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John P Carey - One of the best experts on this subject based on the ideXlab platform.
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Superior Semicircular Canal dehiscence syndrome diagnostic criteria consensus document of the committee for the classification of vestibular disorders of the barany society
Journal of Vestibular Research-equilibrium & Orientation, 2021Co-Authors: Bryan K Ward, Timothy E Hullar, Miriam S Welgampola, Raymond Van De Berg, Vincent Van Rompaey, Alexandre Bisdorff, John P CareyAbstract:This paper describes the diagnostic criteria for Superior Semicircular Canal dehiscence syndrome (SCDS) as put forth by the classification committee of the Barany 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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Superior Semicircular Canal dehiscence syndrome diagnostic criteria consensus document of the committee for the classification of vestibular disorders of the barany society
Journal of Vestibular Research-equilibrium & Orientation, 2021Co-Authors: Bryan K Ward, Timothy E Hullar, Miriam S Welgampola, Raymond Van De Berg, Vincent Van Rompaey, Alexandre Bisdorff, John P CareyAbstract:This paper describes the diagnostic criteria for Superior Semicircular Canal dehiscence syndrome (SCDS) as put forth by the classification committee of the Barany 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 computed tomography (CT) scan 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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flat panel computed tomography in the diagnosis of Superior Semicircular Canal dehiscence syndrome
Otology & Neurotology, 2019Co-Authors: Alexandra Tunkel, John P Carey, Monica S PearlAbstract:Hypothesis Flat panel computed tomography (FPCT) provides more accurate measurements of dimensions for Superior Semicircular Canal dehiscence (SCD) than multislice CT (MSCT). Background SCD syndrome occurs when a bony defect of the Superior Semicircular Canal causes vestibular and auditory symptoms. MSCT can overestimate the size of the Canal defect, with possible over-diagnosis of SCD and suboptimal selection of surgical approach. The higher resolution of FPCT should afford more accurate measurements of these defects. Methods Radiographic and surgical measurements were compared in 22 patients (mean age 49.4) with clinical SCD syndrome and Canal defects confirmed at surgery. Twenty second FPCT scans were acquired before surgery with parameters: 109Kv, small focus, 200 degrees rotation angle, and 0.4 degree per frame angulation step. Dehiscence dimensions were measured from orthogonal multiplanar reconstructions on a high-resolution liquid crystal display monitor and compared with actual measurements recorded during microsurgery. Results SCD dimensions by FPCT (x) were 2.8 ± 1.6 mm for length and 0.72 ± 0.28 mm for width. The surgical measurements (y) were 2.8 ± 1.7 mm for length and 0.72 ± 0.34 mm for width. Linear fits between x and y yielded R values of 0.93 (length) and 0.66 (width). Our previous study using MSCT had R values of 0.28 (length) and 0.48 (width). The average difference between each FPCT and corresponding surgical measurement was not significantly different from zero, whereas the results for MSCT were significantly different. Conclusion FPCT can provide more accurate measurements of SCD than MSCT. Clinicians should consider using FPCT for imaging suspected SCD.
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surgical treatment of Superior Semicircular Canal dehiscence syndrome
2019Co-Authors: Francis X Creighton, John P CareyAbstract:Superior Semicircular Canal dehiscence syndrome (SCDS) is a clinical entity resulting in a myriad of audiological and vestibular symptoms. Pressure and/or sound-induced vertigo/nystagmus, autophony, conductive hearing loss, and conductive hyperacusis are commonly seen in patients with SCDS. The physiologic mechanism of this syndrome is thought to be due to the dehiscence creating a low-impedance outlet for fluid waves in the labyrinth, commonly referred to as a third window. This shunts flow from the cochlea to the labyrinth, which both activates the vestibular system and decreases pressure driving the traveling fluid wave in the cochlea. Diagnosis of SCDS can be difficult and requires both radiographic evidence of a dehiscence and clinical evidence supporting SCDS as the etiology for a patient’s symptoms. Testing for SCDS includes high-resolution CT imaging, audiogram, cervical and ocular VEMP testing, head impulse testing, and visualization of sound- or pressure-induced eye movements in the plane of the affected Superior Canal. Traditional surgical repair of SCDS is via the middle fossa approach. This approach allows for the dehiscence to be seen directly and for it to be both plugged and resurfaced. This approach is preferred in the vast majority of patients, but in certain situations a transmastoid approach can offer benefits over the middle fossa approach. Surgical outcomes for repair of SCDS are quite favorable, with patients having improvement of autophony, imbalance, and vertigo. Patients with predominately audiological symptoms have the highest likelihood of postoperative improvement. The majority of patients report an improvement in their quality of life postoperatively. Complications are rare but do occur in both the middle fossa and transmastoid approaches to SCDS repair. The most common complication is hearing loss, which is typically mild but can be profound in a low percentage of patients. The importance of proper patient selection and preoperative counseling on the risks of surgery cannot be overstated to ensure good surgical outcomes in SCDS repair.
