Intralabyrinthine schwannoma: Surgical management and cochlear lumen preservation.

The present video reports the surgical removal of an intralabyrinthine schwannoma. The video contains patient's medical history, preoperative radiological evaluations and detailed description of surgical steps of the procedure, consisting in labyrinthectomy, cochleostomy and insertion of a dummy electrode in the preserved cochlear lumen within the context of a subtotal petrosectomy.

Cochlear implantation in patients with inner ear schwannomas: a systematic review and meta-analysis of audiological outcomes.

PURPOSE: In patients with inner ear schwannomas (IES), reports on hearing rehabilitation with cochlear implants (CI) have increased over the past decade, most of which are case reports or small case series. The aim of this study is to systematically review the reported hearing results with CI in patients with IES considering the different audiologic outcome measures used in different countries. METHODS: According to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline, a search of published literature was conducted. We included patients with IES (primary or with secondary extension from the internal auditory canal (IAC) to the inner ear, sporadic or NF2 related) undergoing cochlear implantation with or without tumour removal. The audiological results were divided into the categories "monosyllables", "disyllables", "multisyllabic words or numbers", and "sentences". RESULTS: Predefined audiological outcome measures were available from 110 patients and 111 ears in 27 reports. The mean recognition scores for monosyllabic words with CI were 55% (SD: 24), for bisyllabic words 61% (SD: 36), for multisyllabic words and numbers 87% (SD: 25), and 71% (SD: 30) for sentences. Results from for multisyllabic words and numbers in general showed a tendency towards a ceiling effect. Possible risk factors for performance below average were higher complexity tumours (inner ear plus IAC/CPA), NF2, CI without tumour removal ("CI through tumour"), and sequential cochlear implantation after tumour removal (staged surgery). CONCLUSION: Hearing loss in patients with inner ear schwannomas can be successfully rehabilitated with CI with above average speech performance in most cases. Cochlear implantation thus represents a valuable option for hearing rehabilitation also in patients with IES while at the same time maintaining the possibility of MRI follow-up. Further studies should investigate possible risk factors for poor performance. Audiological tests and outcome parameters should be reported in detail and ideally be harmonized to allow better comparison between languages.

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.

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.

Tight modiolar proximity and feasibility of slim modiolar cochlear implant electrode array insertion in diverse etiologies of hearing loss.

PURPOSE: To report on our experience with the slim modiolar electrode (SME) especially focusing on the wide range of etiologies including inner ear anomalies, tumors, ossifications, and even revision surgeries. METHODS: All the cochlear implantation cases performed from June 2018 to September 2019 by a single surgeon was prospectively recruited. The molecular/radiological etiology of hearing loss, intraoperative outcomes, and radiographic studies of cases where the SME was implanted was reviewed to evaluate compatibility of SME for the wide range of etiologies. For cases where SME replaced the other electrode as a revision, audiologic assessment was also made. RESULTS: Among the 99 ears implanted during the study period, the SME was successfully implanted in 86 ears. These SME cases comprised inner ear anomaly/cochear nerve deficiency (n = 21) including cochlear hypoplasia type IV with the modiolus, intracochlear schwannoma (n = 1), far advanced otosclerosis (n = 1) and 7 revision cases. The SME was successfully used in 7 revision surgeries to replace the existing electrode. Shorter spiral diameter and decreased intracochlear position index for SME was found compared with their previous electrodes. Four out of the 6 patients who received revision implantation showed better speech perception after their surgeries. CONCLUSION: The SME can be implanted in any cases unless the integrity of the modiolus is totally compromised. Due to its slim design and tight modiolar-hugging feature, good functional outcome can also be anticipated. Additionally, it is suitable for revision surgeries possibly allowing better hearing outcomes which may be attributed to its closer proximity to the modiolus.

Cochlear Implantation in a Patient with Neurofibromatosis Type 2 and an Intracochlear Schwannoma: A Case Report and Literature Review.

