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1/5. Neurotologic manifestations and treatment of multiple spontaneous tegmental defects.

    OBJECTIVE: To describe the causes, histopathologic features, manifestations, and treatment of symptomatic multiple spontaneous tegmental defects. STUDY DESIGN: Retrospective review of three clinical cases and one temporal bone histopathology report. CLINICAL FEATURES: 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. INTERVENTION AND OUTCOMES: 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. CONCLUSIONS: Multiple tegmen defects constitute a special entity. Successful repair requires a middle fossa craniotomy with extended exposure.
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2/5. Spontaneous cerebrospinal fluid leakage and middle ear encephalocele in seven patients.

    Isolated cases of spontaneous cerebrospinal fluid (CSF) leakage with and without middle ear encephalocele have been reported. These leaks are usually accompanied by episodes of recurrent meningitis, hearing loss, or chronic headache. In this article, we report seven new cases of spontaneous CSF leakage. Six of these patients had conductive hearing loss and serous otitis media, and three had recurrent meningitis. Prior to a definitive diagnosis, six patients had received myringotomy tubes, which produced profuse clear otorrhea. Three patients had positive beta-2 transferrin assays. Computed tomography and magnetic resonance imaging confirmed a defect in the temporal bone tegmen. A combined transmastoid and middle fossa surgical approach with a three-layer closure was used to repair the tegmen defect. All patients had a lumbar drain placed prior to surgery. In addition to describing the seven new cases, we review the history of CSF leakage and discuss diagnostic methods, surgical findings, and our recommendations for management.
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3/5. Epidural blood patch at C2: diagnosis and treatment of spontaneous intracranial hypotension.

    Spontaneous intracranial hypotension in a 37-year-old man with intractable headaches was diagnosed on MR imaging. A generous CSF leak was identified at C2 on CT myelography. Successful treatment was performed with CT-guided blood patch at the leakage site after the patient had failed 2 lumbar blood patches. Imaging-guided precise placement of the blood patch is safe and recommended when a lumbar blood patch away from the leakage site could be ineffective.
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4/5. Otogenic pneumocephalus.

    Fifty-four previously reported cases of otogenic pneumocephalus were analyzed in addition to five new cases which are presented in detail. Forty-one males and 18 females were included with 95% of the patients being over 12 years of age. The most common presenting symptom was headache, and the ventricular system was the intracranial space most commonly involved. Tension pneumocephalus was present in 40 (66%) cases. Trauma (36%) was the most common etiologic factor, while otitis media (30%), otologic surgery (30%), and congenital defects (2%) accounted for the rest. The overall mortality was 12% with all patients succumbing to causes other than pneumocephalus. Because of its lack of specific symptoms, pneumocephalus was usually unsuspected and the diagnosis made only after radiographic evaluation. Despite its rarity, pneumocephalus has to be considered whenever the dura is violated, especially if associated with a CSF leak. Management depends on the degree of tension, symptomatology, and underlying cause. When associated with trauma or surgery, bedrest and close monitoring may suffice, although needle aspiration or re-exploration may be needed. When secondary to otitis media or a congenital defect, control of any infection and repair of the defect are mandatory.
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5/5. Spontaneous CSF communication to the middle ear and external auditory canal. A case report.

    A 27-year-old female with no history of trauma, surgery, infection, or neoplastic process was evaluated for the spontaneous onset of vomiting, headache, and loss of balance. Initial studies demonstrated extensive pneumocephalus. CT revealed a lytic, expansile defect of the right petrous bone, while intrathecal contrast images demonstrated flow of CSF that implied coincidental perforation of the tympanic membrane. MR imaging demonstrated a continuity of CSF signal. The patient underwent surgery to repair the CSF leak and a dural patch was applied. No symptoms of pneumocephalus were seen after surgery and the patient's condition improved.
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