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Purpose: In cochlear implantation (CI) surgery, there are a wide variety of intraoperative tests available. However, no clear guide exists on which tests must be performed as the minimum intraoperative testing battery. Toward this end, we studied the usage patterns, recommendations, and attitudes of practitioners toward intraoperative testing., Methods: This study is a multicentric international survey of tertiary referral CI centers. A survey was developed and administered to a group of CI practitioners (n = 34) including otologists, audiologists and biomedical engineers. Thirty six participants were invited to participate in this study based on a their scientific outputs to the literature on the intraoperative testing in CI field and based on their high load of CI surgeries. Thirty four, from 15 countries have accepted the invitation to participate. The participants were asked to indicate the usage trends, perceived value, influence on decision making and duration of each intraoperative test. They were also asked to indicate which tests they believe should be included in a minimum test battery for routine cases., Results: Thirty-two (94%) experts provided responses. The most frequently recommended tests for a minimum battery were facial nerve monitoring, electrode impedance measurements, and measurements of electrically evoked compound action potentials (ECAPs). The perceived value and influence on surgical decision-making also varied, with high-resolution CT being rated the highest on both measures., Conclusion: Facial nerve monitoring, electrode impedance measurements, and ECAP measurements are currently the core tests of the intraoperative test battery for CI surgery., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: Due to the challenges of direct in vivo measurements in humans, previous studies of cochlear tonotopy primarily utilized human cadavers and animal models. This study uses cochlear implant electrodes as a tool for intracochlear recordings of acoustically evoked responses to achieve two primary goals: (1) to map the in vivo tonotopy of the human cochlea, and (2) to assess the impact of sound intensity and the creation of an artificial "third window" on this tonotopic map., Design: Fifty patients with hearing loss received cochlear implant electrode arrays. Postimplantation, pure-tone acoustic stimuli (0.25 to 4 kHz) were delivered, and electrophysiological responses were recorded from all 22 electrode contacts. The analysis included fast Fourier transformation to determine the amplitude of the first harmonic, indicative of predominantly outer hair cell activity, and tuning curves to identify the best frequency (BF) electrode. These measures, coupled with postoperative imaging for precise electrode localization, facilitated the construction of an in vivo frequency-position function. The study included a specific examination of 2 patients with auditory neuropathy spectrum disorder (ANSD), with preserved cochlear function as assessed by present distortion-product otoacoustic emissions, to determine the impact of sound intensity on the frequency-position map. In addition, the electrophysiological map was recorded in a patient undergoing a translabyrinthine craniotomy for vestibular schwannoma removal, before and after creating an artificial third window, to explore whether an experimental artifact conducted in cadaveric experiments, as was performed in von Békésy landmark experiments, would produce a shift in the frequency-position map., Results: A significant deviation from the Greenwood model was observed in the electrophysiological frequency-position function, particularly at high-intensity stimulations. In subjects with hearing loss, frequency tuning, and BF location remained consistent across sound intensities. In contrast, ANSD patients exhibited Greenwood-like place coding at low intensities (~40 dB SPL) and a basal shift in BF location at higher intensities (~70 dB SPL or greater). Notably, creating an artificial "third-window" did not alter the frequency-position map., Conclusions: This study successfully maps in vivo tonotopy of human cochleae with hearing loss, demonstrating a near-octave shift from traditional frequency-position maps. In patients with ANSD, representing more typical cochlear function, intermediate intensity levels (~70 to 80 dB SPL) produced results similar to high-intensity stimulation. These findings highlight the influence of stimulus intensity on the cochlear operational point in subjects with hearing loss. This knowledge could enhance cochlear implant programming and improve auditory rehabilitation by more accurately aligning electrode stimulation with natural cochlear responses., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To prospectively evaluate the association between hearing preservation after cochlear implantation (CI) and intracochlear electrocochleography (ECochG) amplitude parameters., Study Design: Multi-institutional, prospective randomized clinical trial., Setting: Ten high-volume, tertiary care CI centers., Patients: Adults (n = 87) with sensorineural hearing loss meeting CI criteria (2018-2021) with audiometric thresholds of ≤80 dB HL at 500 Hz., Methods: Participants were randomized to CI surgery with or without audible ECochG monitoring. Electrode arrays were inserted to the full-depth marker. Hearing preservation was determined by comparing pre-CI, unaided low-frequency (125-, 250-, and 500-Hz) pure-tone average (LF-PTA) to LF-PTA at CI activation. Three ECochG amplitude parameters were analyzed: 1) insertion track patterns, 2) magnitude of ECochG amplitude change, and 3) total number of ECochG amplitude drops., Results: The Type CC insertion track pattern, representing corrected drops in ECochG amplitude, was seen in 76% of cases with ECochG "on," compared with 24% of cases with ECochG "off" ( p = 0.003). The magnitude of ECochG signal drop was significantly correlated with the amount of LF-PTA change pre-CI and post-CI ( p < 0.05). The mean number of amplitude drops during electrode insertion was significantly correlated with change in LF-PTA at activation and 3 months post-CI ( p ≤ 0.01)., Conclusions: ECochG amplitude parameters during CI surgery have important prognostic utility. Higher incidence of Type CC in ECochG "on" suggests that monitoring may be useful for surgeons in order to recover the ECochG signal and preventing potentially traumatic electrode-cochlear interactions., (Copyright © 2024, Otology & Neurotology, Inc.)

Objective: To validate electrocochleography (ECochG) between an auditory evoked potential (AEP) machine and an established cochlear implant (CI) manufacturer ECochG system., Methods: Intraoperative validation study at a tertiary referral center. Patients included adults and children undergoing cochlear implantation. Intraoperative ECochG was measured with both the Intelligent Hearing Systems (IHS) Duet AEP machine and Cochlear Corporation (CC) ECochG platform. Recording electrodes captured extracochlear measurements through a standard facial recess. Tone-bursts were presented from 250 Hz to 2 kHz (~110 dB SPL). A fast Fourier transform (FFT) of ECochG waveforms at key frequencies was summed into a total response (ECochG-TR). Pearson's correlation was utilized to evaluate the relationship between IHS-ECochG-TR and CC-ECochG-TR after confirming normality., Results: Thirty patients were enrolled with an average age of 67 years (SD 18.8). In the ear that was implanted, mean preoperative pure-tone average (PTA; 0.5, 1, 2, and 4 kHz) was 87.4 dB HL (SD 19.3) and mean preoperative word-recognition scores (WRS) was 17.0% correct (SD 19.1). There was strong correlation (r = 0.905, 95% confidence interval: 0.809 to 0.954) between IHS-ECochG-TR (median 2.30 μV, range 0.1-148.26) and CC-ECochG-TR (median 3.00 μV, range 0.1-239.63). Four patients underwent transtympanic ECochG with the IHS system for feasibility evaluation and achieved similar responses., Conclusion: Extracochlear ECochG has been predictive of CI speech perception performance. The IHS duet system is a valid measure of extracochlear ECochG for the CI population. Future work will utilize this system for measuring transtympanic ECochG to improve preoperative estimation of CI performance., Level of Evidence: 3 Laryngoscope, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To describe the collection methods for perilymph fluid biopsy during cochlear implantation, detect levels of amyloid β 42 and 40 (Aβ 42 and Aβ 40 ), and total tau (tTau) analytes with a high-precision assay, to compare these levels with patient age and Montreal Cognitive Assessment (MoCA) scores, and explore potential mechanisms and relationships with otic pathology., Study Design: Prospective study., Setting: Tertiary referral center., Methods: Perilymph was collected from 25 patients using polyimide tubing to avoid amyloid adherence to glass, and analyzed with a single-molecule array advanced digital enzyme-linked immunosorbent assay platform for Aβ 40 , Aβ 42 , and tTau. Cognition was assessed by MoCA., Results: Perilymph volumes ranged from ∼1 to 13 µL, with analyte concentrations spanning 2.67 to 1088.26 pg/mL. All samples had detectable levels of tTau, Aβ 40 , and Aβ 42 , with a significant positive correlation between Aβ 42 and Aβ 40 levels. Levels of Aβ 42 , Aβ 40 , and tTau were positively correlated with age, while MoCA scores were inversely correlated with age. tTau and Aβ 42 /Aβ 40 -ratios were significantly correlated with MoCA scores., Conclusion: Alzheimer's disease-associated peptides Aβ 42 , Aβ 40 , and tau analytes are detectable in human perilymph at levels approximately 10-fold lower than those found in cerebrospinal fluid (CSF). These species increase with age and correlate with cognitive impairment indicators, suggesting their potential utility as biomarkers for cognitive impairment in patients undergoing cochlear implantation. Future research should investigate the origin of these analytes in the perilymph and their potential links to inner ear pathologies and hearing loss, as well as their relationships to CSF and plasma levels in individuals., (© 2024 American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

Objectives: A wide variety of intraoperative tests are available in cochlear implantation. However, no consensus exists on which tests constitute the minimum necessary battery. We assembled an international panel of clinical experts to develop, refine, and vote upon a set of core consensus statements., Design: A literature review was used to identify intraoperative tests currently used in the field and draft a set of provisional statements. For statement evaluation and refinement, we used a modified Delphi consensus panel structure. Multiple interactive rounds of voting, evaluation, and feedback were conducted to achieve convergence., Results: Twenty-nine provisional statements were included in the original draft. In the first voting round, consensus was reached on 15 statements. Of the 14 statements that did not reach consensus, 12 were revised based on feedback provided by the expert practitioners, and 2 were eliminated. In the second voting round, 10 of the 12 revised statements reached a consensus. The two statements which did not achieve consensus were further revised and subjected to a third voting round. However, both statements failed to achieve consensus in the third round. In addition, during the final revision, one more statement was decided to be deleted due to overlap with another modified statement., Conclusions: A final core set of 24 consensus statements was generated, covering wide areas of intraoperative testing during CI surgery. These statements may provide utility as evidence-based guidelines to improve quality and achieve uniformity of surgical practice., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 The Authors. Ear & Hearing is published on behalf of the American Auditory Society, by Wolters Kluwer Health, Inc.)

