Hearing preservation and quality of life in small to medium sized vestibular schwannomas after a wait and scan approach or stereotactic radiosurgery: a systematic review and meta-analysis.

BACKGROUND: The management of vestibular schwannomas (VS) encompasses a choice between conservative "wait-and-scan" (WAS) approach, stereotactic radiosurgery (SRS) or open microsurgical resection. Currently, there is no consensus on the optimal management approach for small to medium sized VS. This study aims to compared outcomes related to hearing in patients with small and medium sized VS who underwent initial treatment with WAS versus SRS. METHODS: A systematic review of the available literature was conducted using PubMed/MEDLINE, Embase, and Cochrane up December 08, 2023. Meta-analysis was performed using a random-effect model to calculate mean difference (MD) and relative risk (RR). A leave-one-out analysis was conducted. The risk of bias was assessed via the Risk of Bias in Non-randomized Studies-Interventions (ROBINS-I) and Cochrane Risk of Bias assessment tool (RoB-2). Ultimately, the certainty of evidence was evaluated using the GRADE assessment. The primary outcomes were serviceable hearing, and pure-tone average (PTA). The secondary outcome was the Penn Acoustic Neuroma Quality of Life Scale (PANQOL) total score. RESULTS: Nine studies were eligible for inclusion, comprising a total of 1,275 patients. Among these, 674 (52.86%) underwent WAS, while 601 patients (47.14%) received SRS. Follow-up duration ranged from two to eight years. The meta-analysis indicated that WAS had a better outcome for serviceable hearing (0.47; 95% CI: 0.32 - 0.68; p < 0.001), as well as for postoperative functional measures including PTA score (MD 13.48; 95% CI 3.83 - 23.13; p < 0.01), and PANQOL total score (MD 3.83; 95% CI 0.42 - 7.25; p = 0.03). The overall certainty of evidence ranged from "very low" to "moderate". CONCLUSIONS: Treating small to medium sized VS with WAS increases the likelihood of preserving serviceable hearing and optimized PANQOL overall postoperative score compared to SRS. Nevertheless, the limited availability of literature and the methodological weakness observed in existing studies outline the need for higher-quality studies.

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OBJECTIVES: Acoustic neuroma (AN) is a benign tumor that usually affects a patient's hearing and balance function. For the screening and diagnosis of AN, the traditional approach mainly relies on audiological examination and magnetic resonance imaging (MRI), often ignoring the importance of vestibular function assessment in the affected area. As an emerging method of vestibular function detection, video head impulse test (vHIT) has been widely used in clinic, but research on its use in AN diagnosis is relatively limited. This study aims to explore the value of vHIT in the diagnosis of AN, vestibular dysfunction assessment, and postoperative compensation establishment in unilateral AN patients undergoing unilateral AN resection through labyrinthine approach. METHODS: This retrospective study was conducted on 27 AN patients who underwent unilateral AN resection via labyrinthine approach from October 2020 to March 2022 in the Department of Otolaryngology-Head and Neck Surgery, the Second Xiangya Hospital, Central South University. vHIT was performed 1 week before surgery to assess vestibular function, pure tone audiometry (PTA) was used to assess hearing level, and ear MRI was used to assess tumor size. Follow-up vHIT was conducted at 1 week, 1 month, 6 months, and 1 year post-surgery. The correlation of vHIT with hearing and tumor size was analyzed. RESULTS: Preoperative vHIT showed that the posterior semicircular canal on the affected side was the most common semicircular canal with reduced vestibulo-ocular reflex (VOR) gain. There was a correlation between the VOR gain of vHIT on the affected side and the hearing level (r=-0.47, P<0.05) or tumor size (r=-0.54, P<0.01). The results of vHIT on the affected side showed that the hearing level and mean VOR gain of the anterior semicircular canal increased slightly with time, and the amplitude and saccade percentage of the dominant saccades of the 3 semicircular canals increased, while the latency time decreased, with the most obvious changes occurring 1 week post-surgery. CONCLUSIONS: vHIT can effectively monitor the changes of vestibular function in AN patients before and after surgery and has application value in assisting the diagnosis of vestibular dysfunction in AN patients.

