Recent advances in superior semicircular canal dehiscence syndrome

2015 ◽  
Vol 129 (3) ◽  
pp. 217-225 ◽  
Author(s):  
G Chilvers ◽  
I McKay-Davies
Keyword(s):  
Semicircular Canal ◽  
Health Management ◽  
Case Reports ◽  
False Positive Rate ◽  
Case Series ◽  
Vestibular Evoked Myogenic Potential ◽  
Retrospective Case ◽  
Superior Semicircular Canal Dehiscence ◽  
Superior Semicircular Canal ◽  
Canal Dehiscence

AbstractObjective:This study aimed to review the current advances in superior semicircular canal dehiscence syndrome and to ascertain its aetiology, whether dehiscence size correlates with symptoms, signs and investigation results, the best investigations, and its surgical management.Methods:A literature search using the key words ‘superior semicircular canal dehiscence’ was performed using the Allied and Complementary Medicine Database and the Embase, Health Management Information Consortium, Medline, PsycINFO, British Nursing Index, Cinahl and Health Business Elite databases for the period January 2009 to May 2014. Systematic reviews, meta-analyses, randomised controlled trials, prospective and retrospective case series, case reports, and observational studies were included.Results:Of the 205 papers identified, 35 were considered relevant.Conclusion:The aetiology of superior semicircular canal dehiscence syndrome is unclear. Dehiscence size significantly affects the air–bone gap and ocular vestibular evoked myogenic potential thresholds. Computed tomography evaluation has a high false positive rate. The middle cranial fossa approach is the surgical standard for treating this syndrome; however, the transmastoid approach is gaining popularity.

scholarly journals Superior Semicircular Canal Dehiscence Syndrome: Review of Clinical Manifestations in Adults and Children

2009 ◽  
Vol 24 (2) ◽  
pp. 6-13
Author(s):  
Scheherazade C. Ibrahim ◽  
Charlotte M. Chiong ◽  
Nathaniel W. Yang
Keyword(s):  
Semicircular Canal ◽  
Clinical Manifestations ◽  
Case Series ◽  
Vestibular Evoked Myogenic Potential ◽  
Superior Semicircular Canal Dehiscence ◽  
Computed Tomographic ◽  
Myogenic Potential ◽  
Superior Semicircular Canal ◽  
Computed Tomographic Scan ◽  
Canal Dehiscence

Objective: This report aims to determine the clinical manifestations and management of patients with superior semicircular canal dehiscence syndrome (SSCDS). Methods: Study Design: Case series. Setting: Tertiary hospitals and private clinics Participants: Out of 30 patients with vestibular vertigo or otologic symptom, 14 patients were diagnosed with SSCDS based on high resolution computed tomographic scan (HRCT).  The demographic features, incidence of specific signs and symptoms and management of these patients were described, including the audiograms, vestibular evoked myogenic potential (VEMP) responses and ancillary tests. Results: Vertigo was the most common vestibular symptom of SSCDS. Tullio phenomenon was elicited in 50% of patients with confirmed dehiscence on HRCT scan. Low frequency (250 Hz and 500 Hz) air-bone gap was noted in 21.4% of patients. Lowered VEMP responses were also noted in 66.7% of patients with confirmed SSCDS. Severity of symptoms may determine its management. Conclusion: The diagnosis of SSCDS does not conform to a specific clinical presentation or audiologic result thus good clinical correlation is needed in order to raise suspicion of the disease and prompt the clinician to order confirmatory imaging by computed tomographic scan or magnetic resonance imaging. The presence of this syndrome in a proportion of children that is greater than previously reported needs further study as these children may be genetically predisposed to have thinned out superior semicircular canals that eventually become dehisced albeit at an earlier age. Key words: Superior semicircular canal dehiscence, pure tone audiometry, vestibular evoked myogenic potential

Sealing of superior semicircular canal dehiscence is associated with improved balance outcomes postoperatively versus plugging of the canal in middle fossa craniotomy repairs: a case series

2020 ◽  
Vol 133 (2) ◽  
pp. 462-466
Author(s):  
Vivian Wung ◽  
Prasanth Romiyo ◽  
Edwin Ng ◽  
Courtney Duong ◽  
Thien Nguyen ◽  
...  
Keyword(s):  
Semicircular Canal ◽  
Chart Review ◽  
Case Series ◽  
Superior Semicircular Canal Dehiscence ◽  
Superior Semicircular Canal ◽  
Postoperative Mri ◽  
Mri Scans ◽  
Postoperative Symptoms ◽  
Fluid Signal ◽  
Canal Dehiscence

