Quality of life in patients after vestibular schwannoma surgery.

AIM: To evaluate the most important factors of quality of life in patients after vestibular schwannoma surgery. MATERIALS AND METHODS: Patients with unilateral sporadic occurrence of vestibular schwannoma who underwent surgery via suboccipital-retrosigmoid approach were included in the prospective study (2018-2021). Patients after previous Leksell gamma knife irradiation (or other methods of stereotactic radiosurgery) were excluded. Quality of life was assessed using 10 validated questionnaires that were distributed preoperatively, 3 months and 1 year after the surgery. RESULTS: A total of 76 patients were included in the study, complete data were analysed in 43 of them (response rate 57%). Grade III and IV represented up to 70% of all tumors. Patients with larger tumors had a significantly higher risk of postoperative facial nerve paresis, liquorrhea and lower probability of hearing preservation. Patients with smaller tumors and those, who suffered from headaches before surgery had more frequent and severe headaches after surgery. Postoperative headaches were associated with higher incidence of anxiety and tinnitus. More frequent anxiety was also identified in patients with preoperative serviceable hearing who became deaf after surgery. Nevertheless, tinnitus and hearing impairment appeared to have less impact on overall quality of life compared to headaches and facial nerve function. CONCLUSION: According to our results, tumor size, postoperative function of the facial nerve and occurrence of postoperative headaches had the greatest influence on the overall postoperative quality of life in patients after vestibular schwannoma surgery.

Application of diffusion tensor imaging of the facial nerve in preoperative planning for large vestibular schwannoma: a systematic review.

The accurate identification and preservation of the facial nerve (FN) during vestibular schwannoma (VS) surgery is crucial for maintaining facial function. Investigating the application of diffusion tensor imaging (DTI) in preoperative planning for large VS surgery is provided. PubMed, Cochrane Library, Science Direct, ISI Web of Science, Embase, and additional sources were searched to identify cohort studies about the preoperative DTI usage for the FN tracking before large VS (≥ 2.5 cm) surgery published between 1990 and 2023. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed; the Newcastle-Ottawa Scale was used to assess the risk of bias and to evaluate limitations based on selection/outcome biases. A total of 8 publications yielding 149 VS (mean size 3.66 ± 0.81 cm) were included. Surgical concordance with preoperative DTI FN tracking was 91.67% (range 85-100%). Overall DTI reliability was 88.89% (range 81.81-95.83%). Larger tumor size predicted either DTI inaccurate finding or complete DTI failure (p = 0.001). VS size above > 3.5 cm was associated with a higher risk of DTI failure (p = 0.022), with a higher risk of inaccurate DTI finding preoperatively (p = 0.033), and with a higher House-Brackman score postoperatively (p = 0.007). Application of DTI in larger VS surgery is a valuable FN identification along with electrophysiological monitoring and neuronavigation, therefore also in its preservation and in lowering risk of complications. DTI represents a valuable adjunct to electrophysiological monitoring and neuronavigation in FN identification, applicable not only for smaller, but also larger VS.

Diffusion tensor imaging helps identify shunt-responsive normal pressure hydrocephalus patients among probable iNPH cohort.

