Driscoll, Colin L. W.

Objective: To compare language and audiological outcomes among infants (<9 and <12 mo) and older children receiving cochlear implantation (CI). Study design: Retrospective chart review. Setting: Tertiary academic referral center. Patients: Pediatric patients receiving CI between October 1995 and October 2019. Intervention: Cochlear implantation Main Outcome Measures: Most recent language and audiological assessment scores were evaluated by age group. Results: A total of 118 children were studied, including 19 who were implanted <9 months of age, 19 implanted 9 to <12 months of age, and 80 implanted 12 to <36 months of age. The mean duration of follow-up was 7.4 ± 5.0 years. Most recent REEL-3 receptive (88 ± 12 vs. 73 ± 15; p = 0.020) and expressive (95 ± 13 vs. 79 ± 12; p = 0.013) communication scores were significantly higher in the <9 months group compared to the 9 to <12 months group. PLS and OWLS auditory comprehension and oral expression scores were significantly higher in the <12 months group compared to the 12 to <36 months group. The difference in NU-CHIPS scores between <12 and 12 to <36 months was statistically significant (89% ± 6 vs. 83% ± 12; p = 0.009). LNT scores differed significantly between <9 and 9 to <12 months (94% ± 4 vs. 86% ± 10; p = 0.028). Conclusions: The recent FDA expansion of pediatric CI eligibility criteria to include infants as young as 9 months of age should not serve as a strict clinical cutoff. Rather, CI can be pursued in appropriately selected younger infants to optimize language and audiological outcomes.

Objective: To compare the prevalence of surgical, anesthetic, and device-related complications among infants and older children receiving cochlear implantation (CI). Study Design: Retrospective chart review. Setting: Tertiary academic referral center. Patients: Pediatric patients who underwent CI from November 1990 to January 2020. Intervention: CI. Main Outcome Measures: Surgical, anesthetic, and device-related complication rates were compared by age group (<12 versus 12–23 versus 24+ months with subset analysis of <9 versus 9–11 months). Results: A total of 406 primary pediatric CI surgeries encompassing 482 ears were analyzed, including 45 ears in 23 patients implanted less than 9 months and 89 ears in 49 patients less than 12 months. No anesthetic complications occurred. Postoperative surgical and device-related complication rates were not significantly different among the less than 12, 12 to 23, and 24+ month groups (16% versus 16% versus 12%; p = 0.23) or between the less than 9 and 9 to 11 month groups (22% versus 9%; p = 0.09). Thirty-day readmission was significantly higher for patients less than 12 months compared with patients 24+ months (6% versus <1%; p = 0.011), but was not significantly higher compared with patients 12 to 23 months (6% versus 3%; p = 0.65). Reoperation rates did not differ significantly among the less than 12, 12 to 23, and 24+ month groups (10% versus 7% versus 6%; p = 0.31). Conclusions: The prevalence of surgical, anesthetic, and device related complications was not significantly different among infants implanted less than 9 or less than 12 months of age when compared with older children. These data provide evidence for the continued expansion of pediatric cochlear implant candidacy criteria to include appropriately selected infants less than 9 months of age.

Abstract Background 3D printing—also known as additive manufacturing—has a wide range of applications. Reproduction of low-cost, high-fidelity, disease- or patient-specific models presents a key developmental area in simulation and education research for complex cranial surgery. Methods Using cadaveric dissections as source materials, skull base models were created, printed, and tested for educational value in teaching complex cranial approaches. In this pilot study, assessments were made on the value of 3D printed models demonstrating the retrosigmoid and posterior petrosectomy approaches. Models were assessed and tested in a small cohort of neurosurgery resident subjects (n = 3) using a series of 10 radiographic and 2 printed case examples, with efficacy determined via agreement survey and approach selection accuracy. Results All subjects indicated agreement or strong agreement for all study endpoints that 3D printed models provided significant improvements in understanding of neuroanatomic relationships and principles of approach selection, as compared to 2D dissections or patient cross-sectional imaging alone. Models were not superior to in-person hands-on teaching. Mean approach selection accuracy was 90% (±13%) for 10 imaging-based cases, or 92% (±7%) overall. Trainees strongly agreed that approach decision-making was enhanced by adjunctive use of 3D models for both radiographic and printed cases. Conclusion 3D printed models incorporating skull base approaches and/or pathologies provide a compelling addition to the complex cranial education armamentarium. Based on our preliminary analysis, 3D printed models offer substantial potential for pedagogical value as dissection guides, adjuncts to preoperative study and case preparation, or tools for approach selection training and evaluation.

