Abstract
To the Editor: Neurofibromatosis consists of at least two distinct autosomal dominant hereditary disorders.1 Von Recklinghausen's, or peripheral, neurofibromatosis is characterized by the development during the first and second decades of café au lait spots and neurofibromas of the skin,2 plus iris hamartomas, or Lisch nodules, of the eye.3 The hallmark of the second form, bilateral acoustic neurofibromatosis,4 is the development in the second and third decades of bilateral acoustic schwannomas.5 To our knowledge, eye changes in bilateral acoustic neurofibromatosis have yet to be described. Recently, two relatively young affected patients followed by us at the National Institutes of Health . . .