Natural history of vestibular schwannomas and hearing loss in NF2 patients

Peyre, Matthieu; Bernardeschi, Danièle; Sterkers, Olivier; Kalamaridès, Michel

doi:10.1016/j.neuchi.2015.03.012

Cited by 13 publications

(8 citation statements)

References 36 publications

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“…A literature review showed that 62% to 85% of patients with NF2 present with serviceable hearing loss. 29 We also found that the presence of bilateral VSs was a predictive factor for serviceable hearing loss. Although unclear, these results could indicate that bilateral VSs represent a more progressive and aggressive form, leading to a more rapid hearing decline and serviceable hearing loss.…”

Section: Tumor Control and Serviceable Hearing Preservationsupporting

confidence: 51%

Stereotactic Radiosurgery for Vestibular Schwannoma in Neurofibromatosis Type 2: An International Multicenter Case Series of Response and Malignant Transformation Risk

et al. 2023

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BACKGROUND:Vestibular schwannomas (VSs) related to neurofibromatosis type 2 (NF2) are challenging tumors. The increasing use of stereotactic radiosurgery (SRS) necessitates further investigations of its role and safety.OBJECTIVE:To evaluate tumor control, freedom from additional treatment (FFAT), serviceable hearing preservation, and radiation-related risks of patients with NF2 after SRS for VS.METHODS:We performed a retrospective study of 267 patients with NF2 (328 VSs) who underwent single-session SRS at 12 centers participating in the International Radiosurgery Research Foundation. The median patient age was 31 years (IQR, 21-45 years), and 52% were male.RESULTS:A total of 328 tumors underwent SRS during a median follow-up time of 59 months (IQR, 23-112 months). At 10 and 15 years, the tumor control rates were 77% (95% CI: 69%-84%) and 52% (95% CI: 40%-64%), respectively, and the FFAT rate were 85% (95% CI: 79%-90%) and 75% (95% CI: 65%-86%), respectively. At 5 and 10 years, the serviceable hearing preservation rates were 64% (95% CI: 55%-75%) and 35% (95% CI: 25%-54%), respectively. In the multivariate analysis, age (hazards ratio: 1.03 [95% CI: 1.01-1.05]; P = .02) and bilateral VSs (hazards ratio: 4.56 [95% CI: 1.05-19.78]; P = .04) were predictors for serviceable hearing loss. Neither radiation-induced tumors nor malignant transformation were encountered in this cohort.CONCLUSION:Although the absolute volumetric tumor progression rate was 48% at 15 years, the rate of FFAT related to VS was 75% at 15 years after SRS. None of the patients with NF2-related VS developed a new radiation-related neoplasm or malignant transformation after SRS.

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Section: Tumor Control and Serviceable Hearing Preservationsupporting

confidence: 51%

Stereotactic Radiosurgery for Vestibular Schwannoma in Neurofibromatosis Type 2: An International Multicenter Case Series of Response and Malignant Transformation Risk

et al. 2023

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“…It is the case that the most common challenge in the care of patients with NF2 is the management of their hallmark tumors, namely bilateral VS. With these bilateral lesions comes the lifetime threat of permanent and complete deafness as well as the other risks of VS management and intervention. 15,16 The reassurance to both clinician and patient that the cohort we describe here who, with UVS and MM are 50% less likely to develop bilateral VS than those presenting with UVS and ≥2 NIDS, will be an important factor in both clinical decision making and, equally importantly, counseling for the patient and family.…”

Section: Discussionmentioning

confidence: 91%

Multiple Meningiomas as a Criterion for the Diagnosis of Neurofibromatosis Type 2 and Other Tumor Predisposition Syndromes

