Toward Optimizing cVEMP: 2,000-Hz Tone Bursts Improve the Detection of Superior Canal Dehiscence.

BACKGROUND: The cervical vestibular evoked myogenic potential (cVEMP) test measures saccular and inferior vestibular nerve function. The cVEMP can be elicited with different frequency stimuli and interpreted using a variety of metrics. Patients with superior semicircular canal dehiscence (SCD) syndrome generally have lower cVEMP thresholds and larger amplitudes, although there is overlap with healthy subjects. The aim of this study was to evaluate which metric and frequency best differentiate healthy ears from SCD ears using cVEMP. METHODS: Twenty-one patients with SCD and 23 age-matched controls were prospectively included and underwent cVEMP testing at 500, 750, 1,000 and 2,000 Hz. Sound level functions were obtained at all frequencies to acquire threshold and to calculate normalized peak-to-peak amplitude (VEMPn) and VEMP inhibition depth (VEMPid). Third window indicator (TWI) metrics were calculated by subtracting the 250-Hz air-bone gap from the ipsilateral cVEMP threshold at each frequency. Ears of SCD patients were divided into three groups based on CT imaging: dehiscent, thin or unaffected. The ears of healthy age-matched control subjects constituted a fourth group. RESULTS: Comparing metrics at all frequencies revealed that 2,000-Hz stimuli were most effective in differentiating SCD from normal ears. ROC analysis indicated that for both 2,000-Hz cVEMP threshold and for 2,000-Hz TWI, 100% specificity could be achieved with a sensitivity of 92.0%. With 2,000-Hz VEMPn and VEMPid at the highest sound level, 100% specificity could be achieved with a sensitivity of 96.0%. CONCLUSION: The best diagnostic accuracy of cVEMP in SCD patients can be achieved with 2,000-Hz tone burst stimuli, regardless of which metric is used.

Revisión sobre los conocimientos actuales de dehiscencia del canal semicircular posterior / Review of current knowledge of the dehiscence in the semicircular posterior canal

La dehiscencia del canal semicircular posterior es una patología rara y con baja incidencia, por ello hemos realizado una revisión de los conocimientos actuales de esta entidad. Se ha realizado una búsqueda bibliográfica desde 1998 hasta diciembre de 2016 de toda la literatura publicada sobre la misma en las bases de datos Allied and Complementary Medicine Database and the Embase, Health Management Information Consortium, Scopus, Consortium, Medline, PsycINFO y Scielo. Se han encontrado y revisado 53 trabajos relacionados con el tema. La dehiscencia del canal semicircular posterior presenta una prevalencia variable; 0,3%-4,5% en adultos y 1,2%-20% en niños. Su localización puede ser hacia el golfo de la yugular o fosa cerebral posterior. Los pacientes pueden ser asintomáticos o presentar clínica auditiva y/o vestibular. La tomografía computarizada y la prueba de potenciales vestibulares miogénicos evocados permiten establecer el diagnóstico de certeza. En el tratamiento quirúrgico la vía de abordaje de elección es la transmastoidea y las técnicas del cierre del canal son el "plugging" y el "resurfacing".

The posterior semicircular canal dehiscence is a rare pathology and it has a low incidence. We have realized a review about the current knowledge of this entity. We have performed a bibliographic research from 1998 to 2016 December about the literature published in this subject, in the data basis Allied and Complementary Medicine Database and the Embase, Health Management Information Consortium, Scopus, Consortium, Medline, PsycINFO y Scielo. I thas been found and reviewed 53 papers about the topic. The posterior semicircular canal dehiscence has a variable prevalence: 0,3%-4-5% in adults and 1,2%-20% in children. The location can be in the jugular bulb or in the posterior brain fossa. Some patients can be asymptomatic, whereas others can have auditory and/or vestibular signs and symptoms. Computed tomography and test of vestibular evoked myogenic potentials allow the diagnosis of certainty. In the surgical treatment the approach of choice is transmastoid and techniques to close the canal are plugging and resurfacing.

Upbeat nystagmus after bilateral superior canal plugging: A peripheral cause of vertical nystagmus.

