Clinical practice guideline on the management of vestibular schwannoma.

INTRODUCTION: Vestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy. MATERIAL AND METHODS: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC. RESULTS: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found. CONCLUSIONS: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.

A rare case of vestibular schwannoma manifesting as trigeminal neuralgia.

BACKGROUND: In this case report, the authors reviewed a rare case of a vestibular schwannoma manifesting as trigeminal neuralgia (TN). Intracranial tumors can have a variety of orofacial pain symptoms. Among benign cerebellopontine angle tumors, vestibular schwannoma is the most common cause of a TN-like manifestation. Although the most common symptoms of a vestibular schwannoma are hearing loss and vestibulopathy, the unique feature of this case was the manifestation of symptoms consistent with TN. CASE DESCRIPTION: The patient had right-sided episodic facial pain that was short in duration and severe in intensity. The initial differential diagnoses included short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing and TN. As part of the routine evaluation, the patient was referred for brain magnetic resonance imaging, which revealed a right-sided vestibular schwannoma. The patient was prescribed 200 mg of gabapentin 3 times daily and was referred to neurosurgery for excision of the schwannoma. Surgical excision resulted in complete resolution of pain. PRACTICAL IMPLICATIONS: This case illustrates the importance of interdisciplinary treatment and how it can lead to an optimal outcome for a patient with complex orofacial pain symptoms.

Socioeconomic determinants impact quality of life at vestibular schwannoma diagnosis.

BACKGROUND: Socioeconomic variables including race, education, and income have been shown to affect vestibular schwannoma incidence, treatment, and outcomes. We sought to determine the impact of socioeconomic factors on quality of life at the time of vestibular schwannoma diagnosis. METHODS: Retrospective cohort study conducted at a tertiary academic center. All patients evaluated for vestibular schwannoma from March 1, 2010 to December 31, 2021 who completed at least one Penn Acoustic Neuroma Quality of Life (PANQOL) questionnaire at presentation or prior to any intervention were included. PANQOL scores were compared across income quintiles, racial groups, and health insurance categories. RESULTS: Two-hundred and ninety-six patients who had non-missing information on variables of interest were included. Compared to White/Caucasian patients (84.5 %), Black/African American patients (4.7 %) had significantly lower PANQOL total scores (b = -12.8[-21.7, -4.0], p = 0.005). Compared to patients with Commercial insurance (53 %), patients who were Uninsured/ Self-pay (1.7 %) had significantly lower PANQOL total scores (b = -16.7[-31.4, -1.9], p = 0.027). Patients in higher income quintiles had significantly higher PANQOL total scores (b = 11.7[3.9, 19.5], p = 0.004 comparing highest income quintile to lowest). After controlling for potential confounders, income quintile (b = 9.6[1.3, 17.9], p = 0.023 comparing highest quintile to lowest) and insurance (b = -17.0[-31.9, -21], p = 0.025 comparing Uninsured/Self-pay to Commercial insurance) remained predictors of total PANQOL score. CONCLUSIONS: Socioeconomic factors including race, health insurance, and income appear to contribute to quality of life at the time of vestibular schwannoma diagnosis. These variables are interrelated and the effects of race may be mediated in part by differences in income and health insurance coverage.

Life Expectancy After Diagnosis of a Vestibular Schwannoma in Patients 70 Years and Older.

Importance: Over the past decades, the number of patients, especially in the older adult patient group, diagnosed with vestibular schwannoma (VS) has increased. Assuming that older adult patients have more comorbidities, a longer recovery period after surgery, a higher rate of surgical complications, and a higher mortality rate after VS surgery, a treatment strategy for this group of patients is warranted, based on clinical evidence on postsurgical survival. Objective: To evaluate the survival after diagnosis of a VS in patients 70 years and older, treated with either observation or surgery, and to compare these findings with the life span of an age-matched background population in Denmark. Design, Setting, and Participants: This was a retrospective cohort study of 624 patients 70 years and older diagnosed with VS in Denmark from 1976 to 2016. Since 1976, all patients with a VS have been registered in a national database, which contains 3637 patients. Of the included patients in this study, 477 were treated conservatively with the "wait-and-scan" strategy, and 147 were treated surgically with removal of the tumor. The survival of the patients was compared with a matched background population in Denmark. Data analysis was performed from January 1976 to January 2017. Exposures: Surgery, radiotherapy, or none. Main Outcomes and Measures: The main outcome was survival among the patients and compared with the matched background population. Results: A total of 624 patients were included (317 female patients [50.8%] and 307 male patients [49.2%]). The mean (SD) survival in the observed patients was 9.2 (4.7) years after diagnosis, whereas for the background population, the expected survival was 11 years from the mean age at diagnosis. For the surgically treated patients, the mean (SD) survival was 11.8 (6.6) years, and expected survival was 11 years for the matched background population. The mean (SD) survival was 10.7 (5.5) years in female patients and 8.9 (5.0) years in male patients. There was no significant difference in survival between treatment modalities, irrespective of tumor size. Conclusions and Relevance: In this cohort study, survival after diagnosis of a VS in patients 70 years and older was similar in the surgical group compared with the age-matched background population. In the wait-and-scan group, the survival after diagnosis was marginally shorter, which may be associated with increased comorbidity.

