Objectives: To determine the diagnostic accuracy of ocular vestibular evoked myogenic potentials (oVEMPs) for superior canal dehiscence syndrome (SCDS) in a large cohort of unselected dizzy patients. Most SCDS patients are dizzy (90%); however, only 30% demonstrate archetypical SCDS clinical proxies (Tullio or Hennebert findings). Several case-control studies have addressed specific SCDS markers using VEMP testing, but the diagnostic value of VEMP for SCDS has not been demonstrated in a target population of dizzy patients. The aim of this study was to confirm the diagnostic properties of oVEMP for SCDS in an unselected cohort of dizzy patients. Design: This diagnostic accuracy study was performed in a tertiary referral center and included a consecutive sample of dizzy patients referred for vestibular function testing. One hundred fifty subjects were collected prospectively; five were excluded due to middle ear disorders, 10 had the target condition (SCDS group), and 135 had an alternative condition (non-SCDS group), based on diagnostic criteria for SCDS used in our department as reference standard. The non-SCDS group was subdivided into diagnostic categories including an “undefined dizziness” group. The index test applied to the total sample (missing data: 1%) consisted of oVEMP recording using three different stimulation modalities, that is, air-conducted (AC) sound stimulation and midsagittal bone-conducted (BC) vibration at both forehead (Fz) and vertex (Cz). Data analysis was conducted on four oVEMP parameters: amplitude, latency, amplitude asymmetry ratio, and interaural latency difference. Between-group analysis was conducted with nonparametric tests. The oVEMP diagnostic accuracy for SCDS was determined with uni/multiparametric receiver operating characteristic analysis. Best cutoff points were computed for those parameters or parameter combinations that showed an accuracy level appropriate for clinical use (area under the curve [AUC] > 0.8). Results: Different oVEMP parameters, in particular, the amplitude to AC stimulation (SCDS: 53, inter quartile range [IQR]: 27.6–68.3 µV; non-SCDS: 4.4, IQR: 2.0–8.1 µV; p < 0.001), were able to separate SCDS from non-SCDS conditions with statistical significance. AC oVEMP amplitude had the highest diagnostic accuracy (area under the curve = 0.96) for SCDS, with optimal sensitivity (100%) and high specificity (89%) at a specific cutoff point (16.7 µV); as an SCDS index, it could distinguish these patients not only from those with other vestibular diagnoses but also from patients with undefined dizziness (sensitivity 100%; specificity 81%). Conclusions: oVEMP was able to identify all subjects affected by SCDS, according to our diagnostic criteria, in a large cohort of unselected dizzy patients. The AC oVEMP amplitude parameter showed optimal sensitivity and high specificity for SCDS and may represent an ideal screening test for SCDS among dizzy patients. This is noteworthy when considering that not all SCDS patients express the clinical key features of vestibular hypersensitivity to sound or pressure change, even though most complain of dizziness.

Motor development in infants is dependent upon the function of the inner ear balance organ (vestibular organ). Vestibular failure causes motor delays in early infancy and suboptimal motor skills later on. A vestibular test for newborns and infants that is applicable on a large scale, safe and cost effective is in demand in various contexts: in the differential diagnosis of early onset hearing loss to determine forms associated with vestibular failure; in early hearing habilitation with cochlear implant, indicating the vestibular predominant side; and in the habilitation of children affected by motor skill disorders, revealing the contribution of a vestibular failure. This work explored the feasibility of cervical vestibular evoked myogenic potentials (VEMP) in conjunction with newborn universal hearing screening program. VEMP was measured after the hearing tests and was evoked by bone-conducted stimuli. Moreover, stimulus delivery was regulated by neck muscle activity, with infants rested unconstrained in their parents´ arms and with the head supported by the operator´s hand. This VEMP protocol showed a high level of feasibility in terms of test viability and result reproducibility. VEMP integrated into the newborn hearing screening program may represent a practical method for large-scale assessment of balance function in infants.

Introduction: The aim of this study was to develop a clinical test for body sounds’ hypersensitivity in superior canal dehiscence syndrome (SCDS). Method: Case-control study, 20 patients affected by SCDS and body sounds’ hypersensitivity and 20 control matched subjects tested with a new test called ankle audiometry (AA). The AA consisted of a psychoacoustic hearing test in which the stimulus was substituted by a controlled bone vibration at 125, 250, 500, and 750 Hz, delivered at the medial malleolus by a steel spring-attached bone transducer prototype B250. For each subject, it was defined an index side (the other being non-index), the one with major symptoms in cases or best threshold for each tested frequency in controls. In 3 patients, the AA was measured before and after SCDS surgery. Results: The AA thresholds for index side were significantly lower in SCDS patients (115.6 ± 10.5 dB force level [FL]) than in control subjects (126.4 ± 8.56 dB FL). In particular, the largest difference was observed at 250 Hz (−16.5 dB). AA thresholds in patients were significantly lower at index side in comparison with non-index side (124.2 ± 11.4 dB FL). The response obtained with 250 Hz stimuli outperformed the other frequencies, in terms of diagnostic accuracy for SCDS. At specific thresholds’ levels (120 dB FL), AA showed relevant sensitivity (90%) and specificity (80%) for SCDS. AA did not significantly correlate to other clinical markers of SCDS such as the bone and air conducted hearing thresholds and the vestibular evoked myogenic potentials. The AA thresholds were significantly modified by surgical intervention, passing from 119.2 ± 9.7 to 130.4 ± 9.4 dB FL in 3 patients, following their relief in body sounds’ hypersensitivity. Conclusion: AA showed interesting diagnostic features in SCDS with significantly lower hearing thresholds in SCDS patients when compared to healthy matched subjects. Moreover, AA could identify the affected or more affected side in SCDS patients, with a significant threshold elevation after SCDS surgery, corresponding in body sounds’ hypersensitivity relief. Clinically, AA may represent a first objective measure of body sounds’ hypersensitivity in SCDS and, accordingly, be an accessible screening test for SCDS in not tertiary audiological centers.

Background: Vestibular-evoked myogenic potential (VEMP) is commonly conducted with air-conducted (AC) stimuli whose intensity may exceed the prudential levels of acoustic exposure in susceptible subjects. Objectives: To determine the diagnostic accuracy of AC VEMP for superior canal dehiscence syndrome (SCDS) at lowered acoustic stimulation levels. Material and methods: VEMP was tested in 10 SCDS patients and in 10 age/sex matched control subjects. VEMP were recorded on cervical muscles (cVEMP) and extraocular muscles (oVEMP) in response to short AC tone bursts at 500, 1000 and 2000 Hz delivered at 80 dB nHL (103 dB peSPL). Parameters of interest were the response amplitude and the frequency tuning. Results: VEMP evoked by AC stimuli at safe acoustic stimulation levels did effectively separate SCDS patients from healthy controls. The separation was optimal at all the frequencies tested, however 500 Hz resulted the best VEMP tuning frequency, especially at oVEMP. Conclusions: Lowering the AC stimulation to the levels compatible with testing of patients susceptible to acoustic exposure didn’t affect the VEMP diagnostic properties for SCDS. Significance: SCDS may be screened by VEMP even among subjects susceptible for acoustic exposure.