Effects of Spectral Resolution and Frequency Mismatch on Speech Understanding and Spatial Release From Masking in Simulated Bilateral Cochlear Implants.

OBJECTIVES: Due to interaural frequency mismatch, bilateral cochlear-implant (CI) users may be less able to take advantage of binaural cues that normal-hearing (NH) listeners use for spatial hearing, such as interaural time differences and interaural level differences. As such, bilateral CI users have difficulty segregating competing speech even when the target and competing talkers are spatially separated. The goal of this study was to evaluate the effects of spectral resolution, tonotopic mismatch (the frequency mismatch between the acoustic center frequency assigned to CI electrode within an implanted ear relative to the expected spiral ganglion characteristic frequency), and interaural mismatch (differences in the degree of tonotopic mismatch in each ear) on speech understanding and spatial release from masking (SRM) in the presence of competing talkers in NH subjects listening to bilateral vocoder simulations. DESIGN: During testing, both target and masker speech were presented in five-word sentences that had the same syntax but were not necessarily meaningful. The sentences were composed of five categories in fixed order (Name, Verb, Number, Color, and Clothes), each of which had 10 items, such that multiple sentences could be generated by randomly selecting a word from each category. Speech reception thresholds (SRTs) for the target sentence presented in competing speech maskers were measured. The target speech was delivered to both ears and the two speech maskers were delivered to (1) both ears (diotic masker), or (2) different ears (dichotic masker: one delivered to the left ear and the other delivered to the right ear). Stimuli included the unprocessed speech and four 16-channel sine-vocoder simulations with different interaural mismatch (0, 1, and 2 mm). SRM was calculated as the difference between the diotic and dichotic listening conditions. RESULTS: With unprocessed speech, SRTs were 0.3 and -18.0 dB for the diotic and dichotic maskers, respectively. For the spectrally degraded speech with mild tonotopic mismatch and no interaural mismatch, SRTs were 5.6 and -2.0 dB for the diotic and dichotic maskers, respectively. When the tonotopic mismatch increased in both ears, SRTs worsened to 8.9 and 2.4 dB for the diotic and dichotic maskers, respectively. When the two ears had different tonotopic mismatch (e.g., there was interaural mismatch), the performance drop in SRTs was much larger for the dichotic than for the diotic masker. The largest SRM was observed with unprocessed speech (18.3 dB). With the CI simulations, SRM was significantly reduced to 7.6 dB even with mild tonotopic mismatch but no interaural mismatch; SRM was further reduced with increasing interaural mismatch. CONCLUSIONS: The results demonstrate that frequency resolution, tonotopic mismatch, and interaural mismatch have differential effects on speech understanding and SRM in simulation of bilateral CIs. Minimizing interaural mismatch may be critical to optimize binaural benefits and improve CI performance for competing speech, a typical listening environment. SRM (the difference in SRTs between diotic and dichotic maskers) may be a useful clinical tool to assess interaural frequency mismatch in bilateral CI users and to evaluate the benefits of optimization methods that minimize interaural mismatch.

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.

Radiosurgery treatment is associated with improved facial nerve preservation versus repeat resection in recurrent vestibular schwannomas.

BACKGROUND: Vestibular schwannomas (VSs) are benign neoplasms of the Schwann cells of cranial nerve VIII, and treatment of VS typically involves surgical resection. However, tumor recurrence may necessitate reintervention, and secondary treatment modalities include repeat surgical resection or adjuvant radiosurgery. The purpose of this study is to examine the scientific literature in order to determine whether surgical resection or radiosurgery for recurrent VS results in better tumor control, hearing preservation, and preservation of facial nerve function. METHODS: The PubMed, Scopus, Embase, Cochrane, and Web of Science databases were searched for studies reporting on patients undergoing either radiosurgery or repeat surgical resection after primary surgical resection for recurrent VS. Statistical analyses were performed on the compiled data, primarily outcome data involving tumor control, hearing preservation, and preservation of facial nerve function. RESULTS: We analyzed the data of 15 individual studies involving 359 total patients, and our results reveal that tumor control rates are comparable between adjuvant radiosurgery (91%, CI: 88-94%) and secondary resection (92%, CI 75-98%). However, adjuvant radiosurgery was shown to preserve good facial nerve function better (94%, CI 84-98%) compared to secondary surgical resection (56%, CI 41-69%). CONCLUSION: With comparable tumor control rates and better preservation of good facial nerve function, this study suggests that secondary radiosurgery for recurrent VS is associated with both optimal tumor control and preservation of good facial nerve function.