[Vestibular Schwannoma: Factors in Therapy Decision-Making]. / Vestibularisschwannom: Faktoren bei der Therapieentscheidung.

The treatment of vestibular schwannomas (VS) has always posed a challenge for physicians. Three essential treatment principles are available: wait-and-scan, surgery, and stereotactic radiotherapy. In addition to the type of treatment, decisions must be made regarding the optimal timing of therapy, the combination of different treatment modalities, the potential surgical approach, and the type and intensity of radiation. Factors influencing the therapy decision include tumor location and size or stage, patient age, comorbidities, symptoms, postoperative hearing rehabilitation options, patient preferences, and, not least, the experience of the surgeons and the personnel and technical capabilities of the clinical site. This article begins with a brief overview of vestibular schwannomas, then outlines the fundamental interdisciplinary treatment options, and finally discusses the ENT (ear, nose, and throat)-relevant factors in the therapy decision.

Response to OKN-007 and NAC in a Patient with Unilateral Hearing Loss and Chronic Tinnitus from Vestibular Schwannoma.

BACKGROUND: Tinnitus is the perception of sound in the absence of external acoustic stimulation. Being one of the most common diseases of the ear, it has a global prevalence ranging from 4.1 to 37.2%. To date, it has been difficult to treat tinnitus as its pathophysiology is poorly understood and there are limited treatment options. OBJECTIVE: To investigate the effect of OKN-007 (also known as HPN-07), a nitrone-based investigational drug, in combination with oral N-acetylcycsteine (NAC), for the treatment of hearing loss and chronic tinnitus under an individual expanded access protocol. PATIENT CASE: We report the case of a patient who presented with left-sided ear fullness, mild tinnitus, and mild high frequency sensorineural hearing loss with 100% word recognition. A large enhancing mass seen on MRI revealed a vestibular schwannoma. He underwent subtotal resection of the tumor resulting in a moderate-to-profound sensorineural hearing loss and catastrophic tinnitus. The patient was treated with intravenous OKN-007 at 60 mg/kg dosed three times per week and oral NAC 2500 mg twice daily. RESULTS: Post-treatment audiometric testing revealed an average of 16.66 dB in hearing threshold improvement in three frequencies (125, 250 and 500 Hz) with residual hearing in the affected left ear. His tinnitus loudness matching improved from 90 dB to 19 dB post-treatment. His Tinnitus Handicap Inventory improved from 86/100 (Catastrophic) to 40/100 (Moderate). He also experienced improvements in sleep, concentration, hearing, and emotional well-being, and reported significantly decreased levels of tinnitusrelated distress. CONCLUSIONS: This case report highlights the feasibility and therapeutic potential of the combination of OKN-007 and NAC in treating hearing loss and tinnitus that warrants further investigation.

Sudden Sensorineural Hearing Loss and Facial Palsy in Patients with Vestibular Schwannoma Based on the Population Data of Korea.