The aim of this study was to investigate whether white matter changes as measured by diffusion tensor imaging (DTI) can help differentiate shunt-responsive idiopathic normal pressure hydrocephalus (iNPH) patients from patients with other causes of gait disturbances and/or cognitive decline with ventriculomegaly whose clinical symptoms do not improve significantly after cerebrospinal fluid derivation (non-iNPH). Between 2017 and 2022, 85 patients with probable iNPH underwent prospective preoperative magnetic resonance imaging (MRI) and comprehensive clinical workup. Patients with clinical symptoms of iNPH, positive result on lumbar infusion test, and gait improvement after 120-h lumbar drainage were diagnosed with iNPH and underwent shunt-placement surgery. Fractional anisotropy (FA) and mean diffusivity (MD) values for individual regions of interest were extracted from preoperative MRI, using the TBSS pipeline of FSL toolkit. These FA and MD values were then compared to results of clinical workup and established diagnosis of iNPH. An identical MRI protocol was performed on 13 age- and sex-matched healthy volunteers. Statistically significant differences in FA values of several white matter structures were found not only between iNPH patients and healthy controls but also between iNPH and non-iNPH patients. ROI that showed best diagnostic ability when differentiating iNPH among probable iNPH cohort was uncinate fasciculus, with AUC of 0.74 (p < 0.001). DTI methods of white matter analysis using standardised methods of ROI extraction can help in differentiation of iNPH patients not only from healthy patients but also from patients with other causes of gait disturbances with cognitive decline and ventriculomegaly.

Which Epidemiological Characteristics Drive Decision Making in the Management of Patients with Vestibular Schwannoma?

The incidence of sporadic vestibular schwannoma has significantly increased over the past few decades. However, there is no method currently available to accurately predict the risk of subsequent tumor growth. The difference in the management of five patient groups has been evaluated: wait and scan, conversion to microsurgery, conversion to stereoradiotherapy, sterioradiotherapy, and microsurgery. In total, 463 patients with vestibular schwannoma have been consulted in our department from 2010 through 2016. Of the 250 patients initially indicated for observation, 32.4% were later indicated for active treatment. Younger patients were more frequently indicated for surgery (mean age 48 years) compared to older patients, who were more often indicated for stereoradiotherapy (mean age 62 years). Tumor growth was observed more often in patients under 60 years of age and in patients with tumors greater than 10 mm. In elderly patients, including those with larger tumors, a conservative approach is the optimal solution. If tumor growth occurs in the wait-and-scan strategy, it is still possible to continue with a conservative approach in some situations. The duration of follow-up scans is still a matter of debate, as tumors can begin to grow after 5 years from the initial diagnosis.

Hearing After Vestibular Schwannoma Surgery: Is It Preserved Forever?

INTRODUCTION: Currently, it is possible to preserve the auditory nerve in a large number of cases, but the preservation of the hearing itself is unpredictable. Apart from wait and scan strategy and stereoradiotherapy, hearing after vestibular schwannoma surgery is considered to remain stable even in long-term follow-up. MATERIALS AND METHODS: Twenty-eight patients had preserved hearing after retrosigmoid suboccipital microsurgery of the vestibular schwannoma between 2008 and 2014. A standard audiological protocol was performed together with an magnetic resonance imaging evaluation of the fluid content of the inner ear. RESULTS: The mean difference in pure-tone average between the direct and final postsurgical examination was 12.758 dB ( p = 2.5E - 06). The word recognition score deteriorated by 17.45% ( p = 0.03516). The mean American Academy of Otolaryngology-Head and Neck Surgery score on the second examination was 2.5, and that on the second examination was 3.111 ( p = 0.00483). There was no significant deterioration in the healthy ear.The signal intensity ratio in the basal turn of the cochlea increased by an average of 0.13 points ( p < 0.05).Patients with persistent tumor or nodular enhancement in the internal acoustic meatus deteriorated significantly in hearing according to the American Academy of Otolaryngology-Head and Neck Surgery scale compared with patients without any finding in the meatus ( p = 0.01299). CONCLUSIONS: There is a discrete but gradual deterioration of the hearing in the postoperative period. Hearing impairment is more pronounced in patients with a nodular process in the internal acoustic meatus, regardless of whether it is growth active. After surgery, the pathological content of the inner ear normalizes (evaluated on T2 magnetic resonance imaging sequences).

Boosting phase-contrast MRI performance in idiopathic normal pressure hydrocephalus diagnostics by means of machine learning approach.

