Inherited sensorineural deafness is by far the most important cause of hearing loss in childhood, with an incidence of approximately 1-2 per 1000 births.

However, taken individually, each cause of inherited deafness is extremely rare.

90% are due to autosomal recessive genes.

The risk of recurrence for normal parents with one congenitally deaf child is 1 in 6. This figure is a composite derived from a figure for a new mutation causing autosomal dominant inheritance, and a figure for autosomal recessive inheritance.

The risk for the first child of a congenitally deaf parent with a normal spouse is 1 in 20.