A primary intracochlear schwannoma (ICS) is a unique type of vestibular schwannoma (VS); the tumor originates from the terminal branches of the cochlear nerve and is confined to the cochlea. An ICS is the most common subtype of schwannoma in the inner ear. As an ICS is clinically rare, diagnosis and treatment remain challenging. We report a rare case of cochlear implantation (CI) in a patient with neurofibromatosis type 2 and an ICS. The patient exhibited bilateral, profound, sensorineural hearing loss. The tumor on one side was a common VS treated via tumor and acoustic nerve resection and that on the other side an ICS. To ensure auditory rehabilitation via CI, we performed CI while removing part of the ICS via an enlarged round window. Auditory rehabilitation was satisfactory. Thus, ICS patients, especially those who urgently require auditory rehabilitation, can undergo simultaneous CI and (total or partial) tumor removal. However, the long-term results require close observation.

Diagnostic assessment of magnetic resonance imaging for patients with intralabyrinthine schwannoma: A systematic review.

OBJECTIVES: Recent advancements in high-resolution imaging have improved the diagnostic assessment of magnetic resonance imaging (MRI) for intralabyrinthine schwannoma (ILS). This systematic review aimed to evaluate the diagnostic performance of MRI for patients with ILS. METHODS: Ovid-MEDLINE and EMBASE databases were searched for related studies on the diagnostic performance of MRI for patients with ILS published up to February 10, 2020. The primary endpoint was the diagnostic performance of MRI for ILS. The quality of the enrolled studies was assessed using tailored questionnaires and the Quality Assessment of Diagnostic Accuracy Studies-2 criteria. RESULTS: Overall, 6 retrospective studies that included 122 patients with ILS from a parent population of 364 were included. The sample size, parent population and its composition, reference standard, detailed parameters of MRI, and even the diagnostic methods varied between the studies. The studies had moderate quality. The sensitivity of combination of T2WI and CE-T1WI was over 90%. Relative sensitivity of T2WI comparative to CE-T1WI ranged from 62% to 100%, and the specificity were 100%. CONCLUSIONS: MRI has acceptable diagnostic performance for ILS. There is a need for well-organized research to reduce the factors causing heterogeneity.

Histopathologic Evaluation of Intralabyrinthine Schwannoma.

OBJECTIVES: The aim of this study is to perform a histopathologic analysis of temporal bones with an intralabyrinthine schwannoma (ILS) in order to characterize its extension. METHODS: Archival temporal bones with a diagnosis of sporadic schwannoma were identified. Both symptomatic and occult nonoperated ILS were included for further analysis. RESULTS: A total of 6 ILS were identified, with 4 intracochlear and 2 intravestibular schwannomas. All intracochlear schwannomas involved the osseous spiral lamina, with 2 extending into the modiolus. The intravestibular schwannomas were limited to the vestibule, but growth into the bone next to the crista of the lateral semicircular canal was observed in 1 patient. CONCLUSIONS: Complete removal of an ILS may require partial removal of the modiolus or bone surrounding the crista ampullaris as an ILS may extend into these structures, risking damage of the neuronal structures. Due to the slow growth of the ILS, it remains unclear if a complete resection is required with the risk of destroying neural structures hindering hearing rehabilitation with a cochlear implant.

Management of vestibular dysfunction and hearing loss in intralabyrinthine schwannomas.

