Spontaneous cerebrospinal fluid otorrhea in association with a congenital defect of the cochlear aqueduct and Mondini dysplasia.

Unrecognized spontaneous cerebrospinal fluid (CSF) otorrhea led to recurrent bacterial meningitis in three children. The underlying cause of the spontaneous CSF otorrhea was proved to be a congenital cystic dilatation of the cochlear aqueduct and Mondini dysplasia of the temporal bone. The CSF leak recurred in all patients after an initial surgical attempts to close the defects through a tympanotomy. A suboccipital approach was used successfully to obliterate the CSF fistulas in two children; a translabyrinthine approach was used in the other. A search of the literature revealed that Mondini dysplasia is a congenital anomaly that can commonly cause spontaneous CSF otorrhea in children. The authors discuss the management of this uncommon entity on the basis of a literature review and their own experience.