Carlson, Matthew L.

Objective: Cochlear implantation (CI) under local anesthesia facilitates intraoperative behavioral patient feedback to potentially improve acoustic hearing preservation and limit intracochlear trauma. Patient feedback during electrode insertion is potentially confounded by loudness adaptation, a perception of tone decrease despite stable presentation, or insufficient intensity discrimination, a failure to detect a change in hearing despite one actually occurring. Both loudness adaptation and intensity discrimination are critical factors for awake CI and have not been systematically studied in the CI candidate population. Patients: Adult candidates for CI. Interventions: Under routine audiometric testing conditions in a soundproof booth, a standard behavioral audiogram, audiogram obtained in recorded ambient operating room (OR) noise, loudness adaptation to sustained suprathreshold (+10 and +20 dB) pure tones at 500, 1000, 2000, and 4000 Hz in both quiet and in ambient OR noise, and intensity discrimination to sustained suprathreshold (+10 and +20 dB) pure tones at 500, 1000, 2000, and 4000 Hz were performed. Main outcome measures: Pure tone average (PTA) in quiet and in ambient OR noise, proportion of patients reporting loudness adaptation at different suprathreshold levels and frequencies, and reliability of intensity discrimination. Results: In total, 20 ears were included for study. The median PTA was similar between testing in quiet (68 dB, IQR: 63 to 73) and in ambient OR noise (68 dB, IQR: 62 to 73). Most participants (n = 11, 55%) reported loudness adaptation when testing at +10 dB suprathreshold intensity, with a decreased proportion (n = 8, 40%) demonstrating adaptation at the higher tone intensity (+20 dB suprathreshold). Loudness adaptation varied among frequencies, with a trend toward increasing adaptation at higher frequencies at both testing intensities. Participants demonstrated excellent intensity discrimination, detecting a 5 dB difference when testing at +10 dB both in quiet and with background OR noise in 82 of the 85 (96%) tests performed across the frequency range, although with lower intensity discrimination at +20 dB, particularly in background OR noise. Conclusions: For CI candidates, pure tone audiometric performance did not significantly differ in background OR noise, confirming the reliability of intraoperative audiometric testing. For optimal tone presentation, there is decreasing loudness adaptation with increased tone presentation (+20 dB suprathreshold) and in lower frequencies of tone presentation. Intensity discrimination was reliable at a 5 dB increment across a range of testing parameters.

Objective: Auditory nerve monitoring through electrically evoked compound action potentials (eCAP) can be used to assess cochlear neural integrity during vestibular schwannoma (VS) resection. Herein, we report on 5 cochlear implant (CI) patients who underwent VS resection with present eCAP responses both intraoperatively and postoperatively. Patients either did not perceive any sound through their CI or rapidly adapted to electrical stimulation, suggesting that the presence of eCAP does not guarantee auditory nerve function in patients with VS resection. Patients: Five patients who underwent simultaneous VS translabyrinthine resection and cochlear implantation. Interventions: Simultaneous cochlear implantation and translabyrinthine microsurgical VS resection. Main outcome measure: Electrically evoked compound action potential. Results: All patients had eCAP responses present during their VS resection and for at least 50 days post-surgery. Three of the 5 patients did not perceive any sound with their cochlear implant. This shows that the presence of an eCAP alone does not guarantee a functional auditory nerve or auditory perception. The other 2 patients could hear non-speech sounds, but these sounds quickly became inaudible, indicating that their auditory nerve lacked the ability to integrate the electrical stimulus over time. Conclusions: The presence of an eCAP response does not guarantee auditory sensation through a CI. Although eCAP measurements provide insight into the function of the distal end of the nerve, it does not ascertain integrity or function of the larger auditory nerve pathway. Therefore, eCAPs should not be used in isolation to monitor auditory nerve function during VS resection.

