Conclusions Spontaneous defects between the mastoid and the posterior cranial fossa are exceedingly rare. Patients with these lesions may have a lower BMI compared to those with middle cranial fossa encephaloceles, but are otherwise demographically similar. This study recommends repair via a transtemporal approach to allow for examination of the entire posterior face of the temporal bone. Objective To describe cases of spontaneous posterior cranial fossa defects. Methods This study reviewed all cases of spontaneous posterior fossa defects presenting to a tertiary referral center over the last decade and described clinical presentation, imaging, operative findings, and outcomes. We also compared these lesions to those previously reported in the literature as well as the more common spontaneous encephaloceles of the middle cranial fossa.Results This study identified five cases with a mean age of 61.4 years, female-to-male ratio of 4:1, and a mean BMI of 31. Three cases presented with spontaneous pneumocephalus, one with CSF otorrhea, and one as an incidental imaging finding. Four defects were found medial to the sigmoid sinus and one was in the lateral retrosigmoid air cells.
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