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The objective of the investigation was to document the clinical presentations of a group of patients with neurofibromatosis type two syndrome (NF2), and took the form of a retrospective case note review. The investigation took place in the tertiary referral skull base unit. The dominant presentation of patients with NF2 is with auditory/vestibular symptoms (56%), with a smaller proportion of presentations being due to non-acoustic intracranial and spinal tumours (44%). At the time of presentation the majority of clinically significant tumours could be demonstrated in each patient with MRI of the head and spine, with few new tumours arising during the subsequent follow-up period. The range of presentation of patients with NF2 results in their management being fragmented into a variety of clinical setting, diluting the experience amassed in any one centre.

Objective: To investigate the surgical techniques and speech performance of multichannel auditory brainstem implant (ABI) in patients with bilateral acoustic neuromas (neurofibromatosis type 2)., Methods: The nucleus 21 channel auditory brainstem implant was implanted into the lateral recess of the fourth ventricle through the translabyrinthine approach in 7 patients after removal of the tumor. The accurate placement of electrode array was ensured by the electromyogram monitoring of the 7th and 9th nerves and the electrically evoked auditory brainstem responses (EABR). Initial switch-on occurred six weeks postoperatively. Speech evaluation was performed every 3 months for the first year and annually thereafter., Results: During the surgery, the lateral recess could be found and the typical EABR could be recorded in 6 cases. They later reported a significant benefit from the device. Two of the cases have achieved functional open-set speech understanding. In contrast, one patient with no EABR because of difficulty of the anatomic location during the surgery had no sensations postoperatively., Conclusion: The multichannel ABI could effectively restore auditory sensations in patients deafened by bilateral acoustic neuromas. The accurate location of the cochlear nucleus complex during surgery was the key factor for the success of the operation.

Since 1992 18 patients with bilateral retrocochlear deafness have been provided with a multichannel auditory brainstem implant (ABI). The surgical procedure implies tumour removal and ABI implantation in one stage. Most implantations were via the translabyrinthine approach. The long-term follow-up varied between nine and 80 months. In one case auditory perception could not be achieved and in a second case post-operative stimulation was not possible as the subject died due to lung emboli. In all the other cases auditory perception was achieved and only two subjects became non-users during the follow-up period. The presented long-term results suggest that deaf neurofibromatosis type 2 patients regain acoustic contact with the environment, enlarge their communication skills and improve their quality of life by using a multichannel auditory brainstem prosthesis.

A deaf female patient was diagnosed with bilateral acoustic neurinomas. Diagnosis incorporated the standard audiological battery for sensorineural hearing loss, computed tomography and magnetic resonance imaging. The left side had been operated on four years previously in another clinic using the suboccipital approach. The auditory brainstem implant surgery was performed on the 'second side' using the same approach. It was an uneventful operation with good anatomy and no serious post-operative complications. Post-operatively, the patient performed exceptionally well, with up to 50 per cent of words recognized in the opened set and 85 per cent in the closed set, both without lip-reading.

This paper discusses surgery and radiosurgery from the author's personal experience with both techniques. Multi-discipline assessment will allow the patient to make an informed decision regarding preferred management., (Copyright 2001 S. Karger AG, Basel)

The objective of this study was to present aspects of the current treatment protocol, such as patient evaluation and selection for therapy, multimodality monitoring for optimal auditory brainstem implant (ABI) positioning and radiological evaluation, that might have an impact on the functional results of ABI. Out of a series of 145 patients with bilateral vestibular schwannomas 10 patients received an ABI, eight of which are reported here. Patient selection was based on disease course, clinical and radiological criteria (according to the Hannover evaluation and prognosis scaling of neurofibromatosis type 2 (NF2)), extensive otological test battery and psycho-social factors. ABI placement was controlled by multimodality electrophysiological monitoring in order to activate the auditory pathway and to prevent false stimulation of the cranial nerve nuclei or long sensory or motor tracts. Results of hearing function were correlated with patients' ages, duration of deafness, tumour extension, tumour-induced compression or deformation of the brainstem, and numbers of activated electrodes without any side-effects. Out of 59 patients with pre-operative deafness eight patients received an ABI of the Nucleus 22 type. All these patients became continuous users without any side effects and experienced improved quality of life. Speech reception in combination with lip-reading was markedly improved, with further improvement over a long period. A short duration of deafness may be favourable for achieving good results, while age was not a relevant factor. Lateral recess obstruction may necessitate a more meticulous dissection, but did not prevent good placement of the ABI in the lateral recess. Pre-existing brainstem compression did not prevent good results, but brainstem deformation and ipsi- and contralateral distortion were followed by a less favourable outcome. Among the factors that can be influenced by the therapy management are the selection of patients with a slow progressing NF2 disease, a short duration of deafness, a careful analysis of brainstem deformation and consideration of either side for implantation. Long-standing brainstem deformation might not lead to recovery, but instead lead to a low number of active electrodes and possibly only moderate results. ABI treatment is a safe procedure that can increase a patient's quality of life considerably. ABI placement along with neurophysiological control helps to prevent side effects and to improve acoustic activation. Further studies on structural and functional changes of the brainstem after previous tumour compression and distortion should increase our understanding and facilitate a decision on the best side for ABI implantation.

