Probabilistic Fiber-Tracking Reveals Degeneration of the Contralateral Auditory Pathway in Patients with Vestibular Schwannoma.

BACKGROUND AND PURPOSE: Vestibular schwannomas cause progressive hearing loss by direct damage to the vestibulocochlear nerve. The cerebral mechanisms of degeneration or plasticity are not well-understood. Therefore, the goal of our study was to show the feasibility of probabilistic fiber-tracking of the auditory pathway in patients with vestibular schwannomas and to compare the ipsi- and contralateral volume and integrity, to test differences between the hemispheres. MATERIALS AND METHODS: Fifteen patients with vestibular schwannomas were investigated before surgery. Diffusion-weighted imaging (25 directions) was performed on a 3T MR imaging system. Probabilistic tractography was performed for 3 partial sections of the auditory pathway. Volume and fractional anisotropy were determined and compared ipsilaterally and contralaterally. The laterality ratio was correlated with the level of hearing loss. RESULTS: Anatomically reasonable tracts were depicted in all patients for the acoustic radiation. Volume was significantly decreased on the hemisphere contralateral to the tumor side for the acoustic radiation and diencephalic section, while fractional anisotropy did not differ significantly. Tracking did not yield meaningful tracts in 3 patients for the thalamocortical section and in 5 patients for the diencephalic section. No statistically significant correlations between the laterality quotient and classification of hearing loss were found. CONCLUSIONS: For the first time, this study showed that different sections of the auditory pathway between the inferior colliculus and the auditory cortex can be visualized by using probabilistic tractography. A significant volume decrease of the auditory pathway on the contralateral hemisphere was observed and may be explained by transsynaptic degeneration of the crossing auditory pathway.

Contralateral biopsies in patients with testicular germ cell tumours: patterns of care in Germany and recent data regarding prevalence and treatment of testicular intra-epithelial neoplasia.

The precursor of testicular germ cell tumours (GCTs), called testicular intra-epithelial neoplasia (TIN/CIS), is safely diagnosed immunohistologically. Testicular biopsy provides a valuable tool for early detection of GCTs in risk groups. Although this knowledge is undisputed, testicular biopsies are utilized poorly. The patterns of care regarding the use of biopsies remain unknown. Uncertainty exists about the prevalence and specific treatment of TIN/CIS. We asked clinical urologists in Germany whether or not they employed contralateral biopsies in GCT patients. We evaluated the prevalence of contralateral TIN/CIS in a retrospective analysis of 780 consecutive GCT patients. All had contralateral double biopsies. Discordance of TIN/CIS findings among biopsy pairs as well as age, histology of the primary tumour and clinical stage was noted. Evaluation of data comprised descriptive statistical methods. To evaluate treatment options for TIN/CIS, we performed a literature search. 52.1% of German urologists always perform the biopsy, 17% do it mostly, 27.3% in select cases, 3.5% never. Curiously, there was a geographic north-south gradient regarding biopsy use. Contralateral TIN/CIS was found in 5%. The median ages of patients with TIN/CIS and those without were 31.8 and 34.9 years respectively (p = 0.02). The discordance rate among biopsy pairs was of 33%. Two-site biopsies provide a 17% gain in diagnostic sensitivity. Local radiotherapy with 20 Gy is the safest treatment of TIN/CIS failing in 2%. Chemotherapy has significantly lower efficacy. Contralateral testicular biopsies in GCT patients are well accepted among German urologists. The prevalence of contralateral TIN/CIS found in this series is in accordance with previous reports. Double biopsies should be the diagnostic standard because of their diagnostic superiority. Local radiotherapy with 20 Gy is the safest way of eradicating TIN/CIS. Failures occur in only 2%, usually many years after irradiation. Cisplatin-based chemotherapy is dose dependent and less effective.

Discriminating metastasised from non-metastasised seminoma based on transcriptional changes in primary tumours using NGS.

