULTS: Bilirubin has neurotoxic effects on the auditory pathway in neonates, making it challenging to have strict audiometric criteria for CI. Hearing trials serve the dual purpose of diagnosis and therapy for infants with hearing loss and can identify strong candidates for CI or, conversely, exclude children who may not benefit from CI. Optimal timing of CI balances diagnostic certainty with earlier intervention. Provider counseling is crucial in preparing families for expected outcomes after CI, which are generally favorable, but can vary among infants depending on factors such as interdisciplinary care coordination. CONCLUSION: Audiologic monitoring, careful CI decision-making, and realistic outcome counseling optimize candidate selection and care for neonates with bilirubin-induced hearing loss.