Neuroglial ectopia of the vestibular nerve masquerading as a vestibular schwannoma: A case report.

Neuroglial ectopia is a rare entity of undetermined clinical significance. Here, we report a unique case of neuroglial ectopia of the vestibular nerve. A 27-year-old pharmacy student with a previous radiological diagnosis of vestibular schwannoma presented to our clinic for surgical evaluation. Magnetic resonance imaging (MRI) of the brain revealed a 17-mm T1 hypo- to isointense, T2 iso- to hyperintense, poorly enhancing left cerebellopontine angle mass extending into the left internal auditory canal compatible by imaging with a vestibular schwannoma. The lesion was resected under MRI guidance. The frozen specimen came back as a benign hypocellular lesion. Histological assessment revealed a peripheral nerve engulfed by glial fibrillary acidic protein-positive, S-100-negative cells, suggestive of neuroglial ectopia. There was no evidence of schwannoma. The main concerns were benign neoplasm with potential for progression or sampling artifact. The patient had an uncomplicated hospital course. This case report demonstrates an unusual case of neuroglial ectopia of the vestibular nerve. The differential diagnoses for a poorly enhancing cerebellopontine angle mass should include neuroglial ectopia.

Middle cranial fossa approach for the repair of superior semicircular canal dehiscence is associated with greater symptom resolution compared to transmastoid approach.

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. METHODS: We performed independent searches of a popular database to identify studies that described outcomes following the surgical repair of SSCD through MCF and TM approaches. The primary outcome was symptom resolution. RESULTS: Our analysis included 24 studies that described 230 patients that underwent either an MCF (n = 148, 64%) approach or a TM (n = 82, 36%) approach for primary surgical repair of SSCD. A greater percentage of patients in the MCF group experienced resolution of auditory symptoms (72% vs 59%, p = 0.012), aural fullness (83% vs 55%, p = 0.049), hearing loss (57% vs 31%, p = 0.026), and disequilibrium (75% vs 44%, p = 0.001) when compared to the TM group. The MCF approach was also associated with higher odds of symptom resolution for auditory symptoms (odds ratio [OR] 1.79, 95% confidence interval [CI] 1.14-2.82), aural fullness (OR 4.02, 95% CI 1.04-15.53), hearing loss (OR 2.91, 95% CI 1.14-7.42), and disequilibrium (OR 3.94, 95% CI 1.78-8.73). The mean follow-up was 9 months. CONCLUSIONS: The literature suggests that the MCF approach for the repair of SSCD is associated with greater symptom resolution when compared to the TM approach. This information could help facilitate patient discussions.

Evaluating the utility of a scoring system for lipomas of the cerebellopontine angle.

BACKGROUND: Cerebellopontine angle (CPA) lipomas are rare, benign, slow-growing masses. Resections are considered in symptomatic patients who are refractory to targeted medical therapies, but at those stages the lipomas have often reached considerable sizes and encompass critical neurovascular structures. The objective of this study is to develop and to evaluate the utility of a scoring system for CPA lipomas. The hypothesis is that CPA lipomas with lower scores are probably best managed with early surgery. METHODS: The PubMed database was searched using relevant terms. Data on patient and lipoma characteristics were extracted and used to design a scoring system. CPA lipomas were stratified by scores with corresponding managements and outcomes analyzed. RESULTS: One hundred and seventeen patients with CPA lipomas were identified and 40 CPA lipomas were scored. The remaining CPA lipomas were deficient in data and not scored. No lipomas were scored as 1. Score 2 lipomas (n = 12; 30%) most often underwent serial surveillances (n = 5; 41.6%), with the majority of symptoms remaining unimproved (n = 2; 40%). Patients with score 2 CPA lipomas treated with medical therapies (n = 3; 25%) often experienced symptom resolution (n = 2; 66.6%) (p = 0.0499). Patients with score 2 CPA lipomas undergoing surgical resections (n = 3; 25%) all experienced symptom resolution (n = 3; 100%) (p = 0.0499). Score 3 was most common (n = 16; 40%) and these lipomas were often surgically resected (n = 10; 62.5%). The majority of patients with score 3 CPA lipomas having undergone surgical resections (n = 10; 62.5%) experienced symptom improvement (n = 1; 10%) or resolution (n = 4; 40%). CONCLUSIONS: Score 2 CPA lipomas are smaller and would be deemed non-surgical in general practice. However, our data suggest that these lipomas may benefit from either medical therapies or early surgical resections. The advantages of early surgery are maximal resection, decreased surgical morbidity, and improved symptom relief.