Objective: To evaluate hearing outcomes after treatment with stereotactic radiosurgery (SRS) for sporadic vestibular schwannoma (VS) among patients with baseline 100% word recognition scores (WRS). Study design: Historical cohort. Setting: Tertiary academic medical center. Patients: Patients with 100% WRS on audiometric testing at initial treatment with SRS. Interventions: Gamma Knife SRS. Main outcome measures: Rate of maintaining serviceable hearing (SH), defined as American Academy of Otolaryngology-Head and Neck Surgery hearing class A or B. Results: In total, 125 patients had 100% WRS at SRS for sporadic VS. Median age at SRS was 56 years (IQR: 51 to 62) and median pure tone average (PTA) at SRS was 20 dB HL (IQR: 13 to 31). Sixty-eight patients progressed to non-SH at a median of 3.4 years after SRS (IQR: 1.0 to 8.6); the median duration of follow-up for the 57 patients who maintained SH was 5.1 years (IQR: 2.8 to 9.6). Rates of maintaining SH (95% CI, number still at risk) at 1, 3, 5, 7, and 10 years after SRS were 88% (82 to 94, 101), 75% (68 to 84, 81), 61% (53 to 71, 55), 58% (49 to 68, 43), and 44% (35 to 56, 26), respectively. Each 10-dB HL increase in PTA at SRS was associated with a 52% increased risk of progression to non-SH (hazard ratio: 1.52, 95% CI: 1.24-1.87, P <0.001). Conclusions: Despite having 100% pretreatment WRS, most patients with sporadic VS treated with SRS experienced clinically significant hearing decline over time. Even among this cohort of patients with excellent baseline hearing, at 10 years after treatment, only 44% of patients maintained SH.

OBJECTIVE The objective of this study was to describe the long-term efficacy of single-fraction stereotactic radiosurgery (SRS) for the primary treatment of sporadic vestibular schwannoma. METHODS Adult (≥ 18 years of age) patients with sporadic vestibular schwannoma who underwent SRS from 2000 through 2022 were included. RESULTS A total of 749 patients met inclusion criteria, the majority (n = 566, 76%) of whom had tumors extending into the cerebellopontine angle at SRS. The median patient age at SRS was 62 years, half (50%) of the patients were women, and 744 (99%) exhibited House-Brackmann grade I facial nerve function at SRS. A total of 42 patients experienced radiosurgical failure and underwent salvage treatment; the median duration of follow-up for the patients who did not undergo salvage treatment was 7.0 years. Overall tumor control rates (95% CI, number still at risk) at 1, 3, 5, 10, and 15 years after SRS were 100% (100%–100%, 718), 98% (97%–99%, 589), 96% (94%–97%, 464), 92% (90%–95%, 258), and 91% (89%–94%, 125), respectively. Patient age (hazard ratio [HR] for a 10-year increase of 0.92, 95% CI 0.71–1.19; p = 0.5), presence of a macrocystic tumor (HR 0.59, 95% CI 0.14–2.46; p = 0.5), and treated tumor volume (HR for a 1-cm 3 increase of 1.02, 95% CI 0.88–1.18; p = 0.8) were not significantly associated with the risk of salvage. Three distinct post-SRS tumor behavior patterns were observed, with 13% of patients demonstrating tumor pseudoprogression, all but 4 of whom demonstrated pseudoprogression by year 5 post-SRS. CONCLUSIONS SRS demonstrates durable tumor control through 15 years of follow-up in most patients (91%); however, a minority are found to still experience SRS failure even beyond 10 years of surveillance. Although occurring in only a minority of patients, tumor pseudoprogression can be evident out to 5 years or longer post-SRS.

OBJECTIVE Wait-and-scan surveillance is now commonly employed for initial management of small- and medium-sized vestibular schwannomas. Although small differences in tumor size are unlikely to impact outcomes significantly, treatment with either radiosurgery or microsurgery is usually recommended following radiological detection of tumor growth. The objective of the current study was to identify potential inflection points in vestibular schwannoma tumor size, where the risks of treatment with single-fraction stereotactic radiosurgery (SRS) accelerate to inform timing of intervention. METHODS Adult (≥ 18 years old) patients with sporadic vestibular schwannoma who underwent SRS from 2000 through 2022 were included. RESULTS A total of 749 patients with a median age at SRS of 62 years were studied, the majority (n = 566 [76%]) of whom had tumors extending into the cerebellopontine angle (CPA) at SRS. The optimal tumor size cut point to predict SRS failure and need for salvage treatment was 4 mm or more of CPA extension (c-index 0.59, HR 3.60, p = 0.01). The optimal tumor size cut point to predict the outcome of facial nerve paresis was 13 mm or more of CPA extension, resulting in a c-index of 0.63 (HR 2.88, p = 0.01). Among patients with at least 3 months of surveillance before SRS, those with a tumor growth rate ≥ 2.5 mm/year were more likely to undergo salvage treatment than those with a growth rate < 2.5 mm/year, although this difference did not achieve statistical significance (HR 1.82, p = 0.18). CONCLUSIONS The risk of SRS failure requiring salvage treatment and the risk of post-SRS facial nerve paralysis increase at sizes of approximately 4 and 13 mm extension into the CPA, respectively, providing a size threshold anchor to help guide treatment decision-making regarding timing of SRS. Furthermore, rapid tumor growth during the initial wait-and-scan period may be associated with an increased risk of radiosurgical failure, which may influence choice of treatment.