et al. 2022

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BACKGROUND:Bilateral vestibular schwannomas (VS) are pathognomonic of neurofibromatosis type 2 (NF2), but the diagnostic criteria also include unilateral VS (UVS) in combination with multiple meningiomas (MM) and other schwannomas, as well as MM without VS.OBJECTIVE:To investigate the diagnostic value of these criteria and establish the presence of other genetic conditions in patients presenting in this manner.METHODS:The Manchester International NF2 database was accessed to obtain information on patients presenting with a UVS and MM or ≥2 nonintradermal schwannomas (NIDS). We gathered data on patients diagnosed with NF2 due to MM without VS and on patients presenting with MM without meeting NF2 criteria. Analysis was performed for pathogenic variants (PVs) in NF2, SMARCE1, SMARCB1, and LZTR1.RESULTS:A total of 31 of 131 patients presenting with a UVS and MM had a nonrefuted diagnosis of NF2 after molecular studies, in comparison with 85 of 96 patients presenting with UVS and ≥2 NIDS (P ≤ .00001). Fifty percent of patients presenting with a UVS and ≥2 NIDS with NF2 developed bilateral VS, compared with only 26% of those who presented with a UVS and MM (P = .0046). In total, 11 of 152 patients presenting with MM without fulfilling NF2 criteria were found to have a PV in SMARCE1, and 7 of 152 were confirmed to have mosaic NF2.CONCLUSION:Patients presenting with UVS and MM are significantly more likely to have a nonrefuted diagnosis of NF2 than patients presenting with UVS and ≥2 NIDS, but significantly less likely to develop bilateral VS. Seven percent of those presenting with MM without meeting NF2 criteria had PV in SMARCE1, and 5% had mosaic NF2.

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“…Hearing was lost in a short period of observation (4 years) without clear tumor growth, at variance with the much slower hearing decline with NF2 (13,14) or with sporadic VS (15,16). This may be accounted for by direct damage to the organ of Corti by local abnormal metabolites, such as elevated intralabyrinthine protein, or interruption of potassium cycling (12,17).…”

Section: Discussionmentioning

confidence: 99%

NF2-Related Intravestibular Schwannomas: Long-Term Outcomes of Cochlear Implantation

Jia

Nguyen

Hochet

et al. 2020

Otology &Amp; Neurotology

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Objective: Intravestibular schwannomas (IVSs) are uncommon tumors in Neurofibromatosis type 2 (NF2) and are mainly associated with multiple internal auditory meatus (IAM) and cerebellopontine angle (CPA) tumors. They usually induce profound hearing loss which can be rehabilitated by cochlear implantation (CI). The aim of this study was to analyze the longterm outcomes of CI during the unpredictable evolution of NF2 disease. Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Three adults with neurofibromatosis type 2 and intravestibular schwannomas, and who were cochlear implant recipients. Interventions: Periodic radiologic follow-up, tumor resection and hearing rehabilitation. Main outcome measures: Audiological evolution, tumor evolution, surgical outcome, cochlear implant outcome. Results: Three NF2 patients (mean age at diagnosis, 21 ± 2.5 years) were identified with IVS in the period between 2000 and 2014. IVS were first observed by serial MRI and profound hearing loss occurred in this ear after 4±1.5 years of follow-up. IVS were removed via a translabyrinthine (TL) approach, and ipsilateral cochlear implantations were simultaneously performed. In two patients, large contralateral CPA tumors had previously been removed without hearing preservation, whereas in the third patient, a small, growing contralateral VS was excised via a retrosigmoid approach 6 months after IVS removal/cochlear implantation with serviceable hearing preservation. In all cases, CI provided good hearing outcomes. In two cases, hearing outcomes were even better for more than 5 years when ipsilateral intracanalicular vestibular schwannomas were removed in either the same or subsequent procedures. Conclusions: Rehabilitation of hearing with CI provides a favorable long-term outcome in patients with NF2-related IVS which could be altered by the occurrence of other intracanalicular and/or CPA NF2-related tumors.

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Natural history of vestibular schwannomas and hearing loss in NF2 patients

Cited by 13 publications

References 36 publications

Stereotactic Radiosurgery for Vestibular Schwannoma in Neurofibromatosis Type 2: An International Multicenter Case Series of Response and Malignant Transformation Risk

Stereotactic Radiosurgery for Vestibular Schwannoma in Neurofibromatosis Type 2: An International Multicenter Case Series of Response and Malignant Transformation Risk

Multiple Meningiomas as a Criterion for the Diagnosis of Neurofibromatosis Type 2 and Other Tumor Predisposition Syndromes

NF2-Related Intravestibular Schwannomas: Long-Term Outcomes of Cochlear Implantation

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