Middle fossa repair of bilateral large congenital tegmental defects with meningoencephaloceles

June 4, 2012 by Anil Joshi, MS(ENT), MRCS; Wendy Smith, FRCS(ORL–HNS); David A. Moffat, FRCS
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Abstract

Spontaneous temporal meningoencephaloceles are unusual. When they do occur, they present with a variety of signs and symptoms, which can make diagnosis and management challenging. We report the interesting case of a 49-year-old woman with bilateral congenital temporal meningoencephaloceles. She presented with a 12-month history of bilateral fluctuating hearing loss, and she more recently developed right-sided acute otitis media with meningitis. The presentation of bilateral extensive tegmental defects and meningoencephaloceles with a fluctuating hearing loss and meningitis associated with acute otitis media affecting one ear and then subsequently the other ear is extremely rare and difficult to diagnose. It requires a very careful clinical and radiologic assessment. Methods of surgical repair differ depending on the size of the defects.

Introduction A congenital tegmental defect of the temporal bone with a temporal lobe meningoencephalocele is a rare condition. When it does occur, it can manifest with a variety of clinical signs and symptoms. If such a patient presents with spontaneous cerebrospinal fluid (CSF) leakage—either as CSF rhinorrhea or profuse otorrhea of “gin-clear” fluid through a grommet—the diagnosis is straightforward, and it can be confirmed by the presence of β2-transferrin in the CSF and by imaging.

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CitationEar Nose Throat J. 2012 June;91(6):E7-E10