Objective: To examine the association of extent of otosclerotic plaque using high-resolution photon-counting computed tomography (PCCT) with hearing and surgical outcomes. Study design: Historical cohort. Setting: Large academic center. Patients: Patients with otosclerosis who completed PCCT imaging at time of initial evaluation. Interventions: Observation, surgery. Main outcome measures: Audiometric data. Results: A total of 53 ears were eligible for study. The mean age at PCCT was 51 years (SD: 14). The mean extent of oval window otosclerotic plaque measured in radial degrees was 112 (SD: 65) and the mean otosclerotic depth was 1.6mm (SD: 0.6). The mean air-bone gap pure-tone average was 18 dB (SD: 12) and the mean Carhart degree was 9 dB (SD: 10). The radial degrees of oval window extent were not significantly associated with air-bone gap (correlation coefficient: 0.14; P =0.3) or Carhart degree (correlation coefficient: 0.04; P =0.8). Likewise, otosclerotic plaque depth was not significantly associated with air-bone gap (correlation coefficient: −0.19; P =0.2) or Carhart degree (correlation coefficient: −0.08; P =0.6). After PCCT, 22 ears underwent surgery. In this subset, oval window extent and plaque depth were not significantly associated with the postoperative changes in air-bone gap or high-frequency thresholds. Conclusions: Despite enhanced visualization of subtle abnormalities with PCCT imaging, the extent of oval window otosclerotic plaque, measured in angular degrees and depth, was not associated with hearing loss at time of initial evaluation or postoperative audiologic changes in patients with otosclerosis.

Objective: To evaluate the impact of cochlear implantation (CI) and simultaneous endolymphatic sac decompression (ELSD) on vestibular and audiometric outcomes in patients with advanced Ménière disease (MD). Study design: Historical cohort. Setting: Tertiary academic medical center. Patients: Patients with medically refractory MD who underwent CI with or without simultaneous ELSD from 2000 through 2024. Interventions: CI with or without ELSD. Main outcome measures: Post-CI patient-reported changes in vertigo severity and frequency, Consonant-Nucleus-Consonant word (CNCw) scores, and AzBio sentences in quiet scores. Results: Ninety-nine patients with preoperative vertigo secondary to MD were eligible for study, including 17 (17%) who underwent simultaneous ELSD. In total, 15 (88%) and 2 (12%) of the 17 patients with simultaneous ELSD reported improvement and stability in vertigo severity, respectively; in comparison, 26 (32%), 40 (49%), and 16 (20%) of the 82 patients with CI alone reported improvement, stability, and worsening in vertigo severity, respectively ( P <0.001). Likewise, 15 (88%) and 2 (12%) of the patients with simultaneous ELSD reported improvement and stability in vertigo frequency, respectively, whereas 21 (26%), 43 (52%), and 18 (22%) of patients with CI alone reported improvement, stability, and worsening in vertigo frequency, respectively ( P <0.001). Post-CI speech recognition scores were not significantly different between the simultaneous ELSD and CI alone groups [median (IQR) CNCw 70 (38 to 82) vs. 72 (58 to 82) and median (IQR) AzBio quiet 87 (67 to 96) vs. 87 (73 to 94), respectively]. Conclusions: In patients with MD, CI with simultaneous ELSD provides improvement in vertigo symptoms and similar audiometric outcomes when compared with CI alone. In appropriately selected patients with both refractory vestibular symptoms and sensorineural hearing loss meeting criteria for implantation, CI with simultaneous ELSD offers improvement in severity and frequency of vertigo as well as successful auditory rehabilitation.

