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.

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

scholarly journals Case Report: Local Anesthesia Round Window Plugging and Simultaneous Vibrant Soundbridge Implant for Superior Semicircular Canal Dehiscence

2020 ◽  
Vol 11 ◽  
Author(s):  
Giulia Mignacco ◽  
Lorenzo Salerni ◽  
Ilaria Bindi ◽  
Giovanni Monciatti ◽  
Alfonso Cerase ◽  
...  
Keyword(s):  
Semicircular Canal ◽  
Round Window ◽  
Bone Conduction ◽  
Hearing Threshold ◽  
Vestibular Evoked Myogenic Potentials ◽  
Vibrant Soundbridge ◽  
Superior Semicircular Canal Dehiscence ◽  
Superior Semicircular Canal ◽  
The Right ◽  
Canal Dehiscence

The aim of the present study is to report the outcomes of round window reinforcement surgery performed with the application of a Vibrant Soundbridge middle ear implant (VSB; MED-EL) in a patient with superior semicircular canal dehiscence (SSCD) who presented with recurrent vertigo, Tullio phenomenon, Hennebert's sign, bone conduction hypersensitivity, and bilateral moderate to severe mixed hearing loss. Vestibular evoked myogenic potentials (VEMPs) and high-resolution computed tomography (HRCT) confirmed bilateral superior semicircular canal dehiscence while this was not seen in magnetic resonance imaging. The surgical procedure was performed in the right ear as it had worse vestibular and auditory symptoms, a poorer hearing threshold, and greatly altered HRCT and VEMPs findings. With local-assisted anesthesia, round window reinforcement surgery (plugging) with perichondrium was performed with simultaneous positioning of a VSB on the round window niche. At the one and 3 months follow-up after surgery, VSB-aided hearing threshold in the right ear improved to mild, and loud sounds did not elicit either dizziness or pain in the patient.

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 High-Resolution Three Dimensional MRI Findings After Plugging Surgery for Superior Semicircular Canal Dehiscence: A Case Report

2020 ◽  
Vol 17 (4) ◽  
Author(s):  
Ji Ye Lee ◽  
Ra Gyoung Yoon ◽  
Hyun Joon Shim
Keyword(s):  
High Resolution ◽  
Semicircular Canal ◽  
Three Dimensional ◽  
Bony Defect ◽  
3D Mri ◽  
Mri Findings ◽  
Superior Semicircular Canal Dehiscence ◽  
Superior Semicircular Canal ◽  
View Reconstruction ◽  
Canal Dehiscence

: Superior semicircular canal dehiscence (SSCD) is known as abnormal communication of the superior semicircular canal (SCC) to the intracranial space secondary to a bony defect in the canal. Patients who are subjected to surgical repair usually have intractable symptoms, and recently, plugging of SCC using a transmastoid approach has been widely recommended. In this report, we describe a case of incomplete plugging for SSCD in a 37-year-old woman, along with the high-resolution three dimensional magnetic resonance imaging (3D MRI) findings using Pöschl view reconstruction. Postoperative MRI of 3D T2-wieghted sampling perfection with application optimized contrasts using different flip angle evolution (SPACE) Pöschl plane demonstrated an incomplete plugging of the SCC with partially visible perilymphatic fluid in the posterior limb above the common crus. A 3D fluid-attenuated inversion recovery (FLAIR) sequence showed an enhancement involving the vestibule and SCC, suggesting labyrinthitis. Although there are few reports about incomplete plugging for SSCD, this case could demonstrate postoperative status and complication after plugging of SSCD using a high-resolution 3D MRI sequences with Pöschl view reconstruction.

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 Head rotation evoked tinnitus due to superior semicircular canal dehiscence

2009 ◽  
Vol 124 (3) ◽  
pp. 333-335 ◽  
Author(s):  
E-C Nam ◽  
R Lewis ◽  
H H Nakajima ◽  
S N Merchant ◽  
R A Levine
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Semicircular Canal ◽  
Head Rotation ◽  
Bony Defect ◽  
Case Presentation ◽  
Superior Semicircular Canal Dehiscence ◽  
Superior Semicircular Canal ◽  
The Right ◽  
Canal Dehiscence

AbstractIntroduction:Superior semicircular canal dehiscence affects the auditory and vestibular systems due to a partial defect in the canal's bony wall. In most cases, sound- and pressure-induced vertigo are present, and are sometimes accompanied by pulse-synchronous tinnitus.Case presentation:We describe a 50-year-old man with superior semicircular canal dehiscence whose only complaints were head rotation induced tinnitus and autophony. Head rotation in the plane of the right semicircular canal with an angular velocity exceeding 600°/second repeatedly induced a ‘cricket’ sound in the patient's right ear. High resolution temporal bone computed tomography changes, and an elevated umbo velocity, supported the diagnosis of superior semicircular canal dehiscence.Conclusion:In addition to pulse-synchronous or continuous tinnitus, head rotation induced tinnitus can be the only presenting symptom of superior semicircular canal dehiscence without vestibular complaints. We suggest that, in our patient, the bony defect of the superior semicircular canal (‘third window’) might have enhanced the flow of inner ear fluid, possibly producing tinnitus.

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