The History of Superior Semicircular Canal Dehiscence: A Bibliometric Analysis.

OBJECTIVE: The treatment and understanding of superior semicircular canal dehiscence (SSCD) has seen significant developments over the past 25 years. Bibliographic analysis can provide insight into the evolution of research as well as highlight emerging areas. Reviewing a large volume of publications provides valuable insights into the citation patterns and collaborations of research groups. This study seeks to offer a comprehensive historical overview of SSCD and leading authors. DATABASE REVIEWED: Web of Science Core Collection. METHODS: A bibliometric analysis of the current literature on SSCD was conducted. A performance analysis and science mapping of the top 100 most cited articles was completed using a citation analysis. Two independent reviewers evaluated articles for relevance and adjugated by a third reviewer. Author and institution networks were examined. RESULTS: Seven hundred ninety-one articles on the topic of SSCD were identified. The top 100 articles spanned over 23 years from 1998 to 2019 and were published in 30 different journals. The top 100 articles were cited 8253 times in literature. The document contents revealed 233 keywords and 238 author keywords. The articles were authored by a total of 291 authors, with only 4 single-authored documents. CONCLUSIONS: SSCD has been highly researched in aspects of symptomatology, diagnosis, and treatment, as indicated by many highly cited articles that relate. However, the production of highly cited articles on SSCD displays a significant decrease after 2013, with only 17 of the top cited articles published since then.

Scuba Diving-Induced Inner-Ear Pathology: Imaging Findings of Superior Semicircular Canal and Tegmen Tympani Dehiscence.

BACKGROUND Superior semicircular canal dehiscence is an inner-ear pathology which presents with vertigo, disequilibrium, and hearing loss. Although the exact etiology of superior semicircular canal dehiscence is unknown, it is thought that an increase in middle-ear pressure disrupts a thin overlying temporal bone. Superior semicircular canal dehiscence is frequently seen in association with dehiscence of the tegmen tympani, which overlies the middle ear. Here, we present a case report of a 52-year-old Puerto Rican man with vertigo, dizziness, vomiting, and mild hearing loss associated with superior semicircular canal and tegmen tympani dehiscence after performing improper scuba diving techniques. CASE REPORT A 52-year-old Puerto Rican man presented to the emergency department with vertigo, dizziness, vomiting, and mild hearing loss in the right ear. The symptoms began shortly after scuba diving with inadequate decompression techniques on ascent. He was treated with recompression therapy with mild but incomplete improvement in symptoms. Bilateral temporal magnetic resonance imaging was suggestive of segmental dehiscence of the right superior semicircular canal and tegmen tympani. High-resolution computed tomography of the temporal bone confirmed right superior semicircular canal and tegmen tympani dehiscence with an intact left inner ear. CONCLUSIONS The increased inner-ear pressure that occurs during scuba diving can lead to dehiscence of the superior semicircular canal and tegmen tympani, causing vertigo and hearing loss. Performance of improper diving techniques can further increase the risk of dehiscence. Therefore, appropriate radiologic evaluation of the inner ear should be performed in such patients.

The importance of bone density and anatomical structure in superior semicircular canal dehiscence.

OBJECTIVE: This study investigates the importance of bone density, surface area, and diameter of anatomical structures of the superior semicircular canal (SSC), lateral semicircular canal (LSC), posterior semicircular canal (PSC), utricle, and saccule in patients diagnosed with superior semicircular canal dehiscence (SSCD). MATERIALS AND METHODS: The bone density, surface area, and diameter of SSC, LSC, PSC, utricle, and saccule were measured and compared between the SSCD group and control group. Fifteen ears in the SSCD group and 60 ears in the control group were evaluated. Additionally, within the SSCD group, the dehiscent and healthy sides were evaluated independently. RESULTS: SSC's bone density was significantly lower in the SSCD group compared to the control group (p = 0.008). No significant differences were found in surface area and diameter between the groups (p > 0.05). While most of the anatomical structures showed no significant difference in bone density between dehiscent and healthy ears (p > 0.05), SSC bone density was significantly lower in affected ears (p = 0.000) in SSCD group. CONCLUSION: Based on the data obtained in this study, bone density and anatomical structure may be useful in patients diagnosed with SSCD.

Photon Counting Versus Energy-integrated Detector CT in Detection of Superior Semicircular Canal Dehiscence.