Objectives: Modern cochlear implants (CIs) use varying-length electrode arrays inserted at varying insertion angles within variably sized cochleae. Thus, there exists an opportunity to enhance CI performance, particularly in postlinguistic adults, by optimizing the frequency-to-place allocation for electrical stimulation, thereby minimizing the need for central adaptation and plasticity. There has been interest in applying Greenwood or Stakhovskaya et al. function (describing the tonotopic map) to postoperative imaging of electrodes to improve frequency allocation and place coding. Acoustically-evoked electrocochleography (ECochG) allows for electrophysiologic best-frequency (BF) determination of CI electrodes and the potential for creating a personalized frequency allocation function. The objective of this study was to investigate the correlation between early speech-perception performance and frequency-to-place mismatch., Design: This retrospective study included 50 patients who received a slim perimodiolar electrode array. Following electrode insertion, five acoustic pure-tone stimuli ranging from 0.25 to 2 kHz were presented, and electrophysiological measurements were collected across all 22 electrode contacts. Cochlear microphonic tuning curves were subsequently generated for each stimulus frequency to ascertain the BF electrode or the location corresponding to the maximum response amplitude. Subsequently, we calculated the difference between the stimulus frequency and the patient's CI map's actual frequency allocation at each BF electrode, reflecting the frequency-to-place mismatch. BF electrocochleography-total response (BF-ECochG-TR), a measure of cochlear health, was also evaluated for each subject to control for the known impact of this measure on performance., Results: Our findings showed a moderate correlation (r = 0.51; 95% confidence interval: 0.23 to 0.76) between the cumulative frequency-to-place mismatch, as determined using the ECochG-derived BF map (utilizing 500, 1000, and 2000 Hz), and 3-month performance on consonant-nucleus-consonant words (N = 38). Larger positive mismatches, shifted basal from the BF map, led to enhanced speech perception. Incorporating BF-ECochG-TR, total mismatch, and their interaction in a multivariate model explained 62% of the variance in consonant-nucleus-consonant word scores at 3 months. BF-ECochG-TR as a standalone predictor tended to overestimate performance for subjects with larger negative total mismatches and underestimated the performance for those with larger positive total mismatches. Neither cochlear diameter, number of cochlear turns, nor apical insertion angle accounted for the variability in total mismatch., Conclusions: Comparison of ECochG-BF derived tonotopic electrode maps to the frequency allocation tables reveals substantial mismatch, explaining 26.0% of the variability in CI performance in quiet. Closer examination of the mismatch shows that basally shifted maps at high frequencies demonstrate superior performance at 3 months compared with those with apically shifted maps (toward Greenwood and Stakhovskaya et al.). The implications of these results suggest that electrophysiological-based frequency reallocation might lead to enhanced speech-perception performance, especially when compared with conventional manufacturer maps or anatomic-based mapping strategies. Future research, exploring the prospective use of ECochG-based mapping techniques for frequency allocation is underway., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To examine the association between preoperative comorbidities and cochlear implant speech outcomes., Study Design: Retrospective cohort., Setting: Tertiary referral center., Patients: A total of 976 patients who underwent cochlear implantation (CI) between January 2015 and May 2022. Adult patients with follow-up, preoperative audiologic data, and a standardized anesthesia preoperative note were included., Exposure: Adult Comorbidity Evaluation 27 (ACE-27) based on standardized anesthesia preoperative notes., Main Outcome Measures: Postoperative change in consonant-nucleus-consonant (CNC) score, AzBio Sentence score in quiet, and AzBio + 10 dB signal-to-noise ratio (SNR). Sentence score of the implanted ear at 3, 6, and 12 months., Results: A total of 560 patients met inclusion criteria; 112 patients (20%) had no comorbidity, 204 patients (36.4%) had mild comorbidities, 161 patients (28.8%) had moderate comorbidities, and 83 patients (14.8%) had severe comorbidities. Mixed model analysis revealed all comorbidity groups achieved a clinically meaningful improvement in all speech outcome measures over time. This improvement was significantly different between comorbidity groups over time for AzBio Quiet ( p = 0.045) and AzBio + 10 dB SNR ( p = 0.0096). Patients with severe comorbidities had worse outcomes. From preop to 12 months, the estimated marginal mean difference values (95% confidence interval) between the no comorbidity group and the severe comorbidity group were 52.3 (45.7-58.9) and 32.5 (24.6-40.5), respectively, for AzBio Quiet; 39.5 (33.8-45.2) and 21.2 (13.6-28.7), respectively, for AzBio + 10 dB SNR; and 43.9 (38.7-49.0) and 31.1 (24.8-37.4), respectively, for CNC., Conclusions: Comorbidities as assessed by ACE-27 are associated with CI performance. Patients with more severe comorbidities have clinically meaningful improvement but have worse outcome compared to patients with no comorbidities., Competing Interests: Conflicts of interests: DK and JFP own stock options for PotentiaMetrics, but the work of the company is not related to the present article. CCW is a consultant for Stryker Corporation and Cochlear Ltd.; JAH is a consultant for Cochlear Ltd., CAB is a consultant for Advanced Bionics, Cochlear Ltd., Envoy, and IotaMotion and has equity interest in Advanced Cochlear Diagnostics, LLC.; MAS is a consultant for Cochlear Ltd., (Copyright © 2024, Otology & Neurotology, Inc.)

Objective: To evaluate the predictive value of intracochlear electrocochleography (ECochG) for identifying tip fold-over during cochlear implantation (CI) using the slim modiolar electrode (SME) array., Study Design: Prospective cohort study., Setting: Tertiary referral center., Methods: From July 2022 to June 2023, 142 patients, including adults and children, underwent intracochlear ECochG monitoring during and after SME placement. Tone-bursts were presented from 250 Hz to 2 kHz at 108 to 114 dB HL. A fast Fourier transform (FFT) allowed for frequency-specific evaluation of ECochG response. ECochG patterns during insertion and postinsertion were evaluated using sensitivity and specificity analysis to predict tip fold-over. Intraoperative plain radiographs served as a reference standard., Results: Fifteen tip fold-over cases occurred (10.6%) with significant ECochG response (>2 µV). Sixty-one cases without tip fold-over occurred (43.0%) with significant ECochG response. All tip fold-overs had both a nontonotopic postinsertion sweep and nonrobust active insertion pattern. No patients with robust insertion or tonotopic sweep patterns had tip fold-over. Sensitivity of detecting tip fold-over when having both nonrobust insertion and nontonotopic sweep patterns was 100% (95% confidence inteval [CI] 78.2%-100%), specificity was 68.9% (95% CI 55.7%-80.1%), and the overall accuracy was 72.0% (95% CI 60.5%-81.7%)., Conclusion: Intracochlear ECochG monitoring during cochlear implantation with the SME can be a valuable tool for identifying properly positioned electrode arrays. In cases where ECochG patterns are nonrobust on insertion and nontonotopic for electrode sweeps, there may be a concern for tip fold-over, and intraoperative imaging is necessary to confirm proper insertion., (© 2023 American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

Vestibular schwannomas (VS) are benign tumors that lead to significant neurologic and otologic morbidity. How VS heterogeneity and the tumor microenvironment (TME) contribute to VS pathogenesis remains poorly understood. In this study, we perform scRNA-seq on 15 VS, with paired scATAC-seq (n = 6) and exome sequencing (n = 12). We identify diverse Schwann cell (SC), stromal, and immune populations in the VS TME and find that repair-like and MHC-II antigen-presenting SCs are associated with myeloid cell infiltrate, implicating a nerve injury-like process. Deconvolution analysis of RNA-expression data from 175 tumors reveals Injury-like tumors are associated with larger tumor size, and scATAC-seq identifies transcription factors associated with nerve repair SCs from Injury-like tumors. Ligand-receptor analysis and in vitro experiments suggest that Injury-like VS-SCs recruit myeloid cells via CSF1 signaling. Our study indicates that Injury-like SCs may cause tumor growth via myeloid cell recruitment and identifies molecular pathways that may be therapeutically targeted., (© 2023. The Author(s).)

