Case Report: Fremitus Nystagmus in Superior Canal Dehiscence Syndrome.
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SUBMITTER: Morrison M
PROVIDER: S-EPMC9124807 | biostudies-literature | 2022
REPOSITORIES: biostudies-literature
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Case Report: Fremitus Nystagmus in Superior Canal Dehiscence Syndrome.
Morrison Miranda M Korda Athanasia A Wagner Franca F Caversaccio Marco Domenico MD Mantokoudis Georgios G
Frontiers in neurology 20220509
Superior canal dehiscence syndrome (SCDS) is a structural bony defect of the roof of the superior semi-circular canal into the middle cranial fossa and is responsible for the creation of a third window, which alters the dynamics of the inner ear. During humming, vibratory waves entering the vestibulum and cochlea are re-routed through the dehiscence, leading to stimulation of the otolithic and ampullary vestibular organs. This is responsible for the torsional-vertical nystagmus known as "fremitu ...[more]
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Project description:The cochlear aqueduct (CA) connects the scala tympani to the subarachnoid space and is thought to assist in pressure regulation of perilymph in normal ears, however, its role and variation in inner ear pathology, such as in superior canal dehiscence syndrome (SCDS), is unknown. This retrospective radiographic investigation compared CA measurements and classification, as measured on flat-panel computerized tomography, among three groups of ears: controls, n = 64; anatomic superior canal dehiscence without symptoms (SCD), n = 28; and SCDS, n = 64. We found that in a multinomial logistic regression adjusted for age, sex, and BMI, an increase in CA length by 1 mm was associated with a lower odds for being in the SCDS group vs. control (Odds ratio 0.760 p = 0.005). Hierarchical clustering of continuous CA measures revealed a cluster with small CAs and a cluster with large CAs. Another multinomial logistic regression adjusted for the aforementioned clinical covariates showed an odds ratio of 2.97 for SCDS in the small CA cluster as compared to the large (p = 0.004). Further, no significant association was observed between SCDS symptomatology—vestibular and/or auditory symptoms—and CA structure in SCDS ears. The findings of this study lend support to the hypothesis that SCDS has a congenital etiology.
| S-EPMC10204506 | biostudies-literature
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:BackgroundIntraoperative identification of a superior semicircular canal (SSC) dehiscence via the middle cranial fossa approach (MCFA) remains a difficult endeavor without a neuronavigation system. To address these challenges, we propose a technique to localize the SSC dehiscence intraoperatively using certain anatomical landmarks.MethodThree anatomical landmarks should be identified on preoperative radiological images: the distance from the squamous part of the temporal bone to the dehiscent SSC, the lower limit of the craniotomy, and the exact location of the craniotomy in relation to the bony external auditory canal. The use of these landmarks intraoperatively can allow the surgeon to correctly identify the position of the SSC. Two instructional videos explaining this technique are presented.ConclusionThe proposed manual neuronavigation technique seems to be an accurate, safe, and cost-effective alternative technique for use in SSC dehiscence surgery.
| S-EPMC10098155 | 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:Outcomes following repair of superior canal dehiscence (SCD) are variable, and surgery carries a risk of persistent or recurrent SCD symptoms, as well as a risk of hearing loss and vestibulopathy. Poor outcomes may occur from inadequate repair of the SCD or mechanical insult to the membranous labyrinth. Repair of SCD using a customized, fixed-length prosthesis may address current operative limitations and improve surgical outcomes. We aim to 3-dimensionally print customized prostheses to resurface or occlude bony SCD defects. Dehiscences were created along the arcuate eminence of superior semicircular canals in cadaveric temporal bones. Prostheses were designed and created using computed tomography and a 3-dimensional printer. The prostheses occupied the superior semicircular canal defect, reflected in postrepair computed tomography scans. This novel approach to SCD repair could have advantages over current techniques. Refinement of prosthesis design and materials will be important if this approach is translated into clinical use.
| S-EPMC6939300 | 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.
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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.
