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In patients with Neurofibromatosis Type 2 (NF2) and VS, we previously found an association between pre-contrast fluid-attenuated inversion recovery magnetic resonance imaging (FLAIR MRI) signal in the labyrinth and hearing loss. Here, we asked whether FLAIR hyper-intensity could serve as a predictive biomarker for hearing loss in NF2 patients with VS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      A prospective longitudinal study (NCT00598351) of NF2 enrolled 168 subjects between 2008 and 2013. This study included 34 patients with small VS (total volume\u2009\u2264\u2009500mm\n                      <jats:sup>3<\/jats:sup>\n                      ). Middle fossa decompression surgery (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20094 patients) was provided via a standard-of-care trial (NCT00060541).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>From 34 eligible subjects (mean age 26.8y) with NF2 and small VS, 53 ears met inclusion criteria. Abnormal hearing was recorded in 18 ears at study entry; all 18 ears had FLAIR hyper-intensity. Of the 35 ears with normal hearing, 16 had FLAIR hyper-intensity at study entry, 6 (37.5%) of which developed new hearing loss (median time to hearing loss of 4.45 years). Conversion to FLAIR hyper-intensity occurred in 11 ears, 3 of which proceeded to hearing loss. No hearing loss developed in the eight ears that remained FLAIR negative. FLAIR conversion has high sensitivity (1\u00b700, 95% CI 0\u00b739\u20131) and negative predictive value (1\u00b700, 95% CI 0\u00b763\u20131) for new-onset hearing loss. In 4 patients undergoing middle fossa decompression surgery, we found that surgery stabilized hearing but did not reverse FLAIR hyperintensity.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Our findings suggest that intralabyrinthine FLAIR hyper-intensity is a sensitive, non-invasive biomarker for hearing loss related to VS. Hearing decline followed FLAIR hyper-intensity by approximately 4 years but was absent in ears with normal FLAIR signal.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Trial registration<\/jats:title>\n                    <jats:p>Registry: ClinicalTrials.gov, TRN: NCT00060541, Registration date: 4 June 2003.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s11060-026-05422-9","type":"journal-article","created":{"date-parts":[[2026,1,26]],"date-time":"2026-01-26T15:52:38Z","timestamp":1769442758000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Intralabyrinthine MRI FLAIR as a predictive marker for hearing loss in vestibular schwannomas in Neurofibromatosis Type 2"],"prefix":"10.1007","volume":"176","author":[{"suffix":"III","given":"Robert L.","family":"Walker","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxwell T.","family":"Laws","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. 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All methods were carried out in accordance with the ethical standards of the Declaration of Helsinki and NIH institutional guidelines. All experimental protocols were reviewed and approved by the Combined Neuroscience Institutional Review Board of the NIH Clinical Center under protocol NCT00598351. All participants (or their legal guardians, when appropriate) provided written informed consent prior to enrollment. The middle fossa decompression procedures included in this analysis were performed as part of a standard-of-care clinical trial approved by the same Institutional Review Board (NCT00060541), with written informed consent obtained from all participants.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"182"}}