[Neurofibromatosis type 2 in the otorhinolaryngological practice]. / Neirofibromatoz 2-go tipa v praktike vracha-otorinolaringologa.

Neurofibromatosis type 2 (NF2) is a rare autosomal dominant disease (frequency 1 in 25-90 000) characterized by the formation of tumors of the central nervous system due to a mutation in the NF2 gene on chromosome 22q12. Bilateral vestibular schwannomas are recognized as absolute diagnostic criteria of NF2 and occur in 95% of patients, are accompanied by hearing impairment, manifest at the age of 18-24 years. Skin manifestations can precede vestibular schwannomas for several years and predict the course of the disease: neurofibromas, cafe-au-lait macules, hypopigmented spots, recently described mesh capillary malformations. Despite the benign nature of schwannomas, they can lead to hearing loss, vestibular dysfunction, facial nerve paralysis, gait disorders, pain and convulsions, there is a risk of early death from compression of the brain stem. The probability of progressive hearing loss is partly determined by the type of mutation. We described a clinical case of NF2 in a 21-year-old patient with bilateral vestibular schwannomas without hearing loss, whose skin examination by ENT specialist revealed this disease. The importance of the presented observation is that the doctor should assume neurofibromatosis type 2 in a young patient with bilateral vestibular schwannomas. It is necessary to undertake a further examination of this patient, including: skin examination for the identification of characteristic neurofibromas and cafe-au-lait macules, consultation with an ophthalmologist, neurologist, MRI of the brain and spinal cord with contrast, genetic analysis - for timely initiation of therapy that prevents hearing loss and vestibular disorders.

Transmastoid Approach to Repair Iatrogenic Cerebrospinal Fluid Otorhinorrhea After Cerebellopontine Angle Tumor Resection.

BACKGROUND: The precise treatment of iatrogenic cerebrospinal fluid (CSF) otorhinorrhea has been poorly studied. The purpose of the study was to investigate the clinical manifestation, surgical results, and management of CSF leak. METHODS: Electronic medical record database of iatrogenic CSF leaks after erebellopontine angle(CPA) surgery from 2019 to 2022 was retrospectively analyzed. Three patients returned to the hospital with the complication of CSF leak. After failed attempts of conservative strategies or reverse surgical repair, adipose tissue was applied to the mastoid cracks repair. RESULTS: With the techniques described above, the CSF leaks were successfully settled. The identified patients were observed for at least 10 months. and there was no recurrence or other complications. CONCLUSION: Conservative treatment and initial surgical methods for occult postoperative CSF leaks are prone to delay effective results, particularly in patients with well-evaporated temporal bone. This complication can be minimized with transmastoid closure utilizing autologous fat.

Salvage Microsurgery Following Failed Primary Radiosurgery in Sporadic Vestibular Schwannoma.

