Severe and profound hearing loss (> 70 dB) were analysed in a retrospective study of 226 children, born between 1976 and 1985, and recruited from three French administrative departments. The prevalence was 0.54 per 1000 children under 9 years old, with no decrease over the study period. A hereditary origin was identified in 20.8% of cases and an infectious origin in 11.5%. Perinatal risk factors were present in 11.5%, while the aetiology was undetermined in more than half the cases. In 85.8% of the children there was no other severe impairment. Marked learning difficulties were observed: 36% of the children were two years behind their age group and 28% were more than two years behind. The age of initial care decreased over the study period but is still too advanced. Systematic neonatal screening would enable earlier care, which should limit the social and educational impact of hearing loss.