In patients with reduced auditory nerve function, for example due to tumour removal or an accident, hearing rehabilitation can be elicited by an auditory brainstem implant (ABI). The electrode array of the ABI manufactured by Cochlear Ltd., Sydney, consists of 21 circled contacts in a silicon carrier. This is inserted in the lateral recess of the fourth ventricle. Since 1996, in Hannover eight patients have been implanted with a cochlear ABI Nucleus 21 + 1. All of them were profoundly deaf on both sides due to neurofibromatosis type 2 (NF2). To find the optimal electrode position during surgery, a multimodal monitoring by auditory evoked potentials (AEP), electromyography (EMG) and somatosensory evoked potentials (SEP) was performed. When monitoring AEPs, the function of the implant can be checked first by the stimulus artefact. By analysing the AEPs in more detail, the optimal positioning of the electrode on the cochlear nucleus can be found. If systems other than the auditory system are stimulated this will be revealed in one or more of the AEP, EMG and SEP recordings. According to the literature, AEPs stimulated by an ABI consist of three vertex positive peaks with latencies shorter than 4 ms. Typical AEPs are correlated with good post-operative hearing sensation. Comparing these AEPs with AEPs stimulated acoustically or electrically at different sites of the auditory system, it can be assumed that the first peak corresponds to J3, the second to J4 and the last to J5. From this comparison it can also be concluded that no potentials should occur later than 5 ms. This corresponds to our findings. Post-operatively, side-effects occurred when areas of the electrode array were stimulated that showed potentials with latencies longer than 5 ms intra-operatively. Our results indicate that monitoring is an essential aid for the surgeon in finding the optimal electrode position. Positioning solely with reference to anatomical landmarks may not be enough to find the optimal functional position.

Since 1994, a US Food and Drug Administration clinical trial evaluated the multichannel auditory brainstem implant (ABI) on 92 subjects with neurofibromatosis type 2 (NF2). The trial has shown that 85 per cent of patients receive auditory sensations. A small number of patients demonstrate a clinically significant degree of open-set sentence recognition in the sound-alone condition; however, when the ABI is combined with lip-reading cues, 93 per cent of patients demonstrate improved sentence understanding at three to six months. In addition, the majority of recipients report daily use of their devices, and satisfaction with the decision to receive the ABI.

An auditory brainstem implant (ABI) is indicated for patients suffering from bilateral neural deafness. The most affected patients are those with neurofibromatosis type 2 (NF2). An implantation is possible either at the same time as, or after, surgical removal of an acoustic neuroma. This paper demonstrates the results of eight out of 11 patients with NF2, seven of whom received an ABI after tumour removal. Pre-operatively, all of them were deaf. Post-operatively, the first fitting served to determine the individual stimulation parameters for each electrode. The stimulation-dependent side-effects were eliminated by reducing the stimulus intensity without causing negative effects on the hearing with the ABI. Only in one case was an open set understanding achieved within the first year. However, all patients had a better speech understanding when they combined their hearing with the ABI and their lip-reading abilities. There is no correlation between the performance with ABI and the tumour size or the duration of deafness.

The multichannel auditory brainstem implant (ABI) provides the potential for hearing restoration in patients with neurofibromatosis type 2 (NF2). Programmes for auditory brainstem implantation have been established in two Australian centres. Eight patients have been implanted under the protocol of an international multi-centre clinical trial. Three patients had ABI insertion at the time of first side tumour removal, four at second side tumour removal and one after previous bilateral surgery where there was some residual tumour. The translabyrinthine approach was used in all cases. Successful positioning of the electrode array was achieved in seven of eight patients, all of whom achieved auditory perception with electrical stimulation. Intra-operative electrically evoked auditory brainstem response testing was successful in four patients and was useful in confirming correct electrode position. In six cases post-operative psychophysical and auditory perception testing demonstrated that useful auditory sensations were achieved. Five of these patients regularly used the implant. In one patient electrode placement was unsuccessful and only non-auditory sensations occurred on stimulation. In the remaining patients non-auditory sensations were minimal and avoidable by selective electrode programming. Auditory brainstem implantation should be considered in patients with NF2. The greatest benefit is seen in patients without debilitating disease who have non-aidable hearing in the contralateral ear.

The present paper reports our experience with the surgical retrosigmoid-transmastoid (RS-TM) technique for implanting auditory brainstem implants (ABIs). From April 1997 to August 1998, four patients with neurofibromatosis type 2 (NF2) were operated on for vestibular schwannoma removal with ABI implantation. The subjects (three men and one women) ranged in age from 22 to 31 years. Tumour size ranged from 12 to 30 mm. A classical RS-TM approach was performed. After tumour excision, identification of landmarks (VIIth, VIIIth and IXth cranial nerves, choroid plexus) to the foramen of Luschka was carefully carried out. The choroid plexus was partially removed and the tela choroidea divided and deflected. The floor of the lateral recess of the fourth ventricle and the convolution of the dorsal cochlear nucleus became visible. The electrode array was then inserted into the lateral recess and placed in the correct position with the help of electrically-evoked auditory brain stem responses. Auditory sensations were induced in all patients with various numbers of electrodes. Different pitch sensations could be identified with different electrode stimulation. Details of the results are presented. In our series, the RS-TM approach represents the elective route for ABI insertion.