Importance: Management of sporadic vestibular schwannoma with radiosurgery is becoming increasingly common globally; however, limited data currently characterize patient outcomes in the setting of microsurgical salvage for radiosurgical failure. Objective: To describe the clinical outcomes of salvage microsurgery following failed primary stereotactic radiosurgery (SRS) or fractionated stereotactic radiotherapy (FSRT) among patients with sporadic vestibular schwannoma. Design, Setting, and Participants: This was a cohort study of adults (≥18 years old) with sporadic vestibular schwannoma who underwent salvage microsurgery following failed primary SRS/FSRT in 7 vestibular schwannoma treatment centers across the US and Norway. Data collection was performed between July 2022 and January 2023, with data analysis performed between January and July 2023. Exposure: Salvage microsurgical tumor resection. Main Outcomes and Measures: Composite outcome of undergoing less than gross total resection (GTR) or experiencing long-term facial paresis. Results: Among 126 patients, the median (IQR) age at time of salvage microsurgery was 62 (53-70) years, 69 (55%) were female, and 113 of 117 (97%) had tumors that extended into the cerebellopontine angle at time of salvage. Of 125 patients, 96 (76%) underwent primary gamma knife SRS, while 24 (19%) underwent linear accelerator-based SRS; the remaining patients underwent FSRT using other modalities. Postoperative cerebrospinal fluid leak was seen in 15 of 126 patients (12%), hydrocephalus in 8 (6%), symptomatic stroke in 7 (6%), and meningitis in 2 (2%). Each 1-mm increase in cerebellopontine angle tumor size was associated with a 13% increased likelihood of foregoing GTR (64 of 102 patients [63%]) or long-term postoperative House-Brackmann grade higher than I (48 of 102 patients [47%]) (odds ratio, 1.13; 95% CI, 1.04-1.23). Following salvage microsurgery, tumor growth-free survival rates at 1, 3, and 5 years were 97% (95% CI, 94%-100%), 93% (95% CI, 87%-99%), and 91% (95% CI, 84%-98%), respectively. Conclusions: In this cohort study, more than half of patients who received salvage microsurgery following primary SRS/FSRT underwent less than GTR or experienced some degree of facial paresis long term. These data suggest that the cumulative risk of developing facial paresis following primary SRS/FSRT by the end of the patient's journey with treatment approximates 2.5% to 7.5% when using published primary SRS/FSRT long-term tumor control rates.

Navigating Uncharted Waters: Comparative Analysis of Clinical Progression and Outcomes in Vestibular Schwannoma Patients with Papilledema and without Hydrocephalus, Versus Those without Papilledema and Hydrocephalus: A Comprehensive Institutional Insight.

BACKGROUND: Papilledema's association with hydrocephalus (HCP)-linked larger vestibular schwannoma (VS) is established but cases lacking concurrent HCP require further investigation. METHODS: This retrospective comparative observational study, conducted from July 2018 to July 2023, examined 120 VS patients undergoing surgery. Patients were categorized into Group 1 (papilledema without HCP) and Group 2 (no papilledema or HCP), with comprehensive data analyzed. RESULTS: In this study, Group 1 (14 patients with papilledema) and Group 2 (106 patients without papilledema or HCP) were compared. Group 1 was younger (mean age 27.21 ± 11.73 years) than Group 2 (mean age 54.66 ± 11.44 years). Both groups had similar symptom durations and tumor detection times. Group 1 had increased vascularity (P = 0.001), elevated cisterna magna protein levels (P = 0.001), and a higher incidence of neurofibromatosis 2 (P = 0.003). They also experienced longer surgeries (P = 0.001) and more blood loss (P = 0.001), leading to extended postoperative complications. Group 2 showed improved postsurgery visual outcomes (P = 0.001), better Glasgow Outcome Scores (P = 0.001), enhanced facial nerve preservation (P = 0.002), and improved hearing on follow-up (P = 0.003). Logistic regression analysis highlighted prolonged surgery duration (P = 0.057) and papilledema (P = 0.0001) as significant factors influencing visual improvement. CONCLUSIONS: Patients with VS require preoperative fundoscopy evaluation due to potential visual loss and papilledema, even without HCP. Early treatment initiation enhances visual and hearing outcomes. Meticulous surgery is vital given the lesion's hypervascular nature and adherence to surrounding structures. Preoperative embolization may aid in preserving neurovascular structures. In developing countries with higher blindness rates, judicious noncontrast computed tomography brain evaluation is crucial for timely detection and treatment initiation of lesions like VS.

Vestibular Decompensation Following COVID-19 Infection in a Person With Compensated Unilateral Vestibular Loss: A Rehabilitation Case Study.