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a cohort study of hearing outcomes between middle fossa craniotomy and transmastoid approach for surgical repair of Superior Semicircular Canal dehiscence syndrome
Otology & Neurotology, 2018Co-Authors: Lisa Zhang, Francis X Creighton, Bryan K Ward, Stephen Bowditch, John P CareyAbstract:Objective:To compare postoperative hearing outcomes between transmastoid and middle fossa craniotomy (MFC) approaches for surgical repair of Superior Semicircular Canal dehiscence syndrome (SCDS) in a tertiary referral center.Study Design:Historical cohort study.Setting:Tertiary referral center.Pati
Isaac Yang - One of the best experts on this subject based on the ideXlab platform.
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Superior Semicircular Canal dehiscence outcomes in a consecutive series of 229 surgical repairs with middle cranial fossa craniotomy
World Neurosurgery, 2021Co-Authors: Khashayar Mozaffari, Michael Johanis, Tyler Miao, Courtney Duong, Shelby Willis, Ansley Unterberger, Michelle K Hong, Russell De Jong, Mahlet Mekonnen, Isaac YangAbstract:BACKGROUND Superior Semicircular Canal dehiscence (SSCD) is the appearance of a third mobile window between the middle fossa and the Superior Semicircular Canal. Surgical management is indicated in patients with persistent and debilitating symptoms. The purpose of this study was to evaluate the association between preoperative variables that may impact postoperative symptomatic resolution. METHODS A single-institution retrospective analysis was performed on patients who were surgically treated for SSCD. Patients were divided to different cohorts based on unilateral or bilateral nature of the disease. A p-value <0.05 was considered statistically significant. RESULTS A total of 229 surgical repairs were analyzed. Mean age was 51 years (± 7.8 years), and females made up 55.9% of patients. All cohorts were similar with respect to baseline demographics. The most commonly reported preoperative symptoms were tinnitus, dizziness and autophony. The greatest symptomatic resolution was seen in autophony, internal sound amplification, hyperacusis, and oscillopsia. The unilateral SSCD cohort had significantly higher improvement of autophony (p=0.003), aural fullness (p=0.05), tinnitus (p=0.006), hearing loss (p=0.02), dizziness (p=0.006), and headache (p=0.007), compared to the bilateral SSCD cohorts. Among patients with bilateral disease, those with unilateral surgery reported greater symptomatic resolution with respect to hyperacusis (p=0.03), hearing loss (p=0.02), dizziness (p=0.03), and disequilibrium (p<0.001), than those with bilateral operations. CONCLUSION Surgical management of SSCD leads to high rates of postoperative symptomatic improvement. Patients with unilateral SSCD benefit greater symptomatic resolution compared to those with bilateral pathology.