First-Side and Second-Side Repair of Bilateral Superior Canal Dehiscence.

OBJECTIVE: Bilateral superior canal dehiscence (SCD) may warrant surgeries on both sides. With repairs of unilateral SCD as reference, we investigate the comparative effectiveness of first-side and second-side repairs, in hopes of establishing knowledge that can guide clinical decision-making pertaining the appropriateness of second-side surgeries. METHODS: Middle fossa SCD repairs at an institution between 2011 and 2022 were analyzed. Multivariable regression models assessed symptom resolution and audiometric improvement with surgery cohort (unilateral SCD repair vs. first-side repair vs. second-side repair) as the primary predictor. All models controlled for patient age, sex, surgery duration, prior ear surgery, and follow-up. RESULTS: A total of 407 repairs (180 unilateral SCD, 172 first-side, and 55 second-side repairs) were analyzed. The rates of overall symptom improvement for auditory and vestibular symptoms were as follows: 81% and 67% for unilateral SCD repairs; 73% and 54% for first-side repairs; and 43% and 51% for second-side repairs, respectively. Compared with first-side repairs, which resolved auditory symptoms at similar rates (aOR 95% C.I. 0.36-1.07) but resolved vestibular symptoms at significantly lower rates (aOR 95% C.I. 0.35-0.93) compared with unilateral SCD repairs, second-side repairs resolved auditory symptoms at significantly lower rates (aOR 95% C.I. 0.10-0.51) but resolved vestibular symptoms at similar rates (aOR 95% C.I. 0.45-2.01). CONCLUSIONS: Careful consideration of perioperative symptomatology may inform the appropriateness of second-side surgeries. If auditory symptoms persisted following first-side surgeries, second-side surgeries are less likely to yield resolution. If vestibular symptoms persisted following first-side repairs, second-side repairs may lead to resolution at similar rates as first-side repairs. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:1882-1888, 2024.

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.

Narrowing of Low-Frequency Air-Bone Gap and Its Association with Symptom Resolution Following Superior Canal Dehiscence Repair.

OBJECTIVE: Low-frequency air-bone gap (LABG) on pure tone audiometry is an expected clinical finding of superior canal dehiscence (SCD) syndrome. We investigate how narrowing of LABG following SCD repairs translates to symptom resolution. STUDY DESIGN: Cohort study. SETTING: Tertiary Care Center. METHODS: We analyzed consecutive SCD repairs at an institution between 2012 and 2022. Pure tone audiometry and symptom questionnaires were administered pre- and post-operatively. The independent variable assessed whether the LABG narrowed (≥5 dB) following surgery. Outcome measures were rates of Overall Symptom Improvement (OSI, net resolution of ≥1 symptom) and Symptom Resolution Score (SRS, % symptoms resolved). We conducted multivariable regression analyses with LABG narrowing as the primary predictor. All models controlled for demographics, bilateral disease, prior ear surgery, and follow-up. RESULTS: Among total of 217 repairs analyzed, 161 (74%) reached OSI, and mean SRS was 39 (out of 100). LABG narrowing at 250 Hz (65%), 500 Hz (52%), and 1000 Hz (47%) was associated with a 41-point (ß 95% confidence interval [CI] 5-77) increase in auditory, 15-point (ß 95% CI 1-30) increase in auditory, and 23-point (ß 95% CI 2-45) increase in vestibular SRS, respectively. However, LABG narrowing was not significantly associated with the rates of auditory and vestibular OSI at all frequencies assessed. CONCLUSION: Lack of LABG narrowing following repair was associated with the persistence of a greater proportion of preoperative symptoms but similar likelihood of OSI. This relationship was more prominent for auditory symptoms at 250 to 500 Hz and for vestibular symptoms at 1000 Hz. Additional research is warranted to elucidate the mechanism through which symptoms resolve despite LABG persistence.

Low-Lying Tegmen and Surgical Outcomes Following the Middle Cranial Fossa Repair of Superior Canal Dehiscence.

