Neff, Brian A.

OBJECTIVE Preoperative differentiation of facial nerve schwannoma (FNS) from vestibular schwannoma (VS) can be challenging, and failure to differentiate between these two pathologies can result in potentially avoidable facial nerve injury. This study presents the combined experience of two high-volume centers in the management of intraoperatively diagnosed FNSs. The authors highlight clinical and imaging features that can distinguish FNS from VS and provide an algorithm to help manage intraoperatively diagnosed FNS. METHODS Operative records of 1484 presumed sporadic VS resections between January 2012 and December 2021 were reviewed, and patients with intraoperatively diagnosed FNSs were identified. Clinical data and preoperative imaging were retrospectively reviewed for features suggestive of FNS, and factors associated with good postoperative facial nerve function (House-Brackmann [HB] grade ≤ 2) were identified. A preoperative imaging protocol for suspected VS and recommendations for surgical decision-making following an intraoperative FNS diagnosis were created. RESULTS Nineteen patients (1.3%) with FNSs were identified. All patients had normal facial motor function preoperatively. In 12 patients (63%), preoperative imaging demonstrated no features suggestive of FNS, with the remainder showing subtle enhancement of the geniculate/labyrinthine facial segment, widening/erosion of the fallopian canal, or multiple tumor nodules in retrospect. Eleven (57.9%) of the 19 patients underwent a retrosigmoid craniotomy, and in the remaining patients, a translabyrinthine (n = 6) or transotic (n = 2) approach was used. Following FNS diagnosis, 6 (32%) of the tumors underwent gross-total resection (GTR) and cable nerve grafting, 6 (32%) underwent subtotal resection (STR) and bony decompression of the meatal facial nerve segment, and 7 (36%) underwent bony decompression only. All patients undergoing subtotal debulking or bony decompression exhibited normal postoperative facial function (HB grade I). At the last clinical follow-up, patients who underwent GTR with a facial nerve graft had HB grade III (3 of 6 patients) or IV facial function. Tumor recurrence/regrowth occurred in 3 patients (16%), all of whom had been treated with either bony decompression or STR. CONCLUSIONS Intraoperative diagnosis of an FNS during a presumed VS resection is rare, but its incidence can be reduced further by maintaining a high index of suspicion and undertaking further imaging in patients with atypical clinical or imaging features. If an intraoperative diagnosis does occur, conservative surgical management with bony decompression of the facial nerve only is recommended, unless there is significant mass effect on surrounding structures.

Intraoperative electrocochleography (ECOG) is performed using a single low-frequency acoustic stimulus (e.g., 500 Hz) to monitor cochlear microphonics (CM) during cochlear implant (CI) electrode insertion. A decrease in CM amplitude is commonly associated with cochlear trauma and is used to guide electrode placement. However, advancement of the recording electrode beyond the sites of CM generation can also lead to a decrease in CM amplitude and is sometimes interpreted as cochlear trauma, resulting in unnecessary electrode manipulation and increased risk of cochlear trauma during CI electrode placement. In the present study, multi-frequency ECOG was used to monitor CM during CI electrode placement. The intraoperative CM tracings were compared with electrode scan measurements, where CM was measured for each of the intracochlear electrodes. Comparison between the peak CM amplitude measured during electrode placement and electrode scan measurements was used to differentiate between different mechanisms for decrease in CM amplitude during CI electrode insertion. Analysis of the data shows that both multi-frequency electrocochleography and electrode scan could potentially be used to differentiate between different mechanisms for decreasing CM amplitude and providing appropriate feedback to the surgeon during CI electrode placement.

Objective To characterize the effect of the COVID-19 pandemic on national cochlear implantation utilization by age using inclusive cochlear implantation data from two manufacturers between 2015 and 2020. Study Design Analysis of prospectively registered consecutive patient data from two major cochlear implant (CI) manufacturers in the United States. Patients Children or adults who received CIs. Interventions Cochlear implantation. Main Outcome Measures Annual implantation utilization by age. Results A total of 46,804 patients received CIs from the two participating manufacturers between 2015 and 2020. The annual number of implant recipients increased significantly during the first 5 years of the study period for both children and adults, from a total of 6,203 in 2015 to 9,213 in 2019 (p < 0.001). During 2020, there was a 13.1% drop in national cochlear implantation utilization across all ages compared with 2019, including a drop of 2.2% for those ≤3 years old, 3.8% for those 4–17 years old, 10.1% for those 18–64 years old, 16.6% for those 65–79 years old, and 22.5% for those ≥80 years old. In a multivariable linear regression model, the percent drop in CIs differed significantly by age-group (p = 0.005). Conclusions Especially in light of the prepandemic projected CI counts for 2020, the COVID-19 pandemic reduced national cochlear implantation utilization by over 15% among Medicare-aged patients and by almost 25% among those ≥80 years old, resulting in more than a 3-year setback in total annual CIs. Children were less affected, with those ≤3 years old experiencing minimal interruption during 2020.