BACKGROUND: We aimed to better discriminate (occult) metastasised from non-metastasised seminoma based on transcriptional changes of small RNAs in the primary tumour. METHODS: Total RNAs including small RNAs were isolated from five testicular tumours of each, lymphogenic, occult and non-metastasised patients. Next-generation sequencing (SOLID, Life Technologies) was used to examine transcriptional changes. Small RNAs showing ⩾50 reads and a significant ⩾2-fold difference using non-metastasised tumours as the reference group were examined in univariate logistic regression analysis and combinations of two small RNAs were further examined using support vector machines. RESULTS: On average, 1.3 × 10(7), 1.4 × 10(7) and 1.7 × 10(7) small RNA reads were detectable in non-metastasised, occult and lymphogenic metastasised seminoma, respectively, of which 30-32% remained after trimming. Between 59 and 68% represented annotated reads and between 8.6 and 11% were annotated small RNA tags. Of them, 137 small RNAs showed>50 reads and a two-fold difference to the reference. In univariate analysis, 32-38 small RNAs significantly discriminated lymphogenic/occult from non-metastasised seminoma, and among these different comparisons, it were the same small RNAs in 51-88%. Many combinations of two of these small RNAs allowed a complete discrimination of metastasised from non-metastasised seminoma irrespective of the metastasis subtype. CONCLUSIONS: Metastasised and non-metastasised seminoma can be completely discriminated with a combination of two small RNAs.

Prediction of metastatic status in non-seminomatous testicular cancer.

PURPOSE: To examine the significance of 90 biomarkers for predicting metastatic status in non-seminomatous germ cell tumors (NSGCT). By predicting metastatic status, it may be possible to eliminate unnecessary therapeutic or diagnostic efforts. MATERIALS AND METHODS: We investigated 552 males who were diagnosed with non-metastatic (n = 273) and metastatic (n = 279) NSGCT between 2000 and 2011. The sample included cancers of different histologies: embryonal cell carcinoma (n = 131), teratoma (n = 55), and mixed histology (n = 366). We collected and analyzed more than 90 parameters via logistic regression: demographic characteristics, medical history, histopathological parameters, and levels of tumor markers and hormones. RESULTS: Testis histology (p = 0.004), clinical symptoms (p = 0.0005), tumor length (p = 0.005), infiltration of the rete testis (p = 0.008), invasion of lymphatic (pL1) and blood vessels (pV1) (p < 0.0001), and levels of enzymes such as LDH, ßHCG, AFP, and FSH (p values as small as <0.0001) were associated with metastatic status. With one model, we identified 14 out of 76 (18.4 %) metastatic NSGCT cases with 93-100 % certainty (positive predictive value) at 99 % specificity by the peripheral blood levels of LDH (day of operation) in combination with FSH measurements (1 day after operation). A second model included pV, tumor length, and FSH (1 day after operation). It identified 25 out of 90 (27.8 %) non-metastatic NSGCT with approximately 90 % certainty (negative predictive value) at 94-98 % sensitivity. CONCLUSIONS: No single parameter was able to discriminate metastatic from non-metastatic NSGCT, but combinations of parameters in two predictive models accurately identified the metastatic status in 23 % of the cases in our sample.

The search for biomarkers of metastatic seminoma.

PURPOSE: We screened 90 potential parameters as biomarkers of metastatic seminoma to facilitate detection and eliminate unnecessary therapeutic or diagnostic efforts. MATERIALS AND METHODS: A total of 527 men with pure seminoma (diagnosed 2000 to 2011) were followed during therapy. More than 90 demographic/anamnestic (eg age, height, weight) histopathological parameters (testicular/tumor size, testicular intraepithelial neoplasia) and levels of tumor markers (eg α-fetoprotein, ß-human chorionic gonadotropin, lactate dehydrogenase) in peripheral blood and testicular vein were collected for analysis via logistic regression. Previously described risk factors (tumors larger than 4 cm, infiltration of rete testis) were assessed separately. RESULTS: Established parameters such as tumor length (p = 0.0003), involvement of lymphatic (p <0.0001) or vascular channels (p = 0.0009), extent of primary tumor (p <0.0001) and infiltration of the tunica albuginea (p = 0.02) as well as new biomarkers such as absence of testicular intraepithelial neoplasia in tumor bearing testis (p = 0.03), testicular volume (p = 0.04) and tumor volume (p = 0.02) showed a significant association with metastatic disease. This association was also true of lactate dehydrogenase, human chorionic gonadotropin and α-fetoprotein (p <0.0001 at maximum). However, the discriminatory capacity of these biomarkers (concordance or ROC area) did not exceed 65% when examined alone or in combination, and higher values (up to 80%) were detected for enzyme levels. A subset of metastatic seminoma (2% to 27%) was detectable with high accuracy (positive predictive value 92% to 100%) based on enzyme measurements (p <0.0006). CONCLUSIONS: New biomarkers of metastatic seminoma were identified and previously described risk factors were validated. Further prospective studies of these novel parameters are warranted to verify our findings and to explore a potential use for detecting occult metastases.