Hearing loss is a highly prevalent disease state affecting almost 1 in 5 individuals globally. Growing research shows that untreated or undertreated hearing loss carries significant long-term sequelae, including increased risk of social isolation, depression, and cognitive decline, among other ramifications. Limited awareness and common misperceptions among the general US adult population and primary care providers contribute to low rates of referral to hearing specialty clinics and underutilization of hearing aids, cochlear implants, and other rehabilitative devices. Comprised of diverse membership, the Hearing Health Collaborative (HHC) was formed in May 2021 to identify and overcome challenges impacting good health care practices and public policy on hearing care in connection with healthy aging. This editorial documents a recent outcome of the HHC derived from a structured A3 process. Specifically, initiative 2, a proposal to develop a simple, clinically anchored staging system primarily for use by frontline providers and patients, is presented. Unlike other common disorders that harbor long-term health sequelae, a corresponding pragmatic clinical staging system—leveraging precedent disease staging convention, based on clinically meaningful outcomes and prognosis for acquired hearing loss—does not exist. The development of this staging system will potentially allow for improved communication across providers and patients, facilitate referral pathways, and ultimately enable patient access to timely and appropriate care.

Objective: To evaluate the ethical implications, clinical benefits, and challenges associated with centralizing the surgical care of vestibular schwannoma (VS) care. Databases reviewed: PubMed, Embase, CINAHL, Web of Science, Cochrane, Scopus. Methods: A scoping review was conducted per the PRISMA-ScR (Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews) guidelines. A comprehensive search of 6 electronic databases yielded 1,231 articles using keywords related to vestibular schwannoma, centralization of care, centers of excellence, and ethical considerations. After removing duplicates (n=344) and screening titles and abstracts (n=887), 17 full-text articles were reviewed. Two additional articles were identified through manual screening. A total of 15 studies were included in the final analysis. Results: Hospitals were categorized by surgical case volume (high, medium, low) to compare patient demographics and clinical outcomes. Patients seen at low-volume hospitals (LVHs) were generally older and more racially diverse, and more likely to have public insurance (Medicare or Medicaid), whereas high-volume hospitals (HVHs) served a younger, predominantly White, and privately insured population. Of the 14 studies evaluating outcomes, 12 demonstrated a positive association between higher surgical volume and improved outcomes, including reduced complications, lower mortality, shorter length of stay, and decreased hospitalization costs. High-volume hospitals also had significantly higher rates of routine discharge. Conclusion: Centralization of vestibular schwannoma care to high-volume centers is associated with improved clinical outcomes and reduced health care costs. However, these benefits must be carefully weighed against potential ethical concerns, including disparities in access related to socioeconomic status, insurance coverage, and geographic location.

Objective: The Complete Cochlear Implant Care (CCIC) model is a highly coordinated care delivery model reducing and condensing in-person visits for cochlear implant (CI) patients, leveraging telehealth and remote programming. Clinical, quality-of-life, implementation, patient experience, and financial outcomes of the clinical trial are reported. Study design: Prospective, nonrandomized, 2-arm clinical trial Setting: Tertiary referral CI center Patients: Adult CI candidates Interventions: Cochlear implantation Main outcome measures: Speech perception, quality of life, implementation, patient experience, and financial outcomes for both CCIC and traditional care delivery models. Results: Patients participating in the traditional and CCIC delivery models demonstrated 12-month postoperative speech perception scores comparable to nationally reported outcomes (CNC 61% and 72% and AzBio in quiet 75% and 87% for traditional and CCIC cohorts, respectively). Quality-of-life evaluation using the CIQOL-10 demonstrated similar 12-month postoperative scores between groups (52 and 54 for traditional and CCIC cohorts, respectively). The CI Patient Experience Survey demonstrated high satisfaction outcomes in ease of communication, preparedness for surgery, and overall care in both cohorts. Implementation analysis of the CCIC model revealed perceived benefits from staff and clinicians; however, support for expansion of the program was contingent upon the availability of app-based or web-based remote programming technology. Finally, financial analysis revealed decreased out-of-pocket costs for CCIC patients. Conclusion: The CCIC model has the potential to dramatically streamline hearing health care delivery and reduce out-of-pocket costs for CI recipients. Clinical outcomes between cohorts were comparable in this feasibility study; however, a sufficiently powered trial is required to definitively comment on clinical outcomes with the new care delivery model.