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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.
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