BACKGROUND AND PURPOSE: Surgical removal of a vestibular schwannoma (vestibular schwannoma resection; VSR) results in a unilateral vestibular hypofunction with complaints of dizziness and imbalance. Although the anatomic lesion is permanent, recovery of balance and diminution of dizziness occurs through central neurophysiologic compensation. Compensation of the system is maintained through daily activity. Unfortunately, interruption of stimulus, such as decreased activities due to illness, can cause decompensation. Decompensation is described as the return of symptoms consistent with that experienced during the initial insult/injury (eg, dizziness, oscillopsia, balance difficulty). This case study describes a reoccurrence of vestibular dysfunction in a person with a history of VSR following hospitalization and protracted recovery from a COVID-19 infection. It further documents her recovery that may be a result of vestibular rehabilitation. CASE DESCRIPTION: A 49-year-old woman (M.W.) with a surgical history of VSR (10 years prior) and a medical history of significant COVID-19 infection, resulting in an intensive care unit stay and prolonged use of supplemental oxygen, presented to physical therapy with persistent dizziness and imbalance. The video head impulse test confirmed unilateral vestibular hypofunction. INTERVENTION: M.W. attended biweekly vestibular rehabilitation for 6 weeks and completed daily home exercises. OUTCOMES: At discharge, M.W. demonstrated improvements in patient-reported outcomes (Dizziness Handicap Inventory), functional testing (MiniBEST, 2-Minute Walk Test), and gaze stability measures (video head impulse testing, dynamic visual acuity). DISCUSSION: Vestibular decompensation preluded by a COVID-19 infection caused a significant decrease in functional mobility. Vestibular rehabilitation targeted at gaze and postural stability effectively reduced symptoms and facilitated recovery to M.W.'s pre-COVID-19 level of function. Video Abstract available for more insights from the authors (see the Video, Supplemental Digital Content 1 available at: http://links.lww.com/JNPT/A458 ).

Does Location of Intralabyrinthine Vestibular Schwannoma Determine Objective and Subjective Vestibular Function?

BACKGROUND AND OBJECTIVE: The incidence of intralabyrinthine schwannomas is increasing, and a growing attention is given to the detrimental effects on hearing function. On the contrary, the vestibular profile of intralabyrinthine vestibular schwannomas (VSs) is still not well understood. We aimed to investigate and report the observed relationships between the intralabyrinthine location of the schwannomas and objective and subjective vestibular profile of the patients. METHODS: Retrospective cohort study of 20 consecutive individuals with sporadic intralabyrinthine schwannomas and grouped according to the intralabyrinthine location of the schwannomas. Vestibular testing consisted of the video head impulse test of all three semicircular canals, the caloric test, cervical and ocular vestibular evoked myogenic potentials, and the dizziness handicap inventory. A nonparametric unpaired t test was performed to compare groups, and Fisher's exact test was used for categorical data. RESULTS: The median video head impulse test gains (lateral, anterior, posterior) were 0.40, 0.50, and 0.75 for intravestibular schwannomas and 0.93, 1.52, and 0.91 for intracochlear schwannomas ( p = 0.0001, p = 0.009, p = 0.33), respectively. Caloric unilateral weakness had a median of 100% for intravestibular schwannomas and 14% for intracochlear schwannomas ( p = 0.0001). The mean dizziness handicap inventory was 21 for intravestibular schwannomas and 1 for cochlear schwannomas ( p = 0.02). There were no significant differences in vestibular evoked myogenic potentials according to intralabyrinthine location. CONCLUSION: By both objective and subjective measures, intralabyrinthine schwannomas with an intravestibular component has significantly worse vestibular function than schwannomas with purely cochlear involvement.

Audiovestibular Findings in Patients with Concurrent Superior Canal Dehiscence and Vestibular Schwannoma.