Vertical nystagmus without a torsional component is generally considered a finding indicative of central nervous system pathology. We report two cases of purely upbeat nystagmus elicited with mastoid vibration after bilateral superior canal plugging, to highlight the vestibular pathophysiology involved in this unusual peripheral cause for upbeat nystagmus. Laryngoscope, 127:1698-1700, 2017.

Lateral Semicircular Canal Asymmetry in Idiopathic Scoliosis: An Early Link between Biomechanical, Hormonal and Neurosensory Theories?

INTRODUCTION: Despite its high incidence and severe morbidity, the physiopathogenesis of adolescent idiopathic scoliosis (AIS) is still unknown. Here, we looked for early anomalies in AIS which are likely to be the cause of spinal deformity and could also be targeted by early treatments. We focused on the vestibular system, which is suspected of acting in AIS pathogenesis and which exhibits an end organ with size and shape fixed before birth. We hypothesize that, in adolescents with idiopathic scoliosis, vestibular morphological anomalies were already present at birth and could possibly have caused other abnormalities. MATERIALS AND METHODS: The vestibular organ of 18 adolescents with AIS and 9 controls were evaluated with MRI in a prospective case controlled study. We studied lateral semicircular canal orientation and the three semicircular canal positions relative to the midline. Lateral semicircular canal function was also evaluated by vestibulonystagmography after bithermal caloric stimulation. RESULTS: The left lateral semicircular canal was more vertical and further from the midline in AIS (p = 0.01) and these two parameters were highly correlated (r = -0.6; p = 0.02). These morphological anomalies were associated with functional anomalies in AIS (lower excitability, higher canal paresis), but were not significantly different from controls (p>0.05). CONCLUSION: Adolescents with idiopathic scoliosis exhibit morphological vestibular asymmetry, probably determined well before birth. Since the vestibular system influences the vestibulospinal pathway, the hypothalamus, and the cerebellum, this indicates that the vestibular system is a possible cause of later morphological, hormonal and neurosensory anomalies observed in AIS. Moreover, the simple lateral SCC MRI measurement demonstrated here could be used for early detection of AIS, selection of children for close follow-up, and initiation of preventive treatment before spinal deformity occurs.

Superior canal dehiscence length and location influences clinical presentation and audiometric and cervical vestibular-evoked myogenic potential testing.

Superior canal dehiscence (SCD) is caused by an absence of bony covering of the arcuate eminence or posteromedial aspect of the superior semicircular canal. However, the clinical presentation of SCD syndrome varies considerably, as some SCD patients are asymptomatic and others have auditory and/or vestibular complaints. In order to determine the basis for these observations, we examined the association between SCD length and location with: (1) auditory and vestibular signs and symptoms; (2) air conduction (AC) loss and air-bone gap (ABG) measured by pure-tone audiometric testing, and (3) cervical vestibular-evoked myogenic potential (cVEMP) thresholds. 104 patients (147 ears) underwent SCD length and location measurements using a novel method of measuring bone density along 0.2-mm radial CT sections. We found that patients with auditory symptoms have a larger dehiscence (median length: 4.5 vs. 2.7 mm) with a beginning closer to the ampulla (median location: 4.8 vs. 6.4 mm from ampulla) than patients with no auditory symptoms (only vestibular symptoms). An increase in AC threshold was found as the SCD length increased at 250 Hz (95% CI: 1.7-4.7), 500 Hz (95% CI: 0.7-3.5) and 1,000 Hz (95% CI: 0.0-2.5), and an increase in ABG as the SCD length increased at 250 Hz (95% CI: 2.0-5.3), 500 Hz (95% CI: 1.6-4.6) and 1,000 Hz (95% CI: 1.3-3.3) was also seen. Finally, a larger dehiscence was associated with lowered cVEMP thresholds at 250 Hz (95% CI: -4.4 to -0.3), 500 Hz (95% CI: -4.1 to -1.0), 750 Hz (95% CI: -4.2 to -0.7) and 1,000 Hz (95% CI: -3.6 to -0.5) and a starting location closer to the ampulla at 250 Hz (95% CI: 1.3-5.1), 750 Hz (95% CI: 0.2-3.3) and 1,000 Hz (95% CI: 0.6-3.5). These findings may help to explain the variation of signs and symptoms seen in patients with SCD syndrome.