Treatment of testicular intraepithelial neoplasia (intratubular germ cell neoplasia unspecified) with local radiotherapy or with platinum-based chemotherapy: a survey of the German Testicular Cancer Study Group.

BACKGROUND: The treatment of testicular intraepithelial neoplasia (TIN), the progenitor of testicular germ cell tumours (GCTs), is based on little data. PATIENTS AND METHODS: Two hundred and twenty-eight GCT patients with contralateral TIN were retrospectively enrolled. Ten had surveillance, 122 radiotherapy to testis with 18-20 Gy, 30 cisplatin-based chemotherapy (two cycles), 51 chemotherapy (three cycles), and 15 carboplatin. The study end point was a malignant event (ME), defined as detection of TIN upon control biopsy or occurrence of a second GCT. The Secondary end point was hypogonadism during follow-up. RESULTS: Numbers, proportions of ME, and median event-free survival (EFS) times were: radiotherapy N = 3, 2.5%, 11.08 years; chemotherapy (two cycles) N = 15, 50%, 3.0 years; chemotherapy (three cycles) N = 12, 23.5%, 9.83 years; carboplatin N = 10, 66%, 0.9 years; surveillance N = 5, 50%, 7.08 years. EFS is significantly different among the groups. Hypogonadism rates were in radiotherapy patients 30.8%, chemotherapy (two cycles) 13%, chemotherapy (three cycles) 17.8%, carboplatin 40%, surveillance 40%. CONCLUSIONS: Local radiotherapy is highly efficacious in curing TIN. Chemotherapy is significantly less effective and the cure rates are dose-dependent. Though hypogonadism occurs in one-third of patients, radiotherapy with 20 Gy remains the standard management of TIN.

[Coincidence of adenocarcinoma and MALT lymphoma of the prostate--first report]. / Koinzidenz Adenokarzinom und MALT-Lymphom der Prostata--Eine Erstbeschreibung.

INTRODUCTION: First description of a prostate adenocarcinoma coinciding with the rare MALT lymphoma of the prostate. CASE REPORT: 68-year-old patient with a pT2c, pN0, M0, Gleason 3 + 3 prostate carcinoma (retropubic radical prostatectomy with pelvic lymphadenectomy) and systemic indolent MALT lymphoma of the prostate (bone marrow biopsy). Therapy and course are described and further explained by referring to case reports found in the literature. In this context, possible pathogenetic mechanisms, the prognosis for this special disease and possible therapeutic approaches are discussed. CONCLUSION: MALT lymphomas of the prostate are rare, and so far a coincidence with a prostate carcinoma is unprecedented. The prostate carcinoma is treated according to stage, while the MALT lymphoma is managed according to individual treatment strategies. The histopathological classification is more important for the prognosis. Treatment with antibiotics to eradicate possible copathogenic Helicobacter pylori is discussed as an innovative therapeutic approach. The pathogenesis of the MALT lymphoma of the prostate cannot yet be clarified definitively according to a literature review.

Vestibular schwannomas and auditory function: options in large T3 and T4 tumors?