Objectives: Early hearing detection and intervention (EHDI) is a newborn hearing screening system created to detect infants with hearing loss (HL) and intervene to reduce language and communication impairment. Early hearing detection (EHD) consists of three sequential stages: identification, screening, and diagnostic testing. This study longitudinally reviews each stage of EHD in each state and proposes a framework to improve utilization of EHD data., Design: A retrospective public database review was conducted, accessing publicly available data from the Centers for Disease Control and Prevention. Summary descriptive statistics were utilized to generate a descriptive study of EHDI programs in each U.S. state from 2007 to 2016., Results: Data over 10 years from 50 states as well as Washington, DC were included in this analysis, creating up to 510 data points per analysis. Hundred percent (85 to 105) (median [min to max]) of newborns were identified by and entered EHDI programs. Ninety-eight percent (51 to 100) of identified infants completed screening. Of the infants who screened positive for HL, the proportion that received diagnostic testing was 55% (1 to 100). The overall proportion of infants who failed to complete EHD was 3% (1 to 51). Of the infants who fail to complete EHD 70% (0 to 100) are from missed screenings, 24% (0 to 95) are from missed diagnostic testing, and 0% (0 to 93) are from missed identification. Although there are more infants missed at screening, it was estimated, with limitations, that there is an order of magnitude more infants with HL among those who did not complete diagnostic testing compared with those who did not complete screening., Conclusions: Analysis demonstrates high completion rates at both identification and screening stages, whereas the diagnostic testing stage demonstrates low and highly variable completion rates. The low completion rates at diagnostic testing create a bottleneck in the EHD process and the large variability impedes the comparison of HL outcomes across states. Analysis also demonstrates that among all stages of EHD, whereas the largest number of infants are missed at screening, the largest number of children with HL are likely missed at diagnostic testing. Therefore, a focus by individual EHDI programs on addressing causes of low diagnostic testing completion rates would yield the greatest increase in the identification of children with HL. Potential causes of low diagnostic testing completion rates are further discussed. Finally, a new vocabulary framework is proposed to facilitate further study of EHD outcomes., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Importance: Cochlear implantation produces remarkable results in postlingual deafness, although auditory outcomes vary. Electrocochleography (ECochG) has emerged as a valuable tool for assessing the cochlear-neural substrate and evaluating patient prognosis., Objective: To assess whether ECochG-total response (ECochG-TR) recorded at the best-frequency electrode (BF-ECochG-TR) correlates more strongly with speech perception performance than ECochG-TR measured at the round window (RW-ECochG-TR)., Design, Setting, and Participants: This single-center cross-sectional study recruited 142 patients from July 1, 2021, to April 30, 2022, with 1-year follow-up. Exclusions included perilymph suctioning, crimped sound delivery tubes, non-native English speakers, inner ear malformations, nonpatent external auditory canals, or cochlear implantation revision surgery., Exposures: Cochlear implantation., Main Outcomes and Measures: Speech perception testing, including the consonant-nucleus-consonant (CNC) words test, AzBio sentences in quiet, and AzBio sentences in noise plus 10-dB signal to noise ratio (with low scores indicating poor performance and high scores indicating excellent performance on all tests), at 6 months postoperatively; and RW-ECochG-TR and BF-ECochG-TR (measured for 250, 500, 1000, and 2000 Hz)., Results: A total of 109 of the 142 eligible postlingual adults (mean [SD] age, 68.7 [15.8] years; 67 [61.5%] male) were included in the study. Both BF-ECochG-TR and RW-ECochG-TR were correlated with 6-month CNC scores (BF-ECochG-TR: r = 0.74; 95% CI, 0.62-0.82; RW-ECochG-TR: r = 0.67; 95% CI, 0.54-0.76). A multivariate model incorporating age, duration of hearing loss, and angular insertion depth did not outperform BF-ECochG-TR or RW-ECochG-TR alone. The BF-ECochG-TR correlation with CNC scores was significantly stronger than the RW-ECochG-TR correlation (r difference = -0.18; 95% CI, -0.31 to -0.01; z = -2.02). More moderate correlations existed between 6-month AzBio scores in noise, Montreal Cognitive Assessment (MoCA) scores (r = 0.46; 95% CI, 0.29-0.60), and BF-ECochG-TR (r = 0.42; 95% CI, 0.22-0.58). MoCA and the interaction between BF-ECochG-TR and MoCA accounted for a substantial proportion of variability in AzBio scores in noise at 6 months (R2 = 0.50; 95% CI, 0.36-0.61)., Conclusions and Relevance: In this case series, BF-ECochG-TR was identified as having a stronger correlation with cochlear implantation performance than RW-ECochG-TR, although both measures highlight the critical role of the cochlear-neural substrate on outcomes. Demographic, audiologic, and surgical factors demonstrated weak correlations with cochlear implantation performance, and performance in noise was found to require a robust cochlear-neural substrate (BF-ECochG-TR) as well as sufficient cognitive capacity (MoCA). Future cochlear implantation studies should consider these variables when assessing performance and related interventions.

Objective: To evaluate the impact of preoperative and perioperative factors on postlinguistic adult cochlear implant (CI) performance and design a multivariate prediction model., Study Design: Prospective cohort study., Setting: Tertiary referral center., Patients and Interventions: Two hundred thirty-nine postlinguistic adult CI recipients., Main Outcome Measures: Speech-perception testing (consonant-nucleus-consonant [CNC], AzBio in noise +10-dB signal-to-noise ratio) at 3, 6, and 12 months postoperatively; electrocochleography-total response (ECochG-TR) at the round window before electrode insertion., Results: ECochG-TR strongly correlated with CNC word score at 6 months ( r = 0.71, p < 0.0001). A multivariable linear regression model including age, duration of hearing loss, angular insertion depth, and ECochG-TR did not perform significantly better than ECochG-TR alone in explaining the variability in CNC. AzBio in noise at 6 months had moderate linear correlations with Montreal Cognitive Assessment (MoCA; r = 0.38, p < 0.0001) and ECochG-TR ( r = 0.42, p < 0.0001). ECochG-TR and MoCA and their interaction explained 45.1% of the variability in AzBio in noise scores., Conclusions: This study uses the most comprehensive data set to date to validate ECochG-TR as a measure of cochlear health as it relates to suitability for CI stimulation, and it further underlies the importance of the cochlear neural substrate as the main driver in speech perception performance. Performance in noise is more complex and requires both good residual cochlear function (ECochG-TR) and cognition (MoCA). Other demographic, audiologic, and surgical variables are poorly correlated with CI performance suggesting that these are poor surrogates for the integrity of the auditory substrate., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Objectives: To examine long-term speech and cognition outcomes in older adult cochlear implant (CI) recipients. First, by evaluating if CI performance was maintained over an extended follow-up period regardless of preoperative cognitive status. Secondly, by evaluating if there was a difference in the rate of cognitive decline between preoperative mild and normal cognition following CI over an extended period of time., Study Design and Setting: Retrospective cohort study., Methods: CI recipients ≥65 years of age implanted between 2009 and 2014 with 4+ years follow up. Pre- and postoperative audiometric and speech outcome assessments were collected. Cognitive status was measured using the mini mental status examination (MMSE) at numerous time points., Results: Fifty-three patients met inclusion. Patients were divided into two groups based on preoperative MMSE with scores considered normal (28-30) and those with mildly impaired cognition (MIC, scores 25-27). Audiometric and speech performance improved significantly at one-year post implantation and this was maintained without significant change at 4+ years, regardless of cognitive status. Mixed modeling analysis controlling for age demonstrated no significant difference in the rate of cognitive decline at 4+ years post implantation between the normal cognition cohort (1.74; 95%CI 0.89-2.6) and MIC (2.9; 95%1.91-3.88)., Conclusion: Speech performance was significantly improved and sustained after CI in both normal cognition and MIC patients. The rate of cognitive decline in older adult CI patients appears to be similar regardless of preoperative cognitive status. Although results demonstrate rates of cognitive decline following CI did not differ between cognition groups over 4+ years, future studies will need to further investigate this over extended time periods with a more comprehensive cognitive testing battery., Level of Evidence: 4 Laryngoscope, 133:2379-2387, 2023., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: Compare postoperative speech outcomes in hearing preservation (HP) cochlear implantation (CI) patients with a low-frequency pure-tone average (LFPTA) ≤ 60 dB using 2 electrode array designs., Study Design: Retrospective cohort study., Setting: Large academic cochlear implant referral center., Methods: We reviewed adult HP CI cases using either the slim modiolar electrode (SME) (CI 532/CI 632) or th slim lateral wall electrode (SLWE) (CI 624). One-year speech outcomes and HP status were the primary outcomes., Results: A total of 132 implanted ears were analyzed (mean age 73.1 years, standard deviation [SD] 12.6), with 72% (N = 95) with CI 532/632 and 28% (N = 37) with CI 624. The mean preoperative LFPTA was 44.8 dB, SD 11.8. One-year functional HP was 27.2% (mean LFPTA shift 46.1 dB, SD 22.1) and was as follows: SME 23.9% and SLWE 36.4%, p = .168. The mean age at implantation was significantly younger only in SLWE patients with preserved hearing (66.9 vs 80.3 years, p = .008). At 6 months, speech measures were significantly better in all outcomes in HP patients with an SLWE than nonpreserved SLWE patients; this effect abated at 1 year as performance among nonpreserved SLWE patients became equivalent to the remaining cohort. Speech outcomes in SME patients were similar regardless of HP status. Age at implantation and datalogging was correlated with speech outcomes., Conclusion: In this cohort of HP patients, a 1-year functional HP rate of 23.9% (SME) and 36.4% (SLWE) was observed (p = 0.168). This was initially 57.1% (SME) and 70.3% (SLWE) at activation, p = .172. Datalogging and age at implantation were correlated with postoperative speech outcomes., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Cochlear synaptopathy is the loss of synapses between the inner hair cells and the auditory nerve despite survival of sensory hair cells. The findings of extensive cochlear synaptopathy in animals after moderate noise exposures challenged the long-held view that hair cells are the cochlear elements most sensitive to insults that lead to hearing loss. However, cochlear synaptopathy has been difficult to identify in humans. We applied novel algorithms to determine hair cell and neural contributions to electrocochleographic (ECochG) recordings from the round window of animal and human subjects. Gerbils with normal hearing provided training and test sets for a deep learning algorithm to detect the presence of neural responses to low frequency sounds, and an analytic model was used to quantify the proportion of neural and hair cell contributions to the ECochG response. The capacity to detect cochlear synaptopathy was validated in normal hearing and noise-exposed animals by using neurotoxins to reduce or eliminate the neural contributions. When the analytical methods were applied to human surgical subjects with access to the round window, the neural contribution resembled the partial cochlear synaptopathy present after neurotoxin application in animals. This result demonstrates the presence of viable hair cells not connected to auditory nerve fibers in human subjects with substantial hearing loss and indicates that efforts to regenerate nerve fibers may find a ready cochlear substrate for innervation and resumption of function., Competing Interests: DF, OA, CB, HP, and KB have consulting arrangements and research projects with MED-EL, Cochlear Corp, and/or Advanced Bionics. CB also consults for Envoy Medical and IotaMotion. OA and CB have an equity interest in Advanced Cochlear Diagnostics. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Haggerty, Hutson, Riggs, Brown, Pillsbury, Adunka, Buchman and Fitzpatrick.)