An indicator of probable semicircular canal dehiscence: ocular vestibular evoked myogenic potentials to high frequencies.

The n10 component of the ocular vestibular evoked myogenic potential (oVEMP) to sound and vibration stimuli is a crossed response that has enhanced amplitude and decreased threshold in patients with CT-verified superior semicircular canal dehiscence (SSCD). However, demonstrating enhanced VEMP amplitude and reduced VEMP thresholds requires multiple trials and can be very time consuming and tiring for patients, so a specific indicator of probable SCD that is fast and not tiring would be preferred. Here we report a 1-trial indicator: that the oVEMP n10 in response to a very high frequency stimulus (4000 Hz), either air-conducted sound (ACS) or bone conducted vibration (BCV), is such a fast indicator of probable SCD. In 22 healthy subjects, oVEMP n10 at 4000 Hz was not detectable; however, in all 22 CT-verified SSCD patients tested, oVEMP n10 responses were clearly present to 4000 Hz to either ACS or BCV stimuli.

Air-conducted oVEMPs provide the best separation between intact and superior canal dehiscent labyrinths.

OBJECTIVE: First, to define the best single-step suprathreshold screening test for superior canal dehiscence syndrome (SCDS); second, to obtain further insight into the relative sensitivity of vestibular afferents to sound vibration in the presence of a superior canal dehiscence. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS: Eleven patients with surgically confirmed SCDS (mean, 50 yr; range, 32-66 yr) and 11 age-matched, healthy subjects (right ear only) with no hearing or vestibular deficits (mean, 50 yr; range, 33-66 yr). INTERVENTION: All subjects completed ocular and cervical vestibular evoked myogenic potential (o- and cVEMP) testing in response to air conduction (click and 500 Hz tone burst) and midline bone conduction (reflex hammer and Mini-shaker) stimulation. MAIN OUTCOME MEASURES: OVEMP n10 amplitude and cVEMP corrected peak-to-peak amplitude. RESULTS: OVEMP n10 amplitudes were significantly higher in SCDS when compared with healthy controls in response to all stimuli with the exception of reflex hammer. Likewise, cVEMP-corrected peak-to-peak amplitudes were significantly higher in SCDS when compared with healthy controls for air conduction stimulation (click and 500 Hz toneburst). However, there were no significant differences between groups for midline taps (reflex hammer or mini-shaker). Receiver operating characteristic curves demonstrated that oVEMPs in response to air conduction stimulation provided the best separation between SCDS and healthy controls. CONCLUSION: OVEMPs in response to air conduction stimulation (click and 500 Hz toneburst) provide the best separation between SCDS and healthy controls and are therefore the best single-step screening test for SCDS.

Ocular versus cervical VEMPs in the diagnosis of superior semicircular canal dehiscence syndrome.

OBJECTIVES: To determine whether cervical vestibular evoked myogenic potential (cVEMP) thresholds or ocular VEMP (oVEMP) amplitudes are more sensitive and specific in the diagnosis of superior semicircular canal dehiscence syndrome (SCDS). STUDY DESIGN: Prospective case-control study. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Twenty-nine patients with SCDS (mean age 48 yr; range, 31-66 yr) and 25 age-matched controls (mean age 48 yr; range, 30-66 yr). INTERVENTION(S): cVEMP and oVEMP in response to air-conducted sound. All patients underwent surgery for repair of SCDS. MAIN OUTCOME MEASURE(S): cVEMP thresholds; oVEMP n10 and peak-to-peak amplitudes. RESULTS: cVEMP threshold results showed sensitivity and specificity ranging from 80% to 100% for the diagnosis of SCDS. In contrast, oVEMP amplitudes demonstrated sensitivity and specificity greater than 90%. CONCLUSION: oVEMP amplitudes are superior to cVEMP thresholds in the diagnosis of SCDS.