BACKGROUND: Superior semicircular canal dehiscence (SSCD), an osseous defect overlying the SSC, is associated with a constellation of audiovestibular symptoms. This study sought to compare conventional energy-integrated detector (EID) computed tomography (CT) to photon-counting detector (PCD)-CT in the detection of SSCD. MATERIAL AND METHODS: Included patients were prospectively recruited to undergo a temporal bone CT on both EID-CT and PCD-CT scanners. Two blinded neuroradiologists reviewed both sets of images for 1) the presence or absence of SSCD (graded as present, absent, or indeterminate), and 2) the width of the bone overlying the SSC (if present). Any discrepancies in the presence or absence of SSCD were agreed upon by consensus. RESULTS: In the study 31 patients were evaluated, for a total of 60 individual temporal bones (2 were excluded). Regarding SSCD presence or absence, there was substantial agreement between EID-CT and PCD-CT (k = 0.76; 95% confidence interval, CI 0.54-0.97); however, SSCD was present in only 9 (15.0%) temporal bones on PCD-CT, while EID-CT examinations were interpreted as being positive in 14 (23.3%) temporal bones. This yielded a false positive rate of 8.3% on EID-CT. The bone overlying the SSC was thinner on EID-CT images (0.66 mm; SD = 0.64) than on PCD-CT images (0.72 mm; SD = 0.66) (p < 0.001). CONCLUSION: The EID-CT examinations tend to overcall the presence of SSCD compared to PCD-CT and also underestimate the thickness of bone overlying the SSC.

Trans-mastoid plugging of superior semicircular canal dehiscence: long-term follow-up.

OBJECTIVE: To evaluate the long-term outcomes of trans-mastoid plugging of superior semicircular canal dehiscence (SSCD), focusing on complicated cases. METHODS: In this cohort study, we included all patients who underwent trans-mastoid plugging of SSCD between 2009 and 2019. We evaluated the symptoms (autophony, sound-/pressure-induced vertigo, disequilibrium, aural fullness and pulsatile tinnitus) before and 1 year after surgery in the medical records. We systematically assessed the current symptoms 6.2 ± 3 years postoperative (range 2.2-12.3 years) using questionnaires sent by post and validated by telephone interviews. We also documented any complications and the need for further procedures. We compared pure tone and speech audiometry before and 1 year after surgery. Finally, the degree of mastoid pneumatisation and mastoid tegmen anatomy were reviewed on preoperative CT scans. RESULTS: We included 24 ears in 23 patients. No complications were recorded, and none required a second procedure for SSCD. Following surgery, oscillopsia and Tullio phenomena resolved in all patients. Hyperacusis, autophony, and aural fullness were also settled in all patients except one. Balance impairment persisted to some degree in 35% of patients. No deterioration over the years was reported regarding the above symptoms. On average, bone conduction pure tone average pre- and 1 year postoperative were 13.7 ± 17 and 20.5 ± 18 dB, respectively (P = 0.002). Air bone gaps were reduced from 12.7 ± 8 to 5.9 ± 6 (P = 0.001). Two patients had a significant sclerotic mastoid, three had a prominent low-lying mastoid tegmen, and two had both. Anatomy had no effect on outcome. CONCLUSION: Trans-mastoid plugging of SSCD is a reliable and effective technique which achieves long-lasting symptom control, even in cases with sclerotic mastoid or low-lying mastoid tegmen.

Superior canal dehiscence syndrome: A review.

Superior canal dehiscence syndrome (SCDS) is a vestibular disorder in which the presence of a pathological third window in the labyrinth causes several vestibular and cochlear symptoms. Herein, we review the diagnostic criteria and treatment of SCDS. The cause of SCDS is considered to be congenital or acquired; however, its etiology is not well known. Symptoms: Vertigo and/or oscillopsia induced by loud sounds (Tullio phenomenon) or stimuli that change the middle ear or intracranial pressure (fistula symptoms) with vestibular symptoms and hyperacusis and aural fullness with cochlear symptoms are characteristic clinical complaints of this syndrome. Neurological tests: Vertical-torsional eye movements can be observed when the Tullio phenomenon or fistula symptoms are induced. Conductive hearing loss with both a decrease in the bone conduction threshold at lower frequencies and an increase in the air conduction threshold at lower frequencies may be present on audiometry. Cervical and/or ocular vestibular evoked myogenic potentials are effective in strongly suspecting the presence of a pathologic third window in the labyrinth. Computed tomography (CT) imaging: High-resolution CT findings with multiplanar reconstruction in the plane of the superior semicircular canal consistent with dehiscence indicate SCDS. The Pöschl view along the plane of the superior semicircular canal and the Stenvers view perpendicular to it are recommended as CT imaging conditions. Findings from all three major diagnostic categories (symptoms, neurological tests, and/or CT imaging) are needed to diagnose SCDS. The surgical approaches for SCDS are as follows: the 1) middle cranial fossa approach, 2) transmastoid approach, and 3) round window and oval window reinforcement. Each technique has advantages and disadvantages.

