Labyrinthine fistula, defined by a destruction of the bony labyrinth is one of the most feared complications during the evolution of a chronic otitis media with cholesteatoma. Relatively common, it represents 4-12% cases of chronic otitis media with cholesteatoma and essentially occurs in the lateral semi-circular canal (70-80% of cases). The abnormal opening of the bony labyrinth in the middle ear runs the risk of recurrent labyrinthitis with dizziness, sensorineural hearing loss, purulent labyrinthitis and meningitis. Its treatment is surgical, however, an alteration of cochleovestibular function during surgery may occur. We propose in this development to review signs that suggest this complication and to establish a practical management of labyrinthine fistula in chronic otitis media with cholesteatoma.