Hierarchical Cluster Analysis of Semicircular Canal and Otolith Deficits in Bilateral Vestibulopathy.

Background: Gait imbalance and oscillopsia are frequent complaints of bilateral vestibular loss (BLV). Video-head-impulse testing (vHIT) of all six semicircular canals (SCCs) has demonstrated varying involvement of the different canals. Sparing of anterior-canal function has been linked to aminoglycoside-related vestibulopathy and Meniere's disease. We hypothesized that utricular and saccular impairment [assessed by vestibular-evoked myogenic potentials (VEMPs)] may be disease-specific also, possibly facilitating the differential diagnosis. Methods: We searched our vHIT database (n = 3,271) for patients with bilaterally impaired SCC function who also received ocular VEMPs (oVEMPs) and cervical VEMPs (cVEMPs) and identified 101 patients. oVEMP/cVEMP latencies above the 95th percentile and peak-to-peak amplitudes below the 5th percentile of normal were considered abnormal. Frequency of impairment of vestibular end organs (horizontal/anterior/posterior SCC, utriculus/sacculus) was analyzed with hierarchical cluster analysis and correlated with the underlying etiology. Results: Rates of utricular and saccular loss of function were similar (87.1 vs. 78.2%, p = 0.136, Fisher's exact test). oVEMP abnormalities were found more frequent in aminoglycoside-related bilateral vestibular loss (BVL) compared with Meniere's disease (91.7 vs. 54.6%, p = 0.039). Hierarchical cluster analysis indicated distinct patterns of vestibular end-organ impairment, showing that the results for the same end-organs on both sides are more similar than to other end-organs. Relative sparing of anterior-canal function was reflected in late merging with the other end-organs, emphasizing their distinct state. An anatomically corresponding pattern of SCC/otolith hypofunction was present in 60.4% (oVEMPs vs. horizontal SCCs), 34.7% (oVEMPs vs. anterior SCCs), and 48.5% (cVEMPs vs. posterior SCCs) of cases. Average (+/- 1 SD) number of damaged sensors was 6.8 +/- 2.2 out of 10. Significantly (p < 0.001) more sensors were impaired in patients with aminoglycoside-related BVL (8.1 +/- 1.2) or inner-ear infections (8.7 +/- 1.8) compared with Meniere-related BVL (5.5 +/- 1.5). Discussion: Hierarchical cluster analysis may help differentiate characteristic patterns of BVL. With a prevalence of approximate to 80%, utricular and/or saccular impairment is frequent in BVL. The extent of SCC and otolith impairment was disease-dependent, showing most extensive damage in BVL related to inner-ear infection and aminoglycoside-exposure and more selective impairment in Meniere's disease. Specifically, assessing utricular function may help in the distinction between aminoglycoside-related BVL and bilateral Meniere's disease.