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Purpose: This retrospective monocentric study aimed to evaluate long-term auditory brainstem implant (ABI) function in patients with neurofibromatosis type 2, and to investigate the prognostic factors for ABI use., Methods: Between 1997 and 2022, 27 patients with at least five years of follow-up underwent implantation with 32 ABIs. At 1- and 5-years post-implantation and at last follow-up, ABIs were classified as used or non-used and the size of the ipsilateral tumor was recorded. For patients who used their ABIs, we assessed speech perception (disyllabic words, MBAA sentences) in quiet conditions with the ABI only, by lip-reading (LR), and with a combination of the two (ABI + LR). Hearing improvement was calculated as Δ ABI = (ABI + LR)-LR scores. Predictive factors for ABI use were analyzed., Results: One year post-implantation, 74% patients were ABI-users and 66% of the ABIs were used. Two of these patients were non-users at five years, and another two at last follow-up (14 ± 5.2 years); 54% of the patients were ABI-users at last follow-up. Δ ABI revealed a hearing improvement of 32-41% (disyllabic words) and 28-37% (MBAA sentences). Among 16 ABIs with at least LR improvement at 1-year post-implantation, 4 decreased their performance, coinciding with a large growing ipsilateral tumor in 3/4 ABIs. We identified no significant prognostic factors for ABI use., Conclusions: ABIs are indicated in case of bilateral deafness with a non-functional cochlear nerve. Half the patients with ABIs used their implants and auditory performance remained stable over time, except in cases of ipsilateral tumor growth., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Background and Purpose: Large symptomatic Vestibular Schwannoma (VS) often requires surgical resection, regardless the patient's age. The aim of this study was to assess the surgical outcomes of patients in their ninth decade of life., Methods: This monocenter retrospective observational study included patients aged 80 years or older who underwent VS surgery between 2009 and 2020. We retrospectively analyzed their immediate post-surgical and long-term outcomes and complications., Results: Thirteen octogenarians who underwent VS surgery were included, with average age of 83.2 ± 1.97 years old (median 83.5, range 80-86 years). One patient had a Koos-Grade II tumor, and 12 patients had a grade IV. All patients had a preoperative ASA score ≤ 3 and underwent surgery in the supine position. Twelve patients underwent a pre-planned partial resection (PR) and one had a gross-total resection (GTR). Good facial function (House-Brackmann grade ≤ 2) was achieved in 10 patients (77%). We reported three Clavien-Dindo grade ≤ 3 treatment-related complications and no life-threatening complication. Two patients experienced tumor recurrence after PR., Conclusion: In this series of patients who underwent VS surgery in their ninth decade of life, surgical outcomes were acceptable. Therefore, age alone should not serve as a contraindication for surgery. Preplanned PR is a reasonable attitude in elderly patients., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Competing Interests: M. Kalamarides déclare n’avoir aucun lien d’intérêts.

Traumatic intracranial aneurysm (TICA) is a rare and aggressive pathology that requires prompt treatment. Nevertheless, early vascular imaging following head trauma may yield falsely negative results, underscoring the importance of subsequent imaging within the first week to detect delayed TICAs. This study aims to report our experience with delayed TICAs and highlight the clinical importance of repeated angiographic screening for delayed TICAs. In this retrospective analysis, we evaluated patients managed for a TICA at a tertiary care teaching institution over the last decade. Additionally, we conducted a systematic review of the literature, following the PRISMA guidelines, on previously reported TICAs, focusing on the time lag between the injury and diagnosis. Twelve delayed TICAs were diagnosed in 9 patients. The median time interval from injury to diagnosis was 2 days (IQR: 1-22 days), and from diagnosis to treatment was 2 days (IQR: 0-9 days). The average duration of radiological follow-up was 28 ± 38 months. At the final follow-up, four patients exhibited favorable neurological outcomes, while the remainder had adverse outcomes. The mortality rate was 22%. Literature reviews identified 112 patients with 114 TICAs, showcasing a median diagnostic delay post-injury of 15 days (IQR: 6-44 days), with 73% diagnosed beyond the first week post-injury. The median time until aneurysm rupture was 9 days (IQR: 3-24 days). Our findings demonstrate acceptable outcomes following TICA treatment and highlight the vital role of repeated vascular imaging after an initial negative computed tomography or digital subtraction angiography in excluding delayed TICAs., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To evaluate long-term hearing outcomes following cochlear implantation in patients with neurofibromatosis type 2 and ipsilateral vestibular schwannoma., Study Design: Retrospective study., Setting: Tertiary general hospital., Methods: Twenty-two patients undergoing cochlear implantation between 2004 and 2018 with at least 1 year of follow-up were included. Patients were categorized as "users" or "nonusers" of their cochlear implant (CI). For users, speech perception (disyllabic words) without lip-reading was assessed in quiet conditions 1-year postimplantation, and annually thereafter. CI users were classified into 2 groups on the basis of speech intelligibility (≥40% or <40%). Demographic data, treatment options, and tumor size were also recorded., Results: One year after implantation, 16 (73%) patients used their CI daily. Twelve of these patients had a speech intelligibility ≥40% (mean: 74 ± 21.9%). Three had a Koos stage IV tumor. At the last visit (mean duration of follow-up: 6 ± 5 years), 12 of these 16 patients were still using their implant daily, and 6 had a speech intelligibility ≥40%. No predictive factors for good performance at 1 year or performance stability were identified., Conclusion: Neurofibromatosis type 2 is a complex disease profoundly affecting patient quality of life, and cochlear implantation should always be considered on a case-by-case basis. In some individuals, cochlear implantation can provide good speech intelligibility for extended periods, even posttreatment or in cases of large tumors., (© 2024 American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

