Congenital perilymphatic fistula: an overlooked diagnosis?

Congenital perilymphatic fistula (CPLF) often is the sole cause of sensorineural hearing loss in children that is both progressive and subtle and, therefore, can elude detection by physicians. A 3-year prospective study of 244 children with sensorineural hearing loss of unknown etiology demonstrated that at least 6% (15/244) had CPLF. Close audiologic monitoring and radiologic imaging of the temporal bone of children with sensorineural hearing loss are recommended to detect CPLF.