Perilymph fistula is recognized as a clinically significant problem in certain patients. Owing to its lack of specific symptoms, diagnosis is difficult and depends upon a combination of historical data, physical findings, and test results. Early treatment is initiated in patients with overwhelming evidence of perilymph fistula, in order to ameliorate vestibular symptoms and preserve auditory function. Patients with subtle symptoms, however, should not be overlooked, because they will also benefit from the treatment of this disease.