Objective: To address outcome heterogeneity in cochlear implant (CI) research, we built imputation models using multiple imputation by chained equations (MICEs) and K-nearest neighbors (KNNs) to convert between four common open-set testing scenarios: Consonant-Nucleus-Consonant word (CNCw), Arizona Biomedical (AzBio) in quiet, AzBio +5, and AzBio +10. We then analyzed raw and imputed data sets to evaluate factors affecting CI outcome variability., Study Design: Retrospective cohort study of a national CI database (HERMES) and a nonoverlapping single-institution CI database., Setting: Multi-institutional (32 CI centers)., Patients: Adult CI recipients (n = 4,046 patients)., Main Outcome Measures: Mean absolute error (MAE) between imputed and observed speech perception scores., Results: Imputation models of preoperative speech perception measures demonstrate a MAE of less than 10% for feature triplets of CNCw/AzBio in quiet/AzBio +10 (MICE: MAE, 9.52%; 95% confidence interval [CI], 9.40-9.64; KNN: MAE, 8.93%; 95% CI, 8.83-9.03) and AzBio in quiet/AzBio +5/AzBio +10 (MICE: MAE, 8.85%; 95% CI, 8.68-9.02; KNN: MAE, 8.95%; 95% CI, 8.74-9.16) with one feature missing. Postoperative imputation can be safely performed with up to four of six features missing in a set of CNCw and AzBio in quiet at 3, 6, and 12 months postcochlear implantation using MICE (MAE, 9.69%; 95% CI, 9.63-9.76). For multivariable analysis of CI performance prediction, imputation increased sample size by 72%, from 2,756 to 4,739, with marginal change in adjusted R2 (0.13 raw, 0.14 imputed)., Conclusions: Missing data across certain sets of common speech perception tests may be safely imputed, enabling multivariate analysis of one of the largest CI outcomes data sets to date., Competing Interests: Conflicts of Interest: C.P. and D.S.L. do not have conflicts of interest. J.A.H. is a consultant for Cochlear Ltd. C.A.B. is a consultant for Advanced Bionics, Cochlear Ltd., Envoy, and IotaMotion, and has equity interest in Advanced Cochlear Diagnostics, LLC. M.A.S. is a consultant for Cochlear Ltd., (Copyright © 2023, Otology & Neurotology, Inc.)

Objective: This study aimed 1) to measure the effect of cochlear implantation on health-related quality of life (HR-QOL) using the Cochlear Implant Quality of Life (CIQOL) questionnaire and 2) to determine audiologic, demographic, and non-CI/hearing-related QOL factors influencing the CIQOL., Study Design: Prospective observational study., Setting: Tertiary referral center., Patients and Interventions: Thirty-seven adult patients with sensorineural hearing loss undergoing cochlear implantation., Main Outcome Measures: CIQOL-global score preimplantation and 6 months postimplantation. Physical function score as measured by the short-form survey, audiologic, and demographic variables., Results: CIQOL showed significant improvement from preimplantation to 6 months postactivation with a mean difference of 14.9 points (95% confidence interval, 11.3 to 18.5, p < 0.0001). Improvement in CIQOL (ΔCIQOL) correlated linearly with age ( r = -0.49, p = 0.001) and improvement in speech perception testing ( r = 0.63, p < 0.0001). Multivariate modeling using age and change in consonant-vowel nucleus-consonant (CNC) score explained 46% of the variability measured by the ΔCIQOL-global score., Conclusions: Nearly all CI recipients achieve significant gains for all domains as measured by the CIQOL. However, younger patients and those with a greater improvement in speech perception performance (CNC) are more likely to achieve a greater CIQOL benefit. Results here suggest the importance of considering preoperative CIQOL and speech perception measures when evaluating predictors of HR-QOL., Competing Interests: Conflicts of Interest and Source of Funding: AW is supported by NIH/NIDCD institutional training grant no. T32DC000022; JB is supported by NIH/NIDCD institutional training grant no. T32DC000022; ND, SR, SC, MAS, and ND disclose no conflicts of interest; JAH is a consultant for Cochlear Ltd.; CAB is a consultant for Advanced Bionics, Cochlear Ltd., Envoy, and IotaMotion, and has equity interest in Advanced Cochlear Diagnostics, LLC; CCW is a consultant for Stryker and Cochlear Ltd., (Copyright © 2023, Otology & Neurotology, Inc.)

Objective: Assess the real-world performance of popular imputation algorithms on cochlear implant (CI) candidate audiometric data., Methods: 7,451 audiograms from patients undergoing CI candidacy evaluation were pooled from 32 institutions with complete case analysis yielding 1,304 audiograms. Imputation model performance was assessed with nested cross-validation on randomly generated sparse datasets with various amounts of missing data, distributions of sparsity, and dataset sizes. A threshold for safe imputation was defined as root mean square error (RMSE) <10dB. Models included univariate imputation, interpolation, multiple imputation by chained equations (MICE), k-nearest neighbors, gradient boosted trees, and neural networks., Results: Greater quantities of missing data were associated with worse performance. Sparsity in audiometric data is not uniformly distributed, as inter-octave frequencies are less commonly tested. With 3-8 missing features per instance, a real-world sparsity distribution was associated with significantly better performance compared to other sparsity distributions (Δ RMSE 0.3 dB- 5.8 dB, non-overlapping 99% confidence intervals). With a real-world sparsity distribution, models were able to safely impute up to 6 missing datapoints in an 11-frequency audiogram. MICE consistently outperformed other models across all metrics and sparsity distributions (p < 0.01, Wilcoxon rank sum test). With sparsity capped at 6 missing features per audiogram but otherwise equivalent to the raw dataset, MICE imputed with RMSE of 7.83 dB [95% CI 7.81-7.86]. Imputing up to 6 missing features captures 99.3% of the audiograms in our dataset, allowing for a 5.7-fold increase in dataset size (1,304 to 7,399 audiograms) as compared with complete case analysis., Conclusion: Precision medicine will inevitably play an integral role in the future of hearing healthcare. These methods are data dependent, and rigorously validated imputation models are a key tool for maximizing datasets. Using the largest CI audiogram dataset to-date, we demonstrate that in a real-world scenario MICE can safely impute missing data for the vast majority (>99%) of audiograms with RMSE well below a clinically significant threshold of 10dB. Evaluation across a range of dataset sizes and sparsity distributions suggests a high degree of generalizability to future applications., Competing Interests: AH – Consultant for Cochlear Ltd., CAB – consultant for Advanced Bionics, Cochlear Ltd., Envoy, and IotaMotion, and has equity interest in Advanced Cochlear Diagnostics, LLC.; MAS – consultant for Cochlear Ltd. There are no patents, products in development or marketed products to declare. This does not alter our adherence to PLOS ONE policies on sharing data and materials., (Copyright: © 2023 Pavelchek et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Objective: To assess the clinical application of five recently published cochlear implant (CI) candidacy evaluation (CICE) referral screening tools through external validation., Study Design: Retrospective cohort study., Setting: Tertiary otology/neurotology practice., Patients: Adults who underwent CICE between December 2020 and September 2021., Interventions: CICE referral screening tools versus CI candidacy criteria., Main Outcome Measures: CICE screening tool performance, based on the ability to identify patients who met the CI candidacy criteria, was evaluated. CI candidacy criteria were defined as best-aided AzBio sentences at +10 signal-to-noise ratio and either 60% or less accuracy to reflect traditional criteria used in clinical settings or 40% or less accuracy (only patients 65 years or older) to reflect Medicare-eligible criteria., Results: Screening criteria of proposed CICE referral tools vary widely across pure-tone average and word recognition scores. When screened by traditional criteria, the sensitivities and specificities of these referral tools varied from 40 to 77% and from 22 to 86%, respectively. When screened by Medicare-eligible criteria, sensitivities and specificities varied from 41 to 81% and from 24 to 91%, respectively. The screening tool proposed by Zwolan et al. ( Otol Neurotol 2020;41(7):895-900) demonstrated the best overall performance for traditional (Youden's J , 0.37; sensitivity, 62%; specificity, 75%) and Medicare-eligible patients (Youden's J , 0.44; sensitivity, 66%; specificity, 78%). All screening tools performed worse on the validation cohort compared with their respective development cohorts., Conclusions: Current tools for determining CICE referral have diverse screening criteria. These combinations of pure-tone average and word recognition score are modestly successful at identifying CI candidates., Competing Interests: J.A.H. is a consultant for Cochlear Ltd. J.B.F. has served on the Audiology Advisory Board for Cochlear Ltd and Advanced Bionics. C.C.W. is a consultant for Stryker and Cochlear Ltd. C.A.B. is a consultant for Advanced Bionics, Cochlear Ltd., Envoy, and IotaMotion, and has equity interest in Advanced Cochlear Diagnostics, LLC. D.S.L., A.W., K.Y.Z., N.D., and M.A.S. disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Objective: 1) To determine the relationship of electrocochleography (ECochG) responses measured on the promontory with responses measured at the round window (RW) and various intracochlear sites. 2) To evaluate if promontory ECochG responses correlate with postoperative speech-perception performance using the cochlear implant (CI)., Study Design: Prospective cohort study., Setting: Tertiary referral center., Patients and Interventions: Ninety-six adult CI recipients with no cochlear malformations or previous otologic surgery., Main Outcome Measures: Acoustically evoked ECochG responses were measured intraoperatively at both extracochlear and intracochlear locations. ECochG total response (ECochG-TR), a measure of residual cochlear function, was calculated by summing the fast Fourier transformation amplitudes in response to 250-Hz to 2-kHz acoustic stimuli. Speech-perception performance was measured at 3 months., Results: There were strong linear correlations for promontory ECochG-TR with the ECochG-TRs measured at the RW ( r = 0.95), just inside scala tympani ( r = 0.91), and after full insertion ( r = 0.83). For an individual subject, the morphology of the ECochG response was similar in character across all positions; however, the response amplitude increased from promontory to RW (~1.6-fold) to just inside scala tympani (~2.6-fold), with the largest response at full insertion (~13.1-fold). Promontory ECochG-TR independently explained 51.8% of the variability ( r2 ) in consonant-nucleus-consonant at 3 months., Conclusions: Promontory ECochG recordings are strongly correlated with responses previously recorded at extracochlear and intracochlear sites and explain a substantial portion of the variability in CI performance. These findings are a critical step in supporting translation of transtympanic ECochG into the clinic preoperatively to help predict postoperative CI performance., Competing Interests: A.W. was supported by National Institutes of Health/National Institute on Deafness and Other Communication Disorders institutional training grant T32DC000022. C.C.W. is a consultant for Stryker and Cochlear Ltd. D.C.F. has a research contract with Advanced Bionics. J.A.H. is a consultant for Cochlear Ltd. C.A.B. is a consultant for Advanced Bionics, Cochlear Ltd., Envoy, and IotaMotion, and has equity interest in Advanced Cochlear Diagnostics, LLC. M.A.S., D.S.L., S.M.L., N.D., and A.J.O. disclose no conflicts of interest. This project was supported by funding from the American Neurotology Society., (Copyright © 2022, Otology & Neurotology, Inc.)

