Perilymphatic fistulae are the first cause of perceptive deafness that may be amenable to surgical treatment. After a complete study of the literature, we have made a retrospective analysis of 48 ear explorations in 37 children. These children presented with a variable degree of progressive or fluctuating perceptive deafness without any obvious etiology. Computed tomography, especially aimed at detecting an abnormality in the patency of the aqueduct of the cochlea, seems to be the most efficient criterion of selection to establish an indication for surgical exploration. The extent of the perilymphatic fistulae and their location, mainly at the level of the fissura ante fenestram, call to our mind the possibility of an arrest in the differentiation of the otic capsule, with a persistent anomalous patency of the aqueduct of the cochlea. The results of this study mainly demonstrate stabilizations of hearing, while spectacular improvements still are anecdotal. On the other hand, morbidity is very low and mainly results from insufficient surgery to fill in the fistula. Further studies are needed, especially to better understand the pathophysiology of the perilymphatic fistulae, and their results must certainly be appreciated in the longer term.