Surgical decision-making in superior canal dehiscence syndrome with concomitant otosclerosis.

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. CASES: 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. OUTCOMES: 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. CONCLUSION: The subjective severity of symptoms in combination with shared decision-making is key in determining the appropriate treatment plan for SCDS and concomitant otosclerosis.

Unveiling the importance of the endoscope in the sealing of the superior canal dehiscence syndrome, how we do it.

The superior canal dehiscence syndrome is a pathology that affects the arcuate eminence creating a "third window" between the inner ear and the middle fossa. This condition can lead to symptoms such as hearing loss, autophony, or sound-induced vertigo. Traditionally, surgical treatment has been performed by microscope-assisted temporal craniotomy, but when the dehiscence is in the medial part of the arcuate eminence the bone defect may not be seen. We present case series treated at our institution diagnosed of superior canal dehiscence syndrome involving the medial slope of the arcuate eminence. During surgery, the bone defect could not be visible with traditional microscopic techniques. Nonetheless, by introducing the endoscope with the 0º and 30º optics, the dehiscence could be clearly observed and treated correctly. Our results show a clinical improvement without side effects or complications in the patients undergoing this technique. Endoscope-assisted surgery is a safe procedure and provides a better visualization of medial defects.

Evaluation of clinical predictors of postoperative outcomes in tegmen defect patients with and without concurrent superior semicircular canal dehiscence and cerebrospinal fluid leak.

OBJECTIVES: Tegmen and superior semicircular canal defects have been well studied, yet the factors contributing to their onset and progression are widely debated. The clinical utility of intraoperative intracranial pressure measurements has yet to be tested. This report aims to use intraoperative opening pressure and concurrent superior semicircular canal dehiscence (SSCD) to analyze factors influencing disease course and clinical outcomes in patients with tegmen dehiscence. METHODS: A retrospective analysis of 61 patients who underwent tegmen defect repair was performed. Multiple variables of interest including body mass index (BMI), presence of SSCD, presence of dural venous sinus stenosis, opening pressure, and acetazolamide therapy use were recorded. The cohort was divided into those with or without concurrent SSCD and those presenting with or without cerebrospinal fluid (CSF) leak for analysis. RESULTS: A linear relationship between opening pressure and BMI (p = 0.009) was noted; however, intraoperative opening pressure was not associated with disease outcome. Concurrent SSCD was present in 25 % of patients, while 62 % presented with CSF leak. The concurrent SSCD group exhibited higher opening pressure, higher likelihood of having dural sinus stenosis, and higher likelihood of being discharged on acetazolamide. The CSF leak group had higher likelihood of obstructive sleep apnea and persistent symptoms. CONCLUSIONS: In patients undergoing tegmen defect repair, concurrent SSCD suggests increased disease severity. The presence of preoperative CSF leak predicts persistent symptoms following repair. BMI is linearly correlated with intracranial pressure in these patients.

Superior semicircular canal dehiscence postoperative outcomes: a case series of 350 repairs.

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. CONCLUSION: 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.

Impact of superior semicircular canal dehiscence on cochlear implant audiologic outcomes.

PURPOSE: Determine whether adult cochlear implant users with radiographic superior semicircular canal dehiscence experience clinically significant differences in audiological outcomes when compared to cochlear implant users with normal temporal bone anatomy. MATERIALS AND METHODS: Retrospective, single institution review. Adult, post-lingual deaf patients implanted between 2010 and 2020. Inclusion criteria included age 18 years or older, available preoperative computed tomography imaging, and preoperative and postoperative AzBio audiological data for at least 6 months of cochlear implant use. Preoperative and postoperative AzBio Sentence Test scores were compared between patients with normal temporal bone anatomy and those with radiographic superior semicircular canal dehiscence or near dehiscence. RESULTS: 110 patients met inclusion criteria. Mean AzBio score for normal temporal bone anatomy group improved from 35.2 % (SD 28.2) preoperatively to 70.3 % (SD 25.7) postoperatively, an improvement of 35.1 % (SD 28.6). Mean AzBio score for near dehiscent temporal bone anatomy group improved from 26.6 % (SD 28.9) preoperatively to 64.5 % (SD 30.6) postoperatively, an improvement of 37.9 % (SD 27.9). Mean AzBio score for dehiscent temporal bone anatomy group improved from 26.3 % (SD 20.4) preoperatively to 65.1 % (SD 27.6) postoperatively, an improvement of 38.7 % (SD 26.9). Utilizing the one-way analysis of variance test, there was no significant difference in audiologic outcomes between the three groups. CONCLUSIONS: Patients with complete or near complete radiographic superior canal dehiscence at the time of cochlear implantation achieve similar improvements in speech perception scores compared to normal anatomy adult cochlear implant users.

