Authors report one case of bilateral inner ear malformation in a 3 years-old child with congenital sensorineural hearing loss, recurrent meningitis and cerebro-spinal fluid leakage. This anomaly is a pseudo-Mondini's malformation with a meningocele through the oval window and a fistula of C.S.F. of the round window. A close radiological and surgical correlation support some physiopathological hypothesis about fistula between sub arachnoid space and middle ear. Authors discuss the place of high resolution computed tomography in case of congenital sensorineural learning loss.