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ct evaluation of normal bone thickness overlying the Superior Semicircular Canal
Journal of Clinical Neuroscience, 2019Co-Authors: Taranjit Kaur, Michael Johanis, Tyler Miao, Prasanth Romiyo, Courtney Duong, Matthew Z Sun, Regan Ferraro, Noriko Salamon, David L Mcarthur, Isaac YangAbstract:Abstract Superior Semicircular Canal dehiscence (SSCD) is a rare inner ear disorder with variable amounts of auditory and vestibular dysfunction. In addition to the absence of bone overlying the Superior Semicircular Canal, thinning of bone in this area can also initiate the vestibulocochlear symptoms of SSCD. We evaluated normal bone thickness overlying the course of the Semicircular Canal using computed tomography (CT) scans and assessed correlations between bone thickness and age, gender, and location of the thinnest bone. A single-institution retrospective chart review was conducted on 133 high-resolution CT scans from 76 healthy, asymptomatic patients between ages 9 and 96 years. These CT scans of the temporal bone were obtained between January 2012 and August 2017. The Superior Semicircular Canal dome thickness at the apex was reported with a mean of 1.25 mm for all 76 patients; the 10th percentile was 0.60 mm, and the 90th percentile was 2.08 mm. The thinnest area of bone at any location yielded a mean of 0.86 mm. The normal bone thickness overlying the Superior Semicircular Canal does not depend on gender or age. The thinnest location was evenly distributed across the Superior Semicircular Canal. A bone thickness of 0.40 mm or greater was present in 90% of normal patients based on CT scan measurements at the thinnest location.
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incidence of intraoperative hearing loss during middle cranial fossa approach for repair of Superior Semicircular Canal dehiscence
Journal of Clinical Neuroscience, 2018Co-Authors: Michael Johanis, Isaac Yang, Quinton GopenAbstract:Abstract Introduction Superior Semicircular Canal dehiscence is a rare inner ear disorder characterized by an abnormal third opening between the Superior Semicircular Canal and middle fossa. Symptoms include amplification of internal sounds, aural fullness, tinnitus, hearing loss, autophony, sound-induced vertigo (Tullio phenomenon), pressure-induced vertigo (Hennebert sign), disequilibrium, nystagmus, oscillopsia, and headache. While no cure exists for SSCD, surgical treatment has proven to effectively minimize these symptoms. This study reviewed brainstem auditory evoked potentials (BAEPs) that were monitored intraoperatively to better understand hearing loss risks associated with surgical treatment for SSCD. Methods A retrospective chart review was conducted at the University of California, Los Angeles on adult patients with a confirmed diagnosis of SSCD who had undergone a middle cranial fossa repair from March 2011 to October 2017. A total of 142 cases of SSCD in 118 patients were repaired. Results The majority of patients’ BAEPs remained stable and had no intraoperative hearing changes (n = 135; 95.1%). Seven patients experienced intraoperative changes as determined by a prolongation and reduction of Wave V latency (4.9%). Of these seven cases, five experienced a return to baseline prior to the end of surgery, and had no post-operative changes in hearing (71.4%). Overall, only two of the 142 surgeries (1.4%) resulted in failure to normalize and, as such, these patients experienced permanent changes in hearing. Conclusion The results of this retrospective review demonstrate a low risk for hearing loss due to SSCD surgery via the middle fossa craniotomy approach.
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middle cranial fossa approach for the repair of Superior Semicircular Canal dehiscence is associated with greater symptom resolution compared to transmastoid approach
Acta Neurochirurgica, 2018Co-Authors: Thien Nguyen, Quinton Gopen, Prasanth Romiyo, Courtney Duong, Carlito Lagman, John P Sheppard, Giyarpuram N Prashant, Isaac YangAbstract:Background Superior Semicircular Canal dehiscence (SSCD) is a disorder of the skull base that is gaining increasing recognition among neurosurgeons. Traditionally, the middle cranial fossa (MCF) approach has been used for the surgical repair of SSCD. However, the transmastoid (TM) approach is an alternative strategy that has demonstrated promising results.
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outcomes of middle fossa craniotomy for the repair of Superior Semicircular Canal dehiscence
Journal of Clinical Neuroscience, 2017Co-Authors: Nolan Ung, Quinton Gopen, Lawrance K Chung, Carlito Lagman, Nikhilesh S Bhatt, Natalie E Barnette, Vera Ong, Isaac YangAbstract:Abstract Superior Semicircular Canal dehiscence (SSCD) is a rare defect of the arcuate eminence that causes an abnormal connection between the Superior Semicircular Canal and middle cranial fossa. Patients often present with a variety of auditory and vestibular symptoms. Trigger avoidance is the initial strategy, but surgery may be necessary in debilitating cases. We retrospectively reviewed SSCD patients undergoing repair via a middle fossa craniotomy between March 2011 and September 2015. Forty-nine patients undergoing 58 surgeries were identified. Autophony was the most common symptom at presentation (n = 44; 90%). Mean follow-up was 10.9 months, with 100% of patients reporting resolution of at least one symptom. Aural fullness was the most commonly resolved symptom following surgical repair (n = 19/22; 86%). Hearing loss (n = 11/25; 44%) and tinnitus (n = 11/38; 29%) were the most common symptoms to persist following surgery. The most common symptom to develop after surgery was disequilibrium (n = 4/18; 22%). Upon comparing the overall pre-operative and post-operative groups, the number of patients with autophony (p
Katherine D. Heidenreich - One of the best experts on this subject based on the ideXlab platform.