Neurofibromatosis type 2 (NF2) causes bilateral hearing loss due to tumour growth in the cerebellopontine angle. We report the results of promontory testing and transtympanic electrocochleography on subjects with deafness due to NF2 referred for an auditory brainstem implant. All 19 ears tested revealed loss of cochlear microphonics. Nine ears (mainly without previous treatment) revealed auditory perception during promontory stimulation, indicating cochlear deafness. One of these subjects has been successfully provided with a cochlear implant. The other 10 ears (mainly after previous surgery) revealed negative promontory stimulation, indicating additional retrocochlear deafness. These findings indicate that neurofibromas initially cause a cochlear deafness, so that a cochlear implant can be used if the auditory nerve can be preserved. This option has to be considered in rehabilitating patients with bilateral tumours due to NF2.

Auditory brainstem implants (ABIs) are a modern method of treatment of total bilateral deafness in cases of extracochlear origin. In most cases therapy is applied in patients with neurofibromatosis type 2 (NF2). This paper presents the results of surgical treatment and rehabilitation in a 28-year-old woman with bilateral, multiple tumours of the central nervous system causing total deafness. Simultaneous removal of the tumours and implantation of ABI allowed treatment of the potentially lethal pathology and hearing restoration. Improving auditory skills and excellent tests results were noted in the year following implantation.

In the Czech Republic, the first implantation of a stimulation electrode into the brainstem was performed on 11 January 1999 in the Department of ORL, Head and Neck Surgery, The First Medical Faculty, Charles University in Prague, University Hospital Motol. The selected patient was a 40-year-old woman with neurofibromatosis type 2 (NF2) who had previously undergone bilateral vestibular schwannoma surgery. Both tumours had been radically removed, the left-sided tumour in 1987, the right-sided one in 1988. She had been completely deaf since the last operation, i.e., for 11 years. The surgery was realized by the international cooperation of three teams. Placement of the electrode pad of the Nucleus CI21 + 1M system on the ventral and dorsal cochlear nuclei was performed. Electrically evoked auditory brainstem responses (EABRs) proved the correct position of the electrode array. The post-operative course was uneventful. Six weeks after the surgery the patient received her speech processor. Since that time, the patient already absolved several sessions of a speech processor tune-up. She uses the device as an aid in lip-reading. No adverse or pathological side effects have been observed. The patient was the 45th person in Europe to receive an ABI and the first in the Czech Republic.

Object: Stereotactically guided radiosurgery is one of the primary treatment modalities for patients with acoustic neuromas (vestibular schwannomas). The goal of radiosurgery is to arrest tumor growth while preserving neurological function. Patients with acoustic neuromas associated with neurofibromatosis Type 2 (NF2) represent a special challenge because of the risk of complete deafness. To define better the tumor control rate and long-term functional outcome, the authors reviewed their 10-year experience in treating these lesions., Methods: Forty patients underwent stereotactic radiosurgery at the University of Pittsburgh, 35 of them for solitary tumors. The other five underwent staged procedures for bilateral lesions (10 tumors, 45 total). Thirteen patients (with 29% of tumors) had undergone a median of two prior resections. The mean tumor volume at radiosurgery was 4.8 ml, and the mean tumor margin dose was 15 Gy (range 12-20 Gy). The overall tumor control rate was 98%. During the median follow-up period of 36 months, 16 tumors (36%) regressed, 28 (62%) remained unchanged, and one (2%) grew. In the 10 patients for whom more than 5 years of clinical and neuroimaging follow-up results were available (median 92 months), five tumors were smaller and five remained unchanged. Surgical resection was performed in three patients (7%) after radiosurgery; only one showed radiographic evidence of progression. Useful hearing (Gardner-Robertson Class I or II) was preserved in six (43%) of 14 patients, and this rate improved to 67% after modifications made in 1992. Normal facial nerve function (House-Brackmann Grade 1) was preserved in 25 (81%) of 31 patients. Normal trigeminal nerve function was preserved in 34 (94%) of 36 patients., Conclusions: Stereotactically guided radiosurgery is a safe and effective treatment for patients with acoustic tumors in the setting of NF2. The rate of hearing preservation may be better with radiosurgery than with other available techniques.

We present our experience using the Clarion magnetless multichannel cochlear implant with a woman profoundly deafened following bilateral acoustic neuromata as a consequence of neurofibromatosis 2 (NF2). The right neuroma had been previously removed without an attempt at neural preservation. On the left, however, a posterior fossa approach had been taken with the aim of preserving hearing. Although the left cochlear nerve appeared to be undamaged at the end of the operation, no hearing thresholds could be elicited on post-operative audiometry, because of damage either to the cochlear nerve or to the blood supply to the cochlea. Round window electrical stimulation subsequently produced a perception of sound, confirming that the cochlear nerve was capable of functioning and that a cochlear implant would be effective. Because she would need regular magnetic resonance imaging (MRI) to monitor existing and future NF2 lesions, it was decided to use a magnetless Clarion implant, which has been shown to be MRI compatible. We report our experience of using the device in this case and discuss some of the issues related to the provision of cochlear implants to patients with NF2.