Objective To introduce and discuss implementation strategy for the Complete Cochlear Implant Care (CCIC) model, a highly-coordinated cochlear implant (CI) care delivery model requiring a single on-site visit for preoperative workup, surgery, and postoperative programming. Study Design Prospective, nonrandomized, two-arm clinical trial. Setting Tertiary referral CI center. Patients Adults who meet audiologic criteria for cochlear implantation. Interventions Cochlear implantation, coordinated care delivery, including remote programming. Main outcome measures Care delivery model feasibility and process implementation. Results Patients determined to be likely CI candidates based on routine audiometry are eligible for enrollment. The CCIC model uses telemedicine and electronic educational materials to prepare patients for same-day on-site consultation with CI surgery, same or next-day activation, and postoperative remote programming for 12 months. Implementation challenges include overcoming inertia related to the implementation of a new clinical workflow, whereas scalability of the CCIC model is limited by current hardware requirements for remote programming technology. A dedicated CCIC process coordinator is critical for overcoming obstacles in implementation and process improvement through feedback and iterative changes. Team and patient-facing materials are included and should be tailored to fit each unique CI program looking to implement CCIC. Conclusion The CCIC model has the potential to dramatically streamline hearing healthcare delivery. Implementation requires an adaptive approach, as obstacles may vary according to institutional infrastructure and policies.

Objectives: Compare speech perception performance growth and benchmark score achievement among adult cochlear implant (CI) recipients with single-sided deafness (SSD) versus bilateral moderate to profound hearing loss. Study Design: Retrospective matched cohort analysis. Setting: Tertiary referral center. Patients: Adults with SSD or bilateral moderate to profound hearing sensorineural hearing loss who underwent cochlear implantation from 2014 to 2019. Interventions: Cochlear implantation. Main outcome measures: Time-to-benchmark speech perception score (CNC, AzBio in quiet) and speech performance within first postoperative year. Results: Thirty-three SSD patients were matched to 66 bilateral hearing loss patients (referent cohort) for duration of deafness and preoperative ipsilateral CNC scores. Although SSD patients were more likely to achieve benchmark CNC scores more quickly compared with matched referents, this difference did not reach statistical significance (HR 1.72; 95% CI 0.78–3.82; p = 0.18). AzBio scores showed similar trends (HR 1.40; 95% CI 0.66–2.98; p = 0.38). At last follow-up, the SSD cohort had lower CNC (median 54% vs. 62%; p = 0.019) and AzBio scores (median 72% vs. 84%; p = 0.029) compared to the referent cohort. Conclusions: No significant difference in speech perception performance growth (i.e., time-to-benchmark speech perception score) was identified between SSD and bilateral hearing loss CI recipients, although patients with bilateral hearing loss achieved higher scores in the implanted ear within the first year of follow-up.

Objectives/Hypothesis To review hearing preservation after microsurgical resection of sporadic vestibular schwannomas according to tumor size. Study Design Retrospective cohort. Methods Baseline, intraoperative, and postoperative patient and tumor characteristics were retrospectively collected for a cohort who underwent hearing preservation microsurgery. Serviceable hearing was defined by a pure tone average ≤50 dB and word recognition score ≥50%. Results A total of 243 patients had serviceable hearing preoperatively. Fifty (21%) tumors were confined to the internal auditory canal, and the median tumor size was 16.2 mm (interquartile range [IQR] 11.3–23.2) for tumors with cerebellopontine angle extension. Serviceable hearing was maintained in 64% of patients with tumors confined to the internal auditory canal, 28% with cerebellopontine angle extension <15 mm, and 9% with cerebellopontine angle extension ≥15 mm. On multivariable analysis, the odds ratios of acquiring nonserviceable hearing postoperatively for tumors extending <15 mm and ≥15 mm into the cerebellopontine angle were 5.75 (95% confidence interval [CI] 2.13–15.53; P < .001) and 22.11 (95% CI 7.04–69.42; P < .001), respectively, compared with intracanalicular tumors. Conclusions The strongest predictor of hearing preservation with microsurgery after multivariable adjustment is tumor size. Approximately 10% of patients with tumors ≥15 mm of cerebellopontine angle extension will retain serviceable hearing after microsurgery. Furthermore, hearing preservation techniques offer cochlear nerve preservation and cochlear patency allowing for possible future cochlear implantation. An attempt at hearing preservation, including avoiding surgical approaches that necessarily sacrifice hearing, is worthwhile even in larger tumors if serviceable hearing is present preoperatively. Level of Evidence 4 Laryngoscope , 132:1657–1664, 2022

