Superior Semicircular Canal Dehiscence Outcomes in a Consecutive Series of 229 Surgical Repairs With Middle Cranial Fossa Craniotomy.

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 55.9% of patients were female. 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 with 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. CONCLUSIONS: 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.

Analysis of temporal bone thickness outside of the petrous temporal bone between superior semicircular canal dehiscence and normal patients.

BACKGROUND: SSCD is a rare inner ear disorder. This study aims to compare the thickness of the temporal bone beyond the petrous portion between healthy subjects and those with SSCD to determine whether the etiopathology of SSCD is localized to the petrous temporal bone or generalized to other parts of the temporal bone. METHODS: A retrospective chart review of electronic medical records from September 2011 to February 2018 was conducted at a single-institution study at the University of California, Los Angeles. Participants were divided into two groups: Group 1 had a confirmed diagnosis of SSCD, while Group 2 had no known ear or temporal bone pathology. Participants' high-resolution coronal and axial temporal bone computed tomography scans were analyzed. Regions within the temporal bone were measured and compared between the two groups. RESULTS: A total of 262 scans were included. Group 1 consisted of 103 scans, while Group 2 consisted of 159 scans. There was no statistically significant difference in the thickness of temporal bones between patients diagnosed with SSCD and patients without otologic disease. CONCLUSION: The results suggest that the etiology of SSCD is limited to the petrous portion of the temporal bone. SSCD may be unrelated to a larger process of global temporal bone degeneration. Additional clinical screening for regions outside the petrous temporal bone is not warranted unless SSCD patients present with symptoms characteristic of other temporal bone pathologies.

Concurrent superior semicircular canal dehiscence and endolymphatic hydrops: A novel case series.

INTRODUCTION: Superior semicircular canal dehiscence (SSCD) is characterized by CT-confirmed bony erosion over the superior semicircular canal, creating vestibular and auditory symptoms. Endolymphatic hydrops (EH) is characterized by an MRI-confirmed excess of endolymph within the scala media that distorts the membranous labyrinth. While there is overlap in symptoms, the two diseases result from different pathophysiologies and require different interventions. PRESENTATION OF CASES: A retrospective chart review was conducted at the University of California, Los Angeles on a database of 270 adult SSCD patients, gathered between March 2011 and February 2020. A review of clinical notes, post-operative findings, and imaging was performed for 16 patients who had both CT-confirmed SSCD and an MRI of the internal auditory canal (IAC). Three cases of concurrent SSCD and EH were identified. Medical and surgical history, symptom progression pre- and post-operatively, and treatment outcomes were gathered. One patient's symptoms were resolved via mycophenolate mofetil, another's via hydrochlorothiazide, and the third's via hydrochlorothiazide and bilateral hearing aids. DISCUSSION: Post-surgical persistence of SSCD symptoms that are mutually shared with EH is the strongest indicator that a physician should investigate for concurrent EH. VEMP and audiogram testing in these cases can be misleading and should not be relied on as rule-in or rule-out tests. CONCLUSION: Concurrent SSCD and EH is a rare but treatable entity. Physicians should consider ordering an MRI of the IAC if SSCD patients' symptoms persist or recur after a successful surgery.

Reviewing applications of structural and functional MRI for bipolar disorder.

Bipolar disorders (BDs) represent one of the leading causes of disability and morbidity globally. The use of functional magnetic resonance imaging (fMRI) is being increasingly studied as a tool to improve the diagnosis and treatment of BDs. While morphological biomarkers can be identified through the use of structural magnetic resonance imaging (sMRI), recent studies have demonstrated that varying degrees of both structural and functional impairments indicate differing bipolar subtypes. Within fMRI, resting-state fMRI has specifically drawn increased interest for its capability to detect different neuronal activation patterns compared to task-based fMRI. This study aims to review recently published literature regarding the use of fMRI to investigate structural-functional relationships in BD diagnosis and specifically resting-state fMRI to provide an opinion on fMRI's modern clinical application. All sources in this literature review were collected through searches on both PubMed and Google Scholar databases for terms such as 'resting-state fMRI' and 'functional neuroimaging biomarkers of bipolar disorder'. While there are promising results supporting the use of fMRI for improving differential accuracy and establishing clinically relevant biomarkers, additional evidence will be required before fMRI is considered a dependable component of the overall BD diagnostic process.