OBJECTIVE: Phase-contrast MRI allows detailed measurements of various parameters of CSF motion. This examination is technically demanding and machine dependent. The literature on this topic is ambiguous. Machine learning (ML) approaches have already been successfully utilized in medical research, but none have yet been applied to enhance the results of CSF flowmetry. The aim of this study was to evaluate the possible contribution of ML algorithms in enhancing the utilization and results of MRI flowmetry in idiopathic normal pressure hydrocephalus (iNPH) diagnostics. METHODS: The study cohort consisted of 30 iNPH patients and 15 healthy controls examined on one MRI machine. All major phase-contrast parameters were inspected: peak positive, peak negative, and average velocities; peak amplitude; positive, negative, and average flow rates; and aqueductal area. The authors applied ML algorithms to 85 complex features calculated from a phase-contrast study. RESULTS: The most distinctive parameters with p < 0.005 were the peak negative velocity, peak amplitude, and negative flow. From the ML algorithms, the Adaptive Boosting classifier showed the highest specificity and best discrimination potential overall, with 80.4% ± 2.9% accuracy, 72.0% ± 5.6% sensitivity, 84.7% ± 3.8% specificity, and 0.812 ± 0.047 area under the receiver operating characteristic curve (AUC). The highest sensitivity was 85.7% ± 5.6%, reached by the Gaussian Naive Bayes model, and the best AUC was 0.854 ± 0.028 by the Extra Trees classifier. CONCLUSIONS: Feature extraction algorithms combined with ML approaches simplify the utilization of phase-contrast MRI. The highest-performing ML algorithm was Adaptive Boosting, which showed good calibration and discrimination on the testing data, with 80.4% accuracy, 72.0% sensitivity, 84.7% specificity, and 0.812 AUC. Phase-contrast MRI boosted by the ML approach can help to determine shunt-responsive iNPH patients.

First experiences with Miethke M.blue® valve in iNPH patients.

The literature on hydrocephalus treatment shows support for adjustable valves and devices which prevent the so-called "siphon effect". In our study, 21 probable iNPH patients were indicated to shunt surgery with the Miethke M.blue® adjustable gravitational valve. Outcomes at three months were measured using the following tests: Dutch Gait Scale, International Consortium on Incontinence Questionnaire (ICIQ-UI SF), SF12V2-Health Survey, Kiefer Scale, 3T MRI, and a neuropsychological testing battery. Preoperative parameters were studied for any signs of overdrainage risk. Valves were set according to the manufacturer's recommendations. Significant improvement at three months was seen in the Dutch Gait Scale, ICIQ-UI SF, Kiefer Scale, Mental Health Component of the SF12V2-Health Survey (MCS-12) and three neuropsychological tests: Rey-Osterrieth complex figure test (ROCFT 30 min), auditory verbal learning test (AVLT I-V) and the NKP version of verbal fluency test. Seven patients needed more than one adjustment of the valve. This subgroup significantly improved only in Walking Score and Step Score but the trend was toward significant improvement in other variables. Eight patients had subdural effusions that were completely managed with adjustments until the 3-month control. BMI was significantly lower in patients with ≥2 adjustments compared to those with a maximum of one adjustment. Implantation had low complication rates and no mortality. Initial results are promising however more studies are needed to provide rationale for gravitational valves in iNPH. We recommend increasing the initial valve setting by 2-4 cm H2O above manufacturer's recommendation, especially in lean patients.

Prediction of Shunt Responsiveness in Suspected Patients With Normal Pressure Hydrocephalus Using the Lumbar Infusion Test: A Machine Learning Approach.

