[Congenital hearing loss in children].

The identification of congenital hearing loss using Transient Evoked Otoacoustic Emissions and Automatic Auditory Brainstem Response in a newborn hearing screening program is crucial for initiating early rehabilitation with hearing aids or cochlear implants. Specific genetic causes, such as Pendred syndrome, connexin-26, stereocilin, and otoferlin-associated deafness, can be identified today using gene panels. Specifically, for otoferlin-associated deafness, it may be possible to offer gene therapy as a novel treatment for this specific genetic type of hearing loss.