Objective: To investigate the clinical course, growth character and treatment of bilateral acoustic neuromas (BANs)., Methods: Five cases of BANs were reported., Results: From 1986 to 1996, 92 patients with acoustic tumors were managed. Of them, 5(5%) bilateral acoustic neuromas were observed. All patients were males, the age at the time of testing ranged from 16 to 31 years (mean 23.8 years). In all patients, progressive hearing loss and tinnitus were the most common initial symptoms. In addition, signs of other cranial nerve deficits were detected in all patients. Two cases had cafe au lait spots and all cases had neurofibromas of varying sizes and shapes in subcutaneous tissues. Four had unilateral acoustic tumors operated on, one by the translabyrinthine approach, 3 by the suboccipital approach. One patient died due to encephaledema and hernia of brain after operation. According to the MRI data in one case, the average growth rate of acoustic tumor was 1.1 cm (superior/inferior) and 0.7 cm (medical/lateral) per year., Conclusion: The management of BANs patients presents challenging problems different from the unilateral acoustic tumors.

Cochlear implantation has a limited but definite role in the rehabilitation of certain neurofibromatosis type 2 (NF2) patients. The presence of a dead ear either before, or after, tumour removal does not necessarily imply loss of function in the eighth nerve; in some instances the hearing loss will be cochlear. Promontory or round window electrical stimulation may help to identify those individuals with surviving eighth nerve function. In such patients multichannel cochlear implantation promises a better level of audition than the auditory brain stem implant. This paper highlights such a case and the management problems are discussed.

Neurofibromatosis type 2 (NF2), an as-yet incurable disease that predisposes patients to multiple intracranial and spinal tumors, requires a team approach to treatment, because of its multisystem nature. Included on the team should be neuro-otologists, neurosurgeons, ophthalmologists, geneticists, audiologists, speech therapists and other rehabilitative personnel, including counselors, psychologists and, occasionally, psychiatrists. The challenge is to arrive at a treatment strategy that preserves useful hearing and quality of life without increasing the risk of complications to the facial nerve or compromising neurologic status. Choosing the best treatment approach involves considering a complex set of competing factors that affect various aspects of the patient's outcome.

Objectives: This study investigates the use of endoscopy for the placement of an auditory brainstem implant by translabyrinthine, retrosigmoid (suboccipital), and middle cranial fossa approaches., Study Design: Cadaver dissection and endoscope-assisted placement of the auditory brainstem implant., Methods: Translabyrinthine, retrosigmoid, and middle cranial fossa dissections were performed bilaterally in five cadaveric heads. An auditory brainstem implant was placed within the lateral recess of the fourth ventricle under endoscopic visualization. The implantation was performed with all approaches and documented by digital image capture followed by production of dye-sublimation photographic prints., Results: The lateral recess was visualized with the endoscope in all three approaches to the brainstem. The 30 degrees endoscope provided the best visualization by translabyrinthine and retrosigmoid dissection and was essential for the middle cranial fossa approach. Refinement of implant position was readily achieved, as even the deepest portion of the recess could be seen with all three approaches., Conclusions: This study finds that endoscopy provides superior visualization of the lateral recess of the fourth ventricle than the operating microscope with all approaches. The retrosigmoid approach is recommended, as it provides the best view of the implantation site and the easiest angle for placement of the prosthesis. The use of the endoscope may allow for a smaller craniotomy than with conventional microscopic techniques, depending on tumor size. The translabyrinthine approach provides a good view of the lateral recess but had no advantage over other approaches. The middle cranial fossa approach is only possible with angled endoscopes; however, it is technically the most difficult and places the facial nerve at greatest risk.

A 26-year-old man who had neurofibromatosis type-2 with symptoms of unexplained optic disc edema is reported. Magnetic resonance imaging (MRI) revealed bilateral acoustic schwannomas. Obstructive hydrocephalus, however, was not evident in spite of his severe disc edema and visual loss. After partial removal of the right acoustic schwannoma, symptoms of intracranial hypertension, such as vomiting and headache, developed and MRI demonstrated evidence of obstructive hydrocephalus. Placement of a ventricular-peritoneal shunt relieved the symptoms of intracranial hypertension, but visual acuity in his left eye was reduced to hand motion due to secondary optic atrophy. In patients with similar symptoms it is suggested that, in addition to tumor removal, early treatment to decrease intracranial pressure should be considered when visual function is progressively impaired by the symptoms of prolonged papilledema.

Two cases of acute hearing loss are reported following fractionated stereotactic radiosurgery for acoustic neuroma. Both patients had neurofibromatosis type 2 and were treated with a peripheral tumor dose of 21 Gy delivered in three fractions (7 Gy each) with a minimum interfraction interval of 10 hours. One patient who had previously undergone surgical resection of the treated tumor presented with only rudimentary hearing in the treated ear secondary to an abrupt decrease in hearing prior to treatment. That patient reported total loss of hearing before complete delivery of the third fraction. The second patient had moderately impaired hearing prior to treatment; however, within 10 hours after delivery of the final fraction, he lost all hearing. Both patients showed no improvement in response to glucocorticoid therapy. Possible explanations for this phenomenon are presented.