Objectives: To evaluate the utility of intracochlear electrocochleography (ECochG) monitoring during cochlear implant (CI) surgery on postoperative hearing preservation., Study Design: Prospective, randomized clinical trial., Setting: Ten high-volume, tertiary care CI centers., Patients: Adult patients with sensorineural hearing loss meeting the CI criteria who selected an Advanced Bionics CI., Methods: Patients were randomized to CI surgery either with audible ECochG monitoring available to the surgeon during electrode insertion or without ECochG monitoring. Hearing preservation was determined by comparing preoperative unaided low-frequency (125-, 250-, and 500-Hz) pure-tone average (LF-PTA) to postoperative LF-PTA at CI activation. Pre- and post-CI computed tomography was used to determine electrode scalar location and electrode translocation., Results: Eighty-five adult CI candidates were enrolled. The mean (standard deviation [SD]) unaided preoperative LF-PTA across the sample was 54 (17) dB HL. For the whole sample, hearing preservation was "good" (i.e., LF-PTA change 0-15 dB) in 34.5%, "fair" (i.e., LF-PTA change >15-29 dB) in 22.5%, and "poor" (i.e., LF-PTA change ≥30 dB) in 43%. For patients randomized to ECochG "on," mean (SD) LF-PTA change was 27 (20) dB compared with 27 (23) dB for patients randomized to ECochG "off" ( p = 0.89). Seven percent of patients, all of whom were randomized to ECochG off, showed electrode translocation from the scala tympani into the scala vestibuli., Conclusions: Although intracochlear ECochG during CI surgery has important prognostic utility, our data did not show significantly better hearing preservation in patients randomized to ECochG "on" compared with ECochG "off.", (Copyright © 2022, Otology & Neurotology, Inc.)

Objectives: Electrocochleography (ECochG) recordings during cochlear implantation have shown promise in estimating the impact on residual hearing. The purpose of the study was (1) to determine whether a 250-Hz stimulus is superior to 500-Hz in detecting residual hearing decrement and if so; (2) to evaluate whether crossing the 500-Hz tonotopic, characteristic frequency (CF) place partly explains the problems experienced using 500-Hz., Design: Multifrequency ECochG comprising an alternating, interleaved acoustic complex of 250- and 500-Hz stimuli was used to elicit cochlear microphonics (CMs) during insertion. The largest ECochG drops (≥30% reduction in CM) were identified. After insertion, ECochG responses were measured using the individual electrodes along the array for both 250- and 500-Hz stimuli. Univariate regression was used to predict whether 250- or 500-Hz CM drops explained low-frequency pure tone average (LFPTA; 125-, 250-, and 500-Hz) shift at 1-month post-activation. Postoperative CT scans were performed to evaluate cochlear size and angular insertion depth., Results: For perimodiolar insertions ( N = 34), there was a stronger linear correlation between the largest ECochG drop using 250-Hz stimulus and LFPTA shift ( r = 0.58), compared to 500-Hz ( r = 0.31). The 250- and 500-Hz CM insertion tracings showed an amplitude peak at two different locations, with the 500-Hz peak occurring earlier in most cases than the 250-Hz peak, consistent with tonotopicity. When using the entire array for recordings after insertion, a maximum 500-Hz response was observed 2-6 electrodes basal to the most-apical electrode in 20 cases (58.9%). For insertions where the apical insertion angle is >350 degrees and the cochlear diameter is <9.5 mm, the maximum 500-Hz ECochG response may occur at the non-apical most electrode. For lateral wall insertions ( N = 14), the maximum 250- and 500-Hz CM response occurred at the most-apical electrode in all but one case., Conclusion: Using 250-Hz stimulus for ECochG feedback during implantation is more predictive of hearing preservation than 500-Hz. This is due to the electrode passing the 500-Hz CF during insertion which may be misidentified as intracochlear trauma; this is particularly important in subjects with smaller cochlear diameters and deeper insertions. Multifrequency ECochG can be used to differentiate between trauma and advancement of the apical electrode beyond the CF., Competing Interests: CW was a consultant for Stryker and Cochlear Ltd. JH was a consultant for Cochlear Ltd. CB was a consultant for Advanced Bionics, Cochlear Ltd., Envoy, and iotaMotion and has equity interest in Advanced Cochlear Diagnostics, LLC. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Walia, Shew, Lefler, Kallogjeri, Wick, Holden, Durakovic, Ortmann, Herzog and Buchman.)

Objective: Describe early hearing preservation (HP) cochlear implantation (CI) outcomes using a new slim lateral wall electrode (SLWE)., Study Design: Prospective cohort study., Setting: Tertiary referral center., Patients: Adult CI candidates with preoperative low-frequency pure-tone average (LFPTA; 125, 250, 500 Hz) ≤60 dB HL., Intervention: CI with and without intracochlear real-time electrocochleography (RT-ECochG)., Main Outcome Measure: HP (LFPTA ≤80 dB HL), LFPTA shift, speech-perception performance measures, postoperative CT reconstruction., Results: Forty-two subjects were implanted with the SLWE. Thirty patients underwent full insertion without RT-ECochG feedback, and HP was maintained at 3-months postactivation for 7 (23.3%) patients with mean LFPTA shift of 57.5 ± 25.6 dB HL. RT-ECochG feedback was utilized on 12 patients, of whom 6 patients had full insertions and 6 patients had anywhere from 1 to 3 electrodes left outside of the cochlea based on RT-ECochG feedback. At 3 months postoperatively, HP was achieved on 10 (83.3%) patients and mean LFPTA shift was 18.9 c 11.7 dB HL. Mean difference between LFPTA threshold shift at 3-months postactivation with and without RT-ECochG was 38.6 dB HL (95% CI, 25.6-51.67). There was an improvement in delta CNC from preoperative to 3-months postactivation when using RT-ECochG, with mean difference 20.7% (95% CI, 3.3-38.1)., Conclusions: Use of RT-ECochG monitoring during SLWE placement results in fewer full electrode insertions and significantly better HP rates and speech-perception outcomes when compared with unmonitored insertions. Further investigation is needed to evaluate long-term audiologic outcomes to better understand the relationships among ECochG, cochlear trauma, functional outcomes, and HP., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Among the various cochlear implant systems approved by the Food and Drug Administration, current labeling for pediatric usage encompasses (1) bilateral profound bilateral sensorineural hearing loss in children aged 9 to 24 months and bilateral severe to profound sensorineural hearing loss in children older than 2 years; (2) use of appropriately fitted hearing aids for 3 months (this can be waived if there is evidence of ossification); and (3) demonstration of limited progress with auditory, speech, and language development. Pediatric guidelines require children to have significantly worse speech understanding before qualifying for cochlear implantation. The early years of life have been shown to be critical for speech and language development, and auditory deprivation is especially detrimental during this crucial time.Level of evidence: 2.

Although significant progress has been made in understanding outcomes following cochlear implantation, predicting performance remains a challenge. Duration of hearing loss, age at implantation, and electrode positioning within the cochlea together explain ~ 25% of the variability in speech-perception scores in quiet using the cochlear implant (CI). Electrocochleography (ECochG) responses, prior to implantation, account for 47% of the variance in the same speech-perception measures. No study to date has explored CI performance in noise, a more realistic measure of natural listening. This study aimed to (1) validate ECochG total response (ECochG-TR) as a predictor of performance in quiet and (2) evaluate whether ECochG-TR explained variability in noise performance. Thirty-five adult CI recipients were enrolled with outcomes assessed at 3-months post-implantation. The results confirm previous studies showing a strong correlation of ECochG-TR with speech-perception in quiet (r = 0.77). ECochG-TR independently explained 34% of the variability in noise performance. Multivariate modeling using ECochG-TR and Montreal Cognitive Assessment (MoCA) scores explained 60% of the variability in speech-perception in noise. Thus, ECochG-TR, a measure of the cochlear substrate prior to implantation, is necessary but not sufficient for explaining performance in noise. Rather, a cognitive measure is also needed to improve prediction of noise performance., (© 2022. The Author(s).)

Objectives: To compare intraoperative intracochlear electrocochleography (ECochG) with hearing preservation outcomes in cochlear implant (CI) subjects., Design: Intraoperative electrocochleography was performed in adult CI subjects who were recipients of Advanced Bionics' Bionics LLC precurved HiFocus MidScala or straight HiFocus SlimJ electrode arrays. ECochG responses were recorded from the most apical electrode contact during insertion. No changes to the insertions were made due to ECochG monitoring. No information about insertion resistance was collected. ECochG drops were estimated as the change in amplitude from peak (defined as maximum amplitude response) to drop (largest drop) point after the peak during insertion was measured following the peak response. Audiometric thresholds from each subject were obtained before and approximately 1 month after CI surgery. The change in pure tone average for frequencies between 125 Hz and 500 Hz was measured after surgery. No postoperative CT scans were collected as part of this study., Results: A total of 68 subjects from five surgical centers participated in the study. The study sample included 30 MidScala and 38 SlimJ electrodes implanted by approximately 20 surgeons who contributed to the study. Although a wide range of results were observed, there was a moderate positive correlation (Pearson Correlation coefficient, r = 0.56, p < 0.01) between the size of the ECochG drop and the magnitude of pure tone average change. This trend was present for both the MidScala and SlimJ arrays. The SlimJ and MidScala arrays produced significantly different hearing loss after surgery., Conclusion: Large ECochG amplitude drops observed during electrode insertion indicated poorer hearing preservation. Although the outcomes were variable, this information may be helpful to guide surgical decision-making when contemplating full electrode insertion and the likelihood of hearing preservation., Competing Interests: The authors report no conflicts of interest, (Copyright © 2021, Otology & Neurotology, Inc.)