BACKGROUND: The prevalence of sudden sensorineural hearing loss and facial palsy in patients with vestibular schwannoma and the association of sudden sensorineural hearing loss or facial palsy with vestibular schwannoma were investigated based on the population data of Korea. METHODS: This retrospective study used the Korean National Health Insurance Service data. Patients with vestibular schwannoma and those with a previous history of sudden sensorineural hearing loss or facial palsy were identified based on diagnostic, medication, magnetic resonance imaging, or audiometric codes from 2005 to 2020. The control group was established with propensity score matching. The risk for vestibular schwannoma in patients with a previous history of sudden sensorineural hearing loss or facial palsy was analyzed. RESULTS: There were 5751 patients in the vestibular schwannoma group and 23004 in the control group. The rate of patients with a previous history of sudden sensorineural hearing loss in the vestibular schwannoma group (25.8%) was significantly higher than in the control group (P -lt; .0001), as was the rate of patients with a previous history of facial palsy in the vestibular schwannoma group (4.7%) (P -lt; .0001). Previous history of sudden sensorineural hearing loss was a significant risk factor for vestibular schwannoma (hazard ratio=7.109, 95% confidence interval=6.696-7.547). Previous history of facial palsy was also a significant risk factor for vestibular schwannoma (hazard ratio=3.048, 95% confidence interval=2.695-3.447). CONCLUSION: The prevalence of sudden sensorineural hearing loss or facial palsy was significantly higher in patients with vestibular schwannoma than in those without vestibular schwannoma. Based on the population data of Korea, sudden sensorineural hearing loss and facial palsy were significant risk factors for vestibular schwannoma.

Cerebellopontine angle tumor presenting as acute audiovestibular syndrome.

BACKGROUND: Acute audiovestibular deficits may be a harbinger of vestibular schwannoma (VS). OBJECTIVE: To investigate clinical and laboratory features of 25 consecutive patients with VS presenting with acute audiovestibular deficits. METHODS: A symptomatic combination of acute audiovestibular deficits was investigated. Audiometric and vestibular function tests, and internal auditory canal magnetic resonance imaging (IAC MRI) results were evaluated. RESULTS: Varying combinations of symptoms may develop in VS patients with acute audiovestibular deficits, of whom sudden hearing loss (HL) without acute vertigo or acute facial nerve palsy (FNP) was most common. The most common audiometric configuration was high-tone hearing loss, and no patient showed low-tone hearing loss. IAC MRI demonstrated that the tumor had an intracanalicular portion and attachment to the bony IAC wall in all patients and widened the IAC wall in some patients. CONCLUSION: Different symptomatic combinations of acute audiovestibular deficits may develop in patients with VS. Awareness about the possibility of VS as a cause of sudden HL, acute vertigo, and acute FNP, as well as subsequent IAC MRI scanning is vital to earlier diagnosis of VS in these patients.

Identification of immune-related candidate biomarkers in plasma of patients with sporadic vestibular schwannoma.

Vestibular schwannoma (VS) is an intracranial tumor arising from neoplastic Schwann cells and typically presenting with hearing loss. The traditional belief that hearing deficit is caused by physical expansion of the VS, compressing the auditory nerve, does not explain the common clinical finding that patients with small tumors can have profound hearing loss, suggesting that tumor-secreted factors could influence hearing ability in VS patients. We conducted profiling of patients' plasma for 66 immune-related factors in patients with sporadic VS (N > 170) and identified and validated candidate biomarkers associated with tumor size (S100B) and hearing (MCP-3). We further identified a nine-biomarker panel (TNR-R2, MIF, CD30, MCP-3, IL-2R, BLC, TWEAK, eotaxin, and S100B) with outstanding discriminatory ability for VS. These findings revealed possible therapeutic targets for VS, providing a unique diagnostic tool that may predict hearing change and tumor growth in VS patients, and may inform the timing of tumor resection to preserve hearing.

Bilateral intracochlear schwannomas: histopathological confirmation and outcomes following tumour removal and cochlear implantation with lateral wall electrodes.

Intracochlear schwannomas (ICS) are very rare benign tumours of the inner ear. We present histopathological proof of the extremely rare bilateral occurrence of intracochlear schwannomas with negative blood genetic testing for neurofibromatosis type 2 (NF2). Bilateral schwannomas are typically associated with the condition NF2 and this case is presumed to have either mosaicism for NF2 or sporadic development of bilateral tumours. For progressive bilateral tumour growth and associated profound hearing loss, surgical intervention via partial cochleoectomy, tumour removal, preservation of the modiolus, and simultaneous cochlear implantation with lateral wall electrode carrier with basal double electrode contacts was performed. The right side was operated on first with a 14-month gap between each side. The hearing in aided speech recognition for consonant-nucleus-consonant (CNC) phonemes in quiet improved from 57% to 83% 12 months after bilateral cochlear implantation (CI). Bilateral intracochlear schwannomas in non-NF2 patients are extremely rare but should be considered in cases of progressive bilateral hearing loss. Successful tumour removal and cochlear implantation utilizing a lateral wall electrode is possible and can achieve good hearing outcomes.

