Cases reported "Neuroma, Acoustic"

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1/211. Suboccipital resection of a medial acoustic neuroma with hearing preservation.

    BACKGROUND: Many reports indicate that acoustic neuromas greater than 2.0 cm should be removed without hearing preservation attempted, even if hearing is present preoperatively. These studies advocate a translabyrinthine approach because the likelihood of hearing preservation is low. Medial acoustic neuromas, unlike the more common lateral tumors that involve the internal auditory canal, originate medial to that portion of the eighth nerve complex where the cochlear and vestibular nerves are fused. This anatomical feature suggests that these tumors may be amenable to resection with hearing preservation. methods: A patient with a 3.5 cm medial acoustic neuroma and useful preoperative hearing is presented. RESULTS: Gross total tumor removal with functional hearing was achieved after a two-stage procedure using a suboccipital approach. CONCLUSION: Based on the anatomico-pathologic features in this case, we believe that, if a patient has reasonable preoperative hearing (speech discrimination score > 70%) and a medial acoustic neuroma, an approach to preserve hearing should be considered regardless of tumor size.
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2/211. Magnetic resonance cisternography using the fast spin echo method for the evaluation of vestibular schwannoma.

    neuroimaging of vestibular schwannoma was performed with the fat-suppression spoiled gradient recalled acquisition in the steady state (SPGR) method and magnetic resonance (MR) cisternography, which is a fast spin echo method using a long echo train length, for the preoperative evaluation of the lateral extension of the tumor in the internal auditory canal, and the anatomical identification of the posterior semicircular canal and the nerves in the canal distal to the tumor. The SPGR method overestimated the lateral extension in eight cases, probably because of enhancement of the nerves adjacent to the tumor in the canal. The posterior semicircular canal could not be clearly identified, and the cranial nerves in the canal were shown only as a nerve bundle. In contrast, MR cisternography showed clear images of the lateral extension of the tumor and the facial and cochlear nerves adjacent to the tumor in the internal auditory canal. The anatomical location of the posterior semicircular canal was also clearly shown. These preoperative findings are very useful to plan the extent to which the internal auditory canal can be opened, and for intraoperative identification of the nerves in the canal. MR cisternography is less invasive since no contrast material or radiation is required, as with thin-slice high-resolution computed tomography (CT). MR cisternography should replace high-resolution CT for the preoperative neuroradiological evaluation of vestibular schwannoma.
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3/211. Persistent trigeminal neuralgia after removal of contralateral posterior cranial fossa tumor. Report of two cases.

    BACKGROUND: Contralateral trigeminal neuralgia as a false localizing sign in patients with posterior cranial fossa tumors is rare. Persistent contralateral trigeminal neuralgia after removal of the posterior fossa expanding lesion with microsurgical exploration of the affected trigeminal nerve root has been described in only a few reports. Displacement of the brainstem and the trigeminal nerve root, arachnoid adhesions, and vascular compression of the nerve root entry zone have been reported as causes of persistent contralateral trigeminal neuralgia. methods: One patient developed transformation of the contralateral constant burning facial pain into trigeminal neuralgia after removal of a posterior fossa meningioma. A typical right-sided tic douloureux in our second patient did not disappear after removal of a left acoustic neurinoma. CT scan revealed brainstem displacement to the side of trigeminal neuralgia. Microsurgical exploration in both cases demonstrated the squeezed and distorted trigeminal nerve root and displaced brain stem with no vascular involvement. Both patients underwent partial trigeminal rhizotomy for pain control. RESULTS: Complete disappearance of the trigeminal neuralgia was evident in both cases with postoperative facial sensory loss. The postoperative course in the first case was uneventful; the second patient died from purulent meningoencephalitis. CONCLUSION: Persistent contralateral trigeminal neuralgia after removal of a posterior fossa tumor is caused by distortion of the fifth nerve root by the displaced brainstem. Partial trigeminal rhizotomy can be performed for alleviation of facial neuralgic pain in cases without neurovascular compression.
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4/211. Hearing impairment in patients with acoustic neuroma--analysis by electrocochleography.