Tuning of the ocular vestibular evoked myogenic potential (oVEMP) to air- and bone-conducted sound stimulation in superior canal dehiscence.

Recent studies have demonstrated the frequency selectivity of air-conducted (AC) and bone-conducted (BC) stimuli in eliciting ocular vestibular evoked myogenic potentials (oVEMPs). In this study, frequency tuning of the oVEMP was assessed in patients with superior canal dehiscence (SCD) and compared with responses previously reported for healthy subjects. Six (five unilateral) SCD patients were stimulated using AC sound (50-1,200 Hz) and BC transmastoid vibration (50-1,000 Hz). Stimuli were delivered at two standardized intensities: one the same as previously used for healthy controls and the other at 10 dB above vestibular threshold (a similar relative intensity to that used in controls). For AC stimulation, SCD patients had larger oVEMP amplitudes across all frequencies tested for both stimulus intensities. Normalized tuning curves demonstrated greater high-frequency responses with the stronger stimulus. For BC stimulation, larger oVEMP amplitudes were produced at frequencies at and above 100 Hz using standard intensity stimuli. For the matched intensity above vestibular threshold, enhancement of the oVEMP response was present in SCD patients for 500-800 Hz only. We conclude that SCD causes greater facilitation for AC than BC stimuli. The high-frequency response is likely to originate from the superior (anterior) canal and is consistent with models of inner ear changes occurring in SCD.

Effectiveness of different click stimuli in diagnosing superior canal dehiscence using cervical vestibular evoked myogenic potentials.

CONCLUSION: Testing cervical vestibular evoked myogenic potential (cVEMP) in response to 90 dB nHL clicks can, in contrast to high-intensity 500 Hz tone bursts, be used as a screening test for superior canal dehiscence (SCD) syndrome. OBJECTIVES: cVEMP testing has its key clinical significance for evaluating saccular and inferior vestibular nerve function, but also for assessment of vestibular hypersensitivity to sounds in patients with SCD syndrome. The routine stimulus used in cVEMP testing is high-intensity 500 Hz tone bursts. The aim of the present study was to compare the high-intensity tone burst stimulation with less intense click stimulations for the diagnosis of SCD syndrome. METHODS: cVEMP amplitudes in response to 500 Hz tone bursts and clicks were studied in 38 patients with SCD syndrome unilaterally. RESULTS: cVEMP testing using high-intensity 500 Hz tone bursts did not consistently distinguish SCD patients. This nonfunctioning of high-intensity 500 Hz stimulation is most likely due to saturation. With 90 and 80 dB nHL clicks there is low risk for saturation and both these click stimulations were effective. Testing with both 80 and 90 dB nHL clicks did not have any significant advantage over just using 90 nHL dB clicks.

Use of the loud sound stimulation test in diagnosis of semicircular canal dehiscence syndrome.

Semicircular canal dehiscence (SCD) syndrome is rare, and its diagnosis is a significant challenge in clinical practice. Our aim was to explore application of the loud sound stimulation test for diagnosing SCD syndrome. Eight cases of superior semicircular canal dehiscence (SSCD), among them two patients had bilateral dehiscences and one case of lateral semicircular canal dehiscence (LSCD). A total of 11 dehiscences were studied retrospectively. Loud sounds (pure tones, 100 dB, 110 dB nHL) at frequencies of 500, 1,000, and 2,000 Hz were used to stimulate both ears for 5 s. A temporal bone computed tomography (CT) scan with semicircular canal reconstruction was performed in all patients. Vertigo was present in seven of nine cases following loud sound stimulation. In addition, the patient with LSCD demonstrated horizontal eye movement following loud sound stimulation, whereas six patients with SSCD showed rotational eye movement. Among them, two patients with bilateral superior canal dehiscence showed a positive response to the loud sound stimulation in only one ear. The diagnoses of all patients were confirmed with a high-resolution temporal bone CT with corresponding multi-planar reconstruction of the affected semicircular canals with various size dehiscences. We conclude that the characteristic eye movement following loud sound stimulation is valuable for diagnosing SCD syndrome. In addition, the loud sound stimulation test has unique advantages, especially for confirming the affected ear and the corresponding semicircular canal.

