| • | AKA bilateral acoustic neurofibromatosis; central neurofibromatosis |
| • | (I call it “neuromatosis” or “schwannomatosis”) |
| • | autosomal dominant; chromosome 22 |
| • | symptoms appear at age 15-25 (vs birth or early childhood in NF1) |
| • | pathogenesis: NF2 gene product = schwannomin (AKA merlin) – has tumor suppressor activity |
skin:
| • | neurofibromas – less common than in NF1; often pigmented with hair |
| • | café au lait – 1 or 2; large and pale |
schwannomas:
| • | bilateral vestibular schwannomas |
| • | may be complicated by deafness, tinnitis, poor balance |
| • | schwannomas of other cranial nerves |
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