BACKGROUND: Machine learning (ML) approaches can significantly improve the classical Rout-based evaluation of the lumbar infusion test (LIT) and the clinical management of the normal pressure hydrocephalus. OBJECTIVE: To develop a ML model that accurately identifies patients as candidates for permanent cerebral spinal fluid shunt implantation using only intracranial pressure and electrocardiogram signals recorded throughout LIT. METHODS: This was a single-center cohort study of prospectively collected data of 96 patients who underwent LIT and 5-day external lumbar cerebral spinal fluid drainage (external lumbar drainage) as a reference diagnostic method. A set of selected 48 intracranial pressure/electrocardiogram complex signal waveform features describing nonlinear behavior, wavelet transform spectral signatures, or recurrent map patterns were calculated for each patient. After applying a leave-one-out cross-validation training-testing split of the data set, we trained and evaluated the performance of various state-of-the-art ML algorithms. RESULTS: The highest performing ML algorithm was the eXtreme Gradient Boosting. This model showed a good calibration and discrimination on the testing data, with an area under the receiver operating characteristic curve of 0.891 (accuracy: 82.3%, sensitivity: 86.1%, and specificity: 73.9%) obtained for 8 selected features. Our ML model clearly outperforms the classical Rout-based manual classification commonly used in clinical practice with an accuracy of 62.5%. CONCLUSION: This study successfully used the ML approach to predict the outcome of a 5-day external lumbar drainage and hence which patients are likely to benefit from permanent shunt implantation. Our automated ML model thus enhances the diagnostic utility of LIT in management.

Tumor Biology and Microenvironment of Vestibular Schwannoma-Relation to Tumor Growth and Hearing Loss.

Vestibular schwannoma is the most common benign neoplasm of the cerebellopontine angle. It arises from Schwann cells of the vestibular nerve. The first symptoms of vestibular schwannoma include hearing loss, tinnitus, and vestibular symptoms. In the event of further growth, cerebellar and brainstem symptoms, along with palsy of the adjacent cranial nerves, may be present. Although hearing impairment is present in 95% of patients diagnosed with vestibular schwannoma, most tumors do not progress in size or have low growth rates. However, the clinical picture has unpredictable dynamics, and there are currently no reliable predictors of the tumor's behavior. The etiology of the hearing loss in patients with vestibular schwannoma is unclear. Given the presence of hearing loss in patients with non-growing tumors, a purely mechanistic approach is insufficient. A possible explanation for this may be that the function of the auditory system may be affected by the paracrine activity of the tumor. Moreover, initiation of the development and growth progression of vestibular schwannomas is not yet clearly understood. Biallelic loss of the NF2 gene does not explain the occurrence in all patients; therefore, detection of gene expression abnormalities in cases of progressive growth is required. As in other areas of cancer research, the tumor microenvironment is coming to the forefront, also in vestibular schwannomas. In the paradigm of the tumor microenvironment, the stroma of the tumor actively influences the tumor's behavior. However, research in the area of vestibular schwannomas is at an early stage. Thus, knowledge of the molecular mechanisms of tumorigenesis and interactions between cells present within the tumor is crucial for the diagnosis, prediction of tumor behavior, and targeted therapeutic interventions. In this review, we provide an overview of the current knowledge in the field of molecular biology and tumor microenvironment of vestibular schwannomas, as well as their relationship to tumor growth and hearing loss.

Role of DESH, callosal angle and cingulate sulcus sign in prediction of gait responsiveness after shunting in iNPH patients.

Primary endpoint of this single-centre, prospective consecutive cohort study was to evaluate DESH score, CA, CSS and Evans index of suspected iNPH patients against the reference standard of lumbar infusion test (LIT) and external lumbar drainage (ELD) in prediction of gait response after VP shunt implantation in patients with idiopathic normal pressure hydrocephalus (iNPH). Patients were assigned to NPH and non-NPH groups based on LIT and ELD results. Age-matched controls were added for group comparison. 32 NPH, 46 non-NPH and 15 control subjects were enrolled in the study. There were significant differences in mean preoperative DESH scores of NPH, non-NPH and control groups (6.3 ± 2.3 ([±SD]) (range 2-10) vs 4.5 ± 2.4 (range 0-10) vs 1.0 ± 1.2 (range 0-4)). Differences in mean CA and Evans index were not significant between NPH and non-NPH groups. CSS showed 62.5% sensitivity, 60.87% specificity, 52.63% PPV and 70% NPV for differentiation of NPH and non-NPH groups. A CA of 68 degrees had 48.49% sensitivity, 76.09% specificity, 59.26% PPV 67.31% NPV and DESH score of 4 had 93.75% sensitivity, 41.30% specificity, 52.63% PPV and 90.48% NPV for differentiation between NPH and non-NPH groups. The groups of probable iNPH patients with gait impairment diagnosed by high DESH score or positive functional testing did not overlap and DESH score did not correlate with gait improvement after ELD. DESH score should not be used as a simple diagnostic or prognostic marker of iNPH and we could not confirm the benefit of measurement of callosal angle and cingulate sulcus sign.