OBJECTIVE: To describe the clinical-instrumental findings in case of concurrent superior canal dehiscence (SCD) and ipsilateral vestibular schwannoma (VS), aiming to highlight the importance of an extensive instrumental assessment to achieve a correct diagnosis. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Five patients with concurrent SCD and VS. INTERVENTION: Clinical-instrumental assessment and imaging. MAIN OUTCOME MEASURE: Clinical presentation, audiovestibular findings, and imaging. RESULTS: The chief complaints were hearing loss (HL) and unsteadiness (80%). Other main symptoms included tinnitus (60%) and pressure-induced vertigo (40%). Mixed-HL was identified in three patients and pure sensorineural-HL in 1, including a roll-over curve in speech-audiometry in two cases. Vibration-induced nystagmus was elicited in all cases, whereas vestibular-evoked myogenic potentials showed reduced thresholds and enhanced amplitudes on the affected side in three patients. Ipsilesional weakness on caloric testing was detected in three patients and a bilateral hyporeflexia in one. A global canal impairment was detected by the video-head impulse test in one case, whereas the rest of the cohort exhibited a reduced function for the affected superior canal, together with ipsilateral posterior canal impairment in two cases. All patients performed both temporal bones HRCT scan and brain-MRI showing unilateral SCD and ipsilateral VS, respectively. All patients were submitted to a wait-and-scan approach, requiring VS removal only in one case. CONCLUSION: Simultaneous SCD and VS might result in subtle clinical presentation with puzzling lesion patterns. When unclear symptoms and signs occur, a complete audiovestibular assessment plays a key role to address imaging and diagnosis.

Inner Ear Breaches from Vestibular Schwannoma Surgery: Revisiting the Incidence of Otologic Injury from Retrosigmoid and Middle Cranial Fossa Approaches.

OBJECTIVE: To assess the rate of iatrogenic injury to the inner ear in vestibular schwannoma resections. STUDY DESIGN: Retrospective case review. SETTING: Multiple academic tertiary care hospitals. PATIENTS: Patients who underwent retrosigmoid or middle cranial fossa approaches for vestibular schwannoma resection between 1993 and 2015. INTERVENTION: Diagnostic with therapeutic implications. MAIN OUTCOME MEASURE: Drilling breach of the inner ear as confirmed by operative note or postoperative computed tomography (CT). RESULTS: 21.5% of patients undergoing either retrosigmoid or middle fossa approaches to the internal auditory canal were identified with a breach of the vestibulocochlear system. Because of the lack of postoperative CT imaging in this cohort, this is likely an underestimation of the true incidence of inner ear breaches. Of all postoperative CT scans reviewed, 51.8% had an inner ear breach. As there may be bias in patients undergoing postoperative CT, a middle figure based on sensitivity analyses estimates the incidence of inner ear breaches from lateral skull base surgery to be 34.7%. CONCLUSIONS: A high percentage of vestibular schwannoma surgeries via retrosigmoid and middle cranial fossa approaches result in drilling breaches of the inner ear. This study reinforces the value of preoperative image analysis for determining risk of inner ear breaches during vestibular schwannoma surgery and the importance of acquiring CT studies postoperatively to evaluate the integrity of the inner ear.

Significant tumor compression of the middle cerebellar peduncle is associated with worse facial nerve outcomes and lower extent of resection in surgery for medium-sized vestibular schwannomas - A radiographic analysis of a case series.

BACKGROUND AND OBJECTIVE: Optimizing the extent of resection (EOR) and facial nerve outcomes (FNO) remain a challenge in medium to large vestibular schwannomas (VS). Currently, tumor size has been the only consistently reported factor predicting FNO and EOR. Here, we sought to evaluate whether the degree of the tumor's compression on the middle cerebellar peduncle (PC) influences FNO and EOR in medium to large VS. METHODS: This retrospective case series included 99 patients who underwent surgical resection of their VSs from 2014 to 2022. Preoperative MR imaging was used to measure the degree of PC. Patient medical records were queried to determine the EOR and FNO. RESULTS: Patients with unfavorable FNO (HB 3 +) immediately post-op had significantly greater PC than those with favorable FNO (19.9 vs. 15.4 mm, P = .047). This significance was not observed at the last follow-up but there was a trend. When medium-sized tumors (15-30 mm) were analyzed separately, patients with unfavorable FNO immediate post-op and at last follow-up had significantly greater PC than their favorable counterparts (14.1 vs 8.7 mm). Significantly greater PC was also observed in patients who underwent subtotal resection (20.7 mm) compared to near (14.3 mm) and gross total resection (10.8 mm). Multivariate analyses confirmed these findings in medium-sized tumors, but not large-sized tumors. CONCLUSION: The degree of PC as measured on preoperative imaging can predict FNO and EOR in medium-sized vestibular schwannomas. Medium-sized tumors with > 15 mm of PC likely will have worse FNO and lower EOR.