The auditory brain stem implant has been used effectively to provide hearing sensations to individuals deafened by bilateral auditory nerve tumors (neurofibromatosis type 2). During tumor removal, the auditory brain stem implant is implanted into the lateral recess of the fourth ventricle by a translabyrinthine approach and is intended to stimulate auditory neurons of the cochlear nucleus complex. A new eight-electrode multichannel auditory brain stem implant was developed and evaluated in 20 patients who had at least 3 months' experience with the device. Mild nonauditory sensations (primarily tingling in the head or torso) were encountered in some instances but could be managed by changing the stimulus characteristics or excluding electrodes. Testing of perceptual performance indicated significant benefit from the device for communication purposes, including sound-only sentence recognition scores in three patients ranging from 49% to 58% and ability to converse on the telephone. These results indicate that significant auditory benefit can be derived from direct multichannel electrical stimulation of the auditory portion of the human brain stem.

Objective: We conducted a retrospective study of 87 patients with spinal nerve sheath tumors to determine the overall clinical outcome and specific features in 20 patients affected with neurofibromatosis Types 1 and 2 (NF-1 and NF-2, respectively)., Methods: Case records, operation notes, outpatient files, and radiological examinations were analyzed for all patients treated between September 1977 and August 1994. Additional follow-up data were obtained using outpatient examinations, questionnaires, and telephone calls., Results: During the study period, 128 spinal neuromas (i.e., schwannomas) and 6 neurofibromas in 87 patients were treated. Fifty-seven neuromas were associated with NF-2 in 17 patients and six neurofibromas with NF-1 in 3 patients. Patients with NF-2 and symptomatic neuromas presented with more severe neurological deficits compared to patients without NF-2. Eighty-six percent of the neuromas were removed completely. On average, most preoperative deficits or symptoms improved in patients without NF-2, whereas neurological symptoms remained unchanged in patients with NF-2. Multiple regression analysis revealed that partial removal, surgery of a recurrent tumor, NF-2, and old age predisposed for tumor recurrence. No increased risk of recurrence was observed for patients with NF-1. For patients without NF-2, we observed overall recurrence rates of 10.7% after 5 years and 28.2% after 10 and 15 years, respectively, as determined by Kaplan-Meier analysis. For NF-2, the recurrence rate at 5 years was 39.2%, and all tumors had recurred by 9 years., Conclusion: Spinal nerve sheath tumors carry an excellent prognosis in patients with NF-1 and in patients without neurofibromatosis. Symptomatic neuromas occurring in association with NF-2 present with more severe neurological deficits, demonstrate little postoperative improvement, and have a very high recurrence rate.

Vestibular schwannomas (acoustic neuromas) continue to cause significant facial nerve and hearing morbidity, despite marked improvement in diagnosis and treatment. Mutation of a tumor-suppressor gene on human chromosome 22 has been found to be associated with vestibular schwannoma formation. The central hypothesis of this study is that specific mutations in the neurofibromatosis type 2 (NF2) gene may produce specific clinical characteristics or phenotypic expressions. The purposes of this investigation are: 1. to determine what proportion of vestibular schwannomas from patients with spontaneous unilateral and familial bilateral schwannomas have mutations present within the NF2 gene; 2. to determine whether specific types of mutations are associated with a specific clinical manifestation of this disease; and 3. to further define the relationship between newly discovered mutations within the NF2 tumor-suppressor gene and possible clinical applications of this knowledge to advance diagnosis and treatment of patients with NF2 and spontaneous vestibular schwannomas. DNA from 61 schwannomas (29 unilateral vestibular schwannomas and 32 from patients with bilateral vestibular schwannomas [NF2]) were examined, and 33 unique mutations were identified. Significant differences were found in the frequency, distribution, and type of mutation between the NF2 schwannomas and the spontaneous vestibular schwannomas. Three clinical subtypes of NF2 were identified. In tumors from 28 patients, no mutations were identified. Of the 33 mutations identified in the NF2 gene, 30 were likely to result in loss of tumor-suppressor function from protein truncation; however, three milder mutations termed missense mutations were associated with milder clinical manifestations of the disease and had a slower estimated growth rate. Variable clinical presentation in patients whose tumors had severe or truncating types of mutations suggest that factors in addition to the mutation class are likely to be responsible for a portion of the clinical expression of disease. New diagnostic options are now available for NF2 that will improve the likelihood of hearing and facial nerve preservation and ultimately have significant impact on the management of vestibular schwannomas.

NF-2 is an extremely rare form of neurofibromatosis (NF) characterized by central system (CNS) neural crest-derived tumors and frequently cafe au lait spots (CLS). The purpose of this study was to report the clinical and imaging findings of seven patients with this disorder and to stress that value of surgical treatment in its management. Seven patients between 8 and 32 years of age who had NF-2 were included in the study. Clinical charts, surgical and pathological findings, and imaging studies were reviewed retrospectively. Patients were followed up for to 142 months. Clinical evaluation and neuroimaging studies detected the clinical criteria of NF-2 in all patients. Two deaths occurred after surgical intervention in our series. None of the patients suffered from recurrent tumor following surgery. Our results show that NF-2 is an uncommon entity which has a good prognosis after surgical intervention, in spite of the presence of multiple cranial and/or spinal lesions.