    OBJECTIVE: In patients with acoustic neuroma, the site and severity of hearing impairment are important in discussing surgical approaches. Since the effectiveness of conventional auditory psychological testing is limited, we studied objectively hearing impairment of the cochlea and the cochlear nerve due to the tumor. methods: Electrocochleography (ECochG) was carried out in 21 patients with acoustic neuroma. Cochlear microphonic potential (CM) and action potential (AP) in ECochG evoked with clicks and short tone bursts were recorded through a transtympanic needle electrode technique. Cochlear function was studied using the detection thresholds of CM, and cochlear nerve involvement was analyzed by differences between AP and CM detection thresholds. RESULTS: The 1 kHz CM detection threshold was elevated in 17 (81.0%) of 21 patients indicating cochlear impairment. Of seven patients with normal hearing or mild sensorineural hearing loss in pure tone audiometry, three had a slightly elevated CM detection threshold. Of five patients with pronounced pure tone levels, four showed a CM response and were thought to have mild cochlear dysfunction. cochlear nerve impairment was confirmed in three of four patients with well-developed CM based on elevated AP detection thresholds. Three patients had CM response but no AP response, suggesting severe cochlear nerve impairment. CONCLUSION: Disorders of the cochlea and the cochlear nerve can be evaluated with ECochG AP and CM measurement. The findings of ECochG are thought to be important information to judge hearing prognosis, thereby enhancing its clinical utility.
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5/211. facial nerve grafts: from cerebellopontine angle and beyond.

    OBJECTIVES: To review the outcomes from facial nerve cable grafting in a variety of pathologic conditions and to determine the possible causes of poor facial function after facial nerve grafting. STUDY DESIGN: A retrospective review of patients undergoing facial nerve grafts through a neurotologic procedure. SETTING: This study was performed at a university-based tertiary referral center. patients: All 27 patients undergoing a neurotologic procedure that required a facial nerve graft from 1982 to 1997 were reviewed. Fourteen patients had grafts from the facial nerve stump at the brainstem. INTERVENTIONS: All patients had facial nerve function determined by the senior neurotologist and through use of historical data and photographs. MAIN OUTCOME MEASURE: A facial nerve repair recovery score was developed for facial nerve transection and repair. The House-Brackmann scale was found to be inappropriate for transected and repaired nerves. RESULTS: Facial function was found in 23/25 (92%) patients with at least 8 months follow-up. Statistical analysis revealed no significant correlation between graft length and recovery score. patients who had the nerve grafted to a site distal to the meatal foramen had a mean outcome that was nearly one grade level better than those with an anastomosis proximal to the meatal foramen (i.e., at the brainstem), but this did not reach statistical significance. CONCLUSIONS: facial nerve grafting is an effective way of restoring facial motor function. A new facial recovery grading scale is proposed for repaired or grafted facial nerves.
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6/211. Stereotactic radiosurgery, X: clinical isodosimetry of gamma knife versus linear accelerator X-knife for pituitary and acoustic tumours.

    Several review articles have compared gamma unit versus linear accelerator (linac)-based radiosurgery systems, concluding that the dose gradient 'fall-off' at the margin of the target (expressed as the distance between isodoses) is very similar for both techniques as far as single isocentre treatment volumes up to 1.5 cm diameter are concerned, and that the two radiosurgical systems are, in general, comparable. 'Fine tuning' of the gamma unit can be carried out by using multiple isocentre plans, the differential use of small collimator sizes (down to 4 mm) and field weightings, and adroit use of the gamma angle, and selective beam blocking. Multiple isocentre plans, beam modification, restriction of gantry angles and arc lengths, and microcollimation can similarly improve the isodose gradients from linac units. In both instances, the dosimetric advantages occur along selected aspects of the target perimeter border. However, the more frequent use of multiple isocentred 'shots' on the gamma unit achieves greater conformity indices for more complex target volumes, but at the expense of steeper internal dose gradients. We studied two patients with tumours close to or arising from radiosensitive special sensory nerves (optic and cochlear) to compare and contrast fine tuning of the two technologies. In a previously irradiated patient with a pituitary adenoma, the dose gradient achieved at the rostral margin, adjacent to the optic chiasma, was steeper on the gamma unit (due to the concentration of small collimator shots rostrally and beam blocking), which was therefore the dosimetrically preferred technique. In contrast, the vastly smaller internal dose gradient (11% for linac/X-knife versus 100% for Gamma Knife) and the ability to fractionate on the X-knife system, gave a large dosimetric advantage to the X-knife plan in the treatment of an acoustic neuroma, where the intracanalicular component of the cochlear nerve traversed the target volume. This advantage also pertains to the cochlear ramus of the internal auditory (labyrinthine) artery and the facial nerve. Our published work on X-knife radiosurgery of acoustic neuroma has documented improvement of hearing after therapy and may be relevant in this regard. That there are advantages in physical dose distribution and fractionation, producing a reduction in the biological dose in normal tissue, argues for the use of linac technology in acoustic neuromas. Craniopharyngiomas enveloping the optic nerve/chiasma will similarly be better treated by the linac X-knife system. It is apparent that different radiosurgery systems may be indicated in particular neuro-oncological situations.
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7/211. Isolated metastases of adenocarcinoma in the bilateral internal auditory meatuses mimicking neurofibromatosis type 2--case report.