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.

Subclassification of Koos grade 4 vestibular schwannoma: insights into tumor morphology for predicting postoperative facial nerve function.

OBJECTIVE: Koos grade 4 vestibular schwannoma (KG4VS) is a large tumor that causes brainstem displacement and is generally considered a candidate for surgery. Few studies have examined the relationship between morphological differences in KG4VS other than tumor size and postoperative facial nerve function. The authors have developed a landmark-based subclassification of KG4VS that provides insights into the morphology of this tumor and can predict the risk of facial nerve injury during microsurgery. The aims of this study were to morphologically verify the validity of this subclassification and to clarify the relationship of the position of the center of the vestibular schwannoma within the cerebellopontine angle (CPA) cistern on preoperative MR images to postoperative facial nerve function in patients who underwent microsurgical resection of a vestibular schwannoma. METHODS: In this paper, the authors classified KG4VSs into two subtypes according to the position of the center of the KG4VS within the CPA cistern relative to the perpendicular bisector of the porus acusticus internus, which was the landmark for the subclassification. KG4VSs with ventral centers to the landmark were classified as type 4V, and those with dorsal centers as type 4D. The clinical impact of this subclassification on short- and long-term postoperative facial nerve function was analyzed. RESULTS: In this study, the authors retrospectively reviewed patients with vestibular schwannoma who were treated surgically via a retrosigmoid approach between January 2010 and March 2020. Of the 107 patients with KG4VS who met the inclusion criteria, 45 (42.1%) were classified as having type 4V (KG4VSs with centers ventral to the perpendicular bisector of the porous acusticus internus) and 62 (57.9%) as having type 4D (those with centers dorsal to the perpendicular bisector). Ventral extension to the perpendicular bisector of the porus acusticus internus was significantly greater in the type 4V group than in the type 4D group (p < 0.001), although there was no significant difference in the maximal ventrodorsal diameter. The rate of preservation of favorable facial nerve function (House-Brackmann grades I and II) was significantly lower in the type 4V group than in the type 4D group in terms of both short-term (46.7% vs 85.5%, p < 0.001) and long-term (82.9% vs 96.7%, p = 0.001) outcomes. Type 4V had a significantly negative impact on short-term (OR 7.67, 95% CI 2.90-20.3; p < 0.001) and long-term (OR 6.05, 95% CI 1.04-35.0; p = 0.045) facial nerve function after surgery when age, tumor size, and presence of a fundal fluid cap were taken into account. CONCLUSIONS: The authors have delineated two different morphological subtypes of KG4VS. This subclassification could predict short- and long-term facial nerve function after microsurgical resection of KG4VS via the retrosigmoid approach. The risk of postoperative facial palsy when attempting total resection is greater for type 4V than for type 4D. This classification into types 4V and 4D could help to predict the risk of facial nerve injury and generate more individualized surgical strategies for KG4VSs with better facial nerve outcomes.

Comparing Speech Recognition Outcomes Between Cochlear Implants and Auditory Brainstem Implants in Patients With NF2-Related Schwannomatosis.

