OBJECTIVE: To evaluate the literature and summarize cochlear implantation (CI) outcomes after intralabyrinthine schwannoma (ILS) excision and tumor observation with CI.
METHODS: OVID Medline, Embase, Web of Science; conception to 2024.
METHODS: A literature review was performed using subject headings, MeSH terms, and keywords. Abstracts and full texts were reviewed by two independent reviewers and adjudicated by a third. Inclusion criteria included studies with ILS and CI with reported audiologic outcomes. Subjects were analyzed into two groups, ILS resection with CI and in situ ILS with CI. Patients with NF2 were included. The main outcome of interest was CI audiometric performance level, with secondary outcomes of CI user status and open-set speech attainment.
RESULTS: There were 29 articles with a total of 93 patients who met inclusion criteria. The resection group had 17% low performers, 44% intermediate performers, and 38% high performers. The in situ group had 40% low performers, 32% intermediate performers, 27% high performers. The resection group had 69 patients with 97% maintaining user status and 92% with open-set speech recognition. The observation group had 24 patients, with 87% user rate and 86% achieving open-set speech recognition. There was a greater percentage of NF2 diagnosis seen in the in situ group.
CONCLUSIONS: There is a paucity of literature on CI and ILS. Patients are managed with both resection of tumor and implantation in situ. Early data are limited, with improvement in hearing outcomes and high user rates in both populations.
METHODS: N/A Laryngoscope, 134:3910-3920, 2024.

Intralabyrinthine schwannomas (ILS) are rare benign tumours arising from the peripheral branches of the cochlear or vestibular nerves in the membranous labyrinth, intracochlear schwannomas being the most frequent ones. When hearing is no longer feasible on the affected side, surgical removal along with simultaneous cochlear implantation can be proposed to the patient. We hereby present a systematic review of the literature on the topic, as well as two original cases from our centre (Ospedale Università degli Studi di Padova). Cochlear implantation in intracochlear schwannomas is feasible, with overall satisfactory hearing outcomes in accordance with the evidence found in the literature.

OBJECTIVE: 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.

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.

OBJECTIVE: 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.

BACKGROUND: Intralabyrinthine schwannoma is a rare, benign tumor that affects the most terminal portions of the vestibular and cochlear nerves. This tumor can be classified into 10 subtypes, according to its inner ear location.
OBJECTIVE: To carry out a comprehensive review of the most frequent auditory manifestations secondary to the intralabyrinthine schwannoma, describing the possible underlying pathophysiological mechanisms.
METHODS: Systematic review of the literature until October 2017 using the PubMed, Web of Science and Scopus databases. The inclusion criteria were clinical manifestations of the intralabyrinthine schwannoma. Three researchers independently assessed the articles and extracted relevant information. The description of a case of an intravestibular subtype intralabyrinthine schwannoma with multiple forms of clinical presentations was used as an example.
RESULTS: Twenty-seven studies met our inclusion criteria. The most common intralabyrinthine schwannoma subtype was the intracochlear, followed by the intravestibular type. All the cases demonstrated hearing loss, usually progressive hearing loss.
CONCLUSIONS: The diagnosis of intralabyrinthine schwannomas is based on high-resolution magnetic resonance imaging and should be included in the differential diagnosis of patients with vestibulocochlear complaints. Although there are approximately 600 cases in the literature, we still lack a detailed description of the clinical evolution of the patients, correlating it with MRI findings of temporal bones and tumor subtype.

OBJECTIVE: To describe the natural history of primary inner ear schwannomas (PIES) and evaluate management outcomes and relationship between PIES location, clinical presentation, and time to diagnosis.
METHODS: Retrospective chart review and systematic review of the literature.
METHODS: Vestibular schwannoma confined to or arising from the inner ear were included. PIES classification was based on anatomic subsite(s) involved. Detailed clinical history and outcomes were recorded.
RESULTS: In a systematic review (1933-2011), including 14 patients from the authors\' institution (1999-2009), a total of 72 studies comprising 234 patients were evaluated. Mean follow-up was 32.8 ± 39.1 months (range, 0-183 months). The cochlea was the most commonly involved subsite (51%). Hearing loss was the most frequent presenting symptom (99%). Vertigo and abnormal balance were more common among tumors involving the vestibular system (P < .01). Average delay between symptom onset and diagnosis was 7.0 ± 8.0 years (median, 5 years; range, 0-40 years). Recent onset hearing loss was more likely to elicit an earlier diagnosis (P = .01). The majority of patients were observed without treatment (53%). Tumor progression was seen in 52% of patients.
CONCLUSIONS: PIES are rare tumors and most commonly involve the cochlea. Tumor location is often associated with clinical presentation and correlates with delay between symptom onset and diagnosis. A watch-and-scan approach is the management strategy of choice in the absence of intractable vertigo or extensive tumor growth. The majority of patients report stable or improved symptoms over time, regardless of treatment.