R. A. Yavor

Superior semicircular canal dehiscence simulating otosclerosis

This is a report of a patient with an air-bone gap, thought 10 years ago to be a conductive hearing loss due to otosclerosis and treated with a stapedectomy. It now transpires that the patient actually had a conductive hearing gain due to superior semicircular canal dehiscence. In retrospect for as long as he could remember the patient had experienced cochlear hypersensitivity to bone-conducted sounds so that he could hear his own heart beat and joints move, as well as a tuning fork placed at his ankle. He also had vestibular hypersensitivity to air-conducted sounds with sound-induced eye movements (Tullio phenomenon), pressure-induced nystagmus and low-threshold, high-amplitude vestibular-evoked myogenic potentials. Furthermore some of his acoustic reflexes were preserved even after stapedectomy and two revisions. This case shows that if acoustic reflexes are preserved in a patient with an air-bone gap then the patient needs to be checked for sound- and pressure-induced nystagmus and needs to have vestibular-evoked myogenic potential testing. If there is sound- or pressure-induced nystagmus and if the vestibular-evoked myogenic potentials are also preserved, the problem is most likely in the floor of the middle fossa and not in the middle ear, and the patient needs a high-resolution spiral computed tomography (CT) of the temporal bones to show this.

The click-evoked vestibulo-ocular reflex in superior semicircular canal dehiscence

The authors studied eye movement responses to loud (110dB) clicks in 4 patients with Tullio effect due to superior semicircular canal dehiscence and in 9 normal subjects, by averaging the electro-oculogram. All 4 patients had small (0.1–0.3 deg) but easily reproducible vertical vestibulo-ocular reflex eye movement responses to the clicks. Normal subjects had responses that were at least 10 times smaller. The click-evoked vestibulo-ocular reflex test is a simple, robust way to screen dizzy patients for symptomatic superior semicircular dehiscence.

Unilateral vestibular deafferentation causes permanent impairment of the human vertical vestibulo-ocular reflex in the pitch plane

Rapid, passive, unpredictable, low-amplitude (10–20°), high-acceleration (3000–000°/s2) head rota tions were used to study the vertical vestibulo-ocular reflex in the pitch plane (pitch-vVOR) after unilateral vestibular deafferentation. The results from 23 human subjects who had undergone therapeutic unilateral vestibular deafferentation were compared with those from 19 normals. All subjects were tested while seated in the upright position. Group means and two-tailed 95% confidence intervals are reported for the pitch-vVOR gains in normal and unilateral vestibular deafferented subjects. In normal subjects, at a head velocity of 125°/s the pitch-vVOR gains were: upward 0.89±0.06, down ward 0.91±0.04. At a head velocity of 200°/s, the pitchvVOR gains were: upward 0.92±0.06, downward 0.96±0.04. There was no significant up-down asymme try. In the 15 unilateral vestibular deafferented subjects who were studied more than 1 year after unilateral vestibular deafferentation, the pitch-vVOR was signifi cantly impaired. At a head velocity of 125°/s the pitchvVOR gains were: upward 0.67±0.11, downward 0.63 ± 0.07. At a head velocity of 200°/s, the pitch-vVOR gains were: upward 0.67±0.07, downward 0.58±0.06. There was no significant up-down asymmetry. The pitch-vVOR gain in unilateral vestibular deafferented subjects was significantly lower (P<0.05) than the pitch-vVOR gain in normal subjects at the same head velocities. These results show that total, permanent uni lateral loss of vestibular function produces a permanent symmetrical 30% (approximately) decrease in pitchv-VOR gain. This pitch-vVOR deficit is still present more than 1 year after deafferentation despite retinal slip velocities greater than 30°/s in response to head accelerations in the physiological range, indicating that compensation of pitch-vVOR function following unilat eral vestibular deafferention remains incomplete.

Posterior semicircular canal nystagmus is conjugate and its axis is parallel to that of the canal

Article abstract A patient with a postoperative fistula of the left posterior semicircular canal is presented. Negative pressure in the external ear canal produced upbeat-torsional nystagmus, which was recorded in three dimensions using binocular scleral search coils. The nystagmus was conjugate, without skew deviation, and its trajectory corresponded to the anatomic axis of the left posterior canal. The current study helps validate Ewald’s first law in humans: the axis of nystagmus should match the anatomic axis of the semicircular canal that generated it. This law is clinically useful in diagnosing pathology of the vestibular end-organ, such as benign paroxysmal positional vertigo or the superior semicircular canal dehiscence syndrome.

Compensation of the human vertical vestibulo-ocular reflex following occlusion of one vertical semicircular canal is incomplete

The vestibulo-ocular reflex (VOR) was studied in nine human subjects 2–15 months after permanent surgical occlusion of one posterior semicircular canal. The stimuli used were rapid, passive, unpredictable, low-amplitude (10–20°), high-acceleration (3000–4000°/s2) head rotations in pitch and yaw planes. The responses measured were vertical and horizontal eye rotations, and the results were compared with those from 19 normal subjects. After unilateral occlusion of the posterior semi-circular canal, the gain of the head-up pitch vertical VOR — the vertical VOR generated by excitation from only one and disfacilitation from two vertical semicircular canals — was reduced to 0.61±0.06 (normal 0.92±0.06) at a head velocity of 200°/s. In contrast the gain of the head-down pitch vertical VOR — the VOR still generated by excitation from two, but disfacilitation from only one vertical semicircular canal — was within normal limits: 0.86±0.11 (normal 0.96±0.04). The gain of the horizontal VOR in response to yaw head rotations — ipsilesion 0.81±0.06 (normal 0.88±0.05) and contralesion 0.80±0.11 (normal 0.92±0.11) — was within normal limits in both directions (group means ± two-tailed 95% confidence intervals given in each case). These results show that occlusion of just one vertical semicircular canal produces a permanent deficit of about 30% in the vertical VOR gain in response to rapid pitch head rotations in the excitatory direction of the occluded canal. This observation indicates that, in response to a stimulus in the higher dynamic range, compensation of the human VOR for the loss of excitatory input from even one vertical semicircular canal is incomplete.

The Effect of Unilateral Posterior Semicircular Canal Inactivation on the Human Vestibulo-Ocular Reflex

The responses to rapid, passive, unpredictable, low amplitude (10-20 degrees), high acceleration (3,000-4,000 degrees/s2) head rotations were used to study the human vestibulo-ocular reflex (VOR) in pitch and yaw plane after unilateral posterior semicircular canal occlusion (uPCO) in 10 subjects. The results from these 10 uPCO subjects were compared with those from 18 normal subjects. The VOR gains at a head velocity of 200 degrees/s in the uPCO subjects were: pitch upward = 0.62 +/- 0.06, pitch downward = 0.87 +/- 0.11, yew ipsilesion = 0.78 +/- 0.06, yaw contralesion = 0.79 +/- 0.10 and in normal subjects were: pitch upward = 0.92 +/- 0.06, pitch downward = 0.96 +/- 0.04, yaw right = 0.88 +/- 0.05, yaw left = 0.91 +/- 0.12 (group means +/- twotailed 95% confidence intervals). The results showed that the pitch-vVOR gain was significantly (p < 0.05) decreased in response to upward head impulses whereas in response to downward, ipsilesion and contralesion head impulses were not significantly different (p > 0.05) from the normals. This study shows that there is 30% permanent residual deficit of the upward pitch-vVOR with an up-down asymmetry in pitch-vVOR gain following inactivation of a single posterior semicircular canal and that compensation of pitch-vVOR function is incomplete.