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surgical treatment of hearing loss when otosclerosis coexists with Superior Semicircular Canal dehiscence syndrome
Otology & Neurotology, 2014Co-Authors: Cedric V Pritchett, Katherine D. Heidenreich, Matthew E Spector, Paul R Kileny, Hussam K ElkashlanAbstract:OBJECTIVE Document a case of bilateral otosclerosis with coexisting bilateral Superior Semicircular Canal dehiscence syndrome and the treatment of hearing loss in this setting. PATIENT A 33-year-old woman presented with bilateral mixed hearing loss; worse in the left ear. This was gradual in onset, and she denied dizziness. Computerized tomographic scan revealed fenestral otosclerosis and a large dehiscence of the Superior Semicircular Canal bilaterally. She declined amplification. INTERVENTION Sequential laser-assisted stapedotomy with insertion of a Kurz titanium CliP Piston prosthesis. MAIN OUTCOME MEASURE Comparison of audiovestibular symptoms, hearing thresholds, and neurodiagnostic testing results preoperatively and postoperatively. RESULTS Hearing improved bilaterally with closure of the air-bone gaps at most frequencies, and she has not had permanent vestibular symptoms. Postoperative follow-up time is 37 months for the left ear and 13 months for the right ear. CONCLUSION When otosclerosis and Superior Semicircular Canal dehiscence syndrome coexist and hearing loss is the dominant symptom, stapes surgery can be effective for improving hearing without permanent vestibular symptoms.
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Tullio phenomenon in Superior Semicircular Canal dehiscence syndrome
Neurology, 2014Co-Authors: Gregory J. Basura, Scott J. Cronin, Katherine D. HeidenreichAbstract:Tullio phenomenon refers to eye movements induced by sound.1 This unusual examination finding may be seen in Superior Semicircular Canal dehiscence (SSCD) syndrome.2 This disorder is due to absent bone over the Superior Semicircular Canal (figure ). Patients complain of dizziness triggered by loud sound, aural fullness, autophony, and pulsatile tinnitus. When Tullio phenomenon exists in SSCD syndrome, the patient develops a mixed vertical-torsional nystagmus in which the slow phase rotates up and away from the affected ear (video on the Neurology ® Web site at Neurology.org). This pattern of nystagmus aligns in the plane of the dehiscent Semicircular Canal and is due to excitation of its afferent nerves.
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hennebert s sign in Superior Semicircular Canal dehiscence syndrome a video case report
Laryngoscope, 2012Co-Authors: Andrew G Shuman, Syed S Rizvi, Chantale W Pirouet, Katherine D. HeidenreichAbstract:Superior Semicircular Canal dehiscence (SSCD) syndrome has been called the great otologic mimicker because its presentation overlaps with otosclerosis, Meniere's disease, perilymphatic fistula, and patulous eustachian tube. A valuable examination finding that can help distinguish SSCD syndrome from other pathologic conditions is the presence of Hennebert's sign, in which pressure changes in the external auditory Canal evoke stereotyped eye movements that align in the plane of the dehiscent Semicircular Canal. This video case report demonstrates Hennebert's sign associated with SSCD syndrome and discusses its pathophysiological basis.
