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Spontaneous Bilateral Meningoencephalocoeles of the Temporal Bones

DOI: 10.1155/2013/969762

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Abstract:

Spontaneous tegmen tympani defects are rare with even rarer bilateral cases. The symptoms are nonspecific; hence, a high index of suspicion is required to prevent serious intracranial complications. We present a case of spontaneous bilateral tegmen tympani defects with associated meningoencephalocoeles in a 54-year-old male who presented with the signs and symptoms of severe meningitis. After careful workup which included a lumbar puncture, CT and MRI scans, both defects were repaired using a middle fossa approach. The patient made an uneventful recovery with complete cessation of otorrhoea and improvement in his hearing. 1. Introduction CSF leaks from skull base defects are classified as either spontaneous with no obvious cause or secondary due to trauma, surgery, tumours, infection, and inflammation [1, 2]. Patients may be asymptomatic or present with intracranial complications such as meningitis. Meningoencephalocoeles of the temporal bone can herniate through the tegmen tympani. If there is a defect into the CSF space, patients may present with CSF otorrhoea in the presence of a perforated tympanic membrane or CSF rhinorrhoea through the Eustachian tube [3]. A conductive hearing loss may be due to a CSF middle ear effusion. Spontaneous unilateral tegmen tympani defects are uncommon; bilateral defects are rarely encountered [4]. There are distinct patient groups who are at risk of meningitis from acute otitis media: children with congenital inner ear abnormalities (e.g., Mondini dysplasia), dehiscent stapes footplate, tympanomeningeal fistula, patent fallopian canal, and middle-aged adults with no identifiable congenital or acquired causes [1, 2, 5]. Aetiological theories of idiopathic intracranial hypertension and arachnoid granulations in bony erosions have been suggested [6, 7]. We present a case of bilateral meningoencephalocoeles of the temporal bones with CSF leak and review the literature of this entity. 2. Case Report A 54-year-old man presented to the emergency department with agitation and seizures following a generalized febrile illness and a progressively worsening headache over 24 hours. He was treated with ceftriaxone IV, metronidazole, and acyclovir. A contrast CT scan of the brain showed no focal central lesion but features in keeping with bilateral chronic middle ear and mastoid disease. A lumbar puncture showed no evidence of raised intracranial pressure (ICP). CSF microscopy supported the diagnosis of meningitis, and on culture streptococcus sensitive to ceftriaxone and amoxicillin was grown. He responded to a three-week course of

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