A superior semicircular canal dehiscence-induced air-bone gap in chinchilla.

An SCD is a pathologic hole (or dehiscence) in the bone separating the superior semicircular canal from the cranial cavity that has been associated with a conductive hearing loss in patients with SCD syndrome. The conductive loss is defined by an audiometrically determined air-bone gap that results from the combination of a decrease in sensitivity to air-conducted sound and an increase in sensitivity to bone-conducted sound. Our goal is to demonstrate, through physiological measurements in an animal model, that mechanically altering the superior semicircular canal (SC) by introducing a hole (dehiscence) is sufficient to cause such an air-bone gap. We surgically introduced holes into the SC of chinchilla ears and evaluated auditory sensitivity (cochlear potential) in response to both air- and bone-conducted stimuli. The introduction of the SC hole led to a low-frequency (<2000 Hz) decrease in sensitivity to air-conducted stimuli and a low-frequency (<1000 Hz) increase in sensitivity to bone-conducted stimuli resulting in an air-bone gap. This result was consistent and reversible. The air-bone gaps in the animal results are qualitatively consistent with findings in patients with SCD syndrome.

Sound-induced vertigo due to bone dehiscence of the lateral semicircular canal.

Dehiscence of the lateral semicircular canal (LSCD) has been reported much but mainly in association with cholesteatoma and canal wall down mastoidectomy, while idiopathic LSCD was rarely reported. Bassim reported one case with lateral semicircular canal dehiscence, but presented no vestibular or auditory symptoms. The patient in this study complained significant sound-induced vertigo and autophony in his right ear. The axis of nystagmus was orthogonal to the lateral semicircular canal, and no torsional or vertical motions were observed, so pathology of the lateral semicircular canal was preferentially considered. Benign paroxysmal positional vertigo was excluded since vertigo attacks had no relation to the change of head position. The dehiscence of the right lateral semicircular canal was then confirmed through the high-resolution temporal bone computer tomography scan and the reconstructed images. The cause of the LSCD is poorly understood, since no history of head trauma, otological infection or surgery was documented.

Postmaneuver restrictions in benign paroxysmal positional vertigo: an individual patient data meta-analysis.

OBJECTIVE: Treatment of benign paroxysmal positional vertigo (BPPV) with the Epley and Semont maneuvers has been used with and without postmaneuver postural restrictions. Studies examining these restrictions have yielded differing results. This study sought to examine the studies for a more uniform conclusion. DATA SOURCES: Controlled studies with objective identification of unilateral posterior semicircular canal BPPV and symptom resolution were analyzed. A PubMed search identified six studies totaling 523 patients meeting all inclusion criteria. REVIEW METHODS: Cohorts treated with and without restrictions were compared. Individual patient pooled analyses in a one-stage comparison were used for the meta-analysis. RESULTS: Pooled results of all restriction types showed no advantage over no restriction. Studies including each individual restriction were examined against the pooled population to look for technique differences. No restriction was found to be statistically significant. CONCLUSIONS: The restrictions examined in controlled trials did not differ significantly in clinical outcomes, which suggests that restrictions do not appear to significantly affect the efficacy of BPPV maneuvers.

Cervical vestibular evoked myogenic potentials (cVEMPs) in patients with superior canal dehiscence syndrome (SCDS).

OBJECTIVE: To determine the usefulness of both amplitude and threshold data from tone-burst cervical vestibular evoked myogenic potential (cVEMP) testing for the evaluation of superior canal dehiscence syndrome (SCDS). STUDY DESIGN: Case series with chart review. SUBJECTS AND METHODS: Sixty-seven patients underwent cVEMP testing. We correlated mean tone burst cVEMP amplitude and threshold data with temporal bone CT findings. Patients were excluded for Ménière's disease, middle ear disease, or otologic surgery. RESULTS: Superior canal dehiscence patients had higher mean cVEMP amplitudes (SCDS 173.8 microV vs non-SCDS 69.7 microV, P=0.031) and lower mean thresholds (SCDS 72.8 dB nHL vs non-SCDS 80.9 dB nHL) at 500 Hz. CONCLUSION: Patients with SCDS have larger amplitudes and lower thresholds on cVEMP testing at 500 Hz. This study supports the utility of tone burst cVEMPs for the evaluation of SCDS and is one of few large single-center studies to establish normative data.