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electrocochleography as a diagnostic and intraoperative adjunct in Superior Semicircular Canal dehiscence syndrome
Otology & Neurotology, 2011Co-Authors: Meredith E Adams, Katherine D. Heidenreich, Paul R Kileny, Hussam K Elkashlan, Steven A Telian, Gregory Mannarelli, Alexander H ArtsAbstract:ObjectiveTo determine the electrocochleographic characteristics of ears with Superior Semicircular Canal dehiscence (SSCD) and to examine its use for intraoperative monitoring in Canal occlusion procedures.Study DesignCase series.SettingAcademic medical center.PatientsThirty-three patients (45 ears)
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persistent positional nystagmus a case of Superior Semicircular Canal benign paroxysmal positional vertigo
Laryngoscope, 2011Co-Authors: Katherine D. Heidenreich, Gregory J. Basura, Kevin A Kerber, Wendy J Carender, Steven A TelianAbstract:Involvement of the Superior Semicircular Canal (SSC) in benign paroxysmal positional vertigo (BPPV) is rare. SSC BPPV is distinguished from the more common posterior Semicircular Canal (PSC) variant by the pattern of nystagmus triggered by the Dix-Hallpike position: down-beating torsional nystagmus in SSC BPPV versus up-beating torsional nystagmus in PSC BPPV. SSC BPPV may be readily treated at the bedside, which is a key component in excluding central causes of down-beating nystagmus. We present an unusual video case report believed to represent refractory SSC BPPV based on the pattern of nystagmus and the absence of any other central signs.
Quinton Gopen - One of the best experts on this subject based on the ideXlab platform.
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the gopen yang Superior Semicircular Canal dehiscence questionnaire development and validation of a clinical questionnaire to assess subjective symptoms in patients undergoing surgical repair of Superior Semicircular Canal dehiscence
Journal of Laryngology and Otology, 2018Co-Authors: Brittany L Voth, Courtney Duong, Carlito Lagman, Natalie E Barnette, Vera Ong, John P Sheppard, Thien Nguyen, C Jacky H Chen, J J Arsenault, Quinton GopenAbstract:Objective To characterise subjective symptoms in patients undergoing surgical repair of Superior Semicircular Canal dehiscence. Methods Questionnaires assessing symptom severity and impact on function and quality of life were administered to patients before Superior Semicircular Canal dehiscence surgery, between June 2011 and March 2016. Questionnaire sections included general quality of life, internal amplified sounds, dizziness and tinnitus, with scores of 0-100 points. Results Twenty-three patients completed the questionnaire before surgery. Section scores (mean±standard deviation) were: 38.2 ± 25.2 for general quality of life, 52.5 ± 23.9 for internal amplified sounds, 35.1 ± 28.8 for dizziness, 33.3 ± 30.7 for tinnitus, and 39.8 ± 22.2 for the composite score. Cronbach's α statistic averaged 0.93 (range, 0.84-0.97) across section scores, and 0.83 for the composite score. Conclusion The Gopen-Yang Superior Semicircular Canal Dehiscence Questionnaire provides a holistic, patient-centred characterisation of Superior Semicircular Canal dehiscence symptoms. Internal consistency analysis validated the questionnaire and provided a quantitative framework for further optimisation in the clinical setting.
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incidence of intraoperative hearing loss during middle cranial fossa approach for repair of Superior Semicircular Canal dehiscence
Journal of Clinical Neuroscience, 2018Co-Authors: Michael Johanis, Isaac Yang, Quinton GopenAbstract:Abstract Introduction Superior Semicircular Canal dehiscence is a rare inner ear disorder characterized by an abnormal third opening between the Superior Semicircular Canal and middle fossa. Symptoms include amplification of internal sounds, aural fullness, tinnitus, hearing loss, autophony, sound-induced vertigo (Tullio phenomenon), pressure-induced vertigo (Hennebert sign), disequilibrium, nystagmus, oscillopsia, and headache. While no cure exists for SSCD, surgical treatment has proven to effectively minimize these symptoms. This study reviewed brainstem auditory evoked potentials (BAEPs) that were monitored intraoperatively to better understand hearing loss risks associated with surgical treatment for SSCD. Methods A retrospective chart review was conducted at the University of California, Los Angeles on adult patients with a confirmed diagnosis of SSCD who had undergone a middle cranial fossa repair from March 2011 to October 2017. A total of 142 cases of SSCD in 118 patients were repaired. Results The majority of patients’ BAEPs remained stable and had no intraoperative hearing changes (n = 135; 95.1%). Seven patients experienced intraoperative changes as determined by a prolongation and reduction of Wave V latency (4.9%). Of these seven cases, five experienced a return to baseline prior to the end of surgery, and had no post-operative changes in hearing (71.4%). Overall, only two of the 142 surgeries (1.4%) resulted in failure to normalize and, as such, these patients experienced permanent changes in hearing. Conclusion The results of this retrospective review demonstrate a low risk for hearing loss due to SSCD surgery via the middle fossa craniotomy approach.
