Neurotologic manifestations and treatment of multiple spontaneous tegmental defects

To describe the causes, histopathologic features, manifestations, and treatment of symptomatic multiple spontaneous tegmental defects.

Retrospective review of three clinical cases and one temporal bone histopathology report.

Varied, including spontaneous cerebrospinal fluid otorhinorrhea, conductive hearing loss, chronic headaches, pneumocephalus, extradural abscess, and meningitis. A notable common feature was multiple (8-15) tegmental defects, 1 to 6 millimeters in diameter. Three of the four cases also included associated dural defects and small meningoencephaloceles or arachnoid granulations. Imaging studies generally underestimated the number of defects.

Successful middle cranial fossa repair with temporalis fascia was accomplished in the three clinical cases. Extension of exposure anteriorly and medially was necessary. Closure of the defects with a bone graft or equivalent synthetic material was not always possible, given the anatomic and pathologic features. Our data suggest that there are both congenital and acquired causes of the tegmental dehiscences.

Multiple tegmen defects constitute a special entity. Successful repair requires a middle fossa craniotomy with extended exposure.