Neurofibromatosis Type 2: MR Evaluation and Clinical Correlation
The neurofibromatoses consists of at least two distinct autosomal dominant disorders with differing clinical manifestations (Martuza 1988; NIH 1988). Neurofibromatosis 1 (NF-1) was originally described by von Recklinghausen and was previously known as peripheral neurofibromatosis. Recently the gene has been assigned to chromosome 17. Neurofibromatosis 2 (NF-2), also known as central neurofibromatosis, is an autosomal dominant disorder, the hallmark of which is the presence of bilateral acoustic neuromas (AN). NF-2 has recently been assigned to chromosome 22. The affected persons generally become symptomatic in their teens or twenties with hearing loss or tinnitus as a result of the acoustic neuroma.
KeywordsHearing Loss Normal Hearing Acoustic Neuroma Internal Auditory Canal Ophthalmological Evaluation
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