Syndrome of recurrent meninigitis due to congenital perilymph fistula withtwo different-clinical presentations

Recurrent meningitis secondary to a congenital labyrinthine anomaly is a ra re clinical entity, diagnosis of which is dependent upon certain clinical, radiological and intraoperative features. In the following report we descri be two children with congenital labyrinthine fistula and recurrent meningit is whose clinical presentation, radiological features and intraoperative fi ndings were dissimilar and thus, illustrative of two different ways of pres entation of this rare disorder. While one had a classical Mondini defect an d unilateral hearing loss, the other had normal audiometric and radiographi c findings. The fistulae were successfully closed via a tympanotomy approac h in both the patients. (C) 2000 Elsevier Science Ireland Ltd. All rights r eserved.