Horizontal semicircular canal jam: Two new cases and possible mechanisms.
Ontology highlight
ABSTRACT: Introduction
Benign paroxysmal positional vertigo (BPPV) of the horizontal semicircular canal (hSCC) can present with otoconia blocking its lumen (canalith jam), with signs and symptoms that make it difficult to distinguish from central nervous system pathology.Objective
Here we report two cases of canalith jam affecting the hSCC and offer a theoretical mechanism based on known vestibular neurophysiology.Methods
We use video-oculography to document the canalith jam and show the moment the otoconia loosen.Results
Canalith jam is a rare form of BPPV remedied with repositioning maneuvers.Conclusion
Clinicians should consider canalith jam as a mechanism for BPPV when the nystagmus is (a) Direction fixed with fixation removed and during positional testing; (b) Velocity dependent on supine head position; (c) Converts to geotropic directional changing nystagmus.
SUBMITTER: Schubert MC
PROVIDER: S-EPMC7042637 | biostudies-literature | 2020 Feb
REPOSITORIES: biostudies-literature
Publications
Horizontal semicircular canal jam: Two new cases and possible mechanisms.
Schubert Michael C MC Helminski Janet J Zee David S DS Cristiano Elisabetta E Giannone Antonio A Tortoriello Giuseppe G Marcelli Vincenzo V
Laryngoscope investigative otolaryngology 20200116 1
<h4>Introduction</h4>Benign paroxysmal positional vertigo (BPPV) of the horizontal semicircular canal (hSCC) can present with otoconia blocking its lumen (canalith jam), with signs and symptoms that make it difficult to distinguish from central nervous system pathology.<h4>Objective</h4>Here we report two cases of canalith jam affecting the hSCC and offer a theoretical mechanism based on known vestibular neurophysiology.<h4>Methods</h4>We use video-oculography to document the canalith jam and sh ...[more]
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Project description:Spontaneous canalith jam is an uncommon form of benign paroxysmal positional vertigo mimicking acute vestibular neuritis. We described for the first time a spontaneous horizontal semicircular canalith jam associated with a typical canalolithiasis involving contralateral posterior semicircular canal (PSC), illustrating how the latter condition modified direction-fixed nystagmus during head movements. An 81-year-old woman with persistent vertigo referred to our center. Video-Frenzel examination showed horizontal direction-fixed right-beating nystagmus in primary gaze position, inhibited by visual fixation. She exhibited corrective saccades after leftward head impulses. Chin-to-chest positioning at the head-pitch test did not modify spontaneous nystagmus, whereas slight torsional components with the top pole of the eye beating toward the right ear appeared in backward head-bending, resulting in mixed horizontal-torsional nystagmus. At supine positioning tests, direction-fixed nystagmus turned into direction-changing geotropic horizontal nystagmus, which was stronger on the left side, while overlapping upbeat nystagmus with torsional right-beating components appeared on the right. Primary clinical findings were consistent with a left horizontal semicircular canalith jam, inducing a persistent utriculofugal cupular displacement, combined with a typical right-sided PSC-canalolithiasis. Once canalith jam crumbled, resulting in a non-ampullary arm canalolithiasis of the horizontal semicircular canal, both involved canals were freed by debris with appropriate repositioning procedures.
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Project description:We describe a rare case of spontaneous upbeat nystagmus (UBN) attributable to a canalith jam involving the anterior semicircular canal (ASC) in a patient in whom comprehensive vestibular assessment was useful to identify the underlying pathomechanism. A 56-year-old woman with unsteadiness following repositioning procedures for left-sided benign paroxysmal positional vertigo (BPPV) presented with spontaneous UBN that showed slight right torsional components. A vestibular test battery detected isolated left ASC hypofunction on a video-head impulse test (Video-HIT). We postulated a persistent utriculopetal deflection of the left ASC cupula, which was attributable to entrapment of debris in a narrow canal tract, with consequent sustained inhibition of the ampullary afferents. Although spontaneous UBN receded after impulsive physical therapy, unsteadiness deteriorated into positional vertigo secondary to canalolithiasis involving the ipsilateral posterior canal. In our view, physical therapy possibly fragmented the canalith jam and released free-floating otoconia that eventually settled into the ipsilateral posterior canal. Video HIT revealed normalization of ASC hypofunction, and leftsided posterior canal canalolithiasis was successfully treated using appropriate repositioning procedures. We propose that a canalith jam involving the ASC should be considered in the differential diagnosis of spontaneous UBN, particularly in patients with a history of BPPV and isolated ASC hypofunction detected on video HIT.