Structural volumetry in NPH diagnostics and treatment-future or dead end?

To assess automated volumetric analysis as a potential presurgical diagnostic tool or as a method to potentially shed light on normal pressure hydrocephalus (NPH) pathophysiology. MRI imaging according to our protocol was performed in 29 NPH patients, 45 non-NPH (but suspected) patients and 15 controls. Twenty patients underwent a second MRI 3 months after ventriculoperitoneal (VP) shunt surgery. All structures relevant to NPH diagnosis were automatically segmented using commercial software. The results were subsequently tested using ANOVA analysis. Significant differences in the volumes of the corpus callosum, left hippocampus, internal globus pallidus, grey and white matter and ventricular volumes were observed between NPH group and healthy controls. However, the differences between NPH and non-NPH groups were non-significant. Three months after, VP shunt insertion decreased ventricular volume was the only clearly significant result (p value 0.0001). Even though a detailed volumetric study shows several significant differences, volumetric analysis as a standalone method does not provide a simple diagnostic biomarker, nor does it shed a light on an unknown NPH aetiology.

Normal pressure hydrocephalus-an overview of pathophysiological mechanisms and diagnostic procedures.

Normal pressure hydrocephalus (NPH) is an important differential diagnosis of neurodegenerative diseases. The prevalence of dementia is increasing in line with the worldwide increase in life expectancy. NPH can be divided into idiopathic (iNPH) and secondary (sNPH) which is important in terms of clinical symptoms, future progress, and the outcome of possible treatment. The full clinical triad is not prevalent in all of the cases and the pathophysiology of iNPH remains unclear. Diagnosis is based on the evaluation of clinical symptoms (Hakim's triad) combined with an MRI assessment, evaluation of CSF dynamic parameters by different methods such as a tap test, lumbar infusion test (LIT), and external lumbar drainage (ELD). Despite the development of diagnostic techniques and strategies in management, NPH remains to be a challenge for the specialists despite more than 50 years of research. However, results of this research have brought new opportunities in the diagnosis, therapy, and quality of life as well as survival time of NPH patients with improved symptoms. The aim of this article is to present the pathophysiological hypotheses of NPH and an overview of the diagnostic techniques used for the evaluation of NPH patients.

Pediatric Ventriculoperitoneal Shunts Revision Rate and Costs in High-Volume sub-Saharan Department.

BACKGROUND: Ventriculoperitoneal shunt (VPS) placement is one of the most common neurosurgical procedures. VPSs are associated with high costs, which predominantly arise from relatively high complication and revision rates. It is reasonable to assume that revision rates in developing countries would be higher. In this study we report the single-institution revision rates and costs from a high-volume department in sub-Saharan Africa. METHODS: A pediatric neurosurgical database was studied in the extent of 5 years. The 30-day shunt failure rate, overall revision rate, and costs were calculated, and results were compared with previously published studies from developed countries. RESULTS: In the selected time period 1840 VPS surgeries were performed, of which 592 were shunt revisions (32.14%). The majority of revision surgeries was performed in the first year- 501 (representing 84.63%); second year, 64; third year, 21; fourth year, 6; and fifth year, 2. The overall shunt revision rate was 28.94% with a 30-day revision rate of 14.58%. During the course of the study, costs of VPS surgery, the shunt, and daily ward charges did not change significantly. The average total charge for VPS insertion was 60,000 KES (586 USD), VPS removal 30,000 KES (293 USD), and VPS revision 50,000 KES (489 USD). CONCLUSIONS: This retrospective study proves that VPSs, with their known complication risks, can be performed in a sub-Saharan missionary hospital with acceptable costs and results that are comparable with those achieved in some Western hospitals. Keys to those outcomes include high volume and a highly experienced team.