Third window lesions of the inner ear: A pictorial review.

PURPOSE: Radiographic review of pathologies that associate with third window syndrome. METHODS: Case series and literature review. RESULTS: Eight unique third window conditions are described and illustrated, including superior, lateral, and posterior semicircular canal dehiscence; carotid-cochlear, facial-cochlear, and internal auditory canal-cochlear dehiscence, labyrinthine erosion from endolymphatic sac tumor, and enlarged vestibular aqueduct. CONCLUSION: The present study highlights the characteristic imaging features and symptoms to differentiate third window pathologies for expedient diagnosis and management planning.

Prevalence of Cochlear-Facial and Other Non-Superior Semicircular Canal Third Window Dehiscence on High-Resolution Temporal Bone CT.

BACKGROUND AND PURPOSE: The radiologic prevalence of superior semicircular canal dehiscence in the asymptomatic population has been widely studied, but less is known about the rates of other forms of third window dehiscence. Per the existing literature, the radiologic prevalence of cochlear-facial nerve dehiscence, for example, exceeds that seen in histologic studies, suggesting that conventional CT is unreliable for cochlear-facial dehiscence. These studies relied on nonisometric CT acquisitions, however, and underused multiplanar reformatting techniques, leading to false-positive findings. Our purpose was to determine the rate of cochlear-facial dehiscence and other non-superior semicircular canal third window dehiscences on optimized CT in asymptomatic patients. MATERIALS AND METHODS: Sixty-four-channel temporal bone CT scans from 602 patients in emergency departments were assessed for cochlear-facial and other non-superior semicircular canal third window dehiscences by using high-resolution, multiplanar oblique reformats. Confidence intervals for dehiscence prevalence were calculated using the Newcombe 95% interval confidence method. RESULTS: Of 602 patients, 500 were asymptomatic, while 102 had an imaging indication consistent with possible third window syndrome (symptomatic). Eight asymptomatic patients (1.6%) had cochlear-facial dehiscence, while 43 (8.4%) had jugular bulb-vestibular aqueduct dehiscence. There was no statistically significant difference between the prevalence of cochlear-facial dehiscence or jugular bulb-vestibular aqueduct dehiscence in asymptomatic patients compared with symptomatic patients. Cochlear-carotid canal, cochlear-internal auditory canal, and cochlear-petrosal sinus dehiscences were not observed. CONCLUSIONS: Sixty-four-channel CT with multioblique reformatting is sensitive and specific for identifying cochlear-facial dehiscence, with rates similar to those in postmortem series. Jugular bulb-vestibular aqueduct dehiscence is a common incidental finding and is unlikely to produce third window physiology. Other non-superior semicircular canal third window dehiscences are rare in asymptomatic patients.

Audiometric Outcomes After the Middle Cranial Fossa Repair of Superior Semicircular Canal Dehiscence.

OBJECTIVE: To evaluate the audiometric outcomes after the middle cranial fossa approach (MCF) for superior canal dehiscence (SCD) repair. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: SCD cases presented to a single institution between 2012 and 2022. INTERVENTIONS: The MCF repair of SCD. MAIN OUTCOME MEASURES: Air conduction (AC) threshold (250-8,000 Hz), bone conduction threshold (BC) (250-4,000 Hz), and air bone gap (ABG) (250-4,000 Hz) at each frequency, pure tone average (PTA) (500, 1,000, 2,000, 3,000 Hz). RESULTS: Among 202 repairs, 57% were bilateral SCD disease and 9% had previous surgery on the affected ear. The approach significantly narrowed ABG at 250, 500, and 1,000 Hz. The narrowing of ABG was achieved by both decreased AC and increased BC at 250 Hz, but mediated primarily by increased BC at 500 Hz and 1,000 Hz. Among cases without previous ear surgery, mean PTA remained in the normal hearing range (mean: preop, 21 dB; postop, 24 dB) and clinically important hearing loss (PTA increased by ≥10 dB) after the approach was noted in 15% of cases. Among cases with previous ear surgery, mean PTA remained in the mild hearing loss range (mean: preop, 33 dB; postop, 35 dB) and clinically important hearing loss after the approach was noted in 5% of cases. CONCLUSION: This is the largest study to date examining the audiometric outcomes after the middle cranial fossa approach for SCD repair. Findings of this investigation support that the approach is effective and safe with long-term hearing preservation for most.