OBJECTIVE: While hearing preservation has become an accepted treatment goal, there are still major doubts whether and to what extent this aim is feasible in large tumors that cover the auditory nerve and involve the brainstem. METHODS: One thousand eight hundred (1,800) vestibular schwannomas (VS) were operated on at Nordstadt Neurosurgical Department from 1978 to 1999 by the senior surgeon (MS). There were 1,800 tumors in 1,750 patients, 1,605 patients had unilateral tumors, 145 patients had neurofibromatosis type 2 with bilateral tumors, 195 of which were treated surgically in our unit. Preservation of the cochlear nerve was attempted when ever possible. The audiometric data are analyzed by Hannover Classification System graded in steps of 20 dB by audiometry and in steps of 10% to 30% by speech discrimination. Those data were set into relation with the Hannover tumor extension grading T1 to T4. RESULTS: Overall preservation rate was 40% with considerable differences, though, depending on the preoperative hearing quality and the tumor extension. Best results were obtained for intrameatal tumors (T1) with 56% and small intra-extrameatal tumors (T2) with 57% preservation rate. While preservation rate was at 44% in medium-sized tumors (T3), it was at 20% for T4 tumors with brainstem compression or even dislocation. However, satisfactory chances were encountered in cases with normal or good preoperative hearing, on average 54%, specifically 71% in T1, 69% in T2, 58% in T3 and 29% in T4. Regarding the quality of the preserved hearing, up to one third of cases in T1 and T2 may retain their preoperative hearing level, while this ranges around 20% for T3 and 10% to 14% in T4 tumors. DISCUSSION: For long, hearing preservation in medium sized or large tumors has not received any attention because of the presumed lack of realistic chances and the expected bad quality. Obviously, hearing preservation may be achieved in relevant numbers and at satisfactory quality levels. This aspects appears important to be addressed further in view of the fact that over 70% of tumors are presented for surgery at T3 or T4 extensions. CONCLUSION: The summit of hearing preservation has not been reached as improvements are still achieved even in most difficult conditions.

Surgical management of brainstem cavernomas.

OBJECT: A careful retrospective analysis of 36 cases was performed to evaluate the pre- and postoperative rates of morbidity that occur in patients with brainstem cavernous angiomas. METHODS: The authors evaluated immediate postoperative and follow-up outcomes with regard to clinical findings, the incidence of preoperative hemorrhage(s), location and size of the lesions, and the timing of the surgical procedure after the last hemorrhagic event. Specifically. the following parameters were analyzed: 1) number of hemorrhages; 2) the precise brainstem location (pontomesencephalic, pons, and medulla oblongata); 3) pre- and postoperative cranial nerve status; 4) pre- and postoperative motor and sensory deficits; 5) size (volume) of the lesions; and 6) pre- and postoperative Karnofsky Performance Scale (KPS) scores. Multiple hemorrhages were observed in 16 patients, particularly in those with pontomesencephalic cavernous angiomas (75%). The mean preoperative KPS score was 70.3 +/- 16.3 (+/- standard deviation). Twenty-six patients (72.2%) presented with cranial nerve impairment, 13 (36.1%) with motor deficits, and 17 (47.2%) with sensory disturbance. Volume of the lesions ranged from 0.18 to 18.18 cm3 (mean 4.75 cm3). Postoperative complications included new cranial nerve deficits in 17 patients, motor deficits in three, and new sensory disturbances in 12 patients. In a mean follow-up period of 21.5 months, KPS scores were 80 to 100 in 22 patients. Timing of surgery (posthemorrhage) and multiple hemorrhages did not influence the long-term results. Higher preoperative KPS scores and smaller-volume lesions, however, were factors associated with a better final outcome (p < 0.05). Major morbidity was related mainly to preoperative status and less to surgical treatment. The incidence of new postoperative cranial nerve deficits was clearly lower than that demonstrated preoperatively because of the brainstem hemorrhages. CONCLUSIONS: Based on these findings, resection of brainstem cavernomas is the treatment of choice in the majority of these cases because of the high incidence of morbidity related to one or often several brainstem hemorrhages.

Auditory brainstem implant: part I. Auditory performance and its evolution over time.