OBJECTIVE: To compare cochlear implant (CI) and auditory brainstem implant (ABI) performance in patients with NF2-related schwannomatosis (NF2). STUDY DESIGN: Historical cohort. SETTING: Tertiary academic center. PATIENTS: A total of 58 devices among 48 patients were studied, including 27 ABIs implanted from 1997 to 2022 and 31 CIs implanted from 2003 to 2022. Three patients had bilateral ABIs, three had bilateral CIs, three had an ABI on one side and a CI on the other, one had a CI that was later replaced with an ipsilateral ABI, and one had an ABI and CI concurrently on the same side. INTERVENTIONS: CI or ABI ipsilateral to vestibular schwannoma. MAIN OUTCOME MEASURES: Open-set speech perception, consonant-nucleus-consonant word scores, and AzBio sentence in quiet scores. RESULTS: Among all patients, 27 (47%) achieved open-set speech perception, with 35 (61%) daily users at a median of 24 months (interquartile range [IQR], 12-87 mo) after implantation. Comparing outcomes, CIs significantly outperformed ABIs; 24 (77%) CIs achieved open-set speech perception compared with 3 (12%) ABIs, with median consonant-nucleus-consonant and AzBio scores of 31% (IQR, 0-52%) and 57% (IQR, 5-83%), respectively, for CIs, compared with 0% (IQR, 0-0%) and 0% (IQR, 0-0%), respectively, for ABIs. Patients with ABIs were younger at diagnosis and at implantation, had larger tumors, and were more likely to have postoperative facial paresis. CONCLUSION: Many patients with NF2-associated vestibular schwannoma achieved auditory benefit with either a CI or an ABI; however, outcomes were significantly better in those patients who were able to receive a CI. When disease and anatomy permit, hearing rehabilitation with a CI should be considered over an ABI in these patients. Tumor management strategies that increase the ability to successfully use CIs should be strongly considered given the high risk of losing bilateral functional acoustic hearing in this population.

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.

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.

Hydrocephalus in patients with vestibular schwannoma.

BACKGROUND: Hydrocephalus (HC) is common in patients with vestibular schwannoma (VS). This can be managed with a cerebrospinal fluid (CSF) diversion procedure prior to VS resection or with VS resection, keeping CSF diversion in reserve unless required postoperatively. No clear consensus exists as to which approach is superior. This study identifies factors predictive of the development of HC, and analyses outcomes for those managed with primary CSF resection versus tumour resection. METHODS: Single-centre retrospective cohort study of 204 consecutive adult patients with a unilateral VS from May 2009 to June 2021. Data was collected on patient and tumour demographics, management, and outcome. RESULTS: 204 patients, with a mean age at presentation of 59.5 (21-83), with 50% female, and a mean follow-up of 7.5 years (1.8-13.9) were included. 119 were managed conservatively, 36 with stereotactic radiosurgery only, and 49 with surgery. 30 (15%) patients had radiological HC, of which 23 (77%) were obstructive, and 7 (23%) were communicating. Maximum intracranial tumour diameter and Koos grade were higher in patients with HC. Of the patients with HC the majority (20, 67%) were managed initially with CSF diversion, with 12 patients undergoing subsequent tumour resection, and three patients avoiding primary resection. Nine (30%) were managed with primary surgical resection, of whom three required subsequent CSF diversion. Complication rates and Modified Rankin Scale (MRS) were comparable or lower in the CSF diversion group (8%, MRS ≤2 = 83%), versus the primary resection group (67%, MRS ≤2 = 67%), and the primary surgical resection without HC group (25%, MRS ≤2 = 86%). CONCLUSIONS: CSF diversion prior to tumour resection is a safe and acceptable strategy compared to primary VS resection, with improved outcomes and reduced surgical complications. Randomized studies and national databases are needed to determine the long-term outcomes of patients treated with CSF diversion versus primary resection.

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.

COX inhibitor use during definitive radiotherapy is associated with worse hearing preservation in patients with vestibular schwannoma.