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Project description:ObjectivePatients with superior canal dehiscence syndrome (SCDS) can present with a plethora of auditory and/or vestibular symptoms associated with a bony defect of the superior semicircular canal. While surgical repair is a reasonable option for patients with significant localizing symptoms, the degree of clinical improvement will vary among patients and poses challenges in outcome prediction. This study aims to assess the relationship between preoperative and postoperative symptoms and identify predictors of symptom persistence following repair.Study designRetrospective chart review.SettingTertiary neurotology single-institution care center.Main outcome measuresThe primary outcome was to determine the proportion of resolved and persistent primary (most bothersome) and non-primary audiologic and vestibular symptoms following SCD repair. Secondary outcomes included comparison of patient, operative and radiologic characteristics between patients with resolved vs. persistent symptoms. Standardized patient questionnaires including 11 auditory and 8 vestibular symptoms were administered to patients at their preoperative and follow-up visits. Patient pre- vs. postoperative survey results, demographic and clinical characteristics, operative characteristics, audiometric data and cervical vestibular evoked myogenic potential (cVEMP) thresholds were compared via univariate χ2 and multivariate binary logistic regression analyses between those patients reporting full postoperative resolution of symptoms and persistence of one or more symptoms. Radiologic computed tomography (CT) measurements of superior canal dehiscence (SCD) defect size, location, and laterality were also compared between these two groups.ResultsOf 126 patients (132 ears) included in our study, 119 patients (90.2%) reported postoperative resolution (n = 82, 62.1%) or improvement (n = 37, 28.0%) of primary (most bothersome) symptoms, while 13 patients (9.8%) reported persistence of primary symptoms. The median (interquartile range) and range between surgery and questionnaire completion were 9 (4-28), 1-124 months, respectively. Analyzing all symptoms (primary and non-primary) 69 (52.3%) and 68 (51.1%) patients reported complete postoperative auditory and vestibular symptom resolution, respectively. The most likely persistent symptoms included imbalance (33/65/67, 50.8%), positional dizziness (7/20, 35.0%) and oscillopsia (44/15, 26.7%). Factors associated with persistent auditory symptoms included history of seizures (0% vs. 7.6%, p = 0.023), auditory chief complaint (50.0% vs. 70.5%), higher PTA (mean 19.6 vs. 25.1 dB, p = 0.043) and higher cervical vestibular evoked myogenic potential (cVEMP) thresholds at 1000 Hz (mean 66.5 vs. 71.4, p = 0.033). A migraine diagnosis (14.0% vs. 41.9% p < 0.010), bilateral radiologic SCD (17.5% vs. 38.1%, p = 0.034) and revision cases (0.0% vs. 14.0%, p = 0.002) were associated with persistent vestibular symptoms. Neither SCD defect size nor location were significantly associated with symptom persistence (P > 0.05).ConclusionsSurgical repair for SCDS offers meaningful reduction in the majority of auditory and vestibular symptoms. However, the persistence of certain, mostly non-primary, symptoms and the identification of potential associated factors including migraines, PTA thresholds, cVEMP threshold, bilateral SCD, and revision cases emphasize the importance of individualized patient counseling and management strategies.
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Project description:Superior canal dehiscence syndrome (SCDS) can be treated surgically in patients with incapacitating symptoms. However, the ideal treatment has not been determined.This systematic literature review aims to assess available evidence on the comparative effectiveness and risks of different surgical treatments regarding: (1) symptom improvement; (2) objectively measurable auditory and vestibular function; (3) adverse effects, and (4) length of hospitalization.A systematic database search according to PRISMA statement was conducted on Pubmed, Embase, and Cochrane library. In addition, reference lists were searched. No correspondence with the authors was established. The last search was conducted on June 9, 2017.Retrospective and prospective cohort studies were held applicable under the condition that they investigated the association between a surgical treatment method and the relief of vestibular and/or auditory symptoms. Only studies including quantitative assessment of the pre- to postoperative success rate of a surgical treatment method were included. Case reports, reviews, meta-analysis, and studies not published in English, Dutch, or German were excluded.The first author searched literature and extracted data; the first and last analyzed the data.Seventeen studies (354 participants, 367 dehiscences) met the eligibility criteria and were grouped according to surgical approach. Seven combinations of surgical approaches and methods for addressing the dehiscence were identified: plugging, resurfacing, or a combination of both through the middle fossa (middle fossa approach); plugging, resurfacing, or a combination of both through the mastoid (transmastoid approach); round window reinforcement through the ear canal (transcanal approach). Several studies showed high internal validity, but quality was often downgraded due to study design (1). Outcome measures and timing of postsurgical assessment varied among studies, making it unfeasible to pool data to perform a meta-analysis.A standardized protocol including outcome measures and timeframes is needed to compare the effectiveness and safety SCDS treatments. It should include symptom severity assessments and changes in vestibular and auditory function before and after treatment.
| S-EPMC5523725 | biostudies-other