PURPOSE: Intralabyrinthine schwannomas (ILS) are rare, benign, slow-growing tumors arising from schwann cells of the cochlear or vestibular nerves within the bony labyrinth. This study provides insight into the management of this rare tumor through a large case series. MATERIALS AND METHODS: After Institutional Review Board approval, a retrospective chart review was performed of all ILS patients treated at our institution between 2007 and 2019. RESULTS: 20 patients (9 male, 11 female) with ILS were managed at our institution. The right ear was affected in 9 patients (45%) and the left in 11 (55%). Subjective hearing loss was endorsed by all 20 patients. Average pure tone average at presentation was 72 dB nHL. Nine tumors (45%) were intravestibular, 6 (30%) were intracochlear, 4 (20%) were transmodiolar and 1 (5%) was intravestibulocochlear. Hearings aids were used in 3 patients (15%), BiCROS in 2 (10%), CI in 2 (10%), and bone conduction implant in 1 (5%). Vestibular rehabilitation was pursued in 5 patients. Surgical excision was performed for one patient (5%) via translabyrinthine approach due to intractable vertigo. No patients received radiotherapy or intratympanic gentamicin injections. CONCLUSION: ILS presents a diagnostic and management challenge given the similarity of symptoms with other disorders and limited treatment options. Hearing loss may be managed on a case-by-case basis according to patient symptoms while vestibular loss may be mitigated with vestibular therapy. Surgical excision may be considered in patients with intractable vertigo, severe hearing loss with concurrent CI placement, or in other case-by-case situations.

Hearing rehabilitation after subtotal cochleoectomy using a new, perimodiolar malleable cochlear implant electrode array: a preliminary report.

PURPOSE: We here report about the first surgical experience and audiological outcome using a new, perimodiolar malleable cochlear implant electrode array for hearing rehabilitation after subtotal cochleoectomy for intralabyrinthine schwannoma (ILS). METHOD: Based on a cochlear implant with MRI compatibility of the magnet in the receiver coil up to 3 T, a cochlear implant electrode array was developed that is malleable and can be placed perimodiolar after tumor removal from the cochlea via subtotal cochleoectomy. Malleability was reached by incorporating a nitinol wire into the silicone of the electrode array lateral to the electrode contacts. The custom-made device was implanted in four patients with intracochlear, intravestibulocochlear or transmodiolar schwannomas. Outcome was assessed by evaluating the feasibility of the surgical procedure and by measuring sound field thresholds and word recognition scores. RESULTS: After complete or partial tumor removal via subtotal cochleoectomy with or without labyrinthectomy, the new, perimodiolar malleable electrode array could successfully be implanted in all four patients. Six months after surgery, the averaged sound field thresholds to pulsed narrowband noise in the four patients were 36, 28, 41, and 35 dB HL, and the word recognitions scores for monosyllables at 65 dB SPL were 65, 80, 70, and 25% (one patient non-German speaking). CONCLUSION: The surgical evaluation demonstrated the feasibility of cochlear implantation with the new, perimodiolar malleable electrode array after subtotal cochleoectomy. The audiological results were comparable to those achieved with another commercially available type of perimodiolar electrode array from a different manufacturer applied in patients with ILS.

Management of transmodiolar and transmacular cochleovestibular schwannomas with and without cochlear implantation.

INTRODUCTION: Hearing rehabilitation with cochlear implants has attracted increasing interest also for patients with cochleovestibular schwannoma. The authors report their experience with the surgical management of tumors with rare transmodiolar or transmacular extension and outcomes after cochlear implantation (CI). METHODS: This retrospective case series included nine patients with either primary intralabyrinthine tumors or secondary invasion of the inner ear from the internal auditory canal. The primary endpoint with CI, performed in six patients, was word recognition score at 65 dB SPL (sound pressure level). Secondary endpoints were intra- and postoperative electrophysiological parameters, impedance measures, the presence of a wave V in the electrically evoked (via the CI) auditory brainstem responses, the specifics of postoperative CI programming, and adverse events. RESULTS: Hearing rehabilitation with CI in cases of transmodiolar tumor growth could be achieved only with incomplete tumor removal, whereas tumors with transmacular growth could be completely removed. All six patients with CI had good word recognition scores for numbers in quiet conditions (80-100% at 65 dB SPL, not later than 6 to 12 months post CI activation). Four of these six patients achieved good to very good results for monosyllabic words within 1-36 months (65-85% at 65 dB SPL). The two other patients, however, had low scores for monosyllables at 6 months (25 and 15% at 65 dB SPL, respectively) with worsening of results thereafter. CONCLUSIONS: Cochleovestibular schwannomas with transmodiolar and transmacular extension represent a rare entity with specific management requirements. Hearing rehabilitation with CI is a principal option in these patients.

