Table 2 Previous and current diagnostic criteria for neurofibromatosis 2

1. Bilateral 8th nerve masses^b

1. Bilateral VS

1. Bilateral VS aged < 70 years

1. Same as 2016

1. Bilateral VS

2. FDR with NF2, plus

- unilateral 8^th nerve mass, or

-two NF-related lesions^c

2. FDR with NF2, plus

- unilateral VS, or

-two NF-related lesions^c

2. FDR with NF2, plus unilateral VS aged < 70 years

2. FDR other than siblings with NF2, plus unilateral VS aged < 70 years

2. Identical NF2 pathogenic variant in ≥ two distinct NF2-related tumors^{g, h}

3. Unilateral VS, plus

-two NF-related lesions^c

3. FDR with NF2, or unilateral VS, plus two NF-related lesions^{d, e}

3. FDR other than siblings with NF2, or unilateral VS, plus two NF-related lesions^{f, e}

3. Two major or one major and two minor criteria, as follows:

4. Multiple meningioma, plus

-unilateral VS, or

-two NF-related lesions^c

4. Multiple meningioma, plus two of unilateral VS or other NF-related lesions^d

4. Multiple meningioma, plus two of unilateral VS or other NF-related lesions^{f, e}

Major criteria:

-Unilateral VS

-FDR other than siblings with NF2

- ≥ 2 meningiomas

- NF2 pathogenic variant in an unaffected tissue (e.g., blood, saliva)

5. Constitutional pathogenic NF2 gene variant in blood or identical mutations in two distinct tumors

5. Same as 2016

Minor criteria:

- > one NF-related lesion

- Juvenile subcapsular or cortical cataract, retinal hamartoma, epiretinal membrane in a person aged < 40 years

-Specific pattern of genetic changesⁱ