Congenital perilymphatic fistula: a prospective study in infants and children.

A 3-year prospective study of 244 children (aged 5 months to 17 years) with sensorineural hearing loss of unknown cause revealed that 57 children (23%) had radiographic evidence of abnormalities of the temporal bone, detected by computed tomography scan, and/or progression of sensorineural hearing loss. Of these 57 children, 42 (74%) underwent surgical exploration, with 15 children (26%) of this subgroup demonstrating active congenital perilymphatic fistula. The prevalence of congenital perilymphatic fistula in a large, closely monitored population of children with unexplained sensorineural hearing loss appears to be at least 6% (15 of 244 children). The incidence may be higher because some congenital perilymphatic fistulae may leak intermittently and not be observed during middle ear surgery. Therefore, in children with suspected sensorineural hearing loss, early and frequent audiological evaluations should be made to rule out progressive/fluctuating sensorineural hearing loss. These children should also have radiographic imaging of their temporal bone by computed tomography scan. The use of both methods is the best indicator for congenital perilymphatic fistula.