Neurofibromatosis and secondary acoustic neuroma.

von Recklinghausen's disease, neurofibromatosis, is a neurocutaneous, systemic disease characterized by central nervous system tumors and disorders, café-au-lait spots, generalized cutaneous neurofibromas, skeletal deformities, and somatic and endocrine disorders. It is an autosomal dominant, hereditary disorder occurring in approximately 1 in 2500 births with a 50% affliction rate among family members. This patient had both systemic and ocular manifestations of the disease. She had neurofibromas of the face, neck, and iris, café-au-lait spots on neck and arms, short stature, hypothyroidism, and scoliosis. She reported occasional headaches of varying duration and intensity.