Thinning or dehiscence of bone in structures of the middle cranial fossa floor in superior semicircular canal dehiscence.

BACKGROUND: Superior semicircular canal dehiscence (SSCD) is a rare inner ear disorder; currently, it is unknown whether the etiopathology underlying this structural irregularity affects neighboring structures. The goal is to investigate the prevalence of bone thinning in areas of the middle cranial fossa (MCF) floor in SSCD and non-SSCD patients. METHODS: This retrospective study analyzed 100 patients from March 2011 to June 2017 at a tertiary referral center. 100 patients undergoing 118 SSCD repair surgeries (18 bilateral) were identified. 12 SSCD ears were excluded due to lack of pre-operative computed tomography (CT) scans or history of prior SSCD repair at an outside facility. Non-SSCD ears were identified from routinely-obtained CT scans for temporal bone fracture (fractured sides excluded) for a total of 101 ears; 26 non-SSCD ears were excluded due to lack of high-resolution imaging. RESULTS: Univariate analyses reveal that SSCD diagnosis is associated with higher rates of geniculate ganglion (GG) dehiscence compared with non-SSCD controls (42.7 vs. 24%; χ2(1) = 9.69,P = 0.008). Individuals with SSCD depicted significantly thinner bone overlying the geniculate ganglion (GG) (0.23 ± 1.2 mm) compared to controls (0.28 ± 1.8 mm, (t(1 6 4)) = 2.1, P = 0.04). SSCD patients presented thinner bone overlying the internal auditory canal (IAC) (0.33 ± 1.3 mm) compared to patients without SSCD (0.46 ± 1.6 mm, (t(2 5 7) = 6.4, P < 0.001). CONCLUSIONS: The increased prevalence of dehiscence of the MCF in this cohort of SSCD patients compared to non-SSCD patients suggests that the etiology underlying SSCD affects surrounding structures.

Increased cochlear radiation dose predicts delayed hearing loss following both stereotactic radiosurgery and fractionated stereotactic radiotherapy for vestibular schwannoma.

PURPOSE: Stereotactic radiosurgery (SRS) and fractionated stereotactic radiotherapy (fSRT) are noninvasive therapies for vestibular schwannomas providing excellent tumor control. However, delayed hearing loss after radiation therapy remains an issue. One potential target to for improving hearing rates is limiting radiation exposure to the cochlea. METHODS: We retrospectively reviewed 100 patients undergoing either SRS with 12 Gy (n = 43) or fSRT with 50 Gy over 28 fractions (n = 57) for vestibular schwannoma. Univariate and multivariate analysis were carried out to identify predictors of hearing loss as measured by the Gardner Robertson scale after radiation therapy. RESULTS: Deterioration of hearing occurred in 30% of patients with SRS and 26% with fSRT. The overall long term (> 2 year) progression rates were 20% for SRS and 16% for fSRT. Patients with a decrease in their Gardner Robertson hearing score and those that loss serviceable hearing had significantly higher average minimal doses to the cochlea in both SRS and fSRT cohorts. ROC analysis showed that a cut off of 5 Gy and 35 Gy, for SRS and fSRT respectively, predicted hearing loss with high sensitivity/specificity. CONCLUSION: Our data suggests the minimal dose of radiation that the cochlear volume is exposed to is a predictor of delayed hearing loss after either SRS or fSRT. A threshold of 5 Gy/35 Gy may lead to improved hearing preservation after radiotherapy. Further prospective multi center studies can further elucidate this mechanism.

CT evaluation of normal bone thickness overlying the superior semicircular canal.

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.