Bilateral vestibular schwannomas at < 70 years¹

First-degree relative with NF2 AND unilateral vestibular schwannoma at < 70 years¹

First-degree relative with NF2 OR unilateral vestibular schwannoma AND any 2 of²: meningioma, cataract, glioma, neurofibroma, schwannoma, cerebral calcification. If unilateral schwannoma and ≥2 nonintradermal schwannomas needs also negative LZTR1 genetic test¹

≥2 meningiomas AND any 2 of: unilateral vestibular schwannoma, cataract, glioma, neurofibroma, schwannoma, cerebral calcification

Constitutional or mosaic pathogenic NF2 gene mutation in blood or identical mutations in 2 distinct tumors¹