BACKGROUND: Superior Semicircular Canal Dehiscence (SSCD) is a dehiscence of the otic capsule which normally lies over the superior semicircular canal. This database constitutes the largest series of SSCD patients to date.
OBJECTIVE: To determine what preoperative factors, if any, contribute to postoperative outcomes and evaluate symptom resolution in a large SSCD patient cohort.
METHODS: A single-institution, retrospective chart review collected patient demographics, intraoperative findings, and pre-and postoperative symptoms. Fisher\'s exact t-test was performed for unpaired categorical variables, with a significance level of p < 0.05.
RESULTS: 350 SSCD repairs were performed. The median age was 52 years (range: 17-86 years, ± 6.4 years), and the median follow-up duration was 4.6 months (range: 0.03-59.5 months, ± 6.8 months). Preoperative hearing loss was significantly associated with female sex (p = 0.0028). The most reported preoperative symptoms were tinnitus (77.4%), dizziness (74.0%), autophony (66.3%), amplification (63.7%), and disequilibrium (62.6%). Between patients who received unilateral versus bilateral SSCD repair, the greatest postoperative symptomatic resolution was seen in autophony (74.9%, p < 0.001), amplification (77.3%, p = 0.00027), hyperacusis (77.4%, p = 0.023), hearing (62.9%, p = 0.0063), and dizziness (54.6%, p < 0.001) for patients with unilateral SSCD repair.
CONCLUSIONS: Surgical repair via the middle cranial fossa approach can significantly resolve auditory, vestibular, and neurological symptoms of patients with SSCD. Although this is one of the largest single-institution SSCD studies to date, future multi-institutional, prospective studies would be beneficial to validate these results.

OBJECTIVE: The diagnosis and management of Superior Canal Dehiscence Syndrome (SCDS) with concomitant otosclerosis can be a challenge. Otosclerosis can mask SCDS symptoms and stapes surgery may reveal or exacerbate vestibular symptoms. Our aim is to present four cases of SCDS with concomitant otosclerosis and thereby informing the reader about the possibility of this dual occurrence and its implications for treatment.
METHODS: Four patients with SCDS and concomitant otosclerosis are presented. Two patients underwent surgical treatment for both SCDS and otosclerosis and two patients opted for conservative management.
RESULTS: The main differences between surgically and non-surgically treated cases are the presence of autophony and pressure-induced vertigo and a more severe experience of symptoms in surgically treated cases. Surgically treated cases achieved a sizeable reduction in postoperative air-bone gap and resolution of vestibular symptoms.
CONCLUSIONS: The subjective severity of symptoms in combination with shared decision-making is key in determining the appropriate treatment plan for SCDS and concomitant otosclerosis.

UNASSIGNED: The concurrence of otosclerosis and superior semicircular canal dehiscence (SSCD) presents a diagnostic challenge and failure to differentiate between these 2 diagnoses results in mischaracterization and unsuccessful surgery. The objective of this study is to identify the incidence of SSCD in patients who have computed tomography (CT) evidence of otosclerosis.
UNASSIGNED: Retrospective chart review.
UNASSIGNED: Tertiary referral hospital.
UNASSIGNED: Adults with CT scan of the temporal bone diagnosed with radiological unilateral or bilateral fenestral otosclerosis from January 1995 to April 2018.
UNASSIGNED: Retrospective review of patient imaging from a multi-center tertiary-referral health system from January 1995 to April 2018. Imaging was reviewed to quantify the incidence of SSCD among patients with CT-diagnosed bilateral fenestral otosclerosis. Poor quality imaging was excluded from review.
UNASSIGNED: One-thousand two-hundred eight patients (1214 CT scans) were identified with otosclerosis, of which 373 were diagnosed with fenestral otosclerosis (663 ears) with imaging of sufficient quality for review. This population was predominantly female (57.2%) with bilateral fenestral otosclerosis (78%). Of these, 23 ears (3.5%) had definitive evidence of SSCD, with an additional 15 ears (2.3%) with possible radiographic evidence of SSCD. There was no significant difference in laterality between the SSCD and otosclerosis.
UNASSIGNED: Among 373 patients with fenestral otosclerosis per CT temporal bone imaging at a tertiary referral hospital, as many as 8.3% of patients had radiographic evidence of SSCD. Given this incidence, it continues to be important to consider SSCD when diagnosing and treating otosclerosis.