OBJECTIVE: To compare treatment response from the middle cranial fossa repair of superior canal dehiscence (SCD) between cases with and cases without low-lying tegmen (LLT). STUDY DESIGN: Cohort study. SETTING: Single tertiary care institution. METHODS: Two investigators independently reviewed preoperative high-resolution temporal bone computed tomography images and classified the ipsilateral tegmen as either "low-lying" or "control." Patients completed a symptom questionnaire and underwent audiometric testing pre- and post-operatively. Multivariable regression models assessed for symptomatic resolution and audiometric improvement following surgery with tegmen status as the primary predictor. Models controlled for patient age, sex, bilateral SCD disease, dehiscence location, prior ear surgery status, surgery duration, and follow-up duration. RESULTS: Among a total of 410 cases included, we identified 121 (29.5%) LLT cases. Accounting for all control measures, patients with LLT were significantly less likely to experience overall symptom improvement (adjusted odds ratio: 0.32, 95% confidence interval [CI]: 0.18-0.57, p < .001) and reported a significantly lower proportion of preoperative symptoms that resolved following surgery (adjusted ß: -25.6%, 95% CI: -37.0% to -14.3%, p < .001). However, audiometric outcomes following surgery did not differ significantly between patients with and patients without LLT. CONCLUSION: This is the first investigation on the relationship between LLT and surgical outcomes following the middle fossa repair of SCD. Patients with LLT reported less favorable symptomatic response but exhibited a similar degree of audiometric improvement.

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.

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.

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.

Clinical Predictors of Symptom Improvement Following Eustachian Tube Balloon Dilation.

OBJECTIVE: This study aims to identify clinical predictors of treatment response to Eustachian Tube Balloon Dilation (ETBD) as measured by changes in Eustachian Tube Dysfunction Questionnaire-7 (ETDQ-7) scores. METHODS: One hundred thirteen patients who underwent ETBD at an institution from 2017 to 2021 completed ETDQ-7 pre- and post-operatively. We conducted multivariable regression analyses with ETDQ-7 normalization (<2.1 post-op), minimum clinically important difference (MCID) (>0.5 pre-op - post-op), and quantitative improvement in ETDQ-7 score as outcome variables. Pre-operative ETDQ-7 score, tympanogram type, chronic otitis media, chronic rhinosinusitis (CRS), inferior turbinate hypertrophy, deviated septum, allergic rhinitis, and rhinorrhea were included as covariates. Models controlled for age, sex, ethnicity, prior ear or sinus surgery, and follow-up duration. RESULTS: The mean age was 49 years old. 51% were females, and all patients had pre-operative ETDQ-7 above 2.1. After a mean follow-up period of 13 months, 77% achieved MCID and 37% had normalized. Higher pre-operative ETDQ-7 score was associated with greater ETDQ-7 score improvement (B = 0.60, 95% CI = [0.37, 0.83]) and greater odds of achieving MCID (aOR = 1.65; 95% CI = [1.06, 2.59]). A history of CRS improved chances of achieving MCID (aOR = 4.53; 95% CI = [1.11, 18.55]) and a history of chronic otitis media predicted increased odds of ETDQ-7 normalization (aOR = 2.88; 95% CI = [1.09, 7.58]). CONCLUSIONS: Our findings suggest that ETBD was highly effective among patients with pre-operative ETDQ-7 above 2.1. Furthermore, higher pre-operative ETDQ-7 score, CRS, and chronic otitis media predicted more favorable symptomatic benefit from ETBD. These factors may be important to consider when counseling potential candidates for this procedure.

Intraoperative neuromonitoring techniques in the surgical management of acoustic neuromas.

Unfavorable outcomes such as facial paralysis and deafness were once unfortunate probable complications following resection of acoustic neuromas. However, the implementation of intraoperative neuromonitoring during acoustic neuroma surgery has demonstrated placing more emphasis on quality of life and preserving neurological function. A modern review demonstrates a great degree of recent success in this regard. In facial nerve monitoring, the use of modern electromyography along with improvements in microneurosurgery has significantly improved preservation. Recent studies have evaluated the use of video monitoring as an adjunctive tool to further improve outcomes for patients undergoing surgery. Vestibulocochlear nerve monitoring has also been extensively studied, with the most popular techniques including brainstem auditory evoked potential monitoring, electrocochleography, and direct compound nerve action potential monitoring. Among them, direct recording remains the most promising and preferred monitoring method for functional acoustic preservation. However, when compared with postoperative facial nerve function, the hearing preservation is only maintained at a lower rate. Here, the authors analyze the major intraoperative neuromonitoring techniques available for acoustic neuroma resection.