Evaluation of Asymmetries in Speech-in Noise Abilities in Audiologic Screening for Vestibular Schwannoma.

OBJECTIVES: Measures of speech-in-noise, such as the QuickSIN, are increasingly common tests of speech perception in audiologic practice. However, the effect of vestibular schwannoma (VS) on speech-in-noise abilities is unclear. Here, we compare the predictive ability of interaural QuickSIN asymmetry for detecting VS against other measures of audiologic asymmetry. METHODS: A retrospective review of patients in our institution who received QuickSIN testing in addition to a regular audiologic battery between September 2015 and February 2019 was conducted. Records for patients with radiographically confirmed, unilateral, pretreatment VSs were identified. The remaining records excluding conductive pathologies were used as controls. The predictive abilities of various measures of audiologic asymmetry to detect VS were statistically compared. RESULTS: Our search yielded 73 unique VS patients and 2423 controls. Receiver operating characteristic curve analysis showed that QuickSIN asymmetry was more sensitive and specific than pure-tone average asymmetry and word-recognition-in-quiet asymmetry for detecting VS. Multiple logistic regression analysis revealed that QuickSIN asymmetry was more predictive of VS (odds ratio [OR] = 1.23, 95% confidence interval [CI] [1.10, 1.38], p < 0.001) than pure-tone average asymmetry (OR = 1.04, 95% CI [1.00, 1.07], p = 0.025) and word-recognition-in-quiet asymmetry (OR = 1.03, 95% CI [0.99, 1.06], p = 0.064). CONCLUSION: Between-ear asymmetries in the QuickSIN appear to be more efficient than traditional measures of audiologic asymmetry for identifying patients with VS. These results suggest that speech-in noise testing could be integrated into clinical practice without hindering the ability to identify retrocochlear pathology.

Incidence and presentation of vestibular schwannoma: a 3-year cohort registry study.

BACKGROUND: Vestibular schwannoma (VS) is the most common benign tumour arising in the lateral skull base. Reported incidence rates of VS vary across geographical locations and over time. There is scarce updated evidence over the past decade on the epidemiology and mode of presentation of VS. OBJECTIVE: To describe the epidemiology and mode of presentation of VS in the East of England between 2013 and 2016. METHODS: A retrospective epidemiological analysis of data from a national VS registry and electronic patient records was conducted, including all newly diagnosed adult patients in a UK tertiary referral centre, between April 1st, 2013, and March 31st, 2016. RESULTS: There were 391 new cases identified resulting in an overall mean incidence of 2.2 VS cases per 100,000 person-year. The incidence rate for all patients in the <40 age group ranged between 0.3 and 0.7 per 100,000 person-year, increasing to a range of 5.7 to 6.1 per 100,000 person-year in the 60-69 age group. The top three combinations of symptoms on presentation per patient were hearing loss and tinnitus (97, 24.8%), hearing loss alone (79, 20.2%) and hearing loss, tinnitus, and balance symptoms (61, 15.6%). The median duration of symptoms was 12 months, with a wide range from 1.4 to 300 months. Age was negatively correlated with tumour size (r = -0.14 [-0.24 to -0.04], p=0.01) and positively correlated with symptom duration (r = 0.16 [0.03-0.29], p=0.02). CONCLUSIONS: The incidence of vestibular schwannoma has increased compared to previous studies in the UK and is similar to incidence rates reported in other countries during the past decade. It peaks in the seventh decade of life, mainly because of an increase in the diagnosis of small tumours with a long duration of audio-vestibular symptoms in older patients, compared to earlier studies.

A Bilateral Vestibular Schwannoma is Not Always Related to Neurofibromatosis Type 2.

Bilateral vestibular schwannomas are commonly diagnosed in patients affected by neurofibromatosis type 2, a genetic disease caused by a heterozygous mutation in the gene region encoding neurofibromin-2. Sporadic bilateral vestibular schwannomas are very rare entities affecting almost exclusively elderly people. We present the case of a senior woman who was followed up with the "wait-and-scan" strategy for a unilateral vestibular schwannoma that later developed as a contralateral tumor, compatible with vestibular schwannoma, raising questions about its nature and risk of having been transmitted in offspring. Genetic testing excluded mutations of the neurofibromatosis type 2 gene. The presence of bilateral vestibular schwannomas is often considered pathognomonic of neurofibromatosis type 2, but the estimated probability of sporadic bilateral tumors in the absence of other neurofibromatosis type 2 features is 50% over 70 years of age. Therefore, the NF2 gene assessment is in any case recommended in these patients not only for an evaluation of the risk of being transmitted. The treatment strategy should be carefully personalized for each patient, considering the size of the tumors, symptoms, and hearing function together with the patient's age.