Auditory and Vestibular Symptom Improvement With Surgery for Superior Canal Dehiscence Syndrome.

OBJECTIVE: (1) To measure the change in auditory and vestibular symptoms following superior canal dehiscence (SCD) surgery, and (2) to determine differences in clinical features and surgical outcomes between superior canal dehiscence syndrome (SCDS) patients with primarily auditory or vestibular complaints. STUDY DESIGN: Retrospective cohort study. SETTING: Single surgeon series at the tertiary academic medical center from 2002 to 2021. METHODS: Retrospective review of SCDS patients who underwent surgical repair. (1) Patients were administered a standardized symptom questionnaire at preoperative and follow-up visits, and results were compared with paired statistical testing. (2) Patients were divided into 2 cohorts based on either auditory or vestibular chief complaint and differences in demographic, clinical, and outcome variables were examined. RESULTS: Our study included 113 patients with 118 operated ears. Twenty-seven patients (24%) had radiographic bilateral dehiscence. 10/11 auditory symptoms (91%) and 5/8 vestibular symptoms (63%) solicited on the questionnaire improved significantly with surgery, except for nonpulsatile tinnitus, sense of imbalance, positional dizziness, and oscillopsia. Analyses stratified by chief complaint (auditory vs vestibular) revealed overall similar characteristics and surgical outcomes. Patients with chief vestibular complaints underwent surgery at an earlier age (45.5 vs 53.9 years, p < 0.05). CONCLUSION: SCD surgery alleviates a wide range of auditory and vestibular symptoms. Overall, we did not find significant differences between patients with chief auditory versus vestibular complaints, and both groups benefited from surgery. Symptoms are not directly linked to third-window physiology and certain vestibular symptoms may be more likely to persist. Bilateral dehiscence may play an important role in persistent symptoms as well.

Computed tomography approximated superior semicircular canal dehiscence size and location and their association with clinical presentation.

OBJECTIVE: This study investigates the relationship of superior semicircular canal dehiscence (SSCD) size and location with patient symptomatology and audiometry. METHODS: We retrospectively reviewed SSCD cases presented to a tertiary institution between 2011 and 2022. Dehiscence length and width measured on high-resolution temporal bone computed tomography imaging were used to calculate an approximate dehiscence area (ADA). Dehiscence location was classified employing a six-grade system. Multivariable regression analyses were performed with symptomatology and audiogram metrics as outcome variables and ADA and dehiscence location as the primary covariates of interest. RESULTS: A total of 402 SSCD were included in the analysis. Controlling for patient age, sex, dehiscence laterality, and location, greater ADA was independently associated higher rates of autophony (aOR 1.39; 95% C.I. 1.14-1.71; P = 0.004) and hyper-amplification (aOR 1.39; 1.14-1.70; P = 0.004). Additionally, dehiscences with greater ADA exhibited significantly lower bone conduction threshold at 500 Hz (adjusted ß -1.75, P = 0.006) and 1000 Hz (adjusted ß -1.61, P = 0.018) and significantly wider air-bone gap at 500 Hz (adjusted ß 2.22, P < 0.001) and 1000 Hz (adjusted ß 1.00, P = 0.039). Dehiscence location was not independently associated with any outcome variable examined when accounting for dehiscence size. CONCLUSION: The size of dehiscence is more independently related to clinical presentations than the anatomical location of the dehiscence in the superior semicircular canal. Greater dehiscence size is associated with symptomatology and audiometry consistent with more severe bone conduction hyperacusis.

Ankle Audiometry: A Clinical Test for the Enhanced Hearing Sensitivity for Body Sounds in Superior Canal Dehiscence Syndrome.

