Bilateral vestibular Schwannomas are associated with
Correct Answer: Neurofibromatosis Type 2
Description: (D) Neurofibromatosis Type 2# Central neurofibromatosis, or Neurofibromatosis type 2 (NF2), is a multisystem genetic disorder associated with bilateral vestibular schwannomas, spinal cord schwannomas, meningiomas, gliomas, and juvenile cataracts, with a paucity of cutaneous features (which are seen more consistently in neurofibromatosis type 1 ).> Although quite variable in its age of onset and severity of symptoms in affected individuals, NF2 is associated with significant morbidity and decreased life span. Furthermore, diagnosis in childhood is often difficult because of the absence of central nervous system (CNS) involvement at a young age.> Complications of NF2 may include the following: Unilateral or, frequently, bilateral vestibular schwannomas leading to tinnitus, hearing loss, and/or problems with balance Meningiomas, gliomas, ependymomas, and other cerebral, cerebellar, or spinal cord lesions that may result in neurologic deficits, seizures, and/or hydrocephalus Peripheral nerve schwannomas, mixed tumors, and, occasionally, neurofibromas Peripheral neuropathies Visually significant juvenile cataracts
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