Neurofibromatosis type 2

Case contributed by Jerald Garvin Lim


Bilateral hearing loss, gait imbalance, and hemifacial numbness.

Patient Data

Age: 20 years
Gender: Female

There are nodular, heterogeneous and avidly enhancing masses in both cerebellopontine regions indicative of vestibular schwannomas (predominantly intracanalicular with cisternal portions) with cystic changes and foci of GRE hypointensities likely from calcifications.

Enhancing lesions in the region of the left Meckel's cave and cisternal segments of the right trigeminal, left oculomotor and left abducens nerves also likely represent schwannomas

The constellation of findings may relate to neurofibromatosis type 2.

Case Discussion

Cranial nerve lesions present in virtually all patients with neurofibromatosis type 2 (NF2). They are often multiple, with vestibular schwannomas (VS) being the most common NF2-related schwannomas. The finding of bilateral VS is almost pathognomonic of NF2 by imaging. Nonvestibular schwannomas also occur in around 50% of cases (such as trigeminal, oculomotor). The associated imaging features of NF2 are meningiomas (50%), cord ependymomas (often multiple) and schwannomas of spinal nerve roots.

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