[Bilateral intracochlear schwannomas in a patient with no genetic or clinical features of neurofibromatosis type 2. German version]. / Bilaterale intracochleäre Schwannome bei einer Patientin ohne genetische oder klinische Hinweise auf Neurofibromatose Typ 2.

Schwannomas of the eighth cranial nerve are benign tumours commonly found in the internal auditory meatus or in the cerebellopontine angle. In most cases, they arise from the inferior or vestibular portion of the vestibular nerve. Rarely, these tumours present in the inner ear and are then called intralabyrinthine schwannomas. Bilateral schwannomas are known in neurofibromatosis type 2 (NF2). Bilateral and ipsilateral, multilocular sporadic schwannomas of the eighth cranial nerve have been described as extremely rare findings. This report describes the first case of bilateral sporadic intracochlear schwannomas in a patient with no genetic or clinical features of NF2.

Bilateral intracochlear schwannomas in a patient with no genetic or clinical features of neurofibromatosis type 2.

Schwannomas of the eighth cranial nerve are benign tumours commonly found in the internal auditory meatus or in the cerebellopontine angle. In most cases, they arise from the inferior or vestibular portion of the vestibular nerve. Rarely, these tumours present in the inner ear and are then called intralabyrinthine schwannomas. Bilateral schwannomas are known in neurofibromatosis type 2 (NF2). Bilateral and ipsilateral, multilocular sporadic schwannomas of the eighth cranial nerve have been described as extremely rare findings. This report describes the first case of bilateral sporadic intracochlear schwannomas in a patient with no genetic or clinical features of NF2.

[Management of transmodiolar and transmacular cochleovestibular schwannomas with and without cochlear implantation. German version]. / Management transmodiolärer und transmakulärer Vestibularis- und Cochlearisschwannome mit und ohne Cochleaimplantation.

INTRODUCTION: Hearing rehabilitation with cochlear implants has attracted increasing interest also for patients with cochleovestibular schwannoma. The authors report their experience with the surgical management of tumors with rare transmodiolar or transmacular extension and outcomes after cochlear implantation (CI). METHODS: This retrospective case series included nine patients with either primary intralabyrinthine tumors or secondary invasion of the inner ear from the internal auditory canal. The primary endpoint with CI, performed in six patients, was word recognition score at 65 dB SPL (sound pressure level). Secondary endpoints were intra- and postoperative electrophysiological parameters, impedance measures, the presence of a wave V in the electrically evoked (via the CI) auditory brainstem responses, the specifics of postoperative CI programming, and adverse events. RESULTS: Hearing rehabilitation with CI in cases of transmodiolar tumor growth could be achieved only with incomplete tumor removal, whereas tumors with transmacular growth could be completely removed. All six patients with CI had good word recognition scores for numbers in quiet conditions (80-100% at 65 dB SPL, not later than 6 to 12 months post CI activation). Four of these six patients achieved good to very good results for monosyllabic words within 1-36 months (65-85% at 65 dB SPL). The two other patients, however, had low scores for monosyllables at 6 months (25 and 15% at 65 dB SPL, respectively) with worsening of results thereafter. CONCLUSIONS: Cochleovestibular schwannomas with transmodiolar and transmacular extension represent a rare entity with specific management requirements. Hearing rehabilitation with CI is a principal option in these patients.

MRI screening of the internal auditory canal: Is gadolinium necessary to detect intralabyrinthine schwannomas?