Patient Sex Mediates the Influence of Patient Age on Superior Canal Dehiscence Repair Outcome.

OBJECTIVE: To investigate the independent and interactive effects of patient age and sex on superior canal dehiscence (SCD) repair outcomes. METHODS: This was a cohort study of consecutive SCD repairs via the middle fossa approach at an institution between 2011 and 2022. We constructed multivariable regression models assessing surgical outcomes with age and sex as the primary predictors. Models controlled for surgery duration, follow-up duration, and relevant surgical and medical history. Subsequently, we repeated each model with the incorporation of an interaction term between patient age and sex. RESULTS: Among 402 repairs, average age was 50 years, and 63% of cases were females. There was a significant interaction between patient age and sex with respect to symptom resolution score (SRS) (adj. ß 0.80, 95% C.I. 0.04-1.56). Older age was associated with lower SRS among females (-0.84, -1.29 to -0.39 point per year) but not significantly associated with SRS among males (0.04, -0.65 to 0.56 point per year). Furthermore, older age independently predicted a greater magnitude of increase in air conduction at 8000 Hz following surgery regardless of sex (adj. ß 2.1, 0.2-4.0 dB per 10-year increase). CONCLUSIONS: This is the first study on the interactive effect between patient age and sex with respect to SCD repair outcomes. Older age predicted poorer symptomatic response among female patients but did not predict symptomatic response among male patients. Furthermore, older age predicted more severe high-frequency hearing loss following SCD repairs among both female and male patients. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:3363-3370, 2024.

Superior Semicircular Canal Dehiscence Revision Surgery Outcomes: A Single Institution's Experience.

BACKGROUND: Superior semicircular canal dehiscence (SSCD) is an abnormality of the otic capsule, which normally overlies the superior semicircular canal. Surgical management is indicated in patients with persistent and debilitating symptoms. Given the complexity of the disease, there are patients who experience less favorable surgical outcomes and require revision surgery. The purpose of this study was to report to the rate of postoperative symptomatic improvement in patients who required revision surgery. METHODS: A retrospective analysis of patients undergoing SSCD surgical repair at a single institution was performed. Information on patient demographics, primary and secondary surgical approaches, surgical outcomes, and follow-up length was collected. RESULTS: Seventeen patients underwent 20 revision surgeries. There were eleven (65%) females and six (35%) males. Mean age of the cohorts was 50 years (range 30-68 years), and mean follow-up length was 6.8 months (range 0.1-31.1 months). Cerebrospinal fluid leak was noted in 67% of cases. The greatest postoperative symptomatic resolution was reported in oscillopsia (100%), headache (100%), and internal sound amplification (71%), while the least postoperative symptomatic resolution was reported in tinnitus (42%), aural fullness (40%), and dizziness (29%). CONCLUSIONS: Revision surgery can provide symptomatic improvement in select SSCD patients; however, patients should be cautioned about the possibility of less favorable outcomes than in index surgery. Revision surgeries are associated with a considerably higher rate of perioperative cerebrospinal fluid leak.

Relationship Between Superior Semicircular Canal Dehiscence Volume with Clinical Symptoms: Case Series.

BACKGROUND: Superior semicircular canal dehiscence (SSCD) is an osseous defect of the arcuate eminence of the petrosal temporal bone. Patients typically present with auditory and vestibular symptoms, such as hearing loss and disequilibrium. Using advanced imaging segmentation techniques, we evaluated whether the volume of SSCD correlated with preoperative symptoms and postoperative outcomes. METHODS: Our laboratory previously described a novel method of quantifying the size of an SSCD via manual segmentation. High-resolution computed tomography images of the temporal bones were imported into a specialized segmentation software. The volume of the dehiscence was outlined on consecutive slices of the coronal and axial planes via a single-pixel-thick paintbrush tool and was then calculated according to the number of nonzero image voxels. RESULTS: This study included 111 patients (70 women and 41 men; mean age, 55.1 years; age range, 24-87 years) with a total of 164 SSCDs. Mean postoperative follow-up time was 5.2 months (range, 0.03-59.5 months). The most common preoperative and postoperative symptoms were tinnitus (n = 85) and dizziness (n = 45), respectively. Surgery resulted in improvement of symptoms in most patients. The average volume of 164 SSCDs was 1.3 mm3. SSCD volume was not significantly associated with either preoperative symptoms or postoperative outcomes. CONCLUSIONS: Advances in imaging techniques have allowed increased visualization of SSCD. Further research will be necessary to evaluate the potential correlation of volume of the dehiscence with clinical variables.