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middle cranial fossa approach for the repair of Superior Semicircular Canal dehiscence is associated with greater symptom resolution compared to transmastoid approach
Acta Neurochirurgica, 2018Co-Authors: Thien Nguyen, Quinton Gopen, Prasanth Romiyo, Courtney Duong, Carlito Lagman, John P Sheppard, Giyarpuram N Prashant, Isaac YangAbstract:Background Superior Semicircular Canal dehiscence (SSCD) is a disorder of the skull base that is gaining increasing recognition among neurosurgeons. Traditionally, the middle cranial fossa (MCF) approach has been used for the surgical repair of SSCD. However, the transmastoid (TM) approach is an alternative strategy that has demonstrated promising results.
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outcomes of middle fossa craniotomy for the repair of Superior Semicircular Canal dehiscence
Journal of Clinical Neuroscience, 2017Co-Authors: Nolan Ung, Quinton Gopen, Lawrance K Chung, Carlito Lagman, Nikhilesh S Bhatt, Natalie E Barnette, Vera Ong, Isaac YangAbstract:Abstract Superior Semicircular Canal dehiscence (SSCD) is a rare defect of the arcuate eminence that causes an abnormal connection between the Superior Semicircular Canal and middle cranial fossa. Patients often present with a variety of auditory and vestibular symptoms. Trigger avoidance is the initial strategy, but surgery may be necessary in debilitating cases. We retrospectively reviewed SSCD patients undergoing repair via a middle fossa craniotomy between March 2011 and September 2015. Forty-nine patients undergoing 58 surgeries were identified. Autophony was the most common symptom at presentation (n = 44; 90%). Mean follow-up was 10.9 months, with 100% of patients reporting resolution of at least one symptom. Aural fullness was the most commonly resolved symptom following surgical repair (n = 19/22; 86%). Hearing loss (n = 11/25; 44%) and tinnitus (n = 11/38; 29%) were the most common symptoms to persist following surgery. The most common symptom to develop after surgery was disequilibrium (n = 4/18; 22%). Upon comparing the overall pre-operative and post-operative groups, the number of patients with autophony (p
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outcomes of middle fossa craniotomy for the repair of Superior Semicircular Canal dehiscence
Journal of Clinical Neuroscience, 2017Co-Authors: Nolan Ung, Quinton Gopen, Lawrance K Chung, Carlito Lagman, Nikhilesh S Bhatt, Natalie E Barnette, Vera Ong, Isaac YangAbstract:Superior Semicircular Canal dehiscence (SSCD) is a rare defect of the arcuate eminence that causes an abnormal connection between the Superior Semicircular Canal and middle cranial fossa. Patients often present with a variety of auditory and vestibular symptoms. Trigger avoidance is the initial strategy, but surgery may be necessary in debilitating cases. We retrospectively reviewed SSCD patients undergoing repair via a middle fossa craniotomy between March 2011 and September 2015. Forty-nine patients undergoing 58 surgeries were identified. Autophony was the most common symptom at presentation (n=44; 90%). Mean follow-up was 10.9months, with 100% of patients reporting resolution of at least one symptom. Aural fullness was the most commonly resolved symptom following surgical repair (n=19/22; 86%). Hearing loss (n=11/25; 44%) and tinnitus (n=11/38; 29%) were the most common symptoms to persist following surgery. The most common symptom to develop after surgery was disequilibrium (n=4/18; 22%). Upon comparing the overall pre-operative and post-operative groups, the number of patients with autophony (p<0.0001), aural fullness (p=0.0006), hearing loss (p=0.0119), disequilibrium (p=0.0002), sound- and pressure-induced vertigo (p<0.0001), and tinnitus (p<0.0001) were significantly different. Improved clinical outcomes were demonstrated in patients undergoing SSCD repair through a middle cranial fossa approach. The most common presenting symptom (autophony) was also most likely to resolve after surgery. Hearing loss is less amenable to surgical correction. Disequilibrium developed in a small number of patients after repair.