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Project description:ObjectiveTo determine the use of multi-detector computed tomography (MDCT) in the diagnostic interpretation of superior semicircular canal dehiscence (SSCD) or thinning and its association with ear pathologies and to find whether it is an acquired condition and its association with increase in age.Materials and methodsstudy was performed in a tertiary care institute present in a village, following approval of the institutional ethical committee. Retrospective review of temporal bone CT examinations performed between September 2016 and March 2017 was done. 1 mm interval axial images with sagittal and coronal reformatted images were reviewed for the presence of canal dehiscence and thinning by investigators. We characterised the Superior semicircular canal status as normal, frank dehiscence or thinning. Frank dehiscence was further classified anatomically as anterior limb, apex and posterior limb dehiscence.The patient list was then subcategorized into 5 age groups, and the prevalence of SSCD was calculated for each group.ResultsRetrospective review yielded 80 positive cases which included SSC dehiscence (N = 39) and thinning (N = 41). 80 normal scans were selected as control group retrospectively. Statistical analysis was performed to assess for differences between the groups studied. Pearson chi-square test applied. there was a significant association of SSC pathologies prevalence with increasing age (p = < 0.001). No significant relationship was found between SSCD and presence of either CSOM or Cholesteatoma (p = 0.285). Vertigo rather than Tullio phenomenon was the statistically significant complaint (p = <0.001). which brought the patient to the hospital.ConclusionsThe SSCD and thinning belong to the same spectrum and are acquired conditions. Increasing prevalence in old age suggests it to be an acquired condition rather than a congenital one. No significant association of these condition was seen with CSOM and cholesteatoma. Vertigo is the predominat symptom bringing the patient to hospital along with Tullio phenomenon.
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Project description:ObjectiveThe transmastoid plugging of a superior semicircular canal is considered a safe and effective technique for the management of superior semicircular canal dehiscence (SSCD). The aim of this meta-analysis is to assess the postoperative hearing outcomes after the transmastoid plugging of the superior semicircular canal. Search method and data sources: A systematic database search was performed on the following databases until 30 January 2023: MEDLINE, Embase, Cochrane Library, Web of Science, CINAHL, ICTRP, and clinicaltrials.gov. A systematic literature review and meta-analysis of the pooled data were conducted. We also included a consecutive case series with SCDS for those who underwent transmastoid plugging treatment at our clinic.ResultsWe identified 643 citations and examined 358 full abstracts and 88 full manuscripts. A total of 16 studies were eligible for the systematic review and 11 studies for the meta-analysis. Furthermore, 159 ears (152 patients) were included. The postoperative mean air conduction threshold remained unchanged (mean difference, 2.89 dB; 95% CI: -0.05, 5.84 dB, p = 0.58), while the mean bone conduction threshold was significantly worse (mean difference, -3.53 dB; 95% CI, -6.1, -0.95 dB, p = 0.9).ConclusionThe transmastoid plugging technique for superior semicircular canal dehiscence syndrome, although minimally worsening the inner ear threshold, is a safe procedure in terms of hearing preservation and satisfactory symptom relief.
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Project description: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 various sizes and locations along the superior semicircular canal. Auditory brainstem responses (ABRs) and laser Doppler velocimetry were used to measure hearing loss and vibration changes before and after fenestration, as well as after restorative patching. ABR thresholds at low frequencies (e.g., 1000 Hz) increased after fenestration and decreased back to the normal range after we repaired the defect. Energy leakage from the surgically introduced third window was detected in the range of 300-1500 Hz, accompanied by increased vibration at the umbo, stapes head, and the dehiscence site, while decreased vibration was observed at the round window membrane in the same frequency range. After the patching procedure, the deviant vibrations were recovered. The degree of postfenestration energy leakage was proportional to the size of fenestration and the proximity of the fenestration site to the oval window. These results suggest that the bony fenestration of the superior semicircular canal mimics the hearing loss pattern of patients with SSCD. The decrease in perilymph wave impedance likely accounts for the auditory changes.
