Deafness is the most common sensory disability in developed countries affecting more than 2 births in 1000. Eighty percent of congenital deafness is genetic. Universal newborn hearing screening has been in place since 2012 in France. All genetic hearing losses are not congenital and all congenital hearing losses are not genetic. Genetic hearing loss may be syndromic (associated with other symptoms) (10 %) or non-syndromic (isolated) (90 %). Hearing loss may initially be the only symptom of syndromic deafness. A genetic origin can be diagnosed and must therefore be evoked systematically even in the adult.
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