Severe congenital deafness

Severe congenital deafness affects approximately 1 in 1000
infants. This may occur as an isolated deafness as or part of a
syndrome. At least half the cases of congenital deafness have a
genetic aetiology. Of genetic cases, approximately 66% are
autosomal recessive, 31% are autosomal dominant, 3% are
X linked recessive. Over 30 autosomal recessive loci have been
identified. This means that two parents with autosomal
recessive congenital deafness will have no deaf children