Abstract:Defective acoustic transmission in the cochlea is closely related with various auditory and vestibular symptoms. Among them, semicircular canal dehiscence (SCD) with a defective semicircular bone is typical. Currently, the pathogenesis of SCD is usually explained by the third window hypothesis; however, this hypothesis fails to explain the variability in the symptoms and signs experienced by superior SCD (SSCD) patients. We evaluated the mechanism of hearing loss in a guinea pig model of bony dehiscence with v… Show more
“…The large (2 mm) SSCD used in our gerbil model resulted in electrophysiologic findings that mirror findings in patients with SSCD. Our animal model improves upon the fidelity to the human findings from previously reported models ( 10 , 11 ) and provides a more detailed view of the healing process and associated physiological findings. The advances are in several areas.…”
Section: Discussionmentioning
confidence: 77%
“…Because unilateral bone-conduction ABR thresholds in rodents cannot be measured, it is not possible to measure the pseudoconductive hearing loss, but the proxy finding of worsened ABR thresholds is adequate, particularly, when the hearing loss recovers once the surgically created SSCD recloses after bone regrowth. The two previous animal models tried to record cVEMP responses, but they did not appreciate that the sternocleidomastoid evoked potentials (cVEMP) are relaxation potentials, and this explains why the animal SSCD literature has no convincing cVEMP responses shown in the models published to date (10)(11)(12). In contrast, it is well-known that sound-induced otolithic neck extensor responses are excitatory potentials (13); therefore, we developed a c+VEMP method that demonstrates increased c+VEMP amplitudes and decreased c+VEMP thresholds after surgically creating a SSCD in our model, just as we see in patients with SSCD.…”
BackgroundThird window syndrome is a vestibular-cochlear disorder in humans in which a third mobile window of the otic capsule creates changes to the flow of sound pressure energy through the perilymph/endolymph. The nature and location of this third mobile window can occur at many different sites (or multiple sites); however, the most common third mobile window is superior semicircular canal dehiscence (SSCD). There are two essential objective diagnostic characteristics needed to validate a model of SSCD: the creation of a pseudoconductive hearing loss and cVEMP increased amplitude and decreased threshold.MethodsAdult Mongolian gerbils (n = 36) received surgical fenestration of the superior semicircular canal of the left inner ear. ABR and c+VEMP testing were carried out prior to surgery and over acute (small 1 mm SSCD, 1–10 days) or prolonged (large 2 mm SSCD, 28 days) recovery. Because recovery of function occurred quickly, condenser brightfield stereomicroscopic examination of the dehiscence site was carried out for the small SSCD animals post-hoc and compared to both ABRs and c+VEMPs. Micro-CT analysis was also completed with representative samples of control, day 3 and 10 post-SSCD animals.ResultsThe SSCD created a significant worsening of hearing thresholds of the left ear; especially in the lower frequency domain (1–4 kHz). Left (EXP)/right (CTL) ear comparisons via ABR show significant worsening thresholds at the same frequency representations, which is a proxy for the human pseudoconductive hearing loss seen in SSCD. For the c+VEMP measurements, increased amplitude of the sound-induced response (N1 2.5 ms and P1 3.2 ms) was observed in animals that received larger fenestrations. As the bone regrew, the c+VEMP and ABR responses returned toward preoperative values. For small SSCD animals, micro-CT data show that progressive osteoneogenesis results in resurfacing of the SSCD without bony obliteration.ConclusionThe large (2 mm) SSCD used in our gerbil model results in similar electrophysiologic findings observed in patients with SSCD. The changes observed also reverse and return to baseline as the SSCD heals by bone resurfacing (with the lumen intact). Hence, this model does not require a second surgical procedure to plug the SSCD.
“…The large (2 mm) SSCD used in our gerbil model resulted in electrophysiologic findings that mirror findings in patients with SSCD. Our animal model improves upon the fidelity to the human findings from previously reported models ( 10 , 11 ) and provides a more detailed view of the healing process and associated physiological findings. The advances are in several areas.…”
Section: Discussionmentioning
confidence: 77%
“…Because unilateral bone-conduction ABR thresholds in rodents cannot be measured, it is not possible to measure the pseudoconductive hearing loss, but the proxy finding of worsened ABR thresholds is adequate, particularly, when the hearing loss recovers once the surgically created SSCD recloses after bone regrowth. The two previous animal models tried to record cVEMP responses, but they did not appreciate that the sternocleidomastoid evoked potentials (cVEMP) are relaxation potentials, and this explains why the animal SSCD literature has no convincing cVEMP responses shown in the models published to date (10)(11)(12). In contrast, it is well-known that sound-induced otolithic neck extensor responses are excitatory potentials (13); therefore, we developed a c+VEMP method that demonstrates increased c+VEMP amplitudes and decreased c+VEMP thresholds after surgically creating a SSCD in our model, just as we see in patients with SSCD.…”
BackgroundThird window syndrome is a vestibular-cochlear disorder in humans in which a third mobile window of the otic capsule creates changes to the flow of sound pressure energy through the perilymph/endolymph. The nature and location of this third mobile window can occur at many different sites (or multiple sites); however, the most common third mobile window is superior semicircular canal dehiscence (SSCD). There are two essential objective diagnostic characteristics needed to validate a model of SSCD: the creation of a pseudoconductive hearing loss and cVEMP increased amplitude and decreased threshold.MethodsAdult Mongolian gerbils (n = 36) received surgical fenestration of the superior semicircular canal of the left inner ear. ABR and c+VEMP testing were carried out prior to surgery and over acute (small 1 mm SSCD, 1–10 days) or prolonged (large 2 mm SSCD, 28 days) recovery. Because recovery of function occurred quickly, condenser brightfield stereomicroscopic examination of the dehiscence site was carried out for the small SSCD animals post-hoc and compared to both ABRs and c+VEMPs. Micro-CT analysis was also completed with representative samples of control, day 3 and 10 post-SSCD animals.ResultsThe SSCD created a significant worsening of hearing thresholds of the left ear; especially in the lower frequency domain (1–4 kHz). Left (EXP)/right (CTL) ear comparisons via ABR show significant worsening thresholds at the same frequency representations, which is a proxy for the human pseudoconductive hearing loss seen in SSCD. For the c+VEMP measurements, increased amplitude of the sound-induced response (N1 2.5 ms and P1 3.2 ms) was observed in animals that received larger fenestrations. As the bone regrew, the c+VEMP and ABR responses returned toward preoperative values. For small SSCD animals, micro-CT data show that progressive osteoneogenesis results in resurfacing of the SSCD without bony obliteration.ConclusionThe large (2 mm) SSCD used in our gerbil model results in similar electrophysiologic findings observed in patients with SSCD. The changes observed also reverse and return to baseline as the SSCD heals by bone resurfacing (with the lumen intact). Hence, this model does not require a second surgical procedure to plug the SSCD.
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