Table 1.

Clinical diagnostic criteria for neurofibromatosis 2

NIH criteria for NF2	Manchester criteria	NNFF criteria	Baser criteria
				Presentation age <30 years (points)	Presentation age >30 years (points
			Bilateral 8th nerve masses seen by MRI with gadolinium	Bilateral vestibular schwannomas or	Confirmed definite NF2:	First-degree family relative with NF2	2	2
A parent, sibling, or child with NF2 and either unilateral 8th nerve mass or any one of the following	Family history of neurofibromatosis type 2 and	Bilateral VS	Unilateral VS	4	1 (if <70 years)
Neurofibroma	(1) Unilateral acoustic or	First-degree family relative with NF2 and unilateral VS, 30 years or any two of meningioma, glioma, schwannoma, juvenile lens opacity (posterior subcapsular cataract or cortical cataract)	Second VS	2	3 (if <70 years)
Meningioma	(2) Any two of: meningioma, glioma, neurofibroma, schwannoma, posterior subcapsular lenticular opacities	Presumptive or probable NF2:	One meningioma	2	1
Glioma	(3) Unilateral VS and any two of neurofibroma, meningioma, glioma, schwannoma, or posterior subcapsular lenticular opacities	Unilateral VS <30 years and	Second meningioma	2	1
Schwannoma	(4) Multiple meningiomas (two or more) and unilateral VS or any two of neurofibroma, glioma, schwannoma or cataract	At least one of meningioma, glioma, schwannoma, juvenile lens opacity	Cutaneous schwannoma	2	1
Posterior capsular cataract or opacity at a young age		Multiple meningiomas (two or more) and unilateral VS <30 years or at least one of meningioma, glioma, schwannoma, juvenile lens opacity	Cranial nerve tumor; one or more; excluding VS	2	1
			Mononeuropathy	2	1
			Cataract (one or more)	2	0
			>6 points = definite NF2

NF2, neurofibromatosis type 2; NNFF, National Neurofibromatosis Foundation; VS, vestibular schwannomas.