INTRODUCTION: The aim of this study was to develop a clinical test for body sounds' hypersensitivity in superior canal dehiscence syndrome (SCDS). METHOD: Case-control study, 20 patients affected by SCDS and body sounds' hypersensitivity and 20 control matched subjects tested with a new test called ankle audiometry (AA). The AA consisted of a psychoacoustic hearing test in which the stimulus was substituted by a controlled bone vibration at 125, 250, 500, and 750 Hz, delivered at the medial malleolus by a steel spring-attached bone transducer prototype B250. For each subject, it was defined an index side (the other being non-index), the one with major symptoms in cases or best threshold for each tested frequency in controls. In 3 patients, the AA was measured before and after SCDS surgery. RESULTS: The AA thresholds for index side were significantly lower in SCDS patients (115.6 ± 10.5 dB force level [FL]) than in control subjects (126.4 ± 8.56 dB FL). In particular, the largest difference was observed at 250 Hz (-16.5 dB). AA thresholds in patients were significantly lower at index side in comparison with non-index side (124.2 ± 11.4 dB FL). The response obtained with 250 Hz stimuli outperformed the other frequencies, in terms of diagnostic accuracy for SCDS. At specific thresholds' levels (120 dB FL), AA showed relevant sensitivity (90%) and specificity (80%) for SCDS. AA did not significantly correlate to other clinical markers of SCDS such as the bone and air conducted hearing thresholds and the vestibular evoked myogenic potentials. The AA thresholds were significantly modified by surgical intervention, passing from 119.2 ± 9.7 to 130.4 ± 9.4 dB FL in 3 patients, following their relief in body sounds' hypersensitivity. CONCLUSION: AA showed interesting diagnostic features in SCDS with significantly lower hearing thresholds in SCDS patients when compared to healthy matched subjects. Moreover, AA could identify the affected or more affected side in SCDS patients, with a significant threshold elevation after SCDS surgery, corresponding in body sounds' hypersensitivity relief. Clinically, AA may represent a first objective measure of body sounds' hypersensitivity in SCDS and, accordingly, be an accessible screening test for SCDS in not tertiary audiological centers.

Postoperative Pneumolabyrinth Following the Middle Cranial Fossa Approach for Superior Semicircular Canal Dehiscence Repair, 2014-2020.

OBJECTIVE: The present study investigates risk factors and clinical outcomes of postoperative pneumolabyrinth following the middle cranial fossa approach for superior semicircular canal dehiscence repair, a complication that has not been documented previously. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary/quaternary care referral center. METHODS: We conducted a retrospective review of 332 middle cranial fossa procedures from 2014 to 2020 at a tertiary/quaternary care institution. Upon identifying pneumolabyrinth cases from postoperative computed tomography temporal bone scans, we conducted multivariable logistic regression analysis to explore demographic and clinical factors that were independently linked to this complication. We also compared the rates of postoperative symptoms among patients with different grades of pneumolabyrinth. RESULTS: We identified 41 (12.3%) pneumolabyrinth cases. Patients with older age, higher preoperative pure tone average, and frank dehiscence were at higher risk for pneumolabyrinth. Furthermore, patients with pneumolabyrinth reported significantly higher rates of postoperative dizziness and exhibited significantly greater pure tone average and air-bone gap postoperatively as compared with patients without pneumolabyrinth. Finally, higher-grade pneumolabyrinth was associated with increased rates of postoperative hearing loss, and grade III pneumolabyrinth was associated with higher rates of postoperative tinnitus vs grade I and II cases. CONCLUSION: Pneumolabyrinth following the middle cranial fossa approach was associated with poor clinical outcomes, including dizziness and auditory impairment. Old age, high preoperative pure tone average, and frank dehiscence were risk factors for this complication. The highlighted parameters may be important to note when considering this approach as a treatment option and while monitoring postoperative recovery.

A report on quality-of-life outcomes following transmastoid plugging of superior semicircular canal dehiscence in a newly established service in a UK hospital.

OBJECTIVE: This study aimed to evaluate the quality-of-life outcomes following transmastoid plugging of semicircular canal dehiscence in a newly established service in a UK hospital. METHOD: Quality-of-life outcomes were measured using the Glasgow benefit Inventory score in three patients who underwent transmastoid plugging for superior semicircular canal dehiscence between September 2019 and March 2020. Patients also completed pre- and post-operative symptomatic questionnaires and vestibular-evoked myogenic potential testing. RESULTS: All three patients reported an improvement in overall quality-of-life outcomes with a mean overall Glasgow Benefit Inventory score of +37 (range, +22.2-66.6). There were no immediate post-operative complications and hearing was preserved in all patients. CONCLUSION: This study reported an initial successful experience with transmastoid plugging of superior semicircular canal dehiscence. In all patients, improvement in quality-of-life measures and symptoms was reported.

Superior Semicircular Canal Dehiscence: A Case Report and Literature Review of an Otologic Condition Associated With Severe Psychiatric Disturbance and Suicide Attempt.

We report the case of a man suffering from superior semicircular canal dehiscence with severe audiovestibular symptoms. The patient had a history of posttraumatic stress disorder and traumatic brain injury, and he had a presumed diagnosis of schizophrenia, with suicidal ideation. The patient was treated surgically with complete resolution of his symptoms and a retraction of his schizophrenia diagnosis. This case highlights the overlap between tinnitus and auditory hallucinations, as both result in auditory perception without an external stimulus. Ascertaining the appropriate cause of a patient's auditory symptoms is integral to providing appropriate medical and possibly surgical care.