Endoscopic endonasal approach for mass resection of the pterygopalatine fossa.

OBJECTIVES: Access to the pterygopalatine fossa is very difficult due to its complex anatomy. Therefore, an open approach is traditionally used, but morbidity is unavoidable. To overcome this problem, an endoscopic endonasal approach was developed as a minimally invasive procedure. The surgical aim of the present study was to evaluate the utility of the endoscopic endonasal approach for the management of both benign and malignant tumors of the pterygopalatine fossa. METHOD: We report our experience with the endoscopic endonasal approach for the management of both benign and malignant tumors and summarize recent recommendations. A total of 13 patients underwent surgery via the endoscopic endonasal approach for pterygopalatine fossa masses from 2014 to 2016. This case group consisted of 12 benign tumors (10 juvenile nasopharyngeal angiofibromas and two schwannomas) and one malignant tumor. RESULTS: No recurrent tumor developed during the follow-up period. One residual tumor (juvenile nasopharyngeal angiofibroma) that remained in the cavernous sinus was stable. There were no significant complications. Typical sequelae included hypesthesia of the maxillary nerve, trismus, and dry eye syndrome. CONCLUSION: The low frequency of complications together with the high efficacy of resection support the use of the endoscopic endonasal approach as a feasible, safe, and beneficial technique for the management of masses in the pterygopalatine fossa.

DTI-MRI biomarkers in the search for normal pressure hydrocephalus aetiology: a review.

Normal pressure hydrocephalus (NPH) is a clinical syndrome characterized by gait disturbances, urinary incontinence and dementia. Clinical presentation overlaps with Alzheimer disease (AD). Early recognition thus early intervention (shunting) is important for successful treatment, but lack of a diagnostic test with sufficient sensitivity and specificity complicates the diagnosis. We performed literature search and composed a structured review of imaging biomarkers of NPH. Morphometric studies are not sufficient to diagnose NPH. Hydrocephalus is a common finding in elderly people due to the symmetric brain atrophy and is even more pronounced in patients with AD. The key MRI biomarker seems to be diffusion tensor imaging (DTI). According to recent studies, the DTI analysis of the splenium corporis callosi, posterior limb of internal capsule, hippocampus and fornix combined with measurement of Evans index is a promising MRI biomarker of NPH and could be used for NPH diagnostics and in the differential diagnosis from AD and other dementias.

Bobble-head doll syndrome: therapeutic outcome and long-term follow-up in four children.

Bobble-head doll syndrome (BHDS) is a complex syndrome with the dominant symptom of repetitive anteroposterior head movement. Only 57 patients are quoted in the literature. The etiology of this syndrome remains unknown and no standard treatment has yet been established. We hereby report four cases treated at our department. All the patients presented a psychomotor retardation due to an obstructive hydrocephalus. All the patients were treated using neuroendoscopic techniques: two with ventriculocystostomy, and two with ventriculocystocisternostomy. Cyst decompression was achieved in all four cases and clinical recovery was evident in three of the four patients observed. After surgery, BHDS persisted longer the more the subsequent treatment was delayed. In this article, we provide a concise overview of the theories of pathogenesis, presentation, and management of this syndrome. Based on our own experience, we state that the method of choice should be the neuroendoscopy and this must be performed promptly after diagnosis is made.