Introduction: The relationship between hearing and language development has been widely demonstrated, as children learn to speak by imitating sounds and interpreting them as they are orally taught by their parents. Because of this, countries are trying to put more emphasis on universal screening of newborns. In Chile, the first efforts of early detection of deafness have been in screening high risk newborns. En Chile there are no studies on the causes of deafness, and for this reason the present study seeks to investigate this issue in a group of Deaf children in special schools in Santiago. Understanding the causes of congenital and early childhood deafness allows for better prevention during pregnancy and early childhood. Objective: Establish the causes of severe deafness, according to the knowledge of parents or legal guardians and school records, in young students in special schools for the Deaf. Materials and Methods: In this observational study, the universe consisted of all children that attend 4 special schools for the Deaf in Santiago. The sample consisted of 315 cases, which represent 92% of the universe. School records were examined and information was contrasted and completed with and interview with parents or guardians. Conclusions: 63% of children suffered from congenital deafness, 9% of those due to infection during pregnancy. In 41.5% of all cases the cause of deafness was unknown. 13% was due to meningitis, and 5.8% was due to prematurity.