Superior Semicircular Canal Ampullae Dehiscence As Part of the Spectrum of the Third Window Abnormalities: A Case Study.
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SUBMITTER: Ionescu EC
PROVIDER: S-EPMC5742101 | biostudies-literature | 2017
REPOSITORIES: biostudies-literature
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Superior Semicircular Canal Ampullae Dehiscence As Part of the Spectrum of the Third Window Abnormalities: A Case Study.
Ionescu Eugen Constant EC Al Tamami Nasser N Neagu Alexandra A Ltaief-Boudrigua Aicha A Gallego Stephane S Hermann Ruben R Truy Eric E Thai-Van Hung H
Frontiers in neurology 20171219
A 60-year-old man was referred to the ENT department for intense episodic vertigo triggered by loud sounds. Pure tone audiometry and otoneurological assessment, including videonystagmography using auditory stimulation and cervical vestibular evoked myogenic potential measures, conducted to the hypothesis of a third window syndrome in the left ear. Results from the high-resolution computed tomography of the petrous bone confirmed the hypothesis and revealed the presence of a submillimeter semicir ...[more]
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Project description:To 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.study 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.Retrospective 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.The 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.
| S-EPMC5717502 | biostudies-literature
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.
| S-EPMC10604912 | biostudies-literature
Project description:Background and purposeThe complex anatomy of the temporal bone is difficult to understand and constitutes a challenge in the daily diagnostic routine even for experienced neuroradiologists. In the context of otoneurological (oVEMP) and preoperative diagnostics, the diagnosis of superior semicircular canal dehiscence (SSCD) is of great importance for Ear, Nose, and Throat (ENT) specialists. The gold standard for this diagnosis is a high-resolution CT (HRCT) of the temporal bone. In order to correctly diagnose SSCD, special oblique reconstructions are necessary in addition to standard (axial, coronal, sagittal) reconstructions. We evaluated the frequency of diagnosis and its location in HRCT in correlation with otoneurological examination. From this analysis, we present a new SSCD classification. This classification yields the potential of a differentiated analysis of the patient's clinical symptoms with correlation to the cross-sectional anatomy and may lead to a differentiated therapy approach.Study design and settingWe evaluated 1370 temporal bone scans of patients with residual hearing and verified 343 superior semicircular canal dehiscence (SSCD). We conducted a subgroup analysis of these 343 HRCT scans displaying a SSCD and used them as a basis to create a classification.ResultsThree location types of SSCD were identified. These were anterior type 1, superior type 2 and posterior type 3. Type 2 were significantly more frequent in both sexes. SSCD at this location can be overlooked if diagnosis is performed only in the standard axial plane, since it can only be visualized by means of double oblique reconstruction. We present a standardized reconstruction algorithm.ConclusionIn total, three types of SSCD with differing incidences can be extrapolated from the locations. Superior type 2 is the most frequent one. Both sexes are affected with roughly equal incidence. The use of standardized double oblique reconstruction algorithm ensures that all three types are diagnosed in the HRCT.
| S-EPMC8775191 | biostudies-literature
Project description:Surgical plugging to treat superior semicircular canal dehiscence (SCD) has been proven to impede the effect of the third mobile window, abating cochleovestibular symptoms. Knowledge of superior semicircular canal (SC)-plugging status has been proposed to serve as a guide for adjuvant treatment. Here, we investigated disturbances in the inner ear fluid space following SC plugging using a novel three-dimensional (3D) reconstruction-based method. This approach used a semi-automatic segmentation algorithm and a direct volume rendering method derived from conventional magnetic resonance images. The variable extents of filling defects at the sites of SC plugging and the positional relation of the defect to the ampulla and common crus were identified. The success group exhibited markedly reduced volumes following surgery, whereas the failure group displayed no changes in volume. These results indicate that the success or failure of SC plugging was related to 3D volume changes in the labyrinth fluid signal. Collectively, this study presents individualized SC-plugging statuses using a novel 3D reconstruction-based method and it facilitates future work regarding easy-to-measure 3D volume changes. This current technology also aids in the exploration of pathologic changes in various targets of interest.
| S-EPMC8352905 | biostudies-literature
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.