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Project description:BackgroundVestibular symptoms on sitting-up are frequent on patients seen by vestibular specialists. Recently, a benign paroxysmal positional vertigo (BPPV) variant which elicits vestibular symptoms with oculomotor evidence of posterior semicircular canal (P-SCC) cupula stimulation on sitting-up was described and named sitting-up vertigo BPPV. A periampullar restricted P-SCC canalolithiasis was proposed as a causal mechanism.ObjectiveTo describe new mechanisms of action for the sitting-up vertigo BPPV variant.MethodsEighteen patients with sitting-up vertigo BPPV were examined with a pre-established set of positional maneuvers and follow-up until they resolved their symptoms and clinical findings.ResultsAll patients showed up-beating torsional nystagmus (UBTN) and vestibular symptoms on coming up from either Dix-Hallpike (DHM) or straight head-hanging maneuver. Sixteen out of 18 patients presented a sustained UBTN with an ipsitorsional component to the tested side on half-Hallpike maneuver (HH). A slower persistent contratorsional down-beating nystagmus was found in eleven out 18 patients tested on nose down position (ND).ConclusionsPersistent direction changing positional nystagmus on HH and ND positions indicative of P-SCC heavy cupula was found in 11 patients. A sustained UBTN on HH with the absence of findings on ND, which is suggestive of the presence of P-SCC short arm canalolithiasis, was found on 5 patients. All patients were treated with canalith repositioning maneuvers without success, but they resolved their findings by means of Brandt-Daroff exercises. We propose P-SCC heavy cupula and P-SCC short arm canalolithiasis as two new putative mechanisms for the sitting-up vertigo BPPV variant.
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Project description:Located deep in the temporal bone, the semicircular canal is a subtle structure that requires a spatial coordinate system for measurement and observation. In this study, 55 semicircular canal and eyeball models were obtained by segmentation of MRI data. The spatial coordinate system was established by taking the top of the common crus and the bottom of the eyeball as the horizontal plane. First, the plane equation was established according to the centerline of the semicircular canals. Then, according to the parameters of the plane equation, the plane normal vectors were obtained. Finally, the average unit normal vector of each semicircular canal plane was obtained by calculating the average value of the vectors. The standard normal vectors of the and left posterior semicircular canal, superior semicircular canal and lateral semicircular canal were [-0.651, 0.702, 0.287], [0.749, 0.577, 0.324], [-0.017, -0.299, 0.954], [0.660, 0.702, 0.266], [-0.739, 0.588, 0.329], [0.025, -0.279, 0.960]. The different angles for the different ways of calculating the standard normal vectors of the right and left posterior semicircular canal, superior semicircular canal and lateral semicircular canal were 0.011, 0.028, 0.008, 0.011, 0.024, and 0.006 degrees. The technology for measuring the semicircular canal spatial attitudes in this study are reliable, and the measurement results can guide vestibular function examinations and help with guiding the diagnosis and treatment of BPPV.
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Project description:The semicircular canal system of vertebrates helps coordinate body movements, including stabilization of gaze during locomotion. Quantitative phylogenetically informed analysis of the radius of curvature of the three semicircular canals in 91 extant and recently extinct primate species and 119 other mammalian taxa provide support for the hypothesis that canal size varies in relation to the jerkiness of head motion during locomotion. Primate and other mammalian species studied here that are agile and have fast, jerky locomotion have significantly larger canals relative to body mass than those that move more cautiously.
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Project description:BackgroundSodium absorption by semicircular canal duct (SCCD) epithelial cells is thought to contribute to the homeostasis of the volume of vestibular endolymph. It was previously shown that the epithelial cells could absorb Na+ under control of a glucocorticoid hormone (dexamethasone) and the absorptive transepithelial current was blocked by amiloride. The most commonly-observed target of amiloride is the epithelial sodium channel (ENaC), comprised of the three subunits α-, β- and γ-ENaC. However, other cation channels have also been observed to be sensitive in a similar concentration range. The aim of this study was to determine whether SCCD epithelial cells absorb only Na+ or also K+ through an amiloride-sensitive pathway. Parasensory K+ absorption could contribute to regulation of the transduction current through hair cells, as found to occur via vestibular transitional cells [S. H. Kim and D. C. Marcus. Regulation of sodium transport in the inner ear. Hear.Res. doi:10.1016/j.heares.2011.05.003, 2011].ResultsWe determined the molecular and functional expression of candidate cation channels with gene array (GEO GSE6197), whole-cell patch clamp and transepithelial recordings in primary cultures of rat SCCD. α-, β- and γ-ENaC were all previously reported as present. The selectivity of the amiloride-sensitive transepithelial and cell membrane currents was observed in Ussing chamber and whole-cell patch clamp recordings. The cell membrane currents were carried by Na+ but not K+, but the Na+ selectivity disappeared when the cells were cultured on impermeable supports. Transepithelial currents across SCCD were also carried exclusively by Na+.ConclusionsThese results are consistent with the amiloride-sensitive absorptive flux of SCCD mediated by a highly Na+-selective channel, likely αβγ-ENaC. These epithelial cells therefore absorb only Na+ via the amiloride-sensitive pathway and do not provide a parasensory K+ efflux from the canals via this pathway. The results further provide caution to the culture of epithelial cells on impermeable surfaces.
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