Objective: Although the rate of reported facial nerve preservation after surgery for vestibular schwannomas continuously increases, facial nerve paresis or paralysis is a frequent postsurgical sequelae of major concern. The major goal of this study was to define criteria for the right indication, timing, and type of therapy for patients with palsies despite anatomic nerve continuity and those with loss of anatomic continuity., Methods: One thousand vestibular schwannomas were surgically treated at the Department of Neurosurgery at Nordstadt Hospital from 1978 to 1993. Of 979 cases of complete removal and 21 cases of deliberately partial removal, the facial nerve was anatomically preserved in 929 cases (93%). The rate of preservation is increasing, as is evidenced in the most recent cases, and preservation is supported by special electrophysiological monitoring. The facial nerve was anatomically severed in 60 cases (6%). It was anatomically lost in previous operations that were performed elsewhere in 11 cases (1%). In case of nerve discontinuity (42 cases), immediate nerve reconstruction by one of three available intracranial procedures (within the cerebellopontine angle, intracranial-intratemporal, intracranial-extracranial) was performed in the same surgical setting. In case of loss of the proximal facial nerve stump at the brain stem, early reanimation by combination with the hypoglossal nerve was achieved in most patients within weeks after tumor surgery. In a few patients with anatomic nerve continuity but absence of reinnervation for 10 to 12 months, a hypoglossal-facial combination was applied. All the patients with partial or with complete palsies were treated in a special follow-up program of regular controls and of modulation of physiotherapeutic treatment every 3 to 6 months., Results: In intracranial nerve reconstruction at the cerebellopontine angle, 61 to 70% of patients regained complete eye closure and an overall result equivalent to House-Brackmann Grade 3. Hypoglossal-facial reanimation led to Grade 3 in 79%. The duration between the onset of paralysis and the reconstructive procedure is decisive for the quality of the outcome. These data are discussed in view of other treatment options and certain parameters influencing outcome., Conclusions: This management contains three major principles as follows: 1) preservation of facial nerve continuity in function by the aid of intraoperative monitoring, 2) early nerve reconstruction in case of lost continuity, and 3) scheduled follow-up program for all patients with incomplete or complete palsies.

Objective: Vestibular schwannomas (VSs) affect young patients with Neurofibromatosis 2 (NF-2) and cause very serious problems for hearing, facial expression, and brain stem function. Our objective was to determine a therapy concept for the right timing and indication of neurosurgical therapy., Methods: In 1000 consecutive VS resections, 120 tumors in 82 patients with NF-2 were surgically treated by the same surgeon (MS) at the Department of Neurosurgery at Nordstadt Hospital from 1978 to 1993. The mean age of the patients was 27.5 years. Sixty tumors were surgically treated in 41 male patients, and 60 tumors were surgically treated in 41 female patients. Bilateral tumor resection was performed in 38 patients (76 operations, after previous partial surgery in 15 cases elsewhere), and unilateral operations were performed in 44 patients, 5 of whom had undergone ipsi- or contralateral surgery that was performed elsewhere. The operative and clinical findings are evaluated and compared with the data of patients without NF-2., Results: In 105 cases, complete tumor resections were achieved. In 15 cases, deliberate subtotal resections were performed. These were for brain stem decompression in 4 cases and for hearing preservation in the last hearing ear in 11 cases, with successful preservation in 8 of the 11. Pre- and postoperative hearing rates were higher in male than in female patients (70% in male versus 65% in female patients before surgery and 40.5 versus 31%, respectively, after surgery). Hearing was preserved in 29 of 81 ears (36%). The rate of preservation was 24% in cases of large tumors and 57% in cases of small tumors (<30 mm). Twenty-one of 82 patients (26%) were bilaterally deaf before surgery. Twenty-five patients had uni- or bilateral hearing after surgery (i.e., 41 % of those with preoperative hearing or 30.5% of the whole group). Anatomic facial nerve preservation was achieved in 85%. The facial nerve was reconstructed intracranially at the cerebellopontine angle by sural grafting in 17 cases and by hypoglossal-facial reanimation in 5. Two deaths occurred 1 and 3 months postsurgically as a result of malignant tumor growth with brain stem dysfunction and respiratory problems. In summary, for patients with NF-2, the presentation ages are lower, tumor progression is faster, the chances of anatomic and functional nerve preservation are lower, the chances of good outcomes are best when surgery is performed early and when there is good preoperative hearing function, and the danger of sudden hearing loss is higher. The chances and danger often differ from side to side among individual patients., Conclusion: The indication and the timing of tumor resections are in some respects different from normal VS handling and are dependent on the tumor extension and related necessity of brain stem decompression and on the auditory function. As an optimal goal, completeness of resection with functional cochlear nerve preservation is formulated, and as an acceptable compromise, subtotal microsurgical resection with functional cochlear nerve preservation in the last hearing ear is suggested.

