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Neurofibromatosis 2 : Bilateral Vestibular Schwannomas

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Neurofibromatosis 2 (NF2) is an autosomal dominant disorder affecting 1 in 33,000 to 40,000 individuals. The diagnostic hallmark of NF2 is bilateral vestibular schwannomas (acoustic neuromas) affecting vestibular portion of the eighth cranial nerve. They are manifested by tinnitus and hearing loss starting in adolescence or early adulthood. Other manifestations include: cutaneous schwannomas, schwannomas of other nerves intracranially and in the spinal compartment, meningiomas, ependymoma, and glioma.

The case shown here is from an adult patient who presented with bilateral hearing loss. This post contrast T1 axial image demonstrates bilateral enhancing masses in the cerebellopontine angle, larger on the left. Both can be seen extending into the internal acoustic meatii and are consistent with vestibular schwannomas. The fact that they are bilateral almost certainly indicates that the patient has NF2. Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org. From the case rID: 5325

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