| Genetics and Deafness - Neurofibromatosis and Hearing Loss NFI - Neurofibromatosis Type I has also been called Von Recklinghausen's Disease (VRNF) or Peripheral Neurofibromatosis. It used to be said that John Merrick, "The Elephant Man", had VRNF, but now it is felt that he had a different condition called Proteus syndrome, which explains why his condition was so much more severe than is usually seen with NFI. NFI is the most common type of NF. About one in every 3,000 newborns have NFI, and it accounts for 90% of the people with Neurofibromatosis. In about half of the cases of NFI, the person with NFI is the only one in their family with the gene; in other words, a normal gene changed ("mutated") to produce an NFI gene. Genes often change, but usually they are harmless changes that we never notice. Occasionally, however, a change in a gene will produce a disease, and for some reason the "normal" gene is particularly susceptible to changes which produce an NFI gene. | |
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