Steven D Rauch - One of the best experts on this subject based on the ideXlab platform.
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optimized diagnostic approach to patients suspected of Superior Semicircular Canal dehiscence
Ear and Hearing, 2021Co-Authors: Kimberley S Noij, Aaron K Remenschneider, Barbara S Herrmann, John J Guinan, Steven D RauchAbstract:OBJECTIVES Current methods of diagnosing Superior Semicircular Canal dehiscence syndrome (SCDS) include a clinical exam, audiometric testing, temporal bone computer tomography (CT) imaging, and vestibular evoked myogenic potential (VEMP) testing. The main objective of this study was to develop an improved diagnostic approach to SCDS optimized for accuracy, efficiency, and safety that utilizes clinical presentation, audiometric testing, CT imaging, high-frequency cervical VEMP (cVEMP) testing, and patient treatment preference. A secondary aim was to investigate the cost associated with the current versus proposed diagnostic paradigms. DESIGN All patients who underwent cVEMP testing since introduction of the 2 kHz cVEMP in our clinical protocol in July 2018 were screened. Patients suspected of SCDS based upon symptoms who also had available audiogram, CT scan, and 2 kHz cVEMP were included (58 ears). Patients were categorized as dehiscent, thin, or not dehiscent based on their CT scan. Symptom prevalence and cVEMP outcomes were analyzed and compared for all groups. The accuracy of the 2 kHz cVEMP was calculated using CT imaging as the standard. Using a combination of patient symptomatology, audiometric, CT and 2 kHz cVEMP data, as well as patient preference, a best clinical practice approach was developed. The cost associated with this approach was calculated and compared with cost of the current SCDS diagnostic workup using Medicare reimbursement rates. RESULTS In the overall patient population suspected of SCDS based on clinical presentation, the sensitivity and specificity of 2 kHz cVEMP were 76% and 100%, respectively, while the positive and negative predictive values were 100% and 84.6%, assuming that the CT scan finding was correct. Autophony was the most common symptom in patients who had both Superior Semicircular Canal dehiscence on CT imaging plus abnormal 2 kHz cVEMP (p < 0.001). Combining patient symptomatology, 2 kHz normalized peak to peak cVEMP amplitude, and patient treatment preference to determine, which patients should undergo CT scanning resulted in a potential cost reduction between 45% and 61%. CONCLUSION In patients suspected of SCDS based on their clinical presentation, the combination of symptomatology, 2 kHz cVEMP data, and patient preference can be used to determine which patients should undergo CT scanning, resulting in a diagnostic cost reduction and reduced patient radiation exposure.
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vestibular evoked myogenic potential vemp testing for diagnosis of Superior Semicircular Canal dehiscence
Frontiers in Neurology, 2020Co-Authors: Kimberley S Noij, Steven D RauchAbstract:Superior Semicircular Canal dehiscence is a bony defect of the Superior Semicircular Canal, which can lead to a variety of auditory and vestibular symptoms. The diagnosis of Superior Semicircular Canal dehiscence (SCD) can be challenging, time consuming, and costly. The clinical presentation of SCD patients resembles that of other otologic disease, necessitating objective diagnostics. Although temporal bone CT imaging provides excellent sensitivity for SCD detection, it lacks specificity. Because the treatment of SCD is surgical, it is crucial to use a highly specific test to confirm the diagnosis and avoid false positives and subsequent unnecessary surgery. This review provides an update on recent improvements in vestibular evoked myogenic potential (VEMP) testing for SCD diagnosis. Combining audiometric and conventional cervical VEMP results improves SCD diagnostic accuracy. High frequency VEMP testing is Superior to all other methods described to date. It is highly specific for the detection of SCD and may be used to guide decision-making regarding the need for subsequent CT imaging. This algorithmic sequential use of testing can substantially reduce radiation exposure as well as cost associated with SCD diagnosis.