Vestibular-evoked myogenic potentials and caloric tests in infants with congenital rubella.

UNLABELLED: Vestibular-evoked myogenic potentials and caloric tests in infants with congenital rubella. OBJECTIVE: Congenital rubella is rare due to maternal vaccination, but vestibular function can be markedly impaired in affected infants. Currently, vestibular testing is not routinely performed in infants. This study evaluated inner ear function in infants with congenital rubella. METHODOLOGY: The study included 54 3-month-old infants: 40 healthy controls and 14 with congenital rubella that was serologically confirmed in mothers and infants. There were two subgroups of infants with congenital rubella: 6 infants with clinical symptoms of intrauterine rubella infection present at birth and 8 asymptomatic infants. Caloric testing and vestibular-evoked myogenic potential (VEMP) testing were performed on all of the infants. RESULTS: In congenital rubella there was no reaction to caloric stimulation in 43% of the ears, and no VEMPs were recorded in 64%. The hearing thresholds were below 80 dB hearing level in 21% of the ears and greater than 80 dB hearing level in 43% of the ears. CONCLUSIONS: The degree of vestibular organ impairment in infants with congenital rubella is higher in individuals with profound hearing loss. VEMPs were absent more frequently than were caloric responses. Vestibular testing should be routinely performed in all infants with congenital rubella.

Testing vestibular-evoked myogenic potentials with 90-dB clicks is effective in the diagnosis of superior canal dehiscence syndrome.

Vestibular-evoked myogenic potentials (VEMP) in response to 90-dB-nHL clicks were studied in 20 patients (22 ears) with superior canal dehiscence syndrome. Their amplitude was compared to the VEMP from the 'unaffected' ears of 113 patients using the same stimulus level. The 113 control subjects were those from a previous study on 1,000 patients who had had large VEMP amplitudes in response to 500-Hz 129-dB-SPL tone bursts, and, because of this, had been tested with 90-dB-nHL clicks (which are a much weaker sound stimulus than our routine 500-Hz tone burst). It was found that 90-dB-nHL clicks clearly distinguished patients with vestibular hypersensitivity to sounds. In patients, the VEMP amplitude was usually larger than the simultaneously recorded background electromyographic activity (i.e. 'corrected' amplitude >1), whereas this was not the case for the controls. Consequently, it is suggested that 90-dB-nHL clicks can be used to screen for vestibular hypersensitivity to sounds. This finding has clinical implications for patients with suspected Tullio phenomenon because the definitive VEMP test for this (i.e. estimation of VEMP threshold) is not only time-consuming, but there is also difficulty related to the low signal-to-noise ratio close to the threshold.

Saccular function less affected than canal function in bilateral vestibulopathy.

Bilateral vestibulopathy (BV) is characterized by impaired or lost function of both labyrinths or eighth nerves. The diagnosis is routinely established by the head-thrust test, caloric irrigation and rotational testing with electronystagmography to determine the high- and low-frequency deficit of the vestibulo-ocular reflex. All three methods evaluate semicircular canal function only. Vestibular-evoked myogenic potentials (VEMPs) provide a measure of saccular otolith function. The aim of this study was to evaluate the frequency and extent of saccular dysfunction in patients with BV and to correlate saccular with horizontal semicircular canal dysfunction. A total of 84 BV-patients (23 females, mean age 62 +/- 15 (SD) years at the time of diagnosis assessment) were examined with VEMPs, electronystagmography with caloric irrigation and a standardized neuro-ophthalmological and -otological examination; 47 healthy subjects (18 females, mean 56 +/- 19 years) served as controls. Amplitudes P1-N1 were significantly lower in patients with BV compared to controls (mean P1-N1 of all ears 82.1 +/- 50.7 microV in the patients vs. 130.8 +/- 85.9 microV in healthy volunteers). VEMPs were absent unilaterally in four patients with BV and in none bilaterally. In contrast, caloric responses were absent bilaterally in 40 patients. There was no correlation between amplitude P1-N1 and caloric-induced nystagmus. The latencies P1 and N1 were not significantly different between patients and controls. Thus, in our study population saccular function appeared to be less affected than horizontal semicircular canal function.