Background: Previous cross-sectional studies analyzing quality of life (QOL) outcomes in patients with sporadic vestibular schwannoma (VS) have shown surprisingly little difference among treatment modalities. To date, there is limited prospective QOL outcome data available comparing baseline to posttreatment scores. Study Design: Prospective longitudinal study using the disease-specific Penn Acoustic Neuroma Quality of Life (PANQOL) scale. Setting: Large academic skull base center. Patients: Patients diagnosed with unilateral VS who completed a baseline survey before treatment and at least one posttreatment survey. Main Outcome Measures: Change in PANQOL scores from baseline to most recent survey. Results: A total of 244 patients were studied, including 78 (32%) who elected observation, 118 (48%) microsurgery, and 48 (20%) stereotactic radiosurgery. Patients who underwent microsurgery were younger (p < 0.001) and had larger tumors (p < 0.001) than those who underwent observation or radiosurgery; there was no significant difference in duration of follow-up among management groups (mean 2.1 yrs; p = 0.28). When comparing the total PANQOL score at baseline to the most recent survey, the net change was only –1.1, –0.1, and 0.3 points on a 100-point scale for observation, microsurgery, and radiosurgery, respectively (p = 0.89). After multivariable adjustment for baseline features, there were no statistically significant changes when comparing baseline to most recent scores within each management group for facial function, general health, balance, hearing loss, energy, and pain domains or total score. However, the microsurgical group experienced a 10.8-point improvement (p = 0.002) in anxiety following treatment, compared with 1.5 (p = 0.73) and 5.3 (p = 0.31) for observation and radiosurgery, respectively. Conclusions: In this prospective longitudinal study investigating differences in QOL outcomes among VS treatment groups using the disease-specific PANQOL instrument, treatment did not modify QOL in most domains. Microsurgery may confer an advantage with regard to patient anxiety, presumably relating to the psychological benefit of “cure” from having the tumor removed.

Objective:To address variance in clinical care surrounding sporadic vestibular schwannoma, a modified Delphi study was performed to establish a general framework to approach vestibular schwannoma care. A multidisciplinary panel of experts was established with deliberate representation from key stakeholder societies. External validity of the final statements was assessed through an online survey of registered attendees of the 8th Quadrennial International Conference on Vestibular Schwannoma.Study Design:Modified Delphi method.Methods:The panel consisted of 16 vestibular schwannoma experts (8 neurotology and 8 neurosurgery) and included delegates representing the AAOHNSF, AANS/CNS tumor section, ISRS, and NASBS. The modified Delphi method encompassed a four-step process, comprised of one prevoting round to establish a list of focus areas and three subsequent voting rounds to successively refine individual statements and establish levels of consensus. Thresholds for achieving moderate consensus, at ≥67% agreement, and strong consensus, at ≥80% agreement, were determined a priori. All voting was performed anonymously via the Qualtrics online survey tool and full participation from all panel members was required before procession to the next voting round.Results:Through the Delphi process, 103 items were developed encompassing hearing preservation (N = 49), tumor control and imaging surveillance (N = 20), preferred treatment (N = 24), operative considerations (N = 4), and complications (N = 6). As a result of item refinement, moderate (4%) or strong (96%) consensus was achieved in all 103 final statements. Seventy-nine conference registrants participated in the online survey to assess external validity. Among these survey respondents, moderate (N = 21, 20%) or strong (N = 73, 71%) consensus was achieved in 94 of 103 (91%) statements, and no consensus was reached in 9 (9%). Of the four items with moderate consensus by the expert panel, one had moderate consensus by the conference participants and three had no consensus.Conclusion:This modified Delphi study on sporadic vestibular schwannoma codifies 100% consensus within a multidisciplinary expert panel and is further supported by 91% consensus among an external group of clinicians who regularly provide care for patients with vestibular schwannoma. These final 103 statements address clinically pragmatic items that have direct application to everyday patient care. This document is not intended to define standard of care or drive insurance reimbursement, but rather to provide a general framework to approach vestibular schwannoma care for providers and patients.

Objective: To assess vestibular schwannoma (VS) practice patterns among providers. Study Design: Cross-sectional survey. Setting: 8th Quadrennial International Conference on Vestibular Schwannoma and Other CPA Tumors. Subjects: Clinicians who specialize in the management of VS. Main Outcome Measures: Responses to questions on the management and anticipated outcomes of VS for a series of common clinical scenarios were compared by specialty (otolaryngology versus neurosurgery), level of experience, scope of practice (surgery versus radiation and surgery), and geographic location of practice (United States versus international). Results: Responses from 110 participants were analyzed. Overall, 53% of respondents were otolaryngologists, 60% had greater than 10 years of experience, and 57% practiced within the United States. In total, 86% of respondents would pursue initial observation for themselves if diagnosed with a 4 mm distal intracanalicular VS; however, practicing radiosurgeons were more likely to select stereotactic radiosurgery for this scenario compared with providers who solely practice surgery (14 versus 0%; p = 0.032). Otolaryngologists and neurosurgeons alike report that radiosurgery should not be considered a long-term hearing preservation strategy. Otolaryngologists were more optimistic regarding microsurgical hearing preservation outcomes for small distal intracanalicular tumors compared with neurosurgeons (11 versus 3% selected a high likelihood of maintaining class A/B hearing; p = 0.007). Ninety-five percent of respondents prioritized facial nerve outcome over complete disease removal in the context of microsurgical resection of large tumors. Conclusions: Management decision-making and expected outcomes for various clinical scenarios were largely similar among providers; however, variances in several key clinical areas exist. This study points to the feasibility of developing a widely accepted consensus statement among VS experts across specialties.

