["Inner perilymph fistula" of the anterior semicircular canal. A new disease picture with recurrent attacks of vertigo]

Abstract

In 1998 Minor et al. described a new variant of perilymphatic fistula: the "superior canal dehiscence syndrome". This syndrome is clinically characterized by recurrent attacks of vertigo and oscillopsia induced by loud noises or stimuli that result in changes in intracranial or middle ear pressure. It is caused by a dehiscence of bone overlying the superior (anterior) semicircular canal. Due to this dehiscence, a third, mobile window (in addition to the round and oval windows) is formed, and changes in pressure are pathologically transduced to the anterior semicircular canal. Although the superior canal dehiscence syndrome is not a rare condition, no other cases have yet been reported. Therefore, we describe a typical patient who suffered for many years from recurrent attacks of vertigo and oscillopsia induced by coughing and Valsalva's maneuvers. High resolution temporal bone CT scan showed a defect in the bone overlying the left anterior semicircular canal. Three-dimensional eye movement recordings using the search coil technique and subsequent vector analysis demonstrated that the eye movements were induced by excitation of the left anterior semicircular canal. We conclude that superior canal dehiscence syndrome is an important differential diagnosis in patients suffering from symptoms of a perilymphatic fistula, especially since it can be successfully treated by "plugging" of the affected semicircular canal. Such patients are thus spared unnecessary surgery of the middle ear.