Spontaneous cerebrospinal fluid otorrhea.

Spontaneous cerebrospinal fluid (CSF) otorrhea is rare. We present four new cases and an analysis of the literature. Two distinct subtypes occur. Seventy-two percent of cases are the childhood type with congenital defects of the otic capsule. Meningitis, usually pneumococcal and frequently recurrent, occurs in 92% of these cases. CSF otorrhea follows myringotomy for a presumed serous effusion. The child usually has unilateral and sometimes bilateral absence of cochlear and vestibular function and commonly exhibits a Mondini deformity. CSF usually enters the inner ear through a dural defect in the lateral aspect of the internal auditory canal and exists through the oval window. Treatment should consist of stapedectomy and packing of the vestibule with muscle or subtotal petrosectomy. Twenty-eight percent of cases of spontaneous CSF otorrhea are the adult type characterized by bony dehiscenses, most commonly of the tegmen tympani or tegmen mastoideum and less commonly of the posterior fossa plate. The meningeal defects are either meningoencephaloceles or simply holes in the dura. Therapy should consist of a mastoidectomy in conjunction with a transtemporal supralabyrinthine (middle fossa) approach if a meningoencephalocele of the tegmen is found.