Diagnosis of vestibular schwannoma following an 11-year interval of head and neck manifestations: case report.

Vestibular schwannoma (VS) is a benign peripheral nerve sheath tumor involving the vestibulocochlear nerve. Affected patients typically experience a gradual emergence of episodic imbalance and unilateral hearing loss, tinnitus, and headache. Less often, VS may be associated with facial pain; ocular, otic, and taste disturbance; paresthesia of the tongue and face; and temporomandibular disorder-like presentations. There is limited information in the dental literature relating the myriad of oral and maxillofacial manifestations of VS. The objective of this article is to underscore the importance for dental clinicians to seek clinicopathologic correlations with VS-related symptomatology, potentially resulting in a timelier diagnosis and improved patient outcomes. To convey this clinical challenge, a detailed narrative of a 45-year-old patient with an 11-year delay in diagnosis has been reported. In addition, the typical radiographic appearance of an implanted cranial device placed following VS resection has been provided.

Vestibular Schwannoma Stereotactic Radiosurgery in Octogenarians: Case Series.

BACKGROUND AND OBJECTIVES: The management of octogenarians with vestibular schwannomas (VS) has received little attention. However, with the increase in octogenarian population, more effort is needed to clarify the value of stereotactic radiosurgery (SRS) in this population. The aim of this study was to evaluate the safety and efficacy of SRS in this patient age group. METHODS: A retrospective study of 62 patients aged 80 years or older who underwent single-session SRS for symptomatic VS during a 35-year interval was performed. The median patient age was 82 years, and 61.3% were male. SRS was performed as planned adjuvant management or for delayed progression after prior partial resection in 5 patients. RESULTS: SRS resulted in a 5-year tumor control rate of 95.6% with a 4.8% risk of adverse radiation effects (ARE). Tumor control was unrelated to patient age, tumor volume, Koos grade, sex, SRS margin dose, or prior surgical management. Four patients underwent additional management including 1 patient with symptomatic progression requiring surgical resection, 2 patients with symptomatic hydrocephalus requiring cerebrospinal fluid diversion, and 1 patient whose tumor-related cyst required delayed cyst aspiration. Three patients developed ARE, including 1 patient with permanent facial weakness (House-Brackmann grade II), 1 who developed trigeminal neuropathy, and 1 who had worsening gait disorder. Six patients had serviceable hearing preservation before SRS, and 2 maintained serviceable hearing preservation after 4 years. A total of 44 (71%) patients died at an interval ranging from 6 to 244 months after SRS. CONCLUSION: SRS resulted in tumor and symptom control in most octogenarian patients with VS.

Patient-preferred outcomes in patients with vestibular schwannoma: a qualitative content analysis of symptoms, side effects and their impact on health-related quality of life.

PURPOSE: During counseling and management of patients with vestibular schwannoma (VS), the emphasis is shifting from tumour control and nerve preservation towards maintaining or improving health-related quality of life (HRQoL). Understanding the patients' perspective and impact of VS is, therefore, of utmost importance. The current study aimed to identify treatment outcomes preferred by patients and to explore the patient-reported VS symptoms and management-related side effects and their impact on HRQoL. METHODS: Patients with VS were contacted through the Dutch VS association Stichting Hoormij and questioned using a semi-structured, cross-sectional online survey. Patients were asked to report and rank symptoms and side effects, with their impact on HRQoL and frequency of occurrence. Results were structured through qualitative content analysis. Coded symptoms, side effects, impacts, frequencies, and patient-preferred outcomes were analysed and summarized with descriptive statistics. RESULTS: Of the 231 respondents, 71% were actively treated. Hearing (symptoms vs. side effects: 78.8% vs. 63.6%), balance (62.3%; 48.8%), and energy issues (33.8%; 32.6%) were the most frequently mentioned symptoms and management-related side effects. Fatigue, deafness, headaches, and hearing loss had the highest impact on HRQoL. The majority of patients identified hearing preservation (61%), balance preservation (38.5%), and reduced tinnitus (34.6%) to be the patient-preferred outcomes. CONCLUSION: This qualitative study demonstrates that in this population many patients with VS encounter participation difficulties in their daily physical and social activities and value hearing and balance preservation, reduced tinnitus, and restored energy as preferred outcomes as they are hampered by symptoms and side effects related to hearing, balance, and energy. Healthcare professionals should consider these key points and use these and the patient-preferred outcomes in consultation, shared decision making, treatment, and follow-up to optimize patient-centred care.

