Neurofibromatosis 2 (NF2) and von Hippel-Lindau disease (VHL) are both autosomal dominantly inherited tumor syndromes. In these diseases, there is a high probability of tumors developing in the central nervous system, which cause vertigo and equilibrium disturbance in affected patients. An outline of these two tumor syndromes is presented in this review. In addition, the importance of genetic counseling especially for presymptomatic diagnosis is discussed.