OBJECTIVE Detection of vestibular schwannoma (VS) growth during observation leads to definitive treatment at most centers globally. Although ≥ 2 mm represents an established benchmark of tumor growth on serial MRI studies, 2 mm of linear tumor growth is unlikely to significantly alter microsurgical outcomes. The objective of the current work was to ascertain where the magnitude of change in clinical outcome is the greatest based on size. METHODS A single-institution retrospective review of a consecutive series of patients with sporadic VS who underwent microsurgical resection between January 2000 and May 2020 was performed. Preoperative tumor size cutpoints were defined in 1-mm increments and used to identify optimal size thresholds for three primary outcomes: 1) the ability to achieve gross-total resection (GTR); 2) maintenance of normal House-Brackmann (HB) grade I facial nerve function; and 3) preservation of serviceable hearing (American Academy of Otolaryngology–Head and Neck Surgery class A/B). Optimal size thresholds were obtained by maximizing c-indices from logistic regression models. RESULTS Of 603 patients meeting inclusion criteria, 502 (83%) had tumors with cerebellopontine angle (CPA) extension. CPA tumor size was significantly associated with achieving GTR, postoperative HB grade I facial nerve function, and maintenance of serviceable hearing (all p < 0.001). The optimal tumor size threshold to distinguish between GTR and less than GTR was 17 mm of CPA extension (c-index 0.73). In the immediate postoperative period, the size threshold between HB grade I and HB grade > I was 17 mm of CPA extension (c-index 0.65). At the most recent evaluation, the size threshold between HB grade I and HB grade > I was 23 mm (c-index 0.68) and between class A/B and C/D hearing was 18 mm (c-index 0.68). Tumors within 3 mm of the 17-mm CPA threshold displayed similarly strong c-indices. Among purely intracanalicular tumors, linear size was not found to portend worse outcomes for all measures. CONCLUSIONS The probability of incurring less optimal microsurgical outcomes begins to significantly increase at 14–20 mm of CPA extension. Although many factors ultimately influence decision-making, when considering timing of microsurgical resection, using a size threshold range as depicted in this study offers an evidence-based approach that moves beyond reflexively recommending treatment for all tumors after detecting ≥ 2 mm of tumor growth on serial MRI studies.

Abstract Objectives Two pilot studies of AR‐42, a pan‐histone deacetylase inhibitor, in human neurofibromatosis type 2 (NF2), vestibular schwannomas (VS), and meningiomas are presented. Primary endpoints included safety, and intra‐tumoral pharmacokinetics (PK) and pharmacodynamics (PD). Methods Pilot 1 is a subset analysis of a phase 1 study of AR‐42 in solid tumors, which included NF2 or sporadic meningiomas. Tumor volumes and treatment‐related adverse events (TRAEs) are reported (NCT01129193). Pilot 2 is a phase 0 surgical study of AR‐42 assessing intra‐tumoral PK and PD. AR‐42 was administered for 3 weeks pre‐operatively. Plasma and tumor drug concentrations and p‐AKT expression were measured (NCT02282917). Results Pilot 1: Five patients with NF2 and two with sporadic meningiomas experienced a similar incidence of TRAEs to the overall phase I trial. The six evaluable patients had 15 tumors (8 VS, 7 meningiomas). On AR‐42, tumor volume increased in six, remained stable in eight, and decreased in one tumor. The annual percent growth rate decreased in eight, remained stable in three, and increased in four tumors. Pilot 2: Four patients with sporadic VS and one patient with meningioma experienced no grade 3/4 toxicities. Expression of p‐AKT decreased in three of four VS. All tumors had higher AR‐42 concentrations than plasma. Conclusions AR‐42 is safe. Tumor volumes showed a mixed response, but most slowed growth. On a 40‐mg regimen, drug concentrated in tumors and growth pathways were suppressed in most tumors, suggesting this may be a well‐tolerated and effective dose. A phase 2 study of AR‐42 for NF2‐associated tumors appears warranted. Level of Evidence 1b, 4.