OBJECTIVEThe authors compared postoperative symptoms between patients with sealed and those with plugged semicircular canal dehiscence repairs.METHODSIn total, 136 ears from 118 patients who underwent surgical repair for semicircular canal dehiscence were identified via chart review. Data from postoperative MRI scans showing preservation or loss of semicircular canal fluid signal and postoperative reports of autophony, amplification, aural fullness, tinnitus, hyperacusis, hearing loss, vertigo, dizziness, disequilibrium, oscillopsia, and headache were amalgamated and analyzed.RESULTSPatients with preservation of fluid signal were far less likely to have dizziness postoperatively (p = 0.007, OR 0.158, 95% CI 0.041–0.611). In addition, these patients were more likely to have tinnitus postoperatively (p = 0.028, OR 3.515, 95% CI 1.145–10.787).CONCLUSIONSThe authors found that superior semicircular canal dehiscence patients who undergo sealing without plugging have improved balance outcomes but show more tinnitus postoperatively than patients who undergo plugging.

Superior semicircular canal dehiscence postoperative outcomes: A case series of 156 repairs

2019 ◽  
Vol 68 ◽  
pp. 69-72 ◽  
Author(s):  
Prasanth Romiyo ◽  
Courtney Duong ◽  
Edwin Ng ◽  
Vivian Wung ◽  
Methma Udawatta ◽  
...  
Keyword(s):  
Semicircular Canal ◽  
Case Series ◽  
Postoperative Outcomes ◽  
Superior Semicircular Canal Dehiscence ◽  
Superior Semicircular Canal ◽  
Canal Dehiscence

Superior semicircular canal dehiscence simulating otosclerosis

2003 ◽  
Vol 117 (7) ◽  
pp. 553-557 ◽  
Author(s):  
G. Michael Halmagyi ◽  
Swee T. Aw ◽  
Leigh A. McGarvie ◽  
Michael J. Todd ◽  
Andrew Bradshaw ◽  
...  
Keyword(s):  
Semicircular Canal ◽  
Vestibular Evoked Myogenic Potentials ◽  
Patient Needs ◽  
Vestibular Evoked Myogenic Potential ◽  
Superior Semicircular Canal Dehiscence ◽  
Superior Semicircular Canal ◽  
Acoustic Reflexes ◽  
Temporal Bones ◽  
Conductive Hearing ◽  
Canal Dehiscence

This is a report of a patient with an air-bone gap, thought 10 years ago to be a conductive hearing loss due to otosclerosis and treated with a stapedectomy. It now transpires that the patient actually had a conductive hearing gain due to superior semicircular canal dehiscence. In retrospect for as long as he could remember the patient had experienced cochlear hypersensitivity to bone-conducted sounds so that he could hear his own heart beat and joints move, as well as a tuning fork placed at his ankle. He also had vestibular hypersensitivity to air-conducted sounds with sound-induced eye movements (Tullio phenomenon), pressure-induced nystagmus and low-threshold, high-amplitude vestibular-evoked myogenic potentials. Furthermore some of his acoustic reflexes were preserved even after stapedectomy and two revisions. This case shows that if acoustic reflexes are preserved in a patient with an air-bone gap then the patient needs to be checked for sound- and pressure-induced nystagmus and needs to have vestibular-evoked myogenic potential testing. If there is sound- or pressure-induced nystagmus and if the vestibular-evoked myogenic potentials are also preserved, the problem is most likely in the floor of the middle fossa and not in the middle ear, and the patient needs a high-resolution spiral computed tomography (CT) of the temporal bones to show this.

scholarly journals HOUT-22. TWO CASE REPORTS OF PATIENTS WITH SUPERIOR SEMICIRCULAR CANAL DEHISCENCE AND EHLERS-DANLOS SYNDROME

2018 ◽  
Vol 20 (suppl_6) ◽  
pp. vi117-vi118
Author(s):  
Komal Preet ◽  
Courtney Duong ◽  
Methma Udawatta ◽  
Vera Ong ◽  
Jacky Chen ◽  
...  
Keyword(s):  
Semicircular Canal ◽  
Case Reports ◽  
Ehlers Danlos Syndrome ◽  
Superior Semicircular Canal Dehiscence ◽  
Superior Semicircular Canal ◽  
Danlos Syndrome ◽  
Canal Dehiscence

scholarly journals Pediatric superior semicircular canal dehiscence: illustrative case and systematic review