OBJECTIVE: Non-contrast MRI of the internal auditory canal (IAC) using high-resolution T2WI (T2 weighted image) has been proposed as the primary screening study in patients with sudden or asymmetric sensorineural hearing loss (ASNHL). However, there are concerns that non-contrast MRI may not detect labyrinthine pathology, specifically intralabyrinthine schwannomas (ILSs). The purpose of this study was to determine if non-contrast high-resolution T2WI alone are adequate to exclude these uncommon intralabyrinthine tumors. METHODS: 31 patients with ILSs and 36 patients without inner ear pathology that had dedicated MRI of the IAC performed with both non-contrast T2WI and post-contrast T1WI (T1 weighted image) were identified. Three board-certified neuroradiologists reviewed only the T2WI from these 67 cases. When an ILS was identified, its location and size were recorded. Sensitivity, specificity, and accuracy were calculated using the post-contrast T1WI as the "gold standard." A consensus review of cases with discordant results was conducted. RESULTS: The sensitivity, specificity, and accuracy were 1.0, 1.0, and 1.0 for Observer 1; 0.84, 1.0, and 0.96 for Observer 2; 0.90, 1.0, and 0.98 for Observer 3. The 5 ILSs with discordant results were correctly identified upon consensus review. The median size of the ILSs was 4.4mm (±2.9mm) and most (18/31) were intracochlear in location. CONCLUSION: Non-contrast high-resolution T2WI alone can detect ILSs with 84-100% sensitivity, suggesting that gadolinium may be unnecessary to exclude ILSs on screening MRI. These findings have implications for reducing cost, time, and adverse events associated with gadolinium administration in patients presenting with sudden or ASNHL. LEVEL OF EVIDENCE: 4.

Intralabyrinthine schwannomas: a new surgical treatment.

OBJECTIVE: To define a new surgical option, with lower morbidity, for the treatment of intralabyrinthine schwannomas. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral centers. PATIENTS: Eight patients affected by an intralabyrinthine schwannoma, with or without extension to the internal auditory canal, that underwent surgery with a transcanal transpromontorial approach, were included in the study. The average age at presentation was 47 years. Patients' characteristics, symptoms, tumor features, and surgical results were analyzed. INTERVENTIONS: All patients were diagnosed and evaluated pre-operatively with high-resolution, gadolinium-enhanced MRI and CT scan of the temporal bone, and underwent surgery via either transcanal transpromontorial endoscopic approach (TTEA) or enlarged transcanal transpromontorial endoscopic approach (ETTA). MAIN OUTCOME MEASURES: Hearing function was evaluated with the Hearing Classification System according to the Committee on Hearing and Equilibrium Guidelines. Facial nerve function was evaluated using the House-Brackmann grading system (HB). RESULTS: In six patients out of eight, a TTEA was performed, whereas, due to the extension of the pathology to the cerebellopontine angle, two patients underwent an ETTA. The mean follow-up period was 15.5 months (range 1-69). No intra-operative and post-operative major complications were observed. Post-operative facial nerve function was normal in seven out of eight patients (grade I HB). One patient presented a grade II HB. CONCLUSION: The endoscopic approach to cochlear schwannoma represents a good treatment option for patients and should be preferred to other more invasive surgical techniques when indicated, to reduce complications, hospitalization, and offer to patients the chance to eradicate the disease, thus avoiding the stress of a long-life radiological follow-up. LEVEL OF EVIDENCE: 4.

Intracochlear schwannoma : Tumor removal via subtotal cochleoectomy and partial cochlear reconstruction with preservation of semicircular canal function.

We report about a patient, who in 2005, as a 37-year-old, experienced a sudden, mild to moderate, mid-frequency sensorineural hearing loss in the right ear, along with tinnitus and mild dizziness. MRI of the temporal bone revealed a very small (1 mm) enhancing lesion in the second turn of the right cochlea after injection of contrast medium. Hearing gradually deteriorated, with complete hearing loss in 2015. At this time, an MRI scan showed a lesion completely filling the cochlea.The tumor was removed through a subtotal cochleoectomy. The cochlea was partially reconstructed with cartilage and fascia. A cochlea implant dummy electrode was inserted as a placeholder in order to try to preserve a channel within the most likely fibrosing "neocochlea" during follow-up MRI examinations.Vestibular function tests 2 months postoperatively showed preserved caloric excitability and a normal vestibulo-ocular reflex (video head impulse test, vHIT) in all three planes. The patient is free of vertigo.Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss. Surgical removal of an intracochlear ILS through partial or subtotal cochleoectomy with preservation of vestibular function is possible.