Gustatory Function in Patients With Cerebellopontine Angle Masses.

OBJECTIVE: To investigate the impact of cerebellopontine angle (CPA) masses on subjective and measured taste function. STUDY DESIGN: Retrospective cross-sectional study. SETTING: Tertiary referral center. PATIENTS: Consecutive adult patients with untreated CPA masses. INTERVENTIONS: Gustatory function was psychophysically measured with Taste Strips (range, 0-16) on both sides of the tongue. Subjective taste complaints were assessed using a questionnaire. MAIN OUTCOME MEASURES: Half-sided taste impairment (hemi-ageusia) was defined as side-to-side asymmetry ≥4 points with <9 points on the side of the CPA mass. We used the Koos classification for vestibular schwannomas (VS) and, in the case of facial nerve palsy, the House-Brackmann grading system. RESULTS: We included 135 patients (mean [standard deviation (SD)] age, 55.3 ± 14.1 yr; 62 males). The most common CPA mass was VS (77%). Overall, the measured taste function was lower on the affected compared with the healthy side of the tongue (mean score, 9.8 ± 3.3 versus 11 ± 2.9; p < 0.0001). Looking for clinically relevant one-sided taste impairment revealed 18 (13.3%) patients with hemi-ageusia, but only 4 (30.8%) of those subjectively complained of taste dysfunction. Regarding VS, Koos IV masses presented the lowest score on the affected side (mean score, 7.5 ± 3.7). Six patients presented with facial palsy. Having facial palsy did not result in a lower Taste Strips score (p = 0.23). CONCLUSION: Before any CPA mass treatment, a measurable ipsilateral decrease in gustatory function is present in many patients. Most patients do not notice this preexisting taste impairment. From a medicolegal standpoint, this warrants consideration. To avoid postoperative claims regarding taste function, a preoperative assessment may be considered.

Structural connectivity changes in unilateral hearing loss.

Network connectivity, as mapped by the whole brain connectome, plays a crucial role in regulating auditory function. Auditory deprivation such as unilateral hearing loss might alter structural network connectivity; however, these potential alterations are poorly understood. Thirty-seven acoustic neuroma patients with unilateral hearing loss (19 left-sided and 18 right-sided) and 19 healthy controls underwent diffusion-weighted and T1-weighted imaging to assess edge strength, node strength, and global efficiency of the structural connectome. Edge strength was estimated by pair-wise normalized streamline density from tractography and connectomics. Node strength and global efficiency were calculated through graph theory analysis of the connectome. Pure-tone audiometry and word recognition scores were used to correlate the degree and duration of unilateral hearing loss with node strength and global efficiency. We demonstrate significantly stronger edge strength and node strength through the visual network, weaker edge strength and node strength in the somatomotor network, and stronger global efficiency in the unilateral hearing loss patients. No discernible correlations were observed between the degree and duration of unilateral hearing loss and the measures of node strength or global efficiency. These findings contribute to our understanding of the role of structural connectivity in hearing by facilitating visual network upregulation and somatomotor network downregulation after unilateral hearing loss.

Tumors of the nervous system and hearing loss: Beyond vestibular schwannomas.

Hearing loss is a common side effect of many tumor treatments. However, hearing loss can also occur as a direct result of certain tumors of the nervous system, the most common of which are the vestibular schwannomas (VS). These tumors arise from Schwann cells of the vestibulocochlear nerve and their main cause is the loss of function of NF2, with 95 % of cases being sporadic and 5 % being part of the rare neurofibromatosis type 2 (NF2)-related Schwannomatosis. Genetic variations in NF2 do not fully explain the clinical heterogeneity of VS, and interactions between Schwann cells and their microenvironment appear to be critical for tumor development. Preclinical in vitro and in vivo models of VS are needed to develop prognostic biomarkers and targeted therapies. In addition to VS, other tumors can affect hearing. Meningiomas and other masses in the cerebellopontine angle can compress the vestibulocochlear nerve due to their anatomic proximity. Gliomas can disrupt several neurological functions, including hearing; in fact, glioblastoma multiforme, the most aggressive subtype, may exhibit early symptoms of auditory alterations. Besides, treatments for high-grade tumors, including chemotherapy or radiotherapy, as well as incomplete resections, can induce long-term auditory dysfunction. Because hearing loss can have an irreversible and dramatic impact on quality of life, it should be considered in the clinical management plan of patients with tumors, and monitored throughout the course of the disease.