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.

Quantitative three-dimensional image analysis of the superior canal after surgical plugging to treat superior semicircular canal dehiscence.

Surgical plugging to treat superior semicircular canal dehiscence (SCD) has been proven to impede the effect of the third mobile window, abating cochleovestibular symptoms. Knowledge of superior semicircular canal (SC)-plugging status has been proposed to serve as a guide for adjuvant treatment. Here, we investigated disturbances in the inner ear fluid space following SC plugging using a novel three-dimensional (3D) reconstruction-based method. This approach used a semi-automatic segmentation algorithm and a direct volume rendering method derived from conventional magnetic resonance images. The variable extents of filling defects at the sites of SC plugging and the positional relation of the defect to the ampulla and common crus were identified. The success group exhibited markedly reduced volumes following surgery, whereas the failure group displayed no changes in volume. These results indicate that the success or failure of SC plugging was related to 3D volume changes in the labyrinth fluid signal. Collectively, this study presents individualized SC-plugging statuses using a novel 3D reconstruction-based method and it facilitates future work regarding easy-to-measure 3D volume changes. This current technology also aids in the exploration of pathologic changes in various targets of interest.

Superior Semicircular Canal Dehiscence Syndrome Following Head Trauma: A Multi-institutional Review.

OBJECTIVE: To evaluate patients who become symptomatic from superior semicircular canal dehiscence (SSCD) following head trauma. STUDY DESIGN: Case series assessing patients presenting with SSCD after a trauma. METHODS: A case series was completed assessing patients presenting with SSCD after trauma. Data from three academic medical centers were evaluated, including the following: imaging, videonystagmography (VNG)/vestibular evoked myogenic potential (VEMP) testing, audiometric assessment, and surgical repair. Outcome measures included the following: 1) Description of audio-vestibular symptoms, 2) mean pre- and post-operative pure tone average (PTA), word recognition score (WRS), and air bone gap (ABG). RESULTS: A total of 14 patients were included; 86% were male. Approximately 43% were found to have bilateral SSCD on imaging, with 57% of patients pursuing surgical management. The most common presenting symptoms included pulsatile tinnitus (93%), autophony (79%), and hearing loss (64%). Approximately 36% of patients underwent VNG/VEMP testing, with 83.3% of those demonstrating abnormal results. The mean audiometric findings on the symptomatic side included an air-conduction PTA of 38.0 dB, bone-conduction PTA of 24.3 dB, WRS of 81%, and ABG of 17.9 dB. Among patients who underwent surgery (57%), there was no significant change in the air-conduction PTA, bone-conduction PTA, or WRS (P > .05). However, there was an improvement in the ABG (preoperative = 22.8 dB versus postoperative = 9.7 dB; P = .005). CONCLUSION: Head trauma may be a potentiating event for SSCD syndrome. This study advances the hypothesis that these patients likely have underlying radiographic SSCD prior to their trauma, and a traumatic event increases in intra-vestibular or intracranial pressures, unmasking SSCD syndrome. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2810-E2818, 2021.

A Basis for Standardizing Superior Semicircular Canal Dehiscence Management.

