Pulse-synchronous eye oscillations revealing bone superior canal dehiscence

Abstract

Superior canal dehiscence syndrome is a newly recognized syndrome characterized by vertigo and nystagmus induced by sound (Tullio phenomenon) or changes of middle ear (Hennebert sign) or intracranial pressure. We report on a patient with bilateral superior canal dehiscence syndrome who presented with unusual manifestations including pulse-synchronous vertical pendular nystagmus and Valsalva-induced, up and counterclockwise-beating jerk nystagmus. These unusual symptoms may be a clue to a better understanding of the pathophysiology of superior canal dehiscence syndrome. Abnormal communication between the inner ears and the intracranial space may explain the vertical pendular and pulse-synchronous nystagmus, modulated by increased intracranial pressure.