Purpose: Multiple endocrine neoplasia type 1 (MEN1) is thought to increase the risk of meningioma and ependymoma. Thus, we aimed to describe the frequency, incidence, and specific clinical and histological features of central nervous system (CNS) tumors in the MEN1 population (except pituitary tumors)., Experimental Design: The study population included patients harboring CNS tumors diagnosed with MEN1 syndrome after 1990 and followed up in the French MEN1 national cohort. The standardized incidence ratio (SIR) was calculated based on the French Gironde CNS Tumor Registry. Genomic analyses were performed on somatic DNA from seven CNS tumors, including meningiomas and ependymomas from patients with MEN1, and then on 50 sporadic meningiomas and ependymomas., Results: A total of 29 CNS tumors were found among the 1,498 symptomatic patients (2%; incidence = 47.4/100,000 person-years; SIR = 4.5), including 12 meningiomas (0.8%; incidence = 16.2/100,000; SIR = 2.5), 8 ependymomas (0.5%; incidence = 10.8/100,000; SIR = 17.6), 5 astrocytomas (0.3%; incidence = 6.7/100,000; SIR = 5.8), and 4 schwannomas (0.3%; incidence = 5.4/100,000; SIR = 12.7). Meningiomas in patients with MEN1 were benign, mostly meningothelial, with 11 years earlier onset compared with the sporadic population and an F/M ratio of 1/1. Spinal and cranial ependymomas were mostly classified as World Health Organization grade 2. A biallelic MEN1 inactivation was observed in 4/5 ependymomas and 1/2 meningiomas from patients with MEN1, whereas MEN1 deletion in one allele was present in 3/41 and 0/9 sporadic meningiomas and ependymomas, respectively., Conclusions: The incidence of each CNS tumor was higher in the MEN1 population than in the French general population. Meningiomas and ependymomas should be considered part of the MEN1 syndrome, but somatic molecular data are missing to conclude for astrocytomas and schwannomas., (©2024 American Association for Cancer Research.)

Purpose: NF2-related schwannomatosis (NF2) is characterized by bilateral vestibular schwannomas (VS) often causing hearing and neurologic deficits, with currently no FDA-approved drug treatment. Pre-clinical studies highlighted the potential of mTORC1 inhibition in delaying schwannoma progression. We conducted a prospective open-label, phase II study of everolimus for progressive VS in NF2 patients and investigated imaging as a potential biomarker predicting effects on growth trajectory., Methods: The trial enrolled 12 NF2 patients with progressive VS. Participants received oral everolimus daily for 52 weeks. Brain imaging was obtained quarterly. As primary endpoint, radiographic response (RR) was defined as ≥ 20% decrease in target VS volume. Secondary endpoints included other tumors RR, hearing outcomes, drug safety and quality of life (QOL)., Results: Eight participants completed the trial and four discontinued the drug early due to significant volumetric VS progression. After 52 weeks of treatment, the median annual VS growth rate decreased from 77.2% at baseline to 29.4%. There was no VS RR and 3 of 8 (37.5%) participants had stable disease. Decreased or unchanged VS volume after 3 months of treatment was predictive of stabilization at 12 months. Seven of eight participants had stable hearing during treatment except one with a decline in word recognition score. Ten of twelve participants reported only minimal changes to their QOL scores., Conclusions: Volumetric imaging at 3 months can serve as an early biomarker to predict long-term sensitivity to everolimus treatment. Everolimus may represent a safe treatment option to decrease the growth of NF2-related VS in patients who have stable hearing and neurological condition. TRN: NCT01345136 (April 29, 2011)., (© 2024. The Author(s).)