Objective: To evaluate the impact of different open set sentence recognition tests in quiet, +10 dB signal to noise ratio (SNR), and +5 dB SNR in adult cochlear implant (CI) recipients above and below 65 years of age., Study Design and Setting: Multi-institution, prospective, non-randomized, single-subject repeated measures design., Patients: Ninety six adults more than or equal to 18 years old with postlingual bilateral sensorineural hearing loss., Interventions: Participants received a CI532 in one ear. Speech perception measures were evaluated before and 6-months after activation., Main Outcome Measures: Subjects completed consonant-nucleus-constant (CNC) words in quiet and AzBio sentences in noise using +10 and +5 dB SNR, and Montreal Cognitive Assessment (MOCA)., Results: Ninety six adult patients were enrolled (n = 70 older [≥65 yr], n = 26 younger [<65 yr]). There was no difference in CNC scores (CI alone 58.4% versus 67.5%, p = 0.0857; best aided 66.7% versus 76.1%, p = 0.3357). Older adults performed worse on AzBio +10 dB SNR compared with younger patients (CI alone 37.4% versus 56.9%, p = 0.0006; best aided 51.4% versus 68.2%; p = 0.01), and in AzBio +5 dB SNR (CI alone 7.7% versus 11.2%, p = 0.0002; best aided 15.3% versus 22.3%, p = 0.0005). The magnitude of change in AzBio +10 dB SNR was significantly less in older adults in CI alone (15.3% versus 22.3%; p = 0.0493) but not best aided (21.5% versus 31.3%; p = 0.105). The magnitude of change was drastically worse in AzBio +5 dB SNR for older adults (CI alone 6.7% versus 22.1%, p = 0.0014; best aided 9.5% versus 21.5%; p = 0.0142). There was no significant difference in MOCA between the two age groups., Conclusions: While both older and younger patients have similar outcomes with respect to CNC word scores in quiet, the addition of noise disproportionally impacts older patients. Caution should be exercised testing the elderly in noise; testing in noise may disproportionally impact performance expectations and should be more carefully considered when used for candidacy criteria and counseling. Future studies need to further investigate the disproportionate effect of noise on candidacy testing and its impact on how elderly patients are qualified., Competing Interests: Conflicts of Interests: M.A.S.—None; J.A.H.—Consultant Cochlear Ltd.; C.C.W.—Consultant for Stryker; J.L.M.—Consultant for Stryker; ND—None; C.A.B.—consultant for Advanced Bionics, Cochlear Ltd., Envoy, and IotaMotion, and has equity interest in Advanced Cochlear Diagnostics, LLC., (Copyright © 2021, Otology & Neurotology, Inc.)

Objective: Describe audiologic outcomes in hearing preservation (HP) cochlear implant candidates using a slim modiolar electrode (SME)., Study Design: Retrospective., Setting: Tertiary referral center., Patients: Two hundred three adult cochlear implant patients with preoperative low-frequency pure-tone average (LFPTA) ≤ 80 dB HL that received the SME., Intervention: Implantation with a SME electrode., Main Outcome Measures: Primary outcome was postoperative HP, defined as LFPTA ≤80 dB HL. HP status was analyzed at "early" (activation or 3 mo) and "long-term" (6 or 12 mo) time frames using the patient's worst audiogram. Speech perception tests were compared between HP and non-HP cohorts., Results: Of the 203 HP candidates, the tip fold-over rate was 7.4%. The mean shifts in LFPTA at the "early" and "long-term" time points were 25.9 ± 16.2 dB HL and 29.6 ± 16.9 dB HL, respectively. Of 117 patients with preoperative LFPTA ≤60 dB HL, the early and long-term mean LFPTA shifts were 19.5 ± 12.3 dB HL and 32.6 ± 17.2 dB HL, respectively; early and long-term HP rates were 61.1% and 50.8%, respectively. For patients with preoperative LFPTA ≤80 dB HL, early and long-term HP rates were 45.5% and 43.7%, respectively. No significant difference was observed in postoperative speech perception performance (CNC, AzBio, HINT) at 3, 6, or 12 months between HP versus non-HP groups., Conclusions: HP is feasible using the SME. While electroacoustic stimulation was not studied in this cohort, HP provided no clear advantage in speech perception abilities in this group of patients. The current reporting standard of what constitutes HP candidacy (preoperative LFPTA ≤80 dB HL) should be reconsidered., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Importance: Vestibular schwannomas have long been treated as a homogeneous entity. Clinical symptoms at presentation may help elucidate the underlaying pathophysiologic characteristics of tumor subtypes. Describing the heterogeneity of these benign tumors may assist in predicting clinical outcomes associated with their treatment., Objective: To create a tumor staging system that incorporates symptoms at presentation and tumor size to predict severe surgical complications., Design, Setting, and Participants: A retrospective cohort of patients at a single-center tertiary referral center from January 1, 1998, to October 13, 2020, was studied. Patients diagnosed with sporadic vestibular schwannoma surgically treated at Washington University in St Louis, Missouri, were included., Main Outcomes and Measures: Severe surgical complications within 30 days of surgery as determined by the Clavien-Dindo classification system. Patients experiencing a complication of grade 3 or above were determined to have a severe complication., Results: Of 185 patients evaluated, 40 (22%) had severe postoperative complications. Twenty of the 40 patients (50%) were women; mean (SD) age was 46 (13) years. Patients with severe complications were more likely to have large tumors (>2.5 cm in largest diameter), vestibular symptoms, and recent hearing loss at presentation. Using conjunctive consolidation, a 4-stage clinical severity staging system that incorporates clinical symptoms and tumor size at presentation was created to predict severe complications. The clinical severity staging system demonstrated an improvement in the ability to discriminate severe complications (C index, 0.754; 95% CI, 0.67-0.84) from a model of tumor size alone (C index, 0.706; 95% CI 0.62-0.79)., Conclusions and Relevance: This cohort study found that, among patients with vestibular schwannoma, symptoms present at initial evaluation, in addition to tumor size, served as predictors of severe postoperative complications. A new clinical severity staging system incorporating symptoms at presentation can be helpful for clinicians to identify patients at high risk for severe postoperative complications.

Background: Neurofibromatosis type 2 (NF2) often results in profound hearing loss and cochlear implantation is an emerging hearing rehabilitation option. However, cochlear implant (CI) outcomes in this population vary, and intraoperative monitoring to predict cochlear nerve viability and subsequent outcomes is not well-established., Objective: To review the use of intraoperative electrically evoked cochlear nerve monitoring in patients with NF2 simultaneous translabyrinthine (TL) vestibular schwannoma (VS) resection and cochlear implantation., Methods: A retrospective review was performed of 3 patients with NF2 that underwent simultaneous TL VS resection and cochlear implantation with electrical auditory brainstem response (eABR) measured throughout tumor resection. Patient demographics, preoperative assessments, surgical procedures, and outcomes were reviewed., Results: Patients 1 and 3 had a reliable eABR throughout tumor removal. Patient 2 had eABR pretumor removal, but post-tumor removal eABR presence could not be reliably determined because of electrical artifact interference. All patients achieved auditory percepts upon CI activation. Patients 1 and 2 experienced a decline in CI performance after 1 yr and after 3 mo, respectively. Patient 3 continues to perform well at 9 mo. Patients 2 and 3 are daily users of their CI., Conclusion: Cochlear implantation is attainable in cases of NF2-associated VS resection. Intraoperative eABR may facilitate cochlear nerve preservation during tumor removal, though more data and long-term outcomes are needed to refine eABR methodology and predictive value for this population., (© Congress of Neurological Surgeons 2021.)

Objectives/hypothesis: Previous studies have demonstrated that electrocochleography (ECochG) measurements made at the round window prior to cochlear implant (CI) electrode insertion can account for 47% of the variability in 6-month speech perception scores. Recent advances have made it possible to use the apical CI electrode to record intracochlear responses to acoustic stimuli. Study objectives were to determine 1) the relationship between intracochlear ECochG response amplitudes and 6-month speech perception scores and 2) to determine the relationship between behavioral auditory thresholds and ECochG threshold estimates. The hypothesis was that intracochlear ECochG response amplitudes made immediately after electrode insertion would be larger than historical controls (at the extracochlear site) and explain more variability in speech perception scores., Study Design: Prospective case series., Methods: Twenty-two adult CI recipients with varying degrees of low-frequency hearing had intracochlear ECochG measurements made immediately after CI electrode insertion using 110 dB SPL tone bursts. Tone bursts were centered at five octave-spaced frequencies between 125 and 2,000 Hz., Results: There was no association between intracochlear ECochG response amplitudes and speech perception scores. But, the data suggest a mild to moderate relationship between preoperative behavioral audiometric testing and intraoperative ECochG threshold estimates., Conclusion: Performing intracochlear ECochG is highly feasible and results in larger response amplitudes, but performing ECochG before, rather than after, CI insertion may provide a more accurate assessment of a patient's speech perception potential., Level of Evidence: 4 Laryngoscope, 131:E2681-E2688, 2021., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc..)