Novel standardized indexes of brainstem auditory evoked potentials for predicting hearing preservation in vestibular schwannomas.

Hearing preservation (HP) during vestibular schwannomas (VSs) surgery poses a significant challenge. Although brainstem auditory evoked potentials (BAEPs) on the affected side are commonly employed to monitor cochlear nerve function, their low signal-to-noise ratio (SNR) renders them susceptible to interferences, compromising their reliability. We retrospectively analyzed the data of patients who underwent tumor resection, while binaural brainstem auditory evoked potentials (BAEPs) were simultaneously recorded during surgery. To standardize BAEPs on the affected side, we incorporated the synchronous healthy side as a reference (interval between affected and healthy side ≤ 3 min). A total of 127 patients were enrolled. Comparison of the raw BAEPs data pre- and post-tumor resection revealed that neither V-wave amplitude (Am-V) nor latency (La-V) could serve as reliable predictors of HP simultaneously. However, following standardization, V-wave latency (STIAS-La-V) and amplitude (STIAS-Am-V) emerged as stable predictors of HP. Furthermore, the intraoperative difference in V-wave amplitude (D-Am-V) predicted postoperative HP in patients with preoperative HP and remained predictive after standardization. The utilization of intraoperative synchronous healthy side BAEPs as a reference to eliminate interferences proves to be an effective approach in enhancing the reliability of BAEPs for predicting HP in VSs patients.

Natural history of hearing and tumor growth in vestibular schwannoma in neurofibromatosis type 2-related schwannomatosis.

OBJECTIVES: To determine the natural history of hearing loss and tumor volume in patients with untreated neurofibromatosis type 2 (NF2)-related schwannomatosis. Moreover, we statistically examined the factors affecting hearing prognosis. METHODS: This retrospective cohort study was conducted on 37 ears of 24 patients with NF2-related vestibular schwannomatosis followed up without treatment for more than 1 year. We obtained detailed chronological changes in the PTA and tumor volume in each case over time, and the rate of change per year was obtained. Multivariate analysis was also conducted to investigate factors associated with changes in hearing. RESULTS: The average follow-up period was approximately 9 years, and hearing deteriorated at an average rate of approximately 4 dB/year. The rate of maintaining effective hearing decreased from 30 ears (81%) at the first visit to 19 ears (51%) at the final follow-up. The average rate of change in tumor growth for volume was approximately 686.0 mm3/year. This study revealed that most patients with NF2 experienced deterioration in hearing acuity and tumor growth during the natural course. A correlation was observed between an increase in tumor volume and hearing loss (r = 0.686; p < 0.001). CONCLUSIONS: Although the hearing preservation rate in NF2 cases is poor with the current treatment methods, many cases exist in which hearing acuity deteriorates, even during the natural course. Patients with an increased tumor volume during the follow-up period were more likely to experience hearing deterioration. Trial registration number 20140242 (date of registration: 27 October 2014).

Intraoperative assessment of cochlear nerve functionality in various vestibular schwannoma scenarios: Lessons learned.