CLINICAL-AND-FUNCTIONAL ASSESSMENT OF THE EARLY POSTOPERATIVE OUTCOME OF SURGICAL TREATMENT OF PATIENTS WITH VESTIBULAR SCHWANNOMA.

The incidence of vestibular schwannoma (VS) increased largely within the last 50 years from 1,5 cases per 100 thousand persons to 4,2 (for the last decade). The approaches to the management of VS patients vary significantly in different medical centers and different countries. The search for the consensus in selecting strategy of VS treatment based on systemic clinical-and-functional assessment of treatment outcome is topical nowadays. The aim of study - to analyze the clinical-and-functional early postoperative outcome of the surgical treatment of vestibular schwannoma depending on the stage of the disease. The findings of the examination and the outcomes of the surgical treatment of 27 VS patients were retrospectively analyzed. The patients were treated at the Department of Subtentorial Neurosurgery of the State Institution "Romodanov Institute of Neurosurgery of the NAMS of Ukraine" in 2018-2019. According to Koos classification, three groups of the patients were delineated for the analysis of the results of the study, namely, group 1 (Koos II) - 8 (29,6 %) patients; group 2 (Koos III) - 6 (22,2 %); and group 3 (Koos IV) - 13 (48,2 %). The complex clinical examination, in particular clinical-and-instrumental otoneurological examination and the evaluation of the neurological status according to the Scale for the assessment of the functional treatment outcome were performed preoperatively and early postoperatively. The data were statistically processed. In the patients with small tumors (group 1, Koos II), the socially useful hearing on the affected side was preserved preoperatively necessitating the caution for selecting the treatment strategy in these patients. When pre- and postoperative clinical symptoms were compared in group 1, the statistically significant worsening of the hearing to the socially non-useful, the unilateral subjective tinnitus, the dysfunction of the facial nerve, the decreased sense of taste/loss of taste on the anterior 2/3 of the tongue on the affected side were found. Upon the surgical treatment, the rate of the neurological deficit increased, and the severity grade of the neurological deficit increased by about 10 points. The overall preoperative score in group 3 (Koos IV) was significantly different from that in other groups. The progression of the disease to the stage of Koos IV results in the neurological deficit that is equivalent by the set of the neurological symptoms and their severity to that in early postoperative period in patients with Koos III. In group 3, the rate of the dysfunction of the facial nerve and the caudal group of the cranial nerves increased postoperatively with concomitant decreased sense of taste/loss of taste on the anterior 2/3 of the tongue on the affected side and statocoordinatory impairments. The overall preoperative score differed significantly between all groups. In group 3, the postoperative overall score did not differ from preoperative one, although the postoperative overall score in group 3 (Koos ІV) differed significantly from that in other two groups. The proposed scale for the assessment of the functional outcome of the VS treatment is versatile and represents the integral element of the systemic assessment of the clinical-and-functional status of VS patient. There are good reasons to integrate proposed scale into the general scheme of the medical care for VS patients allowing for the objective assessment of the otoneurological patterns in the patients in the dynamics of the treatment. The analysis of our own findings and the literature data proved the relevance of the problem requiring further task-oriented scientific inquiry. The important aspects of the problem relate to the optimization and improvement of the diagnostic and treatment strategy according to the principles of individualization and multimodality allowing for increasing the level of consensus and improving the functional outcome of the treatment.

Increased Labyrinthine T1 Postgadolinium Signal Intensity Is Associated with the Degree of Ipsilateral Sensorineural Hearing Loss in Patients with Sporadic Vestibular Schwannoma.

