Abstract

Sex linked recessive deafness is a rare cause of male genetic deafness, estimated to account for 6.2% of male genetic deafness in 1966. A male excess was found in the deaf population of Ireland in 1851. Reevaluation of this survey of 1851 confirms sex linked deafness as a factor in the disproportionate number of deaf males and suggests that 5% of congenital male deafness was the result of sex linked recessive deafness. This study confirms that a small but constant proportion of male deafness is the result of sex linked recessive deafness. The figure derived is used to calculate an empirical risk for carrier status in female sibs of isolated cases of male deafness.