OBJECTIVE: Evaluation of auditory performance and its evolution over time in patients with the auditory brainstem implant. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS AND METHODS: Between May 1996 and April 2000, 14 patients with neurofibromatosis type 2 underwent implantation with a multichannel auditory brainstem implant. Auditory performance data were obtained in 13 patients who had used their device on a regular daily basis for 1 to 41 months (average 19 months). Hearing evaluation was based on the results of four tests (vowel confusion, consonant confusion, Freiburger numbers, and speech-tracking test), which were performed with and without lip-reading at regular intervals after device activation. RESULTS: 12 patients received auditory sensation through the auditory brainstem implant immediately after device activation. In one patient, because of postoperative electrode migration, device activation was not successful. In this case, after the electrode array was repositioned, activation was successful. The results of the audiovisual mode 2 weeks after device activation revealed a lip-reading enhancement above the chance level in about 50% of the patients in the vowel confusion and speech-tracking tests and in 70% of the patients in the consonant confusion test. Lip-reading enhancement improved within the first 6 months and then entered a plateau phase, which was more prominent in the monosyllabic vowel and consonant tests. In the auditory alone mode, more than half of the patients showed their first positive result in the vowel test 3 months after device activation, but it took about 6 months until half of the patients revealed a result above the chance level in the consonant and Freiburger numbers tests. Open set speech recognition in the auditory alone mode (in the speech-tracking test) was not common and happened relatively late (within 1 year or later). DISCUSSION AND CONCLUSION: Although auditory sensation appeared immediately after device activation, a period of 6 months was necessary for relearning and adaptation of the central auditory system to the altered form of auditory information presented by the auditory brainstem implant.

Trigeminal root recording in normal trigeminal function.

Despite many investigations on the detection of trigeminal evoked potentials (TEP), there is still no consensus on a technique or interpretation of its components. In order to obtain clear TEP waveforms and analyze the origin of early components, we recorded TEP by the conventional far-field technique at the ipsilateral auricula as well as by near-field technique with a bipolar electrode at the trigeminal root just distal to the entry zone of its root. Trigeminal recordings were performed in nine patients with intrameatal vestibular schwannomas and in three with trigeminal neuralgia without trigeminal nerve deficits. In near-field recording at the trigeminal root, a triphasic response was recorded, with the first component occurring at 3.16 ms, 2.98 ms, and 4.16 ms following supraorbital, infraorbital, and mandibular nerve stimulation, respectively. Using conventional farfield TEP, the first component recorded at the trigeminal root occurred later than the first component in all other recordings. This suggests that the N2.0 component of far-field TEP is of extra-axial origin and the N5 component is of brainstem origin. The N13.0 component may be of cortical origin. We conclude that the N5.0 component and N5.0-N13.0 interpeak latency may be used as electrophysiological parameters of brainstem function during posterior fossa surgery.

Impact of computed tomographic and magnetic resonance imaging findings on surgical outcome in petroclival meningiomas.

OBJECTIVE: The preoperative radiological findings of computed tomographic and magnetic resonance imaging scans of 70 patients with petroclival meningioma were evaluated and statistically compared with the degree of surgical resection and patients' outcomes to depict the most important radiological findings that may influence surgical radicality and outcome. METHODS: The following parameters were evaluated: 1) tumor diameters; 2) tumor extension toward the middle fossa, the internal auditory canal, the brainstem, and the foramen magnum; 3) bone changes; 4) peritumoral edema; 5) signs of tumor infiltrative pattern; and 6) surgical radicality. Postoperative results were analyzed immediately after the surgery and in a long-term follow-up study. RESULTS: Larger tumors affected a younger population and presented a significantly shorter time until symptom onset. In the majority of cases (67%), the tumor extended to the parasellar region. Tumor extension toward the jugular foramen was found in 24% of the patients and reached the level of the foramen magnum in 18%. Irregular tumor margins were found in 67% of the tumors, and 50% of them presented peritumoral edema in addition. Interestingly, edema also was found in 20% of tumors with well-delineated margins. CONCLUSION: Tumor size, brainstem compression, and tumor extension laterally to the internal auditory canal did not influence either the degree of surgical resection or the long-term outcome (P > 0.05). Supratentorial tumor extension to the middle fossa and downward involving the caudal cranial nerves displayed a significant importance in regard to the surgical radicality and the patient's outcome, respectively (P < 0.05). Radiological evidence of infiltrative tumor pattern and peritumoral edema at the brainstem surface were important parameters regarding surgical radicality (P < 0.05). However, only peritumoral edema influenced the long-term results significantly.

Acoustic evoked response following transection of the eighth nerve in the rat.