    A 56-year-old male with a history of lung cancer presented with isolated metastases of adenocarcinoma in the bilateral internal auditory meatuses (IAMs), mimicking the bilateral acoustic schwannomas of neurofibromatosis type 2, and manifesting as rapidly worsening tinnitus and bilateral hearing loss. magnetic resonance imaging showed small tumors in both IAMs with no sign of leptomeningeal metastasis. The preoperative diagnosis was neurofibromatosis type 2. Both tumors were removed and the histological diagnoses were adenocarcinoma. neuroimaging differentiation of a solitary metastatic IAM tumor from a benign tumor is difficult, although rapidly progressive eighth cranial nerve dysfunction suggests a malignant process. Metastases should be considered as a rare diagnostic possibility in a patient with small tumors in both IAMs.
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8/211. meningioma of the internal auditory canal with extension into the vestibule.

    Meningiomas account for approximately 18 to 19 per cent of all brain tumours. Although they can arise in numerous locations, meningiomas of the internal auditory canal (IAC) are rare. Most tumours that originate in the IAC are schwannomas of the VIIIth cranial nerve (acoustic neuromas). We report a case of a meningioma which appears to originate from the IAC and extends into the vestibule. The clinical findings and the radiographical features of meningiomas of the IAC are similar to those of acoustic neuromas. Pre-operative differentiation between acoustic neuromas and meningiomas of the IAC may be difficult.
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9/211. Current surgical results of retrosigmoid approach in extralarge vestibular schwannomas.

    BACKGROUND: Vestibular schwannomas (VS) are common tumors that can be cured; they are reported to comprise 6 approximately 8% of all intracranial tumors. The primary objective in the surgery of extralarge VS is total removal of the tumor mass while preserving the facial nerve. In extralarge tumors, complete excision of the tumor carries a significant risk of injuring the facial nerve and adjacent vital brain structures. The authors are reporting the techniques and results of operation on extralarge VS. methods: The material consisted of 30 patients during the last 6 years with surgically treated VS that had a maximal extrameatal diameter exceeding 4 cm. Suboccipital craniotomy and tumor removal was performed with patients in the lateral position. Results and complications of the surgical technique will be reviewed. RESULTS: Average age of patients was 45.2 years; there was a slight female predominance (1.5:1). Size of the mass ranged from 41 to 70 mm; all were removed by the retrosigmoid transmeatal approach. Peritumoral edema on MRI was seen in 50% (15/30). Total removal was achieved in 73.3% (22/30) with no significant relationship to peritumoral edema. In the cases of total removal, the facial nerve preservation rate was 86.4% (19/22). There was no mortality. Surgical complications were hemorrhage and CSF leakage in 1 case (3%) and 8 cases (26.7%), respectively, but in most of these cases, conservative treatment was adequate. In patients in whom anatomic preservation of the facial nerve was achieved, facial nerve function improved progressively within a year. In all cases except for one with gait disturbance, a good outcome was achieved.CONCLUSIONS: Our surgical techniques, including the prediction of facial nerve displacement, not using retractors, and replacement of bone, contributed to good surgical results in a series of extralarge VS.
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10/211. Focal amyotrophy in neurofibromatosis 2.

    Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder characterised by bilateral vestibular schwannomas and other CNS tumours including meningiomas and spinal schwannomas. Occasionally, peripheral neuropathy occurs in these patients but this is the first report of focal amyotrophy. Clinical, electrophysiological, and imaging data from four NF2 patients seen at a specialist neurofibromatosis clinic over a 4 year period are described in whom symptomatic focal amyotrophy preceded the diagnosis of NF2. Two presented with wasting and weakness of a single muscle group, several years before NF2 was diagnosed. In one patient a mononeuritis multiplex was the presenting feature of NF2, and in one patient focal wasting and weakness developed after the diagnosis of NF2 was made. In none of the four cases could a focal peripheral nerve or root neurofibroma be identified despite extensive imaging with MRI, and the limitations of neuroimaging for identifying a structural cause in patients with NF2 with a focal peripheral nerve lesion is discussed. It is likely that NF2 may affect peripheral nerve structures in a manner distinct from a compressive schwannoma.
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