Perilymph fistulas in infants and children.

During the years 1975 through 1981 we performed exploratory tympanotomies on 33 infants and children (44 ears) to verify the presumptive diagnosis of perilymph fistula (PLF). A PLF was identified at the round window, oval window, or both in 29 (66%) of the 44 ears explored. After surgery hearing was unchanged in 86%, improved in 5%, and worsened in 9% of the ears in which PLFs had been observed. Complaints of vertigo subsided in all children in whom a PLF was repaired. Preoperative factors determined to be highly suggestive of the presence of a PLF included the following: sudden onset of sensorineural hearing loss (SNHL), congenital deformities of the head, and abnormal findings on tomograms of the temporal bones, especially Mondini-like inner ear dysplasias. Middle ear abnormalities (primarily congenital) were observed in 20 of the 44 ears. Abnormal results of preoperative vestibular function studies, which included a fistula test, and sex were not consistently found to be associated with an observed PLF at tympanotomy.