The use of cochlear implants (CIs) is on the rise for patients with vestibular schwannoma (VS). Besides CI following tumor resection, new scenarios such as implantation in observed and/or irradiated tumors are becoming increasingly common. A significant emerging trend is the need of intraoperative evaluation of the functionality of the cochlear nerve in order to decide if a CI would be placed. The purpose of this paper is to explore the experience of a tertiary center with the application of the Auditory Nerve Test System (ANTS) in various scenarios regarding VS patients. The results are compared to that of the studies that have previously used the ANTS in this condition. Patients with unilateral or bilateral VS (NF2) who were evaluated with the ANTS prior to considering CI in a tertiary center between 2021 and 2023 were analyzed. The presence of a robust wave V was chosen to define a positive electrical auditory brainstem response (EABR). Two patients underwent promontory stimulation (PromStim) EABR previous to ANTS evaluation. Seven patients, 2 NF-2 and 5 with sporadic VS were included. The initial scenario was simultaneous translabyrinthine (TL) tumor resection and CI in 3 cases while a CI placement without tumor resection was planned in 4 cases. The ANTS was positive in 4 cases, negative in 2 cases, and uncertain in one case. Two patients underwent simultaneous TL and CI, 1 patient simultaneous TL and auditory brainstem implant, 3 patients posterior tympanotomy with CI, and 1 patient had no implant placement. In the 5 patients undergoing CI, sound detection was present. There was a good correlation between the PromStim and ANTS EABR. The literature research yielded 35 patients with complete information about EABR response. There was one false negative and one false positive case; that is, the 28 implanted cases with a present wave V following tumor resection had some degree of auditory perception in all but one case. The ANTS is a useful intraoperative tool to asses CI candidacy in VS patients undergoing observation, irradiation or surgery. A positive strongly predicts at least sound detection with the CI.

Predictive value of Blink reflex and facial corticobulbar motor evoked potential in cerebellopontine angle tumor surgery.

OBJECTIVE: The current study examined the efficacy of the facial corticobulbar motor evoked potentials (FCoMEPs) and blink reflex (BR) on predicting postoperative facial nerve function during cerebellopontine angle (CPA) tumor surgery. METHODS: Data from 110 patients who underwent CPA tumor resection with intraoperative FCoMEPs and BR monitoring were retrospectively reviewed. The association between the amplitude reduction ratios of FCoMEPs and BR at the end of surgery and postoperative facial nerve function was determined. Subsequently, the optimal threshold of FCoMEPs and BR for predicting postoperative facial nerve dysfunction were determined by receiver operating characteristic curve analysis. RESULTS: Valid BR was record in 103 of 110 patients, whereas only 43 patients successfully recorded FCoMEP in orbicularis oculi muscle. A reduction over 50.3% in FCoMEP (O. oris) amplitude was identified as a predictor of postoperative facial nerve dysfunction (sensitivity, 77.1%; specificity, 83.6%). BR was another independent predictor of postoperative facial nerve deficit with excellent predictive performance, especially eyelid closure function. Its optimal cut-off value for predicting long-term postoperative eyelid closure dysfunction was was 51.0% (sensitivity, 94.4%; specificity, 94.4%). CONCLUSIONS: BR can compensate for the deficiencies of the FCoMEPs. The combination of BR and FCoMEPs can be used in CPA tumor surgery. SIGNIFICANCE: The study first proposed an optimal cut-off value of BR amplitude deterioration (50.0%) for predicting postoperative eyelid closure deficits in patients undergoing CPA tumor surgery.

Audiological Characteristics of Vestibular Schwannoma Patients With Normal Pure-Tone Audiometry.

OBJECTIVE: To investigate the audiological characteristics of vestibular schwannoma (VS) patients with normal pure-tone audiometry (PTA) results. STUDY DESIGN: A retrospective study. SETTING: Forty-two VS patients with normal PTA results from October 2016 to October 2022 were included. METHODS: Normal PTA was defined when the hearing threshold is ≤25 dB hearing loss (HL) in each test frequency and the PTA is ≤25 dB HL. Results of multiple audiological tests such as the auditory brainstem response (ABR), distortion product otoacoustic emission (DPOAE), multiple auditory steady-state responses threshold (ASSR), and speech discrimination score were retrospectively reviewed. Demographic data of these patients were also been collected. RESULTS: According to our results, the ABR and average ASSR threshold of the affected side were statistically significantly higher in VS patients with normal PTA. ABR waveforms on the affected side also showed more abnormalities. The DPOAE pass rates of the affected side were lower than the unaffected side while the amplitude and signal-to-noise ratio rate was also lower. In addition, we used magnetic resonance imaging 3-dimensional reconstruction images to measure the volume of tumors in these patients. We also found that higher ABR threshold means lager tumor size in patients with normal PTA. CONCLUSION: VS patients with normal PTA result cannot be assumed to have no impairment of hearing function. ABR, DPOAE, and ASSR results showed the characteristic changes in the affect ear. ABR threshold has the highest sensitivity for hearing abnormalities and is strong relative with tumor size in patients with normal PTA.