Studies have shown that hearing preservation is possible in the context of reimplantation, but residual hearing could not be predicted or expected in these cases. We describe a case in which a patient with mild to profound sensorineural hearing loss who underwent cochlear implantation with a lateral wall array and had hearing preserved postoperatively. She developed facial nerve stimulation which was unresponsive to reprogramming. Using electrocochleography to measure intracochlear trauma during the insertion process, the patient underwent reimplantation with a perimodiolar electrode and hearing was preserved postoperatively. This case demonstrates the potential to use electrocochleography for hearing preservation during reimplantation. Laryngoscope, 131:2348-2351, 2021., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: Electrocochleography (ECochG), obtained before the insertion of a cochlear implant (CI) array, provides a measure of residual cochlear function that accounts for a substantial portion of variability in postoperative speech perception outcomes in adults. It is postulated that subsequent surgical factors represent independent sources of variance in outcomes. Prior work has demonstrated a positive correlation between angular insertion depth (AID) of straight arrays and speech perception under the CI-alone condition, with an inverse relationship observed for precurved arrays. The purpose of the present study was to determine the combined effects of ECochG, AID, and array design on speech perception outcomes., Design: Participants were 50 postlingually deafened adult CI recipients who received one of three straight arrays (MED-EL Flex24, MED-EL Flex28, and MED-EL Standard) and two precurved arrays (Cochlear Contour Advance and Advanced Bionics HiFocus Mid-Scala). Residual cochlear function was determined by the intraoperative ECochG total response (TR) measured before array insertion, which is the sum of magnitudes of spectral components in response to tones of different stimulus frequencies across the speech spectrum. The AID was then determined with postoperative imaging. Multiple linear regression was used to predict consonant-nucleus-consonant (CNC) word recognition in the CI-alone condition at 6 months postactivation based on AID, TR, and array design., Results: Forty-one participants received a straight array and nine received a precurved array. The AID of the most apical electrode contact ranged from 341° to 696°. The TR measured by ECochG accounted for 43% of variance in speech perception outcomes (p < 0.001). A regression model predicting CNC word scores with the TR tended to underestimate the performance for precurved arrays and deeply inserted straight arrays, and to overestimate the performance for straight arrays with shallower insertions. When combined in a multivariate linear regression, the TR, AID, and array design accounted for 72% of variability in speech perception outcomes (p < 0.001)., Conclusions: A model of speech perception outcomes that incorporates TR, AID, and array design represents an improvement over a model based on TR alone. The success of this model shows that peripheral factors including cochlear health and electrode placement may play a predominant role in speech perception with CIs., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

A fundamental property of mammalian hearing is the conversion of sound pressure into a frequency-specific place of maximum vibration along the cochlear length, thereby creating a tonotopic map. The tonotopic map makes possible systematic frequency tuning across auditory-nerve fibers, which enables the brain to use pitch to separate sounds from different environmental sources and process the speech and music that connects us to people and the world. Sometimes a tone has a different pitch in the left and right ears, a perceptual anomaly known as diplacusis. Diplacusis has been attributed to a change in the cochlear frequency-place map, but the hypothesized abnormal cochlear map has never been demonstrated. Here we assess cochlear frequency-place maps in guinea-pig ears with experimentally-induced endolymphatic hydrops, a hallmark of Ménière's disease. Our findings are consistent with the hypothesis that diplacusis is due to an altered cochlear map. Map changes can lead to altered pitch, but the size of the pitch change is also affected by neural synchrony. Our data show that the cochlear frequency-place map is not fixed but can be altered by endolymphatic hydrops. Map changes should be considered in assessing hearing pathologies and treatments.

Objectives/hypothesis: Results from a prospective trial demonstrated better speech recognition for cochlear implant (CI) recipients implanted with a long lateral wall electrode array compared to subjects with a short array after 1 year of listening experience. As short array recipients may require an extended adaptation period, this study investigated whether differences in speech recognition continued through 4 years of CI use., Study Design: Long-term follow-up of a prospective randomized trial., Methods: Subjects were randomized to receive a MED-EL medium (24 mm) or standard (31.5 mm) array. Linear mixed models compared speech recognition between cohorts with word recognition in quiet and sentence recognition in noise at 1, 3, 6, 12, 24, and 48 months postactivation. Postoperative imaging and electric frequency filters were reviewed to assess the influence of frequency-to-place mismatch and angular separation between neighboring contacts, a metric associated with peripheral spectral selectivity., Results: Long (31.5 mm) array recipients demonstrated superior speech recognition out to 4 years postactivation. There was a significant effect of angular separation between contacts, with more closely spaced contacts associated with poorer speech recognition. There was no significant effect of mismatch, yet this may have been obscured by changes in frequency filters over time., Conclusions: Conventional MED-EL CI recipients implanted with 31.5-mm arrays experience better speech recognition than 24-mm array recipients, initially and with long-term listening experience. The benefit conferred by longer arrays in the present cohort can be partially attributed to more widely spaced electrode contacts, presumably a result of reduced channel interaction., Level of Evidence: 2 Laryngoscope, 131:892-897, 2021., (© 2020 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To evaluate the prevalence, surgical management, and audiologic impact of pulsatile tinnitus caused by sigmoid sinus dehiscence., Study Design and Setting: Retrospective chart review at a tertiary care hospital., Patients: Adults with unilateral pulsatile tinnitus attributable to sigmoid sinus dehiscence who underwent resurfacing between January 2010 and January 2020., Interventions: Transmastoid sigmoid resurfacing., Main Outcome Measures: Resolution of pulsatile tinnitus; audiologic outcomes; complications; tinnitus etiologies., Results: Nineteen patients (89.4% women) had surgery for suspected sigmoid sinus dehiscence. The mean dehiscence size was 6.1 mm (range, 1-10.7 mm). Eight patients had concurrent sigmoid sinus diverticulum and one patient also had jugular bulb dehiscence. Only two patients (10.5%) had the defect identified by radiology. Low-frequency pure-tone average, measured at frequencies of 250 and 500 Hz, showed a significant median improvement of 8.8 dB following resurfacing (18.8 dB versus 10.0 dB, p = 0.02). The majority of patients had complete resolution of pulsatile tinnitus (16/19, 84.2%). Of those without complete resolution, two patients had partial response and one patient had no improvement. There were no significant complications. Of 41 consecutively tracked patients with a pulsatile tinnitus chief complaint, sigmoid pathology represented 32% of cases., Conclusions: Sigmoid sinus dehiscence represents a common vascular cause of pulsatile tinnitus that, if properly assessed, may be amenable to surgical intervention. Sigmoid sinus resurfacing is safe, does not require decompression, and may improve low-frequency hearing. Radiographic findings of dehiscence are often overlooked without a high index of clinical suspicion. Its relationship with transverse sinus pathology and idiopathic intracranial hypertension remain unclear.

There is a strong association between endolymphatic hydrops and low-frequency hearing loss, but the origin of the hearing loss remains unknown. A reduction in the number of cochlear afferent synapses between inner hair cells and auditory nerve fibres may be the origin of the low-frequency hearing loss, but this hypothesis has not been directly tested in humans or animals. In humans, measurements of hearing loss and postmortem temporal-bone based measurements of endolymphatic hydrops are generally separated by large amounts of time. In animals, there has not been a good objective, physiologic, and minimally invasive measurement of low-frequency hearing. We overcame this obstacle with the combined use of a reliable surgical approach to ablate the endolymphatic sac in guinea pigs and create endolymphatic hydrops, the Auditory Nerve Overlapped Waveform to measure low-frequency hearing loss (≤ 1 kHz), and immunohistofluorescence-based confocal microscopy to count cochlear synapses. Results showed low- and mid-(1-4 kHz) frequency hearing loss at all postoperative days, 1, 4, and 30. There was no statistically significant loss of cochlear synapses, and there was no correlation between synapse loss and hearing function. We conclude that cochlear afferent synaptic loss is not the origin of the low-frequency hearing loss in the early days following endolymphatic sac ablation. Understanding what is, and is not, the origin of a hearing loss can help guide preventative and therapeutic development., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020. Published by Elsevier B.V.)

Importance: Hearing loss, especially moderate to severe forms, has the potential to negatively affect an individual's physical, social, emotional, and cognitive well-being. Moreover, having ineffective binaural hearing increases difficulty understanding speech in noise and leads to a greater degree of social isolation and loneliness and a reduced quality of life (QoL)., Objective: To explore the audiometric and holistic effects of cochlear implantation in a group of adults 65 years or older compared with an optimized bilateral hearing aid condition., Design, Setting, and Participants: This ad hoc secondary analysis of a prospective, single-subject, repeated-measures nonrandomized clinical trial included 13 cochlear implantation centers across the United States. Participants 65 years or older with postlingual bilateral moderate-to-profound sensorineural hearing loss with aided Consonant-Vowel Nucleus-Consonant (CNC) word scores in quiet of 40% or less in the ear to undergo implantation and 50% or less in the contralateral ear were included in the analysis. Baseline QoL testing was performed after 1 month of optimized bilateral hearing aid use. Participants were enrolled from February 20, 2017, to May 3, 2018, and follow-up was completed December 21, 2018. Data were analyzed from March 25, 2019, to March 31, 2020., Interventions: Unilateral implantation with a slim, modiolar cochlear implant device. Hearing aid use in the contralateral ear was required through the 6-month primary end-point interval., Main Outcomes and Measures: The primary objective was to evaluate speech perception before and 6 months after activation of a new cochlear implant. Secondary objectives were QoL metrics in the everyday listening condition before and 6 months after implantation., Results: Seventy participants (51 men [73%]) with a median age of 74 (range, 65-91) years were included in the analysis. No major adverse events occurred. Mixed-model analysis with estimated marginal means and 95% CIs compared preimplantation baseline performance with 6-month postimplantation performance. A clinically important improvement in CNC words was shown in the bimodal condition, with a mean difference of 37.2% (95% CI, 32.0%-42.4%), and in the unilateral (cochlear implant only) condition, with a mean difference of 44.1% (95% CI, 39.0%-49.2%). A clinically important improvement in noise (AzBio sentences signal-to-noise ratio of +10 dB) was also shown, with a mean difference of 21.6% (95% CI, 15.7%-27.5%) in the bimodal condition and 24.5% (95% CI, 18.3%-30.7%) in the unilateral condition. The Health Utilities Index Mark 3 multiple-attribute score improved by 0.186 (95% CI, 0.136-0.234); the Speech, Spatial, and Qualities of Hearing Scale total score improved by 2.58 (95% CI, 2.18-2.99); and a novel Device Use Questionnaire reported 94% of participants were satisfied with overall hearing in the everyday listening condition., Conclusions and Relevance: This subgroup analysis of patients 65 years or older enrolled in a within-subject clinical trial of cochlear implantation demonstrated clinically meaningful audiometric and QoL benefit with an acceptable risk profile. These findings suggest that cochlear implantation in older adults may facilitate the concept of healthy aging., Trial Registration: ClinicalTrials.gov Identifier: NCT03007472.