[Intracochlear schwannoma : Tumor removal via subtotal cochleoectomy and partial cochlear reconstruction with preservation of semicircular canal function. German version]. / Intracochleäres Schwannom : Tumorentfernung über subtotale Cochleoektomie und partielle Rekonstruktion der cochleären Kapsel unter Erhalt der Bogengangsfunktion.

We report about a patient who in 2005, aged 37 years, experienced sudden mild to moderate, mid-frequency sensorineural hearing loss in the right ear along with tinnitus and mild dizziness. MRI of the temporal bone revealed a very small (1 mm) contrast-enhancing lesion in the second turn of the right cochlea. Hearing gradually deteriorated with complete hearing loss in 2015. At this time, an MRI scan showed a lesion completely filling the cochlea.The tumor was removed via transmeatal subtotal cochleoectomy. The cochlea was partially reconstructed with cartilage and fascia. A cochlea implant dummy electrode was inserted as a placeholder in order to prevent complete fibrosis of the "neocochlea" during follow-up MRI examinations.Vestibular function tests 2 months postoperatively showed preserved caloric excitability and a normal vestibulo-ocular reflex (vHIT) in all three planes. The patient is free of vertigo.Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss, which should be specifically checked for in MRI. Surgical removal of an intracochlear ILS through partial or subtotal cochleoectomy with preservation of vestibular function is possible in principle.

Clinical features of delayed endolymphatic hydrops and intralabyrinthine schwannoma : An imaging-confirmed comparative case series. English version.

BACKGROUND: The aim of this study was to compare the clinical history and audiovestibular function test results of patients suffering from intralabyrinthine schwannoma or delayed endolymphatic hydrops (DEH). PATIENTS AND METHODS: Five patients diagnosed with intralabyrinthine schwannoma by magnetic resonance imaging (MRI) and five patients diagnosed with DEH by locally enhanced inner ear MRI (LEIM) were retrospectively studied. RESULTS: All patients with intralabyrinthine schwannoma or DEH initially presented with hearing loss. Vertigo occurred in two patients with intralabyrinthine schwannoma and in all patients with DEH. While audiometry achieved poorer results for patients with intralabyrinthine schwannomas, vestibular function tests revealed normal results in about half of the patients in both groups. CONCLUSION: Patients with intralabyrinthine schwannomas may present with clinical symptoms similar to patients suffering from other inner ear disorders such as delayed endolymphatic hydrops and they may obtain similar findings in audiovestibular function tests. High-resolution magnetic resonance imaging with locally applied contrast agent may provide evidence of both underlying pathologies.

Meta-analysis on the clinical outcomes in patients with intralabyrinthine schwannomas: conservative management vs. microsurgery.

The objective of this review is to compare the symptomatological evolution following conservative management (CM) or microsurgery (MS) in patients with intralabyrinthine schwannomas (ILS). A thorough systematic review of the English and French literature from 1948 to February 2014 was performed using Ovid Medline. An ancestor search was also completed. The major inclusion criterion consisted of a diagnosis of ILS with magnetic resonance imaging. Patients with a classic vestibular schwannoma, cases of incidentaloma during surgery or an autopsy were the main exclusion criteria. Thirty-one studies met our selective criteria. Descriptive data were collected from the articles. Clinical outcomes regarding the hearing loss, tinnitus, vertigo, dizziness and aural fullness were stated as improved, unchanged or worse at the last follow-up. All data were then separated into two different groups according to the management option: CM and MS. The data were analyzed using a Pearson χ (2) test and Fisher's exact test. This meta-analysis suggests that MS has a statistically significant favorable outcome regarding symptom relief compared to CM in patients with ILS suffering from tinnitus, vertigo and dizziness. Hearing level was not compared between treatment groups, as MS leads to anacusis. An indicative bias was the main limitation of this study, as patients suffering from intractable vertigo with moderate-to-severe hearing loss were referred to MS. Therefore, in the presence of a serviceable hearing, we suggest that CM should be the treatment of choice.