Syndromic disorders in congenital hearing loss

Objectives: To investigate the aetiology of congenital hearing loss detected by the universal neonatal hearing screening programme (Algo (R)) that was introduced in Flanders 15 years ago, and, more specifically, to investigate genetic causes. Methodology: Diagnostic work-up of all children with confirmed hearing loss after referral by the Algo (R) screening programme and screening at the neonatal intensive care unit (NICU) of our university hospital. Results: A hearing loss was confirmed in 505 of the 569 neonates (18% from NICU) referred between 1997 and 2011. After further examination, a genetic origin was identified in 84(17%) of 100 children with a syndromic hearing loss. The most frequent syndromes are discussed. Conclusion: A higher percentage of syndromic hearing loss was found than in the literature. This could be explained by the good cooperation with the human genetics department and the proportion of children retrieved from the neonatal intensive care unit.