2017 ◽  
Vol 20 (2) ◽  
pp. 196-203 ◽  
Author(s):  
Carlito Lagman ◽  
Vera Ong ◽  
Lawrance K. Chung ◽  
Lekaa Elhajjmoussa ◽  
Christina Fong ◽  
...  
Keyword(s):  
Hearing Aids ◽  
Semicircular Canal ◽  
Case Series ◽  
Signs And Symptoms ◽  
Illustrative Case ◽  
Search Term ◽  
Female Ratio ◽  
Superior Semicircular Canal Dehiscence ◽  
Superior Semicircular Canal ◽  
Canal Dehiscence

OBJECTIVEThe purpose of this study is to present an illustrative case of pediatric superior semicircular canal dehiscence (SSCD) and to systematically review the current published literature in the pediatric population.METHODSAn electronic search of the Scopus, Web of Science, PsycINFO, Cochrane, and Embase databases was performed by 2 independent authors through January 2017. Search term combinations included “pediatrics,” “children,” “canal,” and “dehiscence.” Inclusion criteria were as follows: English, full-text clinical studies, case reports, and case series describing pediatric patient(s) (younger than 18 years) with CT evidence of SSCD. Baseline patient demographic characteristics, clinical presentations, dehiscence characteristics, management strategies, and outcome data were extracted.RESULTSA total of 14 studies involving 122 patients were included in the quantitative synthesis. The patients’ mean age was 7.22 years. Male predominance was observed (approximate male-to-female ratio of 1.65:1). Neurodevelopmental disorders were common (n = 14, 11.5%). Auditory signs and symptoms were more common than vestibular signs and symptoms. Hearing loss (n = 62, 50.8%) was the most common auditory symptom and an indicator for imaging evaluation. Vertigo was the most common vestibular symptom (n = 8, 6.6%). Hearing aids were recommended in 8 cases (6.6%), and surgical repair was performed in 1 case (0.8%). Symptom outcomes and follow-up durations were infrequently reported.CONCLUSIONSThe authors’ data suggest that in pediatric SSCD, males are more commonly affected than females. This is different than the adult population in which females are predominantly affected. A history of otologic and/or neurodevelopmental abnormalities was common. There was a preponderance of auditory symptoms in this age group. Conservative management was favored in the majority.

scholarly journals Superior semicircular canal dehiscence syndrome: Diagnostic criteria consensus document of the committee for the classification of vestibular disorders of the bárány society

2021 ◽  
pp. 1-10
Author(s):  
Bryan K. Ward ◽  
Raymond van de Berg ◽  
Vincent van Rompaey ◽  
Alexandre Bisdorff ◽  
Timothy E. Hullar ◽  
...  
Keyword(s):  
High Resolution ◽  
Diagnostic Criteria ◽  
Semicircular Canal ◽  
Bone Conduction ◽  
Pure Tone Audiometry ◽  
Vestibular Evoked Myogenic Potential ◽  
Superior Semicircular Canal Dehiscence ◽  
Superior Semicircular Canal ◽  
Physiological Tests ◽  
Canal Dehiscence

This paper describes the diagnostic criteria for superior semicircular canal dehiscence syndrome (SCDS) as put forth by the classification committee of the Bárány Society. In addition to the presence of a dehiscence of the superior semicircular canal on high resolution imaging, patients diagnosed with SCDS must also have symptoms and physiological tests that are both consistent with the pathophysiology of a ‘third mobile window’ syndrome and not better accounted for by another vestibular disease or disorder. The diagnosis of SCDS therefore requires a combination of A) at least one symptom consistent with SCDS and attributable to ‘third mobile window’ pathophysiology including 1) hyperacusis to bone conducted sound, 2) sound-induced vertigo and/or oscillopsia time-locked to the stimulus, 3) pressure-induced vertigo and/or oscillopsia time-locked to the stimulus, or 4) pulsatile tinnitus; B) at least 1 physiologic test or sign indicating that a ‘third mobile window’ is transmitting pressure including 1) eye movements in the plane of the affected superior semicircular canal when sound or pressure is applied to the affected ear, 2) low-frequency negative bone conduction thresholds on pure tone audiometry, or 3) enhanced vestibular-evoked myogenic potential (VEMP) responses (low cervical VEMP thresholds or elevated ocular VEMP amplitudes); and C) high resolution CT imaging with multiplanar reconstruction in the plane of the superior semicircular canal consistent with a dehiscence. Thus, patients who meet at least one criterion in each of the three major diagnostic categories (symptoms, physiologic tests, and imaging) are considered to have SCDS.

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