OBJECT: The auditory brainstem response (ABR) is the most widely used means of intra-operative monitoring of the integrity of the auditory nerve and brainstem pathways during surgery in the cerebellopontine angle (CPA). Reliability of this and other electrophysiological techniques has been questioned because of persisting potentials in direct nerve recordings despite complete eighth nerve section. The study was designed to assess the extent to which an acoustic evoked response persists after the cochlear nerve is lesioned in the CPA of the adult rat. METHODS: The eighth nerve was exposed microsurgically via a lateral suboccipital approach without damage to surrounding structures. The auditory brainstem response to monaurally presented click stimuli was recorded using needle electrodes and a bandpass of 10 to 5000 Hz. FINDINGS: Complete sharp sectioning of the nerve in the CPA resulted in immediate disappearance of brainstem-generated potentials but persistence of a large primary, vertex-positive wave in all but one case. This response was also abolished in recordings three days later and after emptying the inner ear canal. Provided that the cochlea remained intact, two weeks later a single, vertex-positive potential in the latency range of wave Ia of the ABR reappeared, reaching its peak amplitude six weeks after sectioning of the nerve. CONCLUSIONS: The short-latency electrical potential recorded following damage of the eighth nerve in the cerebellopontine angle can be mistaken for an indication that nerve function is still preserved. The evoked injury potential is probably the major contributor to this potential that resembles wave I of the ABR. Monitoring of functional auditory integrity must neither be limited to early components of the ABR, nor to the electrocochleogram (EcoG) and the peripheral compound nerve action potential (CNAP), respectively.

Subarachnoid fat dissemination after resection of a cerebellopontine angle dysontogenic cyst: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: This case report illustrates the clinical and radiological relevance of extensive intracranial subarachnoid and ventricular dissemination in dysontogenic (dermoid) tumors. CLINICAL PRESENTATION: We describe a patient with a cerebellopontine angle dysontogenic tumor. Postoperatively, the cyst disseminated fat particles extensively into the subarachnoid space. Magnetic resonance imaging (MRI) studies revealed continuous dispersion of the fat particles into the cerebral cisterns, subarachnoid space, and ventricles. INTERVENTION: Eight years of clinical and MRI follow-up demonstrated neither neurological deterioration in the patient nor growth of the multiple lesions. CONCLUSION: Intracranial subarachnoid dissemination of fat material may occur during the preoperative or postoperative course of dermoid and epidermoid cysts. Aseptic meningitis or other complications such as hydrocephalus, seizures, or cranial nerve deficits also may occur owing to spillage of intracranial cyst contents into the subarachnoid space. MRI can detect the presence of fat drops that may adhere to the surrounding structures or migrate with the cerebrospinal fluid flow. Intracranial disseminated fat particles can remain silent without radiological or neurological change, justifying a wait-and-see approach. During long-term postoperative follow-up, however, regular MRI studies and clinical examinations are necessary to avoid potential complications.

Extensive monitoring during auditory brainstem implant surgery.

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.

Auditory brainstem implant in auditory rehabilitation of patients with neurofibromatosis type 2: Hannover programme.

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.

Auditory brainstem implants: current neurosurgical experiences and perspective.

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.

Arachnoid cysts of the posterior fossa.

BACKGROUND: The surgical indications and management of posterior fossa arachnoid cysts (AC) are still controversial. Different surgical techniques and management have already been suggested for arachnoid cysts of the posterior fossa. AC involving the posterior fossa and especially the cerebellopontine angle may carry a high surgical morbidity because of the involvement of important neurovascular structures (e.g., brain stem and cranial nerves). Only long-term follow-up will determine the best surgical technique for such lesions. METHODS: Between 1990 and 1996 a total of 12 patients underwent surgery for arachnoid cysts involving the posterior fossa. In seven cases AC were located within the cerebellopontine angle (CPA), in three cases in the CPA with major extension dorsal to the brainstem, and in two cases at the CPA extending into the internal auditory canal. RESULTS: A suboccipital retrosigmoid approach was performed in all patients. Radical resection of the cyst could be accomplished in all but one case. There was no mortality. Major postoperative morbidity was present in one case because of an intraoperative air embolism in the semisitting position and strong adherence of the cyst wall to the surrounding neurovascular structures. Long-term follow-up (mean, 3.3 years) revealed improvement of most preoperative symptoms. CONCLUSION: Open surgery and radical removal of the AC located at the posterior fossa, based on our retrospective analysis, provide very good long-term postoperative results. The suboccipital approach provides a good and safe exposure of vascular structures and cranial nerves in the CPA and allows radical resection of the cyst, reducing the chance of recurrence.