Mondini dysplasia: spontaneous cerebrospinal fluid otorrhea. New perspectives in management.

The Mondini dysplasia represents a developmental arrest during the embryogenesis of the inner ear. The malformation, commonly associated with deafness, is frequently heralded by meningitis secondary to communication of the middle ear with the subarachnoid space. Early recognition and successful repair of the CSF leak is important to avoid the sequelae of repeated episodes of meningitis. We believe the addition of CSF diversion in the form of a lumbar drain during and after surgery will result in improved success.