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1/13. Congenital malformation of the inner ear associated with recurrent meningitis.

    Congenital deformities of the labyrinth of the inner ear can be associated with meningitis and varying degrees of hearing loss or deafness. A recurrence of meningitis is due to the development of a fistulous communication between the subarachnoid space and the middle ear cavity, and can prove lethal. An illustrative case of a 4-year-old Japanese girl with bilateral severe hearing loss, recurrent meningitis and malformations of the inner ear and stapes footplate is presented. Removal of the stapes during tympanotomy provoked a gush of cerebrospinal fluid. The defect was repaired successfully, and there has been no further episodes of meningitis to date.
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2/13. Spontaneous cerebrospinal fluid otorrhoea via oval window: an obscure cause of recurrent meningitis.

    Spontaneous cerebrospinal fluid (CSF) leak via the oval window is uncommon and can result in recurrent bacterial meningitis. Current understanding of spontaneous CSF otorrhoea is reviewed and a diagnostic algorithm is presented. A seven-year-old boy presented with bilateral congenital deafness and recurrent meningitis. High-resolution computed tomography (HRCT) of the temporal bone showed a labyrinthine deformity and communication between the internal auditory canal (IAC) and the cochlea. Subtotal petrosectomy with closure of the external acoustic meatus and eustachian tube was performed. Post-operatively, the child had no further episodes of meningitis. This rare and obscure cause of recurrent childhood meningitis requires a high index of suspicion and the use of diagnostic tools, especially HRCT.
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3/13. Mondini dysplasia: spontaneous cerebrospinal fluid otorrhea. New perspectives in management.

    The Mondini dysplasia represents a developmental arrest during the embryogenesis of the inner ear. The malformation, commonly associated with deafness, is frequently heralded by meningitis secondary to communication of the middle ear with the subarachnoid space. Early recognition and successful repair of the CSF leak is important to avoid the sequelae of repeated episodes of meningitis. We believe the addition of CSF diversion in the form of a lumbar drain during and after surgery will result in improved success.
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4/13. Arachnoid granulation cerebrospinal fluid otorrhea.

    A case report and review of the temporal bone (TB) collection in the Department of otolaryngology at SUNY health science Center in Syracuse demonstrated the occurrence of arachnoid granulations (AGs) in the posterior fossa surface of the TB and their role in cerebrospinal fluid (CSF) otorrhea. A large AG responsible for CSF otorrhea in a 64-year-old man was excised with soft tissue repair of the dural defect. Sixteen of 188 TBs (8.5%) in the collection contained 24 AGs ranging in size from 0.07 to 80.65 mm3. Nine AGs (37%) were small (less than 1 mm3) and did not demonstrate enlargement. Twelve (50%) were of intermediate size (2.50 to 9.32 mm3), and three (13%) were large (49.82 to 80.65 mm3). The intermediate and large AGs were associated with bone erosion and a high incidence of communication with a pneumatized mastoid complex (serous otitis media or meningitis). These findings suggest that AGs of sufficient size to produce bone erosion are the primary responsible lesions in adult-onset spontaneous CSF otorrhea.
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5/13. Spontaneous atraumatic CSF otorrhea.

    The clinical course of a five-month-old male with CSF otorrhea in the absence of head trauma is presented. The anatomy, workup, and management of this condition are described. The need to consider communication between the subarachnoid space and middle ear in any child with ear drainage, sensorineural hearing loss, or recurrent meningitis is emphasized.
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6/13. Tension pneumocephalus.

    Tension pneumocephalus occurs when intracranial air exists under pressure, resulting in neurologic deterioration. The syndrome is precluded by an extracranial-intracranial communication and a difference in extracranial-intracranial pressure with the latter being greater. Although most frequently associated with head trauma, a variety of situations, including an operative sitting position and use of nitrous oxide anesthesia, have been known to contribute to this potentially life-threatening complication. This article will address pathogenesis, assessment parameters, and medical and nursing approaches utilized to reduce and minimize further entrapment of air. A case report will be presented illustrating this condition.
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7/13. subarachnoid space: middle ear pathways and recurrent meningitis.

    Congenital bony abnormalities of the inner ear may result in a communication between the middle ear and the subarachnoid space. patients with this anomaly often present with recurrent meningitis associated with acute otitis media or with middle ear fluid. This article presents three cases of recurrent meningitis with open middle ear--subarachnoid space connections. The first two cases involve a cerebrospinal fluid leak into the middle ear via the oval window, both patients having a Mondini-type of inner ear deformity. The pathway in the third case opened into the middle ear along the horizontal portion of the facial nerve. Computed tomography (CT) scanning with metrizamide and differential density calculations helped to identify the abnormal pathway and to confirm that the leak has been closed postoperatively. Use of the CT scanner in these cases can be helpful in planning the surgical closure and in postoperative follow-up.
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8/13. Congenital deafness and spontaneous CSF otorrhea.

    Two patients with congenital deafness had spontaneous CSF otorrhea and recurrent bacterial meningitis. A careful workup, including tomograms, is especially important in the treatment of the closed type of CSF otorrhea. At operation of our patients, the leak was found to be at or near the oval window. The stapes was removed, and muscle was packed into the defect. The profuse flow of CSF was believed to be secondary to a wide-open cochlear aqueduct in the first case, and to an abnormal communication between the internal auditory meatus and the vestibule in the second case. These findings were the bases for the recurrent bacterial meningitis.
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9/13. stapes gusher and klippel-feil syndrome.

    The stapes gusher which is the most dramatic complication of stapedectomy arises from an abnormal communication between the subarchnoid and perilymphatic spaces. This congenital defect may be associated with other anomalies such as the klippel-feil syndrome. Two cases of stapes gusher in patients with congenital fixation of the footplate are described. One of them was combined with klippel-feil syndrome. The object of this paper is to emphasize the necessity of a complete preoperative examination of all cases of congenital footplate fixation.
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10/13. 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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