BACKGROUND AND PURPOSE: Vestibular schwannomas are benign, generally slow-growing tumors, commonly presenting with hearing loss. Alterations in the labyrinthine signal are seen in patients with vestibular schwannoma; however, the association between imaging abnormalities and hearing function remains poorly defined. The purpose of this study was to determine whether labyrinthine signal intensity is associated with hearing in patients with sporadic vestibular schwannoma. MATERIALS AND METHODS: This was an institutional review board-approved retrospective review of patients from a prospectively maintained vestibular schwannoma registry imaged in 2003-2017. Signal-intensity ratios of the ipsilateral labyrinth were obtained using T1, T2-FLAIR, and postgadolinium T1 sequences. Signal-intensity ratios were compared with tumor volume and audiometric hearing threshold data including pure tone average, word recognition score, and American Academy of Otolaryngology-Head and Neck Surgery hearing class. RESULTS: One hundred ninety-five patients were analyzed. Ipsilateral labyrinthine signal intensity including postgadolinium T1 images was positively correlated with tumor volume (correlation coefficient = 0.17, P = .02). Among signal-intensity ratios, postgadolinium T1 was significantly positively associated with pure tone average (correlation coefficient = 0.28, P < .001) and negatively associated with the word recognition score (correlation coefficient = -0.21, P = .003). Overall, this result correlated with impaired American Academy of Otolaryngology-Head and Neck Surgery hearing class (P = .04). Multivariable analysis suggested persistent associations independent of tumor volume with pure tone average (correlation coefficient = 0.25, P < .001) and the word recognition score (correlation coefficient = -0.17, P = .02) but not hearing class (P = .14). No consistent significant associations were noted between noncontrast T1 and T2-FLAIR signal intensities and audiometric testing. CONCLUSIONS: Increased ipsilateral labyrinthine postgadolinium signal intensity is associated with hearing loss in patients with vestibular schwannoma.

Long-Term Hearing Outcome After Radiosurgery for Vestibular Schwannoma: A Systematic Review and Meta-Analysis.

BACKGROUND: Stereotactic radiosurgery (SRS) is one of the main treatment options in the management of small to medium size vestibular schwannomas (VSs), because of high tumor control rate and low cranial nerves morbidity. Series reporting long-term hearing outcome (>3 years) are scarce. OBJECTIVE: To perform a systematic review of the literature and meta-analysis, with the aim of focusing on long-term hearing preservation after SRS. METHODS: Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we reviewed articles published between January 1990 and October 2020 and referenced in PubMed or Embase. Inclusion criteria were peer-reviewed clinical study or case series of VSs treated with SRS (single dose), reporting hearing outcome after SRS with a median or mean audiometric follow-up of at least 5 years. Hearing preservation, cranial nerves outcomes, and tumor control were evaluated. RESULTS: Twenty-three studies were included. Hearing preservation was found in 59.4% of cases (median follow-up 6.7 years, 1409 patients). Main favorable prognostic factors were young age, good hearing status, early treatment after diagnosis, small tumor volume, low marginal irradiation dose, and maximal dose to the cochlea. Tumor control was achieved in 96.1%. Facial nerve deficit and trigeminal neuropathy were found in 1.3% and 3.2% of patients, respectively, both significantly higher in Linear Accelerator series than Gamma Knife series ( P < .05). CONCLUSION: Long-term hearing preservation remains one of the main issues after SRS, with a major impact on health-related quality of life. Our meta-analysis suggests that hearing preservation can be achieved in almost 60% of patients after a median follow-up of 6.7 years, irrespective of the technique.

Intralabyrinthine schwannomas: a two-case series and literature review with a focus on hearing rehabilitation.

PURPOSE: Intralabyrinthine schwannomas (ILSs) are an uncommon finding. Diagnosis is challenging and no gold standard treatment exists yet. In this article, we present a two-cases series and review the latest available literature to assess the best diagnostic and therapeutic scheme. METHODS: We reviewed the latest available literature assessing most frequent and relevant sets of symptoms, clinical features of the disease, diagnostic tests and imaging, possible treatments and after-surgery hearing rehabilitation techniques. We then compared literature data to our own series ones. RESULTS: ILSs clinical presentation and development may overlap with other, more common otological conditions. Full audiometric battery test, electrophysiological study of VEMPS and MRI with contrast enhancement all appear to be critical to correctly diagnose these tumors. Several treatments exist: radiological follow-up, radiation therapy, full or partial surgical excision. Hearing rehabilitation is mostly accomplished through simultaneous cochlear implantation. CONCLUSIONS: Our case-series data matches the available literature. ILSs are a rare type of vestibular schwannomas. Diagnosis in challenging and delayed in time as all the diagnostic tests, yet sensitive, are not specific for ILSs. The most suitable treatment seems to be surgical excision of these tumors followed by simultaneous cochlear implantation to restore hearing.