Assessment of Metabolic Markers and Osteoporosis in 250 Patients with Superior Semicircular Canal Dehiscence Treated With Middle Fossa Craniotomy.

BACKGROUND: Superior semicircular canal dehiscence (SSCD) is caused by bony defects in the osseous shell of the arcuate eminence separating the labyrinth and the intracranial space. This pathologic third window causes hydroacoustic transmission resulting in debilitating symptoms. We examine the pathophysiologic association between metabolic markers, previous medical history, and SSCD symptoms before and after middle fossa craniotomy (MFC) treatment. METHODS: This study was conducted between March 2011 and September 2020 with patients with SSCD who underwent MFC. We used a Fisher test to compare variables, including bilateral SSCD, second surgery, ear anomaly, osteoporosis, arthritis, vitamin D, and preoperative/postoperative symptoms, and others. Point-biserial correlation analysis was performed to test correlations between continuous variables and categorical variables. RESULTS: A total of 250 patients with SSCD underwent MFC repair. There was significant postoperative resolution in all symptoms (P < 0.0001). Laboratory 25-hydroxyvitamin D values correlated with preoperative aural fullness (rpb= 0.29; P = 0.03), and preoperative disequilibrium (rpb= -0.32; P = 0.02). Serum calcium values correlated with preoperative hearing loss (rpb= 0.16; P = 0.02). Osteoporosis history (n = 16; 6%) was more prevalent in female patients (P = 0.0001), associated with higher levels of preoperative hearing loss (odds ratio, 4.56; P = 0.02) and higher postoperative hearing loss resolution (odds ratio, 2.89; P = 0.0509). CONCLUSIONS: Certain metabolic markers may predict SSCD presentation before and after surgery. Previous history of osteoporosis, autoimmune conditions, or arthritis may play a role in SSCD pathophysiology and can help predict clinical outcomes. Future evaluation should take metabolic laboratory values and acquire an exact medical history.

Postoperative Outcomes of Patients with Thin Bone Overlying the Superior Semicircular Canal: A Single Institution's Experience.

BACKGROUND: Superior semicircular canal dehiscence (SSCD) is defined by a bony defect overlying the superior semicircular canal (SSC) in the middle cranial fossa floor, causing a myriad of vestibular and auditory symptoms. Patients with thin bone without full dehiscence overlying the SSC also present with similar symptoms. There are currently no guidelines for surgical management of patients with thin bone. The authors offer their experience with thin bone patients to characterize their symptomatology and explore whether these patients benefit from surgical intervention typically offered to SSCD patients. METHODS: Two hundred fifty-six patients evaluated for SSCD from 2011 to 2019 were reviewed. High-resolution coronal computed tomography scans with 0.6-mm slice thickness of the temporal bones were assessed to determine whether the patient had a true dehiscence or a thin bone covering overlying the SSC. Bone that was ≤0.5 mm was considered to be "thin bone." Parameters of interest included patient demographics as well as preoperative and postoperative symptomatology. A P value < 0.05 was considered statistically significant. RESULTS: Forty-eight patients met inclusion criteria of having "thin bone." The mean age was 48.13 ± 12.03 years, and 65.5% of patients were female. Of the preoperative symptoms evaluated, the greatest postoperative symptomatic resolution was noted in hearing loss (92.3%), vertigo (94.4%), and oscillopsia (100%). Dizziness (56.5%) had the lowest symptomatic resolution rate. CONCLUSIONS: Surgical management of thin bone patients via middle fossa craniotomy, a similar technique to SSCD repair, provides significant symptomatic resolution. Therefore, surgery should be considered in thin bone patients with debilitating symptoms, albeit not having a true dehiscence.

Evaluation of Superior Semicircular Canal Dehiscence Anatomical Location and Clinical Outcomes: A Single Institution's Experience.