OBJECTIVE Intracranial facial nerve schwannomas (FNS) requiring treatment are frequently recommended for surgery or stereotactic radiosurgery (SRS). The objective of this study was to compare facial nerve function outcomes between these two interventions for FNS via a systematic review and meta-analysis. METHODS A search of the Ovid EMBASE, PubMed, SCOPUS, and Cochrane databases from inception to July 2019 was conducted following PRISMA guidelines. Articles were screened against prespecified criteria. Facial nerve outcomes were classified as improved, stabilized, or worsened by last follow-up. Incidence was pooled by random-effects meta-analysis of proportions. RESULTS Thirty-three articles with a pooled cohort of 519 patients with FNS satisfied all criteria. Twenty-five articles described operative outcomes in 407 (78%) patients; 10 articles reported SRS outcomes in 112 (22%). In the surgical cohort, facial nerve function improved in 23% (95% CI 15%–32%), stabilized in 41% (95% CI 32%–50%), and worsened in 30% (95% CI 21%–40%). In the SRS cohort, facial nerve function was improved in 20% (95% CI 9%–34%), stable in 66% (95% CI 54%–78%), and worsened in 9% (95% CI 3%–16%). Compared with SRS, microsurgery was associated with a significantly lower incidence of stable facial nerve function (p < 0.01) and a significantly higher incidence of worsened facial nerve function (p < 0.01). Tumor progression and complication rates were comparable. Outcome certainty assessments were very low to moderate for all parameters. CONCLUSIONS Unfavorable facial nerve function outcomes are associated with surgical treatment of intracranial FNS, whereas stable facial nerve function outcomes are associated with SRS. Therefore, SRS should be recommended to patients with FNS who require treatment, and surgery should be reserved for patients with another indication, such as decompression of the brainstem. Further study is required to definitively optimize and validate management strategies for these rare skull base tumors.

Objective: To evaluate adverse events and feasibility of performing 1.5-T MRI in patients with cochlear implants (CI) and auditory brainstem implants (ABI). Setting: Single tertiary academic referral center. Patients: CI and ABI recipients undergoing 1.5-T MRI without internal magnet removal. Intervention(s): MRI after tight headwrap application. Main Outcome Measures: Adverse events, patient tolerance. Results: A total of 131 MR studies in 79 patients were performed, with a total of 157 study ears. Sixty-one patients (77%) had unilateral devices. Four patients (5%) underwent MRI with ABI magnets in place. Sixteen patients (20%) had MRI-compatible devices that did not require a head wrap. There were no instances of device stimulation, device malfunction, or excessive heating of the receiver-stimulator package. Magnet tilt requiring manual repositioning occurred during seven studies (4.5%) and magnet displacement requiring operative intervention occurred during seven studies (4.5%). Significant pain where imaging had to be discontinued occurred during three episodes (2%). No adverse events were noted among patients who underwent MRI with an MRI-compatible magnet. Conclusions: MRI with CI or ABI magnets in place is associated with a low prevalence of adverse events when performed in a controlled setting. Many partial magnet displacements can be corrected with firm manual pressure. Devices with magnets that align with the field within their housing were not associated with any adverse events and do not require immobilization of the magnet during the scan. These may be valuable in patients with known or anticipated need for MRI.

PurposeMore than 460 million people are impacted by disabling hearing loss worldwide. Hearing loss has negative impacts across the life span and varies by individual. Specifically, in children, hearing loss has been shown to affect learning outcomes, behavioral and cognitive growth, and psychosocial development. In adults, hearing loss has been shown to affect quality of life, social isolation, depression, and anxiety. The purpose of this review is to advance the understanding of the latest research in the development of new treatments of hearing loss, including gene therapy, stem cell therapy, and pharmacotherapies. We address the use of animals to model human hearing loss and the importance of animals in previous discoveries and developing new therapies. We highlight the importance of early identification for better speech and developmental outcomes specifically in pediatric individuals. Lastly, we review new possibilities in the prevention of hearing loss and the path to translation into practice.ConclusionsResearch and development of new therapies to treat hearing loss is an ever growing field with many notable successes. However, clinical translation still poses many challenges. Many discoveries related to gene therapy, stem cell therapy, and pharmacotherapy are in the early phases of clinical trials and hold promise. This review empowers audiologists to be aware of these most recent developments and be conscious of the evolving future landscape of treatment and management of hearing loss.