OBJECTIVES: (1) To determine how otologic/neurotologic surgeons counsel patients with superior semicircular canal dehiscence (SSCD). (2) To understand the plethora of presenting symptoms associated with SSCD and appropriate management. (3) To suggest appropriate management; oftentimes avoiding surgery. METHODS: This was a survey study of both community and academic physicians. A 23-question survey was distributed to all members of the American Neurotological (ANS) and American Otologic Societies (AOS) via email in the Fall of 2018. A total of 54 responses were received from a possible pool of 279 for a response rate of 19.4%. Inferences were made about the population through sample proportions and confidence intervals. RESULTS: All respondents use computed tomography (CT) in diagnosing SSCD and 11.1% use CT exclusively. Cervical vestibular evoked myogenic potential (VEMP; 77.8%) are used more often than ocular VEMPs (38.9%). Magnetic resonance imaging (7.4%) is used infrequently; 96.3% of surgeons surveyed have seen patients with SSCD on imaging that are asymptomatic. Following surgical treatment, respondents reported balance issues and mild-to-moderate high-frequency sensorineural hearing loss (88.4%); 32.6% reported that the majority (>50%) of their patients needed further intervention after surgery, typically aggressive vestibular rehabilitation. CONCLUSIONS: There is a discrepancy in the systematic approach to SSCD between both the surgeons and the published literature. Patients with SSCD on ultra-high-resolution CT may have myriad symptoms while others are asymptomatic, and surgery may lead to additional complications. We will present a methodical recommendation to assist in the management of patients with SSCD depending upon their symptoms. This may improve patient selection, counseling, and outcomes.

Outcomes after mini-craniotomy middle fossa approach combined with mastoidectomy for lateral skull base defects.

PURPOSE: Controversy exists regarding the ideal approach for repair of lateral skull base defects. Our goal is to report the outcomes following middle cranial fossa (MCF) mini-craniotomy combined with mastoidectomy for patients with superior semicircular canal dehiscence (SSCD), spontaneous cerebrospinal fluid (CSF) leak, and cholesteatoma. MATERIALS AND METHODS: A retrospective database from chart review was formed consisting of 97 patients who met surgical criteria: SSCD, spontaneous CSF leak, and cholesteatoma. Mini-craniotomy MCF approach (<4 × 2 cm in size) combined with mastoidectomy was performed. All patients were admitted directly to the ICU postoperatively. Multiple factors were assessed, including need for revision surgery, duration of surgery, length of post-operative stay, and hospital readmission. RESULTS: Average surgery time was 110 min with no intraoperative complications. The average length of hospitalization was 2 days with an average ICU stay of 1 day. There were no neurologic complications; however, there were 3 inpatient complications (3%) which included 1 patient (1%) that had wound breakdown and 2 patients (2%) that had severe post-operative vertigo. A total of 8 patients (8%) required revision surgery and these were primarily for SSCD. The 30-day readmission rate was 3%. CONCLUSION: In the current series, all patients that underwent mini-craniotomy MCF surgery combined with mastoidectomy had minimal complications, short surgical time, limited hospital stay, low revision surgery rate and few hospital readmissions. This combined approach offers superior visualization of lateral skull base defects without the morbidity and risk typically associated with traditional, extensive MCF surgery.

Current management of superior semicircular canal dehiscence syndrome.

PURPOSE OF REVIEW: The current article reviews literature on the contemporary management of superior semicircular canal dehiscence syndrome (SSCDS). Approaches to management and surgical techniques are compared along with a discussion of the use of more standardized, objective outcome measures. RECENT FINDINGS: Considerable debate still exists as to what approach and technique is most appropriate for patients with SSCDS and how to best measure postoperative outcomes. However, it is increasingly accepted that multiple factors account for outcomes in SSCDS, including presenting symptoms and presence of vestibular comorbidities. Therefore, surgical intervention is best tailored to each individual patient. Data on SSCDS outcomes is heterogenous, and increased emphasis is being placed on validated measures of outcome. Round window approaches remain controversial and their role is still undefined. SUMMARY: The treatment strategies for SSCDS continue to diversify. A patient-specific approach with systematic documentation of outcomes will continue to inform how these patients are best managed.

Superior Semicircular Canal Dehiscence Syndrome.

Superior canal dehiscence syndrome (SCDS) is a vestibular disorder caused by a pathologic third window into the labyrinth that can present with autophony, sound- or pressure-induced vertigo, and chronic disequilibrium among other vestibulocochlear symptoms. Careful history taking and examination in conjunction with appropriate diagnostic testing can accurately diagnose the syndrome. Key examination techniques include fixation-suppressed ocular motor examination investigating for sound- or pressure-induced eye movements in the plane of the semicircular canal. Audiometry, vestibular evoked myogenic potentials, and computed tomography confirm the diagnosis. Corrective surgical techniques can be curative, but many patients find their symptoms are not severe enough to undergo surgery. Although a primarily peripheral vestibular disorder, as first-line consultants for most dizziness complaints, neurologists will serve their patients well by understanding SCDS and its role in the differential diagnosis of vestibular disorders.