BACKGROUND: Superior semicircular canal dehiscence (SSCD) is becoming increasingly recognized as a pathology underlying various auditory and vestibular complaints. To date, our understanding of the pathology has yet to attribute specific symptoms to the anatomic location of dehiscence in patients with SSCD. This study aims to address this issue by evaluating the relationship between symptomatology and anatomic location of dehiscence. METHODS: A single-institution retrospective review of SSCD patients was performed. Information was collected on patient demographics, symptomatology, and anatomic location of dehiscence. High-resolution computed tomography scans of the temporal bones were used to categorize the anatomic SSCD location into 1 of 3 groups: anterior limb, apex, and posterior limb. Lastly, we performed statistical analysis to determine the degree of association between each of the various perioperative factors and anatomic SSCD location. RESULTS: We studied 54 patients in total (32 women, 22 men). Mean age at diagnosis was 53 years (range: 20-82 years) and mean follow-up length was 5.5 months (range: 0.03-27.0 months). The most common anatomical location of superior semicircular canal dehiscence was the apex, which was seen in 68.5% of cases. While preoperative symptomatology was similar among the 3 cohorts, those with apical dehiscences had a significantly higher rate of postoperative improvement of autophony (P = 0.03), aural fullness (P = 0.03), and tinnitus (P = 0.05) as compared to their counterparts. CONCLUSIONS: Although our results do not support an association between preoperative characteristics-including symptomatology-and anatomic SSCD location, our findings do suggest that apical dehiscences are associated with greater postoperative symptomatic resolution.

The molecular genetics and tumor pathogenesis of meningiomas and the future directions of meningioma treatments.

Meningiomas are mostly benign, slow-growing tumors of the CNS that originate from arachnoidal cap cells. While monosomy 22 is the most frequent genetic abnormality found in meningiomas, a multitude of other aberrant chromosomal alterations, signaling pathways, and growth factors have been implicated in its pathogenesis. Losses on 22q12.2, a region encoding the tumor suppressor gene merlin, represent the most common genetic alterations in early meningioma formation. Malignant meningioma progression, however, is associated with more complex karyotypes and greater genetic instability. Cytogenetic studies of atypical and anaplastic meningiomas revealed gains and losses on chromosomes 9, 10, 14, and 18, with amplifications on chromosome 17. However, the specific gene targets in a majority of these chromosomal abnormalities remain elusive. Studies have also implicated a myriad of aberrant signaling pathways involved with meningioma tumorigenesis, including those involved with proliferation, angiogenesis, and autocrine loops. Understanding these disrupted pathways will aid in deciphering the relationship between various genetic changes and their downstream effects on meningioma pathogenesis. Despite advancements in our understanding of meningioma pathogenesis, the conventional treatments, including surgery, radiotherapy, and stereotactic radiosurgery, have remained largely stagnant. Surgery and radiation therapy are curative in the majority of lesions, yet treatment remains challenging for meningiomas that are recurrent, aggressive, or refractory to conventional treatments. Future therapies will include combinations of targeted molecular agents as a result of continued progress in the understanding of genetic and biological changes associated with meningiomas.

Novel grading system of sigmoid sinus dehiscence for radiologic evaluation of pulsatile tinnitus.

BACKGROUND: Sigmoid sinus dehiscence (SSD) is an important etiology of pulsatile tinnitus (PT) though there is currently no consensus on the prevalence of SSD in non-PT populations. This study establishes a grading system of SSD and analyzes a non-PT cohort for prevalence of SSD. METHODS: In this retrospective study temporal bone CT scans of 91 patients without PT were analyzed for SSD. The dehiscence was divided into three grades: Grade 1 indicating a micro dehiscence of <3.5 mm with an opening to the mastoid air cells, Grade 2 indicating a major dehiscence of >3.5 mm with an opening to the mastoid air cells, and Grade 3 indicating a sigmoid sinus wall dehiscence opening directly to the underlying tissue. RESULTS: In patients without PT, SSD occurred in 34% of the cohort. Of these, 75% were Grade 1 and 25% were Grade 2. The range of dehiscence measurements for Grade 1 dehiscences was 0.9-3.4 mm. The range of dehiscence measurements for Grade 2 was 4-7.5 mm. There were no cases of Grade 3 dehiscence among this cohort. CONCLUSIONS: SSD occurred in over a third of our non-symptomatic cohort. While all grades of SSD may currently be treated surgically, a large portion of non-PT patients may have these sigmoid sinus anomalies asymptomatically. This grading system allows for the standardization of SSD definition and severity in future studies. Grade 3 dehiscences were completely absent in this cohort of non-PT patients.