In recent years, perilymph fistula(PLF) was diagnosed correctly and treated surgically by the improvement of diagnostic tools and surgical technique. The authors experienced two cases of PLF associated with recurrent meningitis, both of them were congenital bilateral deaf and had recurrent boutes of meningitis. In the past, it was misdiagnosed as sudden deaf or simple recurrent meningitis, but we have diagnosed PLF preoperatively with temporal bone CT after injection of metrizamide via lumbar puncture. The purpose of this article is to review pertinent literature on this subject and to present two additional case reports. Emphasis will be placed on the surgical technique employed in repair of the discovered defect.