Perilymphatic fistula are difficult to diagnose, and the associated hearing loss is not easy to treat. In 18 patients with perilymphatic fistula, who visited our department from April 2008 to December 2010 with a confirmed diagnosis by exploratory tympanotomy, 19 ears were retrospectively analyzed from chart review. The purpose of this study was to investigate the clinical findings, treatment efficacies, and the clinical usefulness of the CTP (Cochlin-tomoprotein) detection test for this disease. The primary symptoms were hearing loss, and dizziness or vertigo, which were both observed in over 70% of the patients. Nystagmus was observed in 50% of the patients with vertigo or dizziness, the direction of which varied and exhibited no consistent correlation with the side on which the fistula had occured. The hearing loss recovered in 33%, especially when the operation was performed early after the onset.