Neurofibromatosis type 2 is associated with:: (A) B/L acoustic neuroma, (B) Cafe-au-lait spots, (C) Chromosome 22, (D) All
Answer is D. B/L acoustic neuromas are a hallmark of Neurofibromatosis 2 Neurofibromatosis Type 2 is an autosomal dominant highly penetrant condition Gene for NF-2 is located on chromosome 22q. Patients with NF2 present in second and third decade of life, rarely after the age of 60. M/C symptom/Presenting symptom = Hearing loss Skin tumors are present in nearly two thirds of patients of NF-2 -Current Otolaryngology 3/e