Cerebrospinal fluid (CSF) otorrhea is usually associated with a history of trauma or previous surgery. Spontaneous CSF otorrhea is uncommon. When such spontaneous CSF leakage occurs, it may be associated with dural herniation, with the production of conductive hearing loss from impingement on the ossicles. This type of leakage has characteristic findings which, if recognized, can lead to earlier diagnosis and avoidance of complications. A review of the world literature revealed 44 reported cases. This report presents the authors' experience with 12 patients with spontaneous CSF leakage from temporal bone defects. This entity may be more prevalent than is reported. The differential diagnosis and management of CSF otorrhea (secondary to temporal bone defects) are discussed and representative cases are presented.