Pre- and post-operative semicircular canal function evaluated by video head impulse test in patients with vestibular schwannoma.

OBJECTIVES: To evaluate pre- and post-operative semicircular canal function in patients with vestibular schwannoma (VS) by the video Head Impulse Test (vHIT). METHODS: Nineteen patients with VS who underwent surgery were enrolled in this study. The gain in vestibulo-ocular reflex (VOR) and the degree of scatter in catch-up saccades were examined pre- and post-operatively for the semicircular canals in VS patients. RESULTS: Ten of 19 cases (52.6 %) with VS were defined as demonstrating both superior vestibular nerve (SVN) and inferior vestibular nerve (IVN) impairment from the results of pre-operative vHIT. Hearing level and subjective vestibular symptoms showed significant correlations with pre-operative semicircular canal function. Compared to pre-operative vHIT results, VOR gains within 1 month after surgery were significantly reduced in all three canals; however, significant differences had disappeared in the anterior and posterior semicircular canals at 6 months after surgery. Cases of unknown origin had a significantly greater reduction in posterior semicircular canal function after surgery compared with those with disease of IVN origin. CONCLUSIONS: As vHIT could evaluate pre-operative vestibular nerve impairment, post-operative VOR gain reduction and the degree of vestibular compensation, semicircular canal function evaluated by vHIT provides a good deal of useful information regarding VS patients undergoing surgery compared to caloric testing, and vHIT should be performed pre- and post-operatively for patients with VS.

Imbalance and dizziness caused by unilateral vestibular schwannomas correlate with vestibulo-ocular reflex precision and bias.

Imbalance and dizziness are disabling symptoms for many patients with vestibular schwannomas (VS) but symptom severity typically does not correlate with the vestibulo-ocular reflex (VOR) amplitude-based metrics used to assess peripheral vestibular damage. In this study, we tested the hypothesis that imbalance and dizziness in patients with VS relate to VOR metrics that are not based on response amplitude. Twenty-four patients with unilateral, sporadic VS tumors were studied, and objective (balance) and subjective (dizziness) vestibular dysfunction was quantified. The VOR was tested using two yaw-axis motion stimuli, low-frequency en-bloc sinusoidal, and high-frequency head-on-body impulsive rotations. Imbalance correlated with VOR precision (the inverse of the trial-to-trial variability) and with low-frequency VOR dynamics (quantified with the time constant), and these two metrics were also strongly correlated. Dizziness correlated with the VOR bias caused by an imbalance in static central vestibular tone, but not with dynamic VOR metrics. VOR accuracy (mean response amplitude relative to the ideal response) was not correlated with the severity of imbalance or dizziness or with measures of VOR precision or time constant. Imbalance in patients with VS, therefore, scales with VOR precision and time constant, both of which appear to reflect the central vestibular signal-to-noise ratio, but not with VOR slow-phase accuracy, which is based on the magnitude of the central vestibular signals. Dizziness was related to the presence of a static central tone imbalance but not to any VOR metrics, suggesting that abnormal perception in VS may be affected by factors that are not captured by yaw-axis VOR measurements.NEW & NOTEWORTHY The severity of symptoms associated with unilateral vestibular schwannomas (VS) is poorly correlated with standard yaw-axis vestibulo-ocular reflex (VOR) metrics that are based on response amplitude. In this study, we show that the balance and perceptual dysfunction experienced by patients with VS scales with VOR metrics that capture information about the central signal-to-noise ratio (balance) and central static tone (dizziness), but are not correlated with the VOR gain, which reflects central signal amplitude.

