Determinants of the quality of life of patients with NF2-related schwannomatosis and validation of the Dutch NFTI-QOL questionnaire.
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NF2-Related Schwannomatosis (NF2-SWN) is a rare tumor predisposition syndrome characterized by heterogeneous clinical manifestations that substantially impact quality of life (QOL). In this diverse patient population, a disease-specific QOL instrument is essential for both research and clinical decision-making. Insight into factors associated with QOL may further facilitate the tailoring of management strategies to individual patient needs. This study aimed to validate the Dutch version of the NF2 Impact on Quality of Life questionnaire (NFTI-QOL) and to identify factors associated with QOL in Dutch patients with NF2. Following forward‑ and backward‑translation, the NFTI-QOL was administered to NF2-SWN patients alongside the SF‑36, EQ‑5D and PANQOL. The NFTI-QOL was repeated after one week. Demographic and clinical data were extracted from medical records. To provide clinical context, questionnaires were also administered to patients with sporadic vestibular schwannoma (sVS). 64 NF2-SWN and 51 sVS patients completed the questionnaires. The NFTI-QOL demonstrated good validity and reliability. In multivariate linear regression, dizziness (β = 2.533, p = 0.027) and facial weakness (β = 4.064, p = 0.008) were significantly associated with QOL, whereas hearing complaints were not. Surprisingly, overall QOL did not differ between NF2-SWN and sVS patients, whereas patients with sVS even reported poorer hearing-related QOL. The Dutch NFTI-QOL is a reliable instrument for assessing QOL in NF2-SWN patients. Dizziness and facial weakness were associated with lower QOL, while hearing problems were not.