PURPOSE: Patients with vestibular schwannoma undergoing definitive radiotherapy commonly experience hearing loss due to tumor and treatment effects; however, there is limited data evaluating concurrent medication use and other clinicopathologic factors associated with hearing preservation during and after radiotherapy. We performed a retrospective cohort study reviewing consecutive patients from 2004 to 2019 treated with radiotherapy for vestibular schwannoma at our institution. METHODS: Ninety four patients with concurrent medications, baseline audiograms, and post-radiotherapy audiograms available were evaluable. We performed chi-squared analyses of the frequency of various clinicopathologic factors and t-tests evaluating the degree of hearing loss based on audiograms. RESULTS: At a median follow-up of 35.7 months (mean: 46.5 months), the baseline pure-tone average (PTA) of the ipsilateral ear worsened from 38.4 to 59.5 dB following completion of radiotherapy (difference: 21.1, 95% CI 17.8-24.4 dB, p < 0.001). 36 patients (38.3%) reported regular use of cyclooxygenase (COX) inhibitors (including acetaminophen and NSAIDs) during radiotherapy. The mean increase in PTA was significantly higher for patients taking COX inhibitors (25.8 dB vs 18.1 dB, p = 0.024) in the ipsilateral ear but not for the contralateral side. COX inhibitor use remained independently associated with worse PTA in the multivariate analysis. CONCLUSION: COX inhibitor use during definitive radiotherapy is associated with worse hearing loss in the affected ear but not for the contralateral side. This suggests the ototoxic effects of COX inhibitors may influence the effects of radiotherapy. These results could have clinical implications and warrant further investigation.

A Case of Vestibular Schwannoma with Deafness Showing Remarkable Hearing Recovery Following Hearing-Preserving Surgery.

Surgery for vestibular schwannoma can be divided into hearing-preserving and nonhearing-preserving surgeries. Hearing-preserving surgery is usually not considered in patients with deafness due to vestibular schwannoma, because hearing is unlikely to improve, and surgery aims to maximize the tumor resection at the expense of hearing. We report an extremely rare case of a 46-year-old man with unilateral profound hearing loss due to a vestibular schwannoma with marked cystic degeneration in the left cistern, which significantly recovered to near-normal hearing levels after hearing-preserving surgery. Hearing loss gradually worsened, and preoperative pure-tone evaluation showed complete hearing loss in the left ear. However, the response to the distortion product otoacoustic emission was preserved, and hearing loss was considered to be retrocochlear. Tumor resection was performed using the retrolabyrinthine approach with continuous monitoring using dorsal cochlear nucleus action potential, auditory brainstem response, and facial nerve function muscle action potential. The cistern portion of the tumor was almost completely resected along with the wall. Postoperatively, the pure-tone threshold on the left side markedly improved. The present case clearly demonstrates the possibility of hearing recovery in patients with retrocochlear hearing loss. We should consider expanding the indications for hearing-preserving surgery.

Long-term clinical outcomes of stereotactic radiotherapy for bilateral vestibular schwannomas in neurofibromatosis type 2 patients.

PURPOSE: The evidence for treating patients with neurofibromatosis 2-related vestibular schwannoma (VS-NF2) using hypofractionated stereotactic radiation therapy (HSRT) is limited. This study aimed to investigate clinical outcomes in patients with VS-NF2 treated with Robotic HSRT. METHODS: We retrospectively analyzed 25 NF2 patients with 48 VSs who were treated using Robotic HSRT at Ramathibodi Hospital from January 2009 to January 2020. RESULTS: Median follow-up was 98 months (range, 24-155 months). Median tumor volume was 2.3 cm3 (range, 0.4-28.3 cm3). Median prescribed dose was 18 Gy (range, 18-25 Gy) in three fractions (range, 3-5). The 5- and 10-year local control rates were 87% and 80%, respectively. The 5- and 10-year hearing preservation rates were 59% and 35%, respectively. Three patients developed new symptoms associated with transient volume expansion after treatment: hydrocephalus in one, facial weakness in one, and ataxia in one. No patient developed worsening of trigeminal nerve function. No histologically confirmed of radiation induced malignancy was reported in the study. CONCLUSIONS: Robotic HSRT demonstrated excellent long-term tumor control with a low non-auditory complication rate in patients with VS-NF2. However, preservation of hearing remains a major concern.