Signal and morphological changes in the endolymph of patients with vestibular schwannoma on non-contrast 3D FLAIR at 3 Tesla.

BACKGROUND: Non-contrast FLAIR revealed increased signal within the inner ear in patients with vestibular schwannoma, which is generally assumed to occur in the perilymph; however, the majority of previous studies did not differentiate between the endolymph and perilymph. Therefore, endolymph signal changes have not yet been investigated in detail. The purpose of the present study was three-fold: (1) to assess perilymph signal changes in patients with vestibular schwannoma on heavily T2-weighted (T2W) 3D FLAIR, also termed positive perilymphatic images (PPI), (2) to evaluate signal and morphological changes in the endolymph on PPI, and (3) to establish whether vertigo correlates with the signal intensity ratios (SIR) of the vestibular perilymph or vestibular endolymphatic hydrops. METHODS: Forty-two patients with unilateral vestibular schwannoma were retrospectively recruited. We semi-quantitatively and qualitatively evaluated the perilymph signal intensity on the affected and unaffected sides. We also quantitatively examined the signal intensity of the vestibular perilymph and assessed the relationship between vertigo and the SIR of the vestibular perilymph on the affected side. We semi-quantitatively or qualitatively evaluated the endolymph, and investigated whether vestibular hydrops correlated with vertigo. RESULTS: The perilymph on the affected side showed abnormal signal more frequently (signal intensity grade: overall mean 1.45 vs. 0.02; comparison of signal intensity: overall mean 36 vs. 0 cases) and in more parts (the entire inner ear vs. the basal turn of the cochlea and vestibule) than that on the unaffected side. No significant difference was observed in the SIR of the vestibular perilymph with and without vertigo (5.54 vs. 5.51, p = 0.18). The endolymph of the vestibule and semicircular canals showed the following characteristic features: no visualization (n = 4), signal change (n = 1), or vestibular hydrops (n = 10). A correlation was not observed between vestibular hydrops and vertigo (p = 1.000). CONCLUSIONS: PPI may provide useful information on signal and morphological changes in the endolymph of patients with vestibular schwannoma. Further research is warranted to clarify the relationship between vertigo and the MR features of the inner ear.

Impact of extent of internal acoustic meatus tumor removal using translabyrinthine approach for acoustic neuroma surgery.

OBJECTIVES: Many studies have investigated the surgical outcome and predictive factors of acoustic neuroma using different approaches. The present study focused on large tumors due to the greater likelihood of internal acoustic meatus involvement and the greater application of surgical intervention than radiosurgery. There have been no previous reports on outcomes of internal acoustic meatus tumor removal. We investigated the impact of the extent of internal acoustic meatus tumor removal using a translabyrinthine approach for large acoustic neuroma surgery and predictive factors of tumor control. METHODS: This retrospective study reviewed 104 patients with large cerebellopontine angle tumor >3 cm treated by translabyrinthine approach microsurgery. Predictive factors of postoperative facial palsy, tumor control, and extent of internal acoustic meatus tumor removal were assessed. RESULTS: The mean tumor size was 38.95 ± 6.83 mm. Postoperative facial function showed 76.9% acceptable function (House-Brackmann grade 1 or 2) six months after surgery. The extent of internal acoustic meatus tumor removal was a statistically significant predictor factor of poor postoperative facial function. Younger age, larger tumor size needing radiosurgery, and more extensive removal of tumor were associated with better tumor control. CONCLUSION: More extensive internal acoustic meatus tumor removal was associated with poor postoperative facial function and better tumor control.

Continuous Head Motion is a Greater Motor Control Challenge than Transient Head Motion in Patients with Loss of Vestibular Function.

