Purpose: Increased knowledge of the prevalence of various craniofacial anomalies and their associated risks for hearing loss can help guide 1) development of evidence-based practice regarding detection and documentation of risk factors at birth and 2) health care professionals to make appropriate recommendations for follow-up testing and monitoring.

Method: Records were reviewed for 39,813 infants born at Adventist HealthCare Shady Grove Medical Center and White Oak Medical Center between January 1, 2014 and December 31, 2019 to determine the association between the presence of craniofacial anomalies and newborn hearing screening fail rates, as well as the prevalence of confirmed hearing loss for infants born with and without risk factors. Additionally, surveys were sent to state EHDI programs and newborn hearing screening program coordinators across the United States to determine how facilities document risk factors for hearing loss, specifically craniofacial anomalies.

Results: Study outcomes revealed four primary conclusions: (1) Infants with craniofacial anomalies are at greater risk for failing their newborn hearing screening; (2) There is a need to better delineate craniofacial anomaly risk factors into subgroups; (3) Follow-up audiologic evaluations are not warranted for infants with preauricular sinuses/tags and; (4) A universal protocol needs to be developed for recording risk factors for all infants.

Keywords: newborn hearing screening (NBHS), risk factors, craniofacial anomalies, hearing loss, Early Hearing Detection and Intervention (EHDI), Neonatal Intensive Care Unit (NICU)