The two French registries of childhood disabilities, which cover the departments of Haute-Garonne (RHE31), Isère, Savoie, and Haute-Savoie (RHEOP), are specifically tasked with systematically recording cases of severe and profound bilateral hearing loss in children aged 7 to 8 years. Inclusion in the registries is based on a review of medical records and audiometric test results, with severe or profound bilateral hearing loss defined as a hearing loss in the better ear strictly greater than 70 decibels (dB). The overall prevalence at age 8 for children born between 1997 and 2005 was 0.58 [0.5–0.7] per 1,000 children residing in the departments covered by the registries. The etiology was known in approximately half of the cases, and a genetic cause was identified in more than 30% of cases. Among cases of non-genetic origin, a pre- or postnatal infectious cause was most commonly implicated. Perinatal risk factors were present in 14% of cases for which the precise etiology was not identified. Hearing loss was associated with other disabilities in a number of cases: most often (11%) with mild or more severe intellectual disability, but also with motor disabilities (7%), epilepsy (4%), and pervasive developmental disorders (3%). The educational arrangements offered varied by the departments studied. The widespread adoption of neonatal hearing screening and advances in early diagnosis will allow for better observation and description of hearing loss and its causes. However, the increase in the prevalence of cases during childhood requires continued epidemiological surveillance beyond the neonatal period.