Background. The vestibular system is vital for gaze stability via the vestibulo-ocular reflex, which generates compensatory eye motion in the direction opposite to head motion. Consequently, individuals with peripheral vestibular loss demonstrate impaired gaze stability that reduces functional capacity and quality of life. To facilitate patients' compensatory strategies, two classes of gaze stabilization exercises are often prescribed: (i) transient (eg, ballistic) and (ii) continuous. However, the relative benefits of these two classes of exercises are not well understood. Objective. To quantify head motion kinematics in patients with vestibular loss while they performed both classes of exercises. Methods. Using inertial measurement units, head movements of 18 vestibular schwannoma patients were measured before and after surgical deafferentation and compared with age-matched controls. Results. We found that the head movement during both classes of exercises paralleled those of natural head movement recorded during daily activities. However, head movement patterns were more informative for continuous than transient exercises in distinguishing patients from healthy controls. Specifically, we observed coupling between kinematic measures in control subjects that was absent in patients for continuous but not transient head motion exercises. In addition, kinematic measures (eg, cycle duration) were predictive of standard clinical measures for continuous but not transient head motion exercises. Conclusions. Our data suggest that performing continuous head motion is a greater motor control challenge than transient head motion in patients with less reliable vestibular feedback during the sub-acute stage of recovery, which may also prove to be a reliable measure of progression in vestibular rehabilitation protocols.

Evaluation of Vestibular Function in Diagnosis of Vestibular Schwannomas.

Vestibular schwannomas (VS) are benign tumors of the vestibular nerve. The common first symptoms are hearing loss and tinnitus, followed by imbalance, vertigo, and facial nerve involvement. The subjective symptoms of VS patients are not consistent with the severity of vestibular lesions and the results of vestibular tests, which often interfere with clinicians' diagnoses. Thus, the main screening and diagnostic methods for VS are audiometry and magnetic resonance imaging (MRI), ignoring the evaluation of vestibular function at the source of pathological lesions. With the development and improvement of vestibular evaluation technology and its wide application in the clinic, modern vestibular examination technology can reflect the severity and frequency of vestibular lesions and compensation from multiple perspectives, providing an objective basis for the diagnosis and treatment of vestibular diseases. In this report, we review the results and characteristics of vestibular tests in VS patients and further clarify the clinical value of vestibular function assessment in the diagnosis and treatment of VS.

Reliability and toxicity of bevacizumab for neurofibromatosis type 2-related vestibular schwannomas: A systematic review and meta-analysis.

BACKGROUND: The anti-angiogenic agent bevacizumab is currently the only drug used clinically for neurofibromatosis type 2-related vestibular schwannomas (NF2-VS). Though benefits have been demonstrated in several cases, the standardized dosage remains unclear. OBJECTIVE: Our meta-analysis was performed to systematically and comprehensively investigate the reliability and toxicity of bevacizumab in the treatment of NF2-VS, with particular emphasis on the impact of dosage. METHODS: The literature search was conducted for studies providing data on patients treated with bevacizumab for NF2-VS across PubMed, Embase, and Cochrane Library until December 31, 2020. Two reviewers extracted the incidence rate of results independently. Then we calculated and pooled unadjusted incidence rate with 95% CIs for each study. The subgroups analyzed were conducted. RESULTS: Fourteen citations (prospective or retrospective observational cohort studies) were eligible based on data from a total of 247 patients with NF2 and 332 related VSs. The pooled results showed that the radiographic response rate (RRR) was 30% [95% CI (20%-42%)], the hearing response rate (HRR) was 32% [95% CI (21%-45%)]. The incidence of major complications was: hypertension 29% [95% CI (23%-35%)], proteinuria 30% [95% CI (18%-44%)], menstrual disorders 44% [95% CI (16%-73%)], hemorrhage 14% [95% CI (4%-26%)], grade3/4 events 12% [95% CI (4%-22%)]. CONCLUSIONS: Nearly one-third of NF2-VS patients may benefit significantly from bevacizumab due to hearing improvement and tumor reduction. Menstrual disorders were the most common adverse events. The high-dose regimen didn't show better efficacy, but results varied considerably according to age.

Case series of vestibular schwannoma patients with no asymmetry in hearing.