| S-EPMC5941760 | biostudies-literature
Project description:Superior semicircular canal dehiscence (SCD), which is characterized by a "third mobile window" in the inner ear, causes various vestibular and auditory symptoms and signs. Surgical plugging of the superior semicircular canal (SC) can eliminate the symptoms associated with increased perilymph mobility due to the presence of the third window. However, the natural course of vestibular function after surgical plugging remains unknown. Therefore, we explored longitudinal vestibular function after surgery in 11 subjects with SCD who underwent SC plugging using the middle cranial fossa approach. Changes in vestibulo-ocular reflex (VOR) gain in all planes were measured over 1 year with the video head impulse test. We also evaluated surgical outcomes, including changes in symptoms, audiometric results, and electrophysiological tests, to assess whether plugging eliminated third mobile window effects. The mean VOR gain for the plugged SC decreased from 0.81 ± 0.05 before surgery to 0.65 ± 0.08 on examinations performed within 1 week after surgery but normalized thereafter. Four of seven subjects who were able to perform both VOR tests before surgery and immediately after surgery had pathologic values (SC VOR gain < 0.70). Conversely, the mean VOR gain in the other canals remained unchanged over 1 year. The majority of symptoms and signs were absent or markedly decreased at the last follow-up evaluation, and no complications associated with the surgery were reported. Surgical plugging significantly attenuated the air-bone gap, in particular at low frequencies, because of increased bone conduction thresholds and deceased air conduction thresholds. Moreover, surgical plugging significantly increased vestibular-evoked myogenic potential thresholds and decreased the ratio of summating potential to action potential in plugged ears. Postoperative heavily T2-weighted images were available for two subjects and showed complete obliteration of the T2-bright signal intensity in the patent SC lumen in preoperative imaging based on filling defect at the site of plugging. Our results suggest that successful plugging of dehiscent SCs is closely associated with a transient, rather than persistent, disturbance of labyrinthine activity exclusively involved in plugged SCs, which may have clinical implications for timely and individualized vestibular rehabilitation.
| S-EPMC7385253 | biostudies-literature
Project description:CONCLUSION: In spite of its absence in the control population, there is questionable evidence for the alteration c.114C->T in the HMX3 gene being implicated in the development of superior semicircular canal dehiscence (SSCD). However, the concept of a complex disease is valid for SSCD and a possible molecular origin can neither be confirmed nor excluded by the results of this study. OBJECTIVES: SSCD was first described in 1998 by Minor et al. While the etiology is not clear, findings from both temporal bone CT and histologic studies suggest a congenital or developmental origin. In recent years, a couple of genes regulating inner ear morphogenesis have been described. Specifically, Netrin-1 and HMX3 have been shown to be critically involved in the formation of the SCC. Molecular alterations in these two genes might lead to a disturbed development of this canal and might represent an explanation for SSCD. METHODS: DNA was extracted from whole blood of 15 patients with SSCD. The coding sequences of Netrin-1 and HMX3 were amplified by PCR and sequenced. RESULTS: One sequence alteration, heterozygous c.114C->T (conservative change without alteration of amino acid) in exon 1 of HMX3, was detected in 2 of 15 patients but not in 300 control chromosomes. The study was supported in part by the Emilia-Guggenheim-Schnurr-Foundation, Basel, Switzerland.
| S-EPMC3477893 | biostudies-literature
Project description: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.
| S-EPMC9892720 | biostudies-literature
Project description:The purpose of this study was to evaluate the clinical and genetic findings of 53-year-old monozygotic twins who had bilateral otosclerosis and right-sided superior semicircular canal near dehiscence (SSCND). Monozygotic twins at the age of 53 presented with conductive hearing loss and normal tympanic membranes. Detailed audiovestibular testing and computed tomography scan revealed that both patients had concurrent otosclerosis and SSCND. Conservative management (hearing aids) was the treatment for these patients. Exome sequencing (ES) for the twins and their affected mother identified a heterozygous missense variant in the EYA4 (c.1744G>A; p.Glu582Lys) gene. This is the first case report to present these separate entities identified in monozygotic twins with a heterozygous missense variant in the EYA4 gene. Our ES data may imply a possible causal relationship or association between variants in the EYA4 gene and concurrent otosclerosis and SSCND.
| S-EPMC8996085 | biostudies-literature
Project description:We describe the clinical evaluation and operative management of posterior semicircular canal dehiscence caused by a high jugular bulb.We performed a retrospective case report.The patient had clinical and audiometric findings consistent with semicircular canal dehiscence and imaging findings that demonstrated erosion of the posterior semicircular canal by a high jugular bulb. Resurfacing of the eroded canal provided resolution of the vestibular symptoms without damage to the inner ear.Dehiscence of the posterior semicircular canal can cause clinical and audiometric findings similar to those of superior semicircular canal dehiscence syndrome. Resurfacing of the area of dehiscence can successfully relieve the vestibular symptoms. In the case of dehiscence of the posterior canal from a high jugular bulb, resurfacing may offer advantages over canal plugging for definitive management.
| S-EPMC3972486 | biostudies-literature