Pulsatile tinnitus associated with congenital central nervous system malformations

Pulsatile tinnitus as a manifestation of congenital central nervous system malformations has not been previously described. We present two patients with type I Arnold-Chiari malformation and one patient with congenital stenosis of the sylvian aqueduct with pulsatile tinnitus. Pulsatile tinnitus in these patients is believed to be secondary to increased intracranial pressure. Tinnitus subsided in one patient with Arnold-Chiari malformation after suboccipital decompression. The other two patients declined surgery. Audiologic and radiologic findings are presented, and our diagnostic algorithm for pulsatile tinnitus is described. The pathophysiology of pulsatile tinnitus can be a manifesting symptom in some patients with congenital central nervous system malformations.