OBJECTIVE: (1) Describe the presenting symptoms and tumor characteristics of patients who are diagnosed with a vestibular schwannoma (VS) with normal hearing or symmetric hearing loss, and (2) report the prospective growth and treatment of each tumor. METHODS: This is a retrospective cohort study of acoustic neuroma patients who were found to have a VS and normal or symmetric hearing loss at a single tertiary care academic center from 1999 to 2012. Medical records were reviewed collecting the following information: patient demographics, symptoms, MRI characteristics, and treatment modality. RESULTS: 15/195 (7.7%) patients met criteria for the study. Dizziness was the most common presenting symptom occurring in 9 subjects (60%), followed by headache in 6 (40%), vision changes in 5 (33%), tinnitus in 5 (33%), and unilateral hearing loss in 1 (7%). The average tumor size was 1.07 cm (range 0.3-2.2 cm). Eight (53%) patients presented with intracanalicular tumors. Growth was observed in 6 subjects (40%) with average growth rate in those who exhibited mean growth of 1.8 mm per year. Treatment consisted of surgical removal in 4 cases (27%), gamma knife therapy in 1 (7%), and observation or loss to follow-up in 10 (66%). CONCLUSIONS: The incidence of VS with normal hearing was 7.7% Patients with VSs who do not exhibit unilateral hearing loss present most commonly with dizziness. Most of our patients had small, intracanalicular tumors with the largest tumor measuring 2.2 cm in greatest dimension. Of those who were managed conservatively with repeat imaging and observation, most showed tumor growth.

Reorganization of auditory-visual network interactions in long-term unilateral postlingual hearing loss.

Long-term unilateral hearing loss could reorganize the functional network association between the bilateral auditory cortices, while alterations of other functional networks need to be further explored. We attempted to investigate the pattern of the reorganization of functional network associations between the auditory and visual cortex caused by long-term postlingual unilateral hearing loss (UHI) and its relationship with clinical characteristics. Therefore, 48 patients with hearing loss caused by unilateral acoustic tumors and 52 matched healthy controls were enrolled, and their high-resolution structural MRI and resting-state functional MRI data were also collected to depict the brain network. Degree centrality (DC) was employed to evaluate the functional network association of the auditory-visual network interaction. Group comparisons were performed to investigate the network reorganization, and its correlations with clinical data were calculated. Compared with the healthy control group, patients with UHI showed significantly increased DC between the auditory network (superior temporal gyrus and the medial geniculate body) and the visual network. Meanwhile, this difference was positively correlated with the extent of hearing impairment, and the correlation was more significant with the ipsilateral superior temporal gyrus in cases of acoustic neuroma. These results suggest that long-term unilateral hearing impairment may lead to enhancement of the visual-auditory network interactions and that the degree of reorganization is positively correlated with the pure tone average (PTA) and is more significant for the ipsilateral superior temporal gyrus, which provides clinical evidence regarding cross-modal plasticity in the UHI and its lateralization.

Head movement kinematics are altered during gaze stability exercises in vestibular schwannoma patients.

Gaze stability is the ability of the eyes to fixate a stable point when the head is moving in space. Because gaze stability is impaired in peripheral vestibular loss patients, gaze stabilization exercises are often prescribed to facilitate compensation. However, both the assessment and prescription of these exercises are subjective. Accordingly, here we quantified head motion kinematics in patients with vestibular loss while they performed the standard of care gaze stability exercises, both before and after surgical deafferentation. We also correlate the head kinematic data with standard clinical outcome measures. Using inertial measurement units, we quantified head movements in patients as they transitioned through these two vestibular states characterized by different levels of peripheral damage. Comparison with age-matched healthy control subjects revealed that the same kinematic measurements were significantly abnormal in patients both pre- and post-surgery. Regardless of direction, patients took a longer time to move their heads during the exercises. Interestingly, these changes in kinematics suggest a strategy that existed preoperatively and remained symmetric after surgery although the patients then had complete unilateral vestibular loss. Further, we found that this kinematic assessment was a good predictor of clinical outcomes, and that pre-surgery clinical measures could predict post-surgery head kinematics. Thus, together, our results provide the first experimental evidence that patients show significant changes in head kinematics during gaze stability exercises, even prior to surgery. This suggests that early changes in head kinematic strategy due to significant but incomplete vestibular loss are already maladaptive as compared to controls.