Importance: Cochlear implants were approved for use in adults in the 1980s, but use remains low owing to a lack of awareness regarding cochlear implantation candidacy criteria and expected outcomes. There have been limited, small series examining the safety and effectiveness of cochlear implantation in adult hearing aid (HA) users with and without mild cognitive impairment (MCI)., Objective: To investigate the safety and effectiveness of a single-ear cochlear implant in a group of optimized adult HA users with and without MCI across a variety of domains., Design, Setting, and Participants: In this nonrandomized controlled trial, a multicenter, prospective, repeated-measures investigation was conducted at 13 US institutions. The setting was academic and community-based cochlear implant programs. Eligible participants were 100 adults (aged >18 years) with postlinguistic onset of bilateral moderate sloping to profound or worse sensorineural hearing loss (≤20 years' duration). Fluent English speakers underwent an optimized bilateral HA trial for at least 30 days. Individuals with aided Consonant-Vowel Nucleus-Consonant (CNC) word score in quiet of 40% or less correct in the ear to be implanted and 50% or less correct in the contralateral ear were offered cochlear implants. The first participant was enrolled on February 20, 2017, and the last participant was enrolled on May 3, 2018. The final follow-up was on December 21, 2018., Interventions: Participants received the same cochlear implant system and contralateral HA., Main Outcomes and Measures: The primary outcome measure was speech understanding in quiet (CNC word score) using both the cochlear implant and opposite ear HA. Secondary outcome measures included the following: adverse events; speech understanding in noise (AzBio signal-to-noise ratio of +10 db [+10 SNR]) Health Utilities Index Mark 3 (HUI3); Speech, Spatial, and Qualities of Hearing Questionnaire 49 (SSQ49); and Montreal Cognitive Assessment (MoCA)., Results: The median age at cochlear implantation of the 96 patients included in the trial was 71 years (range, 23-91 years), and 62 patients (65%) were male. Three serious adverse events requiring revision surgery occurred, and all resolved without sequelae. By 6 months after activation, the absolute marginal mean change in CNC word score and AzBio +10 SNR was 40.5% (95% CI, 35.9%-45.0%) and 24.1% (95% CI, 18.9%-29.4%), respectively. Ninety-one percent (87 of 96) of participants had a clinically important improvement (>15%) in the CNC word score in the implant ear. Mild cognitive impairment (MoCA total score ≤25) was observed in 48 of 81 study participants (59%) at baseline. Speech perception marginal mean improvements were similar between individuals with and without baseline MCI, with values of 40.9% (95% CI, 35.2%-46.6%) and 39.6% (95% CI, 31.8%-47.4%), respectively, for CNC word score and 27.5% (95% CI, 21.0%-33.9%) and 17.8% (95% CI, 9.0%-26.6%), respectively, for AzBio +10 SNR. Statistically significant and clinically important improvements in the HUI3 and SSQ49 were evident at 6 months., Conclusions and Relevance: The findings of this nonrandomized controlled trial seem to indicate that cochlear implants are safe and effective in restoring speech understanding in both quiet and noise and improve quality of life in individuals with and without MCI., Trial Registration: ClinicalTrials.gov Identifier: NCT03007472.

Importance: Current indications for Medicare beneficiaries to receive a cochlear implant are outdated. Multichannel cochlear implant systems may be effective when provided to Medicare beneficiaries using expanded indications., Objective: To examine the effectiveness of cochlear implants, as measured by improvement on the AzBio Sentence Test, for newly implanted Medicare beneficiaries who meet the expanded indications of an AzBio Sentence Test score of 41% to 60% in their best-aided condition., Design, Setting, and Participants: A multicenter nonrandomized trial examined preoperative and postoperative speech recognition, telephone communication, hearing device benefit, health utility, and quality of life for 34 participants enrolled at 8 different centers who received a cochlear implant between September 17, 2014, and July 10, 2018. All participants were 65 years or older, had bilateral moderate to profound hearing loss, and had a best-aided preoperative AzBio Sentence Test score in quiet of 41% to 60%. Analysis was performed on an intention-to-treat basis. Statistical analysis of final results took place from July 29 to October 1, 2019., Intervention: Multichannel cochlear implants., Main Outcomes and Measures: The study examined the a priori hypothesis that the cochlear implant would improve the AzBio Sentence Test score in the best-aided condition by 25% or more and in the implanted ear-alone condition by 30% or more. The study additionally examined word and telephone recognition and examined device benefit, health utility, and quality of life., Results: A total of 34 participants received a cochlear implant; 31 (23 men [74%]; median age, 73.6 years [range, 65.7-85.1 years]) completed testing through the 6-month evaluation, and 29 completed testing through the 12-month evaluation. Median preoperative AzBio Sentence Test scores were 53% (range, 26%-60%) for the best-aided condition and 24% (range, 0%-53%) for the cochlear implant-alone condition; median scores 12 months after implantation improved to 89% (range, 36%-100%) for the best-aided condition and 77% (range, 13%-100%) for the cochlear implant-alone condition. This outcome represents a median change of 36% (range, -22% to 75%) for the best-aided condition (lower bound of 1-sided 95% CI, 31%) and a median change of 53% (range, -15% to 93%) for the cochlear implant-alone condition (lower bound of 1-sided 95% CI, 45%)., Conclusions and Relevance: Intervention with a cochlear implant was associated with improved sentence, word, and telephone recognition in adult Medicare beneficiaries whose preoperative AzBio Sentence Test scores were between 41% and 60%. These findings support expansion of the Center for Medicare & Medicaid current indications for cochlear implants., Trial Registration: ClinicalTrials.gov Identifier: NCT02075229.

Importance: Cochlear implants are a treatment option for individuals with severe, profound, or moderate sloping to profound bilateral sensorineural hearing loss (SNHL) who receive little or no benefit from hearing aids; however, cochlear implantation in adults is still not routine., Objective: To develop consensus statements regarding the use of unilateral cochlear implants in adults with severe, profound, or moderate sloping to profound bilateral SNHL., Design, Setting, and Participants: This study was a modified Delphi consensus process that was informed by a systematic review of the literature and clinical expertise. Searches were conducted in the following databases: (1) MEDLINE In-Process & Other Non-Indexed Citations and Ovid MEDLINE, (2) Embase, and (3) the Cochrane Library. Consensus statements on cochlear implantation were developed using the evidence identified. This consensus process was relevant for the use of unilateral cochlear implantation in adults with severe, profound, or moderate sloping to profound bilateral SNHL. The literature searches were conducted on July 18, 2018, and the 3-step Delphi consensus method took place over the subsequent 9-month period up to March 30, 2019., Main Outcomes and Measures: A Delphi consensus panel of 30 international specialists voted on consensus statements about cochlear implantation, informed by an SR of the literature and clinical expertise. This vote resulted in 20 evidence-based consensus statements that are in line with clinical experience. A modified 3-step Delphi consensus method was used to vote on and refine the consensus statements. This method consisted of 2 rounds of email questionnaires and a face-to-face meeting of panel members at the final round. All consensus statements were reviewed, discussed, and finalized at the face-to-face meeting., Results: In total, 6492 articles were identified in the searches of the electronic databases. After removal of duplicate articles, 74 articles fulfilled all of the inclusion criteria and were used to create the 20 evidence-based consensus statements. These 20 consensus statements on the use of unilateral cochlear implantation in adults with SNHL were relevant to the following 7 key areas of interest: level of awareness of cochlear implantation (1 consensus statement); best practice clinical pathway from diagnosis to surgery (3 consensus statements); best practice guidelines for surgery (2 consensus statements); clinical effectiveness of cochlear implantation (4 consensus statements); factors associated with postimplantation outcomes (4 consensus statements); association between hearing loss and depression, cognition, and dementia (5 consensus statements); and cost implications of cochlear implantation (1 consensus statement)., Conclusions and Relevance: These consensus statements represent the first step toward the development of international guidelines on best practices for cochlear implantation in adults with SNHL. Further research to develop consensus statements for unilateral cochlear implantation in children, bilateral cochlear implantation, combined electric-acoustic stimulation, unilateral cochlear implantation for single-sided deafness, and asymmetrical hearing loss in children and adults may be beneficial for optimizing hearing and quality of life for these patients.

Objective: Hearing loss remains a significant morbidity for patients with vestibular schwannomas (VS). A growing number of reports suggest audibility with cochlear implantation following VS resection; however, there is little consensus on preferred timing and cochlear implant (CI) performance., Data Sources: A systematic literature search of the Ovid Medline, Embase, Scopus, and clinicaltrails.gov databases was performed on 9/7/2018. PRISMA reporting guidelines were followed., Study Selection: Included studies reported CI outcomes in an ear that underwent a VS resection. Untreated VSs, radiated VSs, and CIs in the contralateral ear were excluded., Data Extraction: Primary outcomes were daily CI use and attainment of open-set speech. Baseline tumor and patient characteristics were recorded. Subjects were divided into two groups: simultaneous CI placement with VS resection (Group 1) versus delayed CI placement after VS resection (Group 2)., Data Synthesis: Twenty-nine articles with 93 patients met inclusion criteria. Most studies were poor quality due to their small, retrospective design. Group 1 had 46 patients, of whom 80.4% used their CI on a daily basis and 50.0% achieved open-set speech. Group 2 had 47 patients, of whom 87.2% used their CI on a daily basis and 59.6% achieved open-set speech. Group 2 had more NF2 patients and larger tumors. CI timing did not significantly impact outcomes., Conclusions: Audibility with CI after VS resection is feasible. Timing of CI placement (simultaneous versus delayed) did not significantly affect performance. Overall, 83.9% used their CI on a daily basis and 54.8% achieved open-set speech.

Endolymphatic hydrops is an enlargement of scala media that is most often associated with Meniere's disease, though the pathophysiologic mechanism(s) