Auditory brainstem implantation: surgical experience and audiometric outcomes in the pediatric population.

OBJECTIVE: Pediatric data regarding treatment via an auditory brainstem implant (ABI) remains sparse. The authors aimed to describe their experience at their institution and to delineate associated demographic data, audiometric outcomes, and surgical parameters. METHODS: An IRB-approved, retrospective chart review was conducted among the authors' pediatric patients who had undergone auditory brainstem implantation between 2012 and 2021. Demographic information including sex, age, race, coexisting syndrome(s), history of cochlear implant placement, average duration of implant use, and follow-up outcomes were collected. Surgical parameters collected included approach, intraoperative findings, number of electrodes activated, and complications. RESULTS: A total of 19 pediatric patients had an ABI placed at the authors' institution, with a mean age at surgery of 4.7 years (range 1.5-17.8 years). A total of 17 patients (89.5%) had bilateral cochlear nerve aplasia/dysplasia, 1 (5.3%) had unilateral cochlear nerve aplasia/dysplasia, and 1 (5.3%) had a hypoplastic cochlea with ossification. A total of 11 patients (57.9%) had a history of cochlear implants that were ineffective and required removal. The mean length of implant use was 5.31 years (0.25-10 years). Two patients (10.5%) experienced CSF-related complications requiring further surgical intervention. The most recent audiometric outcomes demonstrated that 15 patients (78.9%) showed improvement in their hearing ability: 5 with sound/speech awareness, 5 able to discriminate among speech and environmental sounds, and 5 able to understand common phrases/conversation without lip reading. Nine patients (47.4%) are in a school for the deaf and 7 (36.8%) are in a mainstream school with support. CONCLUSIONS: The authors' surgical experience with a multidisciplinary team demonstrates that the retrosigmoid approach for ABI placement in children with inner ear pathologies and severe sensorineural hearing loss is a safe and effective treatment modality. Audiometric outcome data showed that nearly 79% of these patients had an improvement in their environmental and speech awareness. Further multicenter collaborations are necessary to improve these outcomes and potentially standardize/enhance electrode placement.

Small pretreatment lesion size and high sphericity as favorable prognostic factors after laser interstitial thermal therapy in brain metastases.

OBJECTIVE: The objective of this study was to identify baseline clinical and radiological characteristics of brain metastases (BMs) associated with a higher probability of lesion-specific progression-free survival (PFS-L) after laser interstitial thermal therapy (LITT). METHODS: A total of 47 lesions in 42 patients with BMs treated with LITT were retrospectively examined, including newly diagnosed BM, suspected recurrent BM, and suspected radiation necrosis. The association of baseline clinical and radiological features with PFS-L was assessed using survival analyses. Radiological features included lesion size measurements, diffusion and perfusion metrics, and sphericity, which is a radiomic feature ranging from 1 (perfect sphere) to 0. RESULTS: The probability of PFS-L for the entire cohort was 88.0% at 3 months, 70.6% at 6 months, 67.4% at 1 and 2 years, and 62.2% at 3 years. For lesions progressing after LITT (n = 13), the median time to progression was 3.9 months, and most lesions (n = 11) progressed within 6 months after LITT. In lesions showing response to LITT (n = 17), the median time to response was 12.1 months. All 3 newly diagnosed BMs showed a long-term response. The mean (± SD) follow-up duration for all censored lesions (n = 34) was 20.7 ± 19.4 months (range 12 days to 6.1 years). The mean pretreatment enhancing volume was 2.68 cm3 and the mean sphericity was 0.70. Pretreatment small enhancing volume (p = 0.003) and high sphericity (p = 0.024) computed from lesion segmentation predicted a longer PFS-L after LITT. Lesions meeting optimal cutoffs of either enhancing volume < 2.5 cm3 (adjusted p = 0.004) or sphericity ≥ 0.705 (adjusted p = 0.019) had longer PFS-L, and their probability of PFS-L was 86.8% at 3 years. Lesions meeting both cutoffs showed a cumulative benefit (p < 0.0001), with a 100% probability of PFS-L at 3 years, which was unchanged at the end of follow-up (4.1 years). Manually computed estimates of lesion size (maximal axial diameter, p = 0.011) and sphericity (p = 0.043) were also predictors of PFS-L. Optimal cutoffs of diameter < 2 cm (adjusted p = 0.035) or manual sphericity ≥ 0.91 (adjusted p = 0.092) identified lesions with longer PFS-L, and lesions meeting both cutoffs showed a cumulative benefit (p = 0.0023). Baseline diffusion imaging did not predict PFS-L. A subset of lesions (n = 7) with highly perfused hotspots had worse PFS-L (adjusted p = 0.010), but perfusion signal contamination from vessels and cortex and underlying size differences were possible confounders. CONCLUSIONS: Small size and high sphericity are ideal baseline features for lesions considered for LITT treatment, with a cumulative PFS-L benefit when both features are present, that could aid patient selection.

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.

Neurosurgical Management of Interspinous Device Complications: A Case Series.

Background: Best practice guidelines for treating lumbar stenosis include a multidisciplinary approach, ranging from conservative management with physical therapy, medication, and epidural steroid injections to surgical decompression with or without instrumentation. Marketed as an outpatient alternative to a traditional lumbar decompression, interspinous process devices (IPDs) have gained popularity as a minimally invasive stabilization procedure. IPDs have been embraced by non-surgical providers, including physiatrists and anesthesia interventional pain specialists. In the interest of patient safety, it is imperative to formally profile its safety and identify its role in the treatment paradigm for lumbar stenosis. Case Description: We carried out a retrospective review at our institution of neurosurgical consultations for patients with hardware complications following the interspinous device placement procedure. Eight cases within a 3-year period were identified, and patient characteristics and management are illustrated. The series describes the migration of hardware, spinous process fracture, and worsening post-procedural back pain. Conclusions: IPD placement carries procedural risk and requires a careful pre-operative evaluation of patient imaging and surgical candidacy. We recommend neurosurgical consultation and supervision for higher-risk IPD cases.

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.

Adjuvant radiotherapy for atypical meningiomas is associated with improved progression free survival.

PURPOSE: To assess the progression-free survival benefits of adjuvant radiotherapy (ART) following surgical resection compared to surgery alone in high-grade meningioma patients. METHODS: We retrospectively identified 43 patients with atypical meningiomas, who underwent either radiotherapy post-surgical resection (Surgery+ART) or surgery alone (Surgery alone) at our institution between February 2007 to March 2019. GTR was achieved in 28 patients, and STR, in 11. Patient, meningioma, and treatment data were extracted from records and compared using Kaplan-Meier methodology, log-rank tests, and Cox proportional hazard models. Radiation complications were also evaluated. RESULTS: Overall 32.6% (n = 14) of patients, 6 patients in the Surgery+ART group and 8 in the Surgery alone group, experienced recurrence. In the Surgery+ART group, the median PFS time was 46.5 months (CI: [35.8-50.6]), compared to 24.5 months (CI: [18.3-32.9]) in the Surgery alone group. 2-year PFS for Surgery+ART was 100% vs. 69.0% for Surgery alone, and the 5-year PFS rate was 70.6% and 39.2%, respectively (log-rank p-value = .004). CONCLUSIONS: Our data revealed a significant PFS increase for those treated with adjuvant radiotherapy following surgery compared to surgery alone. Future prospective studies evaluating differing radiation modalities and dosages should be conducted.

Radiotherapy versus combination radiotherapy-bevacizumab for the treatment of recurrent high-grade glioma: a systematic review.

BACKGROUND: High-grade gliomas (HGG) comprise the most common primary adult brain cancers and universally recur. Combination of re-irradiation therapy (reRT) and bevacizumab (BVZ) therapy for recurrent HGG is common, but its reported efficacy is mixed. OBJECTIVE: To assess clinical outcomes after reRT ± BVZ in recurrent HGG patients receiving stereotactic radiosurgery (SRS), hypofractionated radiosurgery (HFSRT), or fully fractionated radiotherapy (FFRT). METHODS: We performed a systematic review of PubMed, Web of Science, Scopus, Embase, and Cochrane databases, following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We identified studies reporting outcomes for patients with recurrent HGG treated via reRT ± BVZ. Cohorts were stratified by BVZ treatment status and re-irradiation modality (SRS, HFSRT, and FFRT). Outcome variables were overall survival (OS), progression-free survival (PFS), and radiation necrosis (RN). RESULTS: Data on 1399 patients was analyzed, with 954 patients receiving reRT alone and 445 patients receiving reRT + BVZ. All patients initially underwent standard-of-care therapy for their primary HGG. In a multivariate analysis that adjusted for median patient age, WHO grade, RT dosing, reRT fractionation regimen, time between primary and re-irradiation, and re-irradiation target volume, BVZ therapy was associated with significantly improved OS (2.51, 95% CI [0.11, 4.92] months, P = .041) but no significant improvement in PFS (1.40, 95% CI [- 0.36, 3.18] months, P = .099). Patients receiving BVZ also had significantly lower rates of RN (2.2% vs 6.5%, P < .001). CONCLUSIONS: Combination of reRT + BVZ may improve OS and reduce RN rates in recurrent HGG, but further controlled studies are needed to confirm these effects.

A systematic review of stereotactic radiofrequency ablation for hypothalamic hamartomas.

BACKGROUND: The seizure activity associated with hypothalamic hamartomas (HHs) is refractory to medical management and surgical intervention is often required. Stereotactic Radiofrequency Ablation (SRFA) is a minimally invasive technique offering targeted lesion ablation with a reduced risk of complications. OBJECTIVE: Here, we review the current literature on the use of SRFA for HHs. METHODS: This study was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines for systematic literature review. A database search of PubMed, Cochrane, Embase, and Web of Science was conducted in July 2020. RESULTS: A total of 12 articles were included in this review, showing outcomes for 185 unique patients. Of these patients, 142 (76.8%) experienced some extent of therapeutic benefit in alleviating seizures following SRFA, with 128 (69.2%) patients achieving complete freedom from seizures. Common transient complications included hyponatremia (52 patients, 28.1%), hyperphagia (48 patients, 25.9%), hyperthermia (47 patients, 25.4%), Horner's syndrome (103 patients, 55.7%), and weight gain (75 patients, 40.5%). CONCLUSIONS: SRFA is a potential therapy for patients with HHs, especially when resection confers significant risk to surrounding structures. Multiple rounds of SRFA treatment may be needed in approximately a quarter of patients to improve gelastic seizure outcomes but may not affect non-gelastic seizures. There is limited data on the use of SRFA for HHs and further case series and clinical trials are needed to establish the use of SRFA for HHs.

Meta-analysis of adjuvant radiotherapy for intracranial atypical and malignant meningiomas.

INTRODUCTION: Meningiomas comprise 33% of all CNS tumors. The World Health Organization (WHO) describes meningiomas as benign (BM), atypical (AM), and malignant/anaplastic (MM). High-grade meningiomas such as AMs and MMs are more aggressive, recur more frequently, and portend a worse prognosis than BMs. Currently, the standard treatment for high-grade meningiomas, especially AMs, is ill-defined. In particular, the benefit to survival outcomes of adjuvant radiotherapy post-surgical resection remains unclear. In this study, we investigated the effect of adjuvant radiotherapy (ART) post-surgery on survival outcomes compared to surgery alone for high-grade meningiomas. METHODS: PRISMA guidelines were a foundation for our literature review. We screened the PubMed database for studies reporting overall survival (OS), progression free survival (PFS), and tumor recurrence for intracranial, primary AM and MMs treated with surgery+ART or surgery alone. Fixed and random effect models compared tumor control rate for AM aforementioned groups. RESULTS: Mean 5-year PFS was 76.9% for AM (surgery+ART) and 55.9% for AM (surgery alone) patients. Mean 5-year OS was 81.3% and 74% for AM (surgery+ART) and AM (surgery alone) groups, respectively. Overall, the mean 5-year PFS for aggregated high-grade meningiomas AM+MM (surgery+ART) was 67.6%. Fixed effect models revealed tumor control rate as 76% for AM (surgery+ART) and 69% for AM (surgery alone) groups. ART induced toxicity incidence ranged from 12.0% to 35.5% for AM and MM patients. CONCLUSIONS: Our analysis suggests that (surgery+ART) may increase PFS, OS, and tumor control rates in high-grade meningiomas. However, further studies involving surgery+ ART should be conducted to fully evaluate the ideal radiosurgical candidate, modality, and dosage.

Bilateral Superior Semicircular Canal Dehiscence Concurrent With Ehlers-Danlos Syndrome: A Case Report.

Superior semicircular canal dehiscence (SSCD) is characterized by temporal bone thinning, which creates an opening between the inner ear and middle cranial fossa. Ehlers-Danlos syndrome, hypermobility type (EDS-HT) is a genetic collagen synthesis disorder, often resulting in bony abnormalities. We present the case of a 39-year-old female with EDS-HT who exhibited the otological symptoms characteristic of bilateral SSCD. High-resolution computed tomography (CT) scans confirmed the diagnosis. The patient elected for middle fossa craniotomy and noted symptomatic improvement. Due to its potential to confer bone fragility, EDS-HT may predispose SSCD development. Further examination of the relationship between these disorders is necessary.

Psychopathy and Pride: Testing Lykken's Hypothesis Regarding the Implications of Fearlessness for Prosocial and Antisocial Behavior.

Despite widespread assumptions that psychopathy is associated with serious and repeated law-breaking, individuals with psychopathic personality traits do not invariably become chronic criminal offenders. As a partial explanation for this finding, Lykken (1995) ventured that a fearless temperament underlies both psychopathic traits and heroic behavior, and that heroic individuals' early exposure to effective socializing forces such as warm parenting or healthy self-esteem often fosters a characteristic adaption that tends to beget "successful" behaviors, thereby differentiating heroes from convicts. In this study, we investigate relations between psychopathy, principally its fearless dominance dimension, pride, and prosocial and antisocial behavior in a community sample (N = 339). Fearless dominance and self-centered impulsivity components of psychopathy yielded differential relations with authentic and hubristic pride (Tracy and Robins, 2004), such that fearless dominance was significantly positively correlated with both facets of pride while self-centered Impulsivity was significantly negatively correlated with authentic pride and significantly positively correlated with hubristic pride. Further, authentic pride moderated (potentiated) the relation between fearless dominance and transformational leadership, one of the two outcome measures for prosocial behavior employed in our investigation. Authentic pride did not moderate the relations between fearless dominance and either our other measure of prosocial behavior (heroism) or antisocial behavior, nor did positive parenting moderate the relations between psychopathy components and social behavior. Unexpectedly, hubristic pride significantly moderated the relation between impulsive-antisocial features and antisocial behavior in a protective manner.