Vestibular-evoked myogenic potential thresholds normalize on plugging superior canal dehiscence.

BACKGROUND: Diagnosis of the superior canal dehiscence syndrome (SCDS) relies on symptoms such as sound- or pressure-induced vertigo or oscillopsia, demonstration of sound or pressure-evoked vertical/torsional eye movements, and the presence of a defect in the bony roof overlying the superior semicircular canal. Lowered thresholds for eliciting vestibular-evoked myogenic potentials (VEMPs) provide additional conformation. OBJECTIVE: To examine VEMP characteristics before and after canal plugging for SCDS. METHODS: VEMPs evoked by air- and bone-conducted tones were measured from the sternocleidomastoid muscles (cVEMP) and periocular sites (oVEMP) of 20 normal volunteers, 10 newly diagnosed subjects with SCDS, and 12 subjects who underwent successful superior canal plugging. RESULTS: In all SCDS ears, thresholds for evoking VEMP using air-conducted tones were pathologically lowered, with average values of 83.85 +/- 1.40 dB sound pressure level (SPL) for cVEMP and 85.38 +/- 1.32 dB SPL for oVEMP, 20 to 30 dB below those of controls. Successful canal plugging resulted in normal reflex thresholds. For bone vibration, average thresholds in SCDS ears were 114.62 +/- 1.54 dB FL (force level) for cVEMP and 116.0 +/- 1.52 dB FL for oVEMP, 10 to 20 dB below controls, yet three SCDS ears had normal thresholds. CONCLUSIONS: Ocular and cervical vestibular-evoked myogenic potentials evoked by air-conducted sound are equally useful in the diagnosis and follow-up of superior canal dehiscence syndrome. Stimulus thresholds are consistently lowered upon presentation and normalize after corrective surgery. Thresholds for bone vibration, in contrast, have a lower diagnostic yield.

Ocular vestibular evoked myogenic potentials in superior canal dehiscence.

OBJECTIVE: Patients with superior canal dehiscence (SCD) have large sound-evoked vestibular reflexes with pathologically low threshold. We wished to determine whether a recently discovered measure of the vestibulo-ocular reflex-the ocular vestibular evoked myogenic potential (OVEMP)-produced similar high-amplitude, low-threshold responses in SCD, and could differentiate patients with SCD from normal control patients. METHODS: Nine patients with CT-confirmed SCD and 10 normal controls were stimulated with 500 Hz, 2 ms tone bursts and 0.1 ms clicks at intensities up to 142 dB peak SPL. Conventional VEMPs were recorded from the ipsilateral sternocleidomastoid muscle to determine threshold, and OVEMPs were recorded from electrode pairs placed superior and inferior to the eyes. Three-dimensional eye movements were measured with scleral dual-search coils. RESULTS: In patients with SCD, OVEMP amplitudes were significantly larger than normal (p<0.001) and thresholds were pathologically low. The n10 OVEMP in the contralateral inferior electrode became particularly large with increasing stimulus intensity (up to 25 microV) and with up-gaze (up to 40 microV). Sound-evoked (slow-phase) eye movements were present in all patients with SCD (vertical: upward; torsional: upper pole away from the affected side; and horizontal: towards or away from the affected side), but began only as the OVEMP response became maximal, which is consistent with the surface potentials being produced by activation of the extraocular muscles that generated the eye movements. CONCLUSIONS: OVEMP amplitude and threshold (particularly the contralateral inferior n10 response) differentiated patients with SCD from normal controls. Our findings suggest that both the OVEMPs and induced eye movements in SCD are a result of intense saccular activation in addition to superior canal stimulation.