OBJECTIVE: To describe the features of antecedent head trauma in patients with superior canal dehiscence syndrome (SCDS).
METHODS: Cross-sectional survey.
METHODS: Tertiary referral center.
METHODS: An online survey was sent to 450 adult patients who underwent surgical repair for SCDS patients asking questions about the nature of internal or external traumatic events preceding symptoms.
RESULTS: One-hundred and thirty-six patients (avg. age, 51.9 years, 62.8% female) completed the survey, of which 61 (44.9%) described either preceding external head trauma (n = 35, 26%), preceding internal pressure event (n = 33, 25%), or both (8, 6%). Of those endorsing external trauma, 22 (63%) described a singular event (head hit by object (n = 8); head hit ground (n = 5); motor vehicle accident (n = 4); assault (n = 2); other (n = 3). One-third experienced loss of consciousness because of the trauma. For those describing internal pressure events (n = 33), the most common events were heavy physical exertion (9, 27%); pressure changes while flying (6, 18%); coughing, nose blowing with illness (5, 15%); childbirth (5, 15%); and self contained underwater breathing apparatus diving events (3, 9%). The interval between trauma and onset of symptoms averaged 5.6 years (SD, 10.7 years). One-third (n = 19) described onset of symptoms immediately after the external trauma or internal pressure event. Symptoms began on the side ipsilateral to the trauma in 91%. Sound- and pressure-induced vertigo/oscillopsia developed more commonly after external trauma versus internal pressure events (68% and 61% vs 44% and 32%, respectively).
CONCLUSIONS: Trauma or internal pressure-related events precede SCDS symptoms in nearly half of cases, with roughly half of preceding events being external.

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 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.
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.
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.
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.

The aim of this study was to develop a clinical test for body sounds\' hypersensitivity in superior canal dehiscence syndrome (SCDS).
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.
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.
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.

UNASSIGNED: Superior semicircular canal dehiscence (SSCD) is defined as a defect in the bone overly the superior semicircular canal (SSC). The purpose of this study is to evaluate the pre-operative imaging examinations of patients who have undergone SSCD repair. We hypothesize that these patients will not have a supralabyrinthine air cell on the side of surgery.
UNASSIGNED: Our group retrospectively reviewed 50 consecutive pre-operative computed tomography (CT) temporal bone examinations who had confirmed SSCD on intraoperative examination and underwent repair for the presence of a supralabyrinthine air cell.
UNASSIGNED: 100% of patients who had confirmed SSCD on intraoperative examination had no supralabyrinthine air cell on pre-operative CT of the temporal bone.
UNASSIGNED: This study shows that a supralabyrinthine air cell is not present in SSCD. When a supralabyrinthine air cell is present, the roof of the SSC is intact. CT and magnetic resonance imaging (MRI) are often performed together to evaluate for SSCD and exclude other etiologies. We propose that if a supralabyrinthine air cell is seen on MRI, no CT is necessary, thus avoiding unnecessary radiation exposure and additional imaging costs to the patient.

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OBJECTIVE: Cartilage cap resurfacing is a method to seal a superior semicircular canal dehiscence. The purpose of this study was to evaluate the detection of new bone formation after surgical placement of a cartilage cap over a dehiscent semicircular canal.
METHODS: In this retrospective review, two neuroradiologists blinded to each other\'s interpretation reviewed the temporal bones of 20 patients, five of which had a pre-operative computed tomography (CT) exam which was interpreted as unilateral superior semicircular canal dehiscence and with new bone formation following repair on follow-up CT. There were also 15 control subjects. Each neuroradiologist was blinded to history, including post-operative changes, and asked to determine if there was a dehiscence or no dehiscence.
RESULTS: Out of the 15 controls, there was 100% inter-observer agreement. On the five post-operative patients, there was agreement in 4/5 that there was no dehiscence post-operatively and 1/5 agreement of dehiscence post-operatively, but ectopic bone adjacent to the dehiscence.
CONCLUSIONS: Our results indicate that new bone formation can be seen at the site of cartilage cap placement over the dehiscence and be interpreted as bony closure of the dehiscence.