A case report of bilateral acoustic neurofibromatosis (NF-2) is presented with temporal bone histopathology. The distinct clinical and genetic features of NF-2 are reviewed in the context of recent data that show it to be a disorder distinct from classic von Recklinghausen's disease (NF-1).
MartuzaRLEldridgeR. Neurofibromatosis 2. N Engl J Med1988; 318: 684–8.
2.
RouleauGAWerteleckiWHainesJL. Genetic linkage of bilateral acoustic neurofibromatosis to a DNA marker on chromosome 22. Nature1987; 329: 246–8.
3.
KanterWREldridgeRFabricantRAllenJCKoerberT. Central neurofibromatosis with bilateral acoustic neuroma: genetic, clinical and biochemical distinctions from peripheral neurofibromatosis. Neurology1980; 30: 851–9.
4.
SchuknechtHF. Pathology of the ear. Cambridge, Mass: Harvard University Press, 1974.
MartuzaR. Neurofibromatosis: neurological and neurosurgical considerations. In: Neurology and neurosurgery update series. Vol 3. Princeton, NJ: Continuing Professional Education Center, 1982: 1–7.
9.
SeizingerBRMartuzaRLGusellaJF. Loss of genes on chromosome 22 in tumorigenesis of human eighth nerve schwannoma. Nature1986; 322: 664–7.
10.
SeizingerBRRouleauGOzeliusLJ. Common pathogenetic mechanism for three tumor types in bilateral acoustic neurofibromatosis. Science1987; 236: 317–9.
11.
WerteleckiWRouleauGASuperneauDW. Neurofibromatosis 2: clinical and DNA linkage studies of a large kindred. N Engl J Med1988; 319: 278–83.
12.
SeizingerBRRouleauGOzeliusLJ. Genetic linkage of von Recklinghausen neurofibromatosis to the nerve growth factor receptor gene. Cell1987; 49: 589–94.
13.
BarkerDWrightTNguyenK. Gene for von Recklinghausen neurofibromatosis is in the pericentromeric region of chromosome 17. Science1987; 236: 1100–2.
14.
SorensenSAMulvihillJJNielsenA. Long-term follow-up of von Recklinghausen neurofibromatosis survival and malignant neoplasms. N Engl J Med1986; 314: 1010–5.