Neurofibromatosis type 2 (NF2) is a complex and progressively disabling disease, resulting from development of multiple central nervous system tumours. Two case studies, one of a woman who suffered hearing problems from the age of 17 and one of a man with cataract as the first symptom at the age of five, illustrate the complex course of the disease. The first patient died of her disease, the second due to a traffic accident. It is necessary to anticipate the frequent development of new neoplasms. The regular communication between the various disciplines involved in the patient's care is instrumental to reach this goal. This multidisciplinary approach should also play a part in the screening of patients at risk for NF2.

Threshold current levels for electrical stimulation of a single human brainstem via an auditory prosthesis are compared with postmortem measures of the distance between the electrode and stimulated structures. The results compare well with the summary of threshold-distance measures from animal experiments compiled by Ranck. The correspondence between the human and animal data gives confidence that the extent of current spread (distance to stimulable neural units) can be well estimated from the current level at threshold for 200 microseconds/phase biphasic pulses. This is of particular interest in electrical stimulation of the human central nervous system, where localization of stimulation is of paramount importance.

A young male patient suffered in cutaneous neurofibromatosis was followed during four years. He developed tissue proliferations of various histological structures manifested in the skin, central nervous system and different splanchnic organs. The classification criteria of neurofibromatosis as well as the diagnostic and therapeutic recommendations are discussed.

This case study describes a therapeutic strategy using a cochlear implant for a bilateral acoustic neuroma deafened patient. The cochlear nerve had previously been sacrificed on one side during tumour removal, but on the remaining side a functioning cochlear nerve was assessed by electric promontory stimulation in spite of a neuroma extending into the vestibular labyrinth. The patient was successfully stimulated with a Nucleus 22-channel implant after removal of the intracanalicular portion of the neuroma via a middle fossa approach.

Neurofibromatosis 2 (NF2) is an autosomal dominant disease predisposing to multiple tumors of the central and peripheral nervous system. Bilateral vestibular schwannomas are the hallmark of the disease. To define the clinical spectrum of the disease, we performed gadolinium-enhanced magnetic resonance imaging of the brain and spine as well as neurological, dermatological, and ocular examinations in 48 patients with NF2 diagnosed with the National Institutes of Health diagnostic criteria. Patients were ascertained from patient workshops and publications and from referral as a result of vestibular schwannoma surgery. Vestibular schwannomas were found in 46 patients (96%, 43 bilateral and 3 unilateral), spinal tumors were found in 43 (90%), posterior subcapsular cataracts were found in 30 (63%), meningiomas were found in 28 (58%), and trigeminal schwannomas were found in 14 (29%). The presenting symptoms included hearing loss or tinnitus in 15 patients (31%), multiple or nonspecific symptoms in 15 (31%), skin tumors in 12 (25%), and ocular symptoms in 6 (13%). When the complete spine was imaged, spinal tumors were more common in patients with NF2 than has previously been reported. This is a noteworthy finding, because spinal tumors are a major cause of NF2 morbidity and mortality.

Intraoperative microendoscopy was performed for eight patients to access the fundus of the internal auditory canal after retrosigmoid transmeatal surgery of vestibular schwannomas. The transmeatal procedure is usually limited laterally by the labyrinth block. The restricted opening of the internal auditory canal bears a potential risk of incomplete tumor resection. For eight patients with vestibular schwannomas, intraoperative microendoscopy was performed after tumor resection to expose the "blind" area of the internal auditory canal fundus. An excellent view of the fundus contents was obtained, including Cranial Nerves VII and VIII and the crista transversa. Tumor remnants were not observed in this series. Microendoscopy was shown to be an ideal adjunct to hearing-preserving transmeatal surgery of vestibular schwannomas, enabling the removal of intracanalicular tumors with direct control of the lateral intracanalicular nerve portions.

Nowadays smaller acoustic tumors are being detected earlier. We started to remove these tumors with attempt of preservation of hearing in 1994. Since 1994, in an attempt to preserve hearing, the retrosigmoid approach was used. Our first results are presented and criteria of preoperative selection and postoperative evaluation are discussed. A fair chance to keep a useful hearing can be given to a patient with a small tumor, preferrably smaller than 1,5 cm and provided that the average hearing levels differ by no more than 30 dB from the other ear. In all the other cases the translabyrinthine route remains the preferred approach.

A case report of a 20-year-old female with bilateral acoustic neurofibromatosis (NF-2) and bilateral facial schwannomas is presented. Multiple segmental schwannomas were found with clinically intact tissue between each tumoral enlargement in the right parotid region. Translocation (2;8) (p2.4:q2.1) was detected in this patient, and has not been reported in a schwannoma until now. The patient's family would not allow any major surgery to be performed.

Progress in molecular biology has provided us with new understanding of neurofibromatosis type 2. The diagnosis of the condition is based on a number of clinical and radiological findings, which include the use of the scanner and MRI. The treatment objective is to preserve neurological function for as long as possible, with a minimum of surgical intervention. The dilemma is particularly great as far as the hearing is concerned. The care of these patients and their families must be carried out in highly specialised multi-disciplinary centres which have available the services of neuro-otologists, neurosurgeons, geneticists, audiologists, ophthalmologists and dermatologists.

