Nodular Growths of NF2 -Related Schwannomatosis Vestibular Schwannoma: Clinical and Therapeutical Consequences.
Journal:
Neurosurgery
Published Date:
Oct 13, 2025
Abstract
BACKGROUND AND OBJECTIVES: Neurofibromatosis type 2-related schwannomatosis ( NF2 -SWN) is marked by the growth of bilateral vestibular schwannoma (VS). VS size and tumor growth rate are not correlated with hearing modifications, making a hearing preservation strategy uncertain. The goal of this study was to identify the volumetric growth of VS in NF2 -SWN using a specific 3-dimensional (3D) volumetric analysis artificial intelligence tool and make correlations with hearing pattern. METHODS: A retrospective data set was extracted from a single institution for NF2 -SWN VS and sporadic VS for comparison. Each patient included in the study had between 2 and 3 follow-up imaging studies over time. For each unique tumor, 3D segmentation masks were created the artificial intelligence tool. These 3D masks represented the same tumor at multiple time points, allowing for longitudinal assessment of tumor morphology and growth dynamics by overlaying these scans. RESULTS: A total of 43 tumors from 32 NF2 -SWN patients and 43 sporadic VS were included. In the NF2 -SWN population, tumors grew by 2.3 cm 3 over an average of 6-year follow-up time, with significant hearing loss observed in 14 cases linked to this growth. NF2 -SWN VS had nodular, multinodular, or homogeneous growth whereas sporadic VS had always homogeneous growth ( P < .01). In NF2 -SWN VS, nodular growth was significantly associated with hearing loss when developing in the posterior-inferior part of the VS in the cerebellopontine angle ( P = .02). By contrast, hearing was preserved if the nodular growth was in the anterosuperior part of the VS ( P = .001). CONCLUSION: NF2 -SWN VS display specific growth patterns different from sporadic VS, frequently with nodules, explained by the genetic of these tumors. The growth of a nodule in the posterior-inferior part was strongly and specifically correlated with hearing loss, probably because of the stretching of the cochlear nerve.
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