Background: Patients with superior canal dehiscence syndrome may experience
vertigo and nystagmus when pressure changes occur in the external auditory
canal, the middle ear, or the intracranial space. The cause is a defect in
the bone of the superior canal.
Objective: To study the mechanisms of pressure sensitivity of the labyrinth
in superior canal dehiscence syndrome and its surgical repair in a chinchi
lla model.
Methods: We investigated the changes in firing rates of vestibular nerve af
ferents in the chinchilla in response to changes in external auditory canal
pressure before and after fenestration of the superior canal, and after re
pair of the fenestra.
Results: Before superior canal fenestration, external auditory canal pressu
re changes caused no responses in horizontal canal or otolith afferents, an
d only 1 of 9 superior canal afferents responded to pressure. After fenestr
ation, all superior canal afferents were excited by positive pressure and i
nhibited by negative pressure. Half of 18 otolith and most (21 of 33) horiz
ontal canal afferents were unaffected by pressure. The superior canal affer
ents had higher pressure gain than the horizontal canal afferents (P = .03)
. Pressure responses could be abolished only by applying a rigid seal to th
e fenestra.
Conclusions: Fenestration of the superior canal rendered all superior canal
afferents sensitive to pressure, whereas less than half of the other affer
ents became pressure sensitive. The direction of the superior canal afferen
t responses agreed with the predictions of our model of endolymph flow with
in the superior canal. A rigid seal applied to the fenestra abolished press
ure sensitivity while maintaining physiologic rotational sensitivity.