Neurofibromatosis (NF) describes the most commonly inherited disorders affecting the nervous system. These autosomal dominant, neurocutaneous syndromes are associated with multiple tumors of the nervous system, including neurofibromas, schwannomas, meningiomas, and intracranial gliomas. Spinal cord involvement in NF is typically from extramedullary growth of spinal nerve root tumors. Intramedullary spinal cord tumors in NF have been reported as scattered, single cases in literature. However, this association has not been clearly defined as have other nervous system neoplasms that are typically linked with NF. We present a series of nine patients with NF with intramedullary spinal cord tumors who were managed at our institution from 1984 to 1994. The patients' ages ranged from 4 to 31 years. There were seven male patients and two female patients. Three patients had NF-1 (von Recklinghausen's), five patients had NF-2, and one patient had NF, type uncertain. There were three cervicomedullary tumors, two cervical tumors, three cervicothoracic tumors, and one thoracic tumor. A histological examination revealed five ependymomas, three astrocytomas, and one intramedullary schwannoma. Two patients with malignant intramedullary spinal ocrd tumors have died with progressive disease. One other patient has required a subsequent operation for recurrent tumor. The other six patients are doing well, and their prognosis is expected to be related to their systemic disease. We conclude that there is a clinical entity of intramedullary spinal cord tumors associated with NF.

As laryngeal schwannomas are a threat to breathing, they must be removed. CT and MR provide an accurate pre-operative work-up of these lesions. The clear delineation of the tumoral attachment to the larynx proved to be very useful in the difficult management of our second patient. Our two laryngeal schwannomas exhibited a similar appearance which differed from those of the few other laryngeal nerve sheath tumors reported in the literature The low attenuating outer part correlated with Antoni B areas. The denser enhancing inner part correlated with Antoni A areas containing large vessels. This unusual tumoral appearance, which has been observed in some other peripheral schwannomas, must bring this diagnostic possibility to mind. However, this clearly contrasting distribution of the two components of schwannomas is not the most commonly observed in other locations. More reports are needed to establish whether this special appearance is characteristic of laryngeal location.

The removal of large acoustic tumors is associated with increased mortality and cranial nerve injury. One method for treating these difficult lesions is staged resection. Between 1972 and 1992, more than 600 acoustic tumors were resected at our institution. Of these, 83 were removed in stages. This represents the largest series of staged acoustic tumor resections reported to date. A review of available films and patient records was performed for all acoustic tumors resected in stages between 1972 and early 1993 to analyze demographic information, tumor size, operative technique, outcome, and complications. The information was collected on standardized data sheets and entered into a computer database. Virtually all tumors were large, with the average size being 4 cm in greatest diameter. The average patient age was 41 years, and there was a slight preponderance of female patients. Ten patients had neurofibromatosis Type 2. The suboccipital approach was used in most patients. Anatomic preservation of the VIIth cranial nerve was achieved in > 72% of patients, with an average House-Brackmann score of Grade 3 at the longest follow-up (mean, 43 mo). Facial reanimation was performed in 19 of 23 patients with transected VIIth cranial nerves. Complications included cerebrospinal fluid fistulas in 11 patients, with 8 of 11 fistulas resolving after lumbar drainage. Six patients had meningitis (bacterial in three and aseptic in three). Two patients developed wound infections, and 10 patients developed exposure keratitis. There were two documented recurrences. There were no operative deaths. In most series, the incidence of cranial nerve deficits as well as morbidity and mortality is directly related to tumor size. Our operative strategy involved debulking the lateral aspect of large tumors during Stage I. Second stage removal is performed after the remaining tumor is shown to decompress out of the pons on computed tomographic or magnetic resonance images. During the second procedure, the residual tumor is less vascular and no longer densely adherent to the brain stem. Although staged removal is not without risk, there seems to be no apparent increase in morbidity when these results are compared with the results of series from the literature. Although there remain no absolute indications for staged resection of acoustic tumors, we think that it may represent the safest option for these difficult lesions.

The outcome of acoustic neuroma (vestibular schwannoma) surgery continues to improve rapidly. Advances can be attributed to several fields, but the most important contributions have arisen from the identification of the genes responsible for the dominant inheritance of neurofibromatosis types 1 (NF1) and 2 (NF2) and the development of magnetic resonance imaging with gadolinium enhancement for the early anatomic confirmation of the pathognomonic, bilateral vestibular schwannomas in NF2. These advances enable early diagnosis and treatment when the tumors are small in virtually all subjects at risk for NF2. The authors suggest that advising young NF2 patients to wait until complications develop, especially hearing loss, before diagnosing and operating for bilateral eighth nerve schwannomas may not always be in the best interest of the patient. To the authors' knowledge, this is the first reported case of preservation of both auditory and vestibular function in a patient after bilateral vestibular schwannoma excision.

Central electrical stimulation of the auditory pathway can allow hearing in patients suffering from deafness localized in the auditory nerve. Developments in a multi-channel auditory brainstem implant based on the Nucleus Mini 22 Cochlear implant with transcutaneous signal transmission is discussed. The devices have been implanted in nine European patients suffering from Neurofibromatosis Type 2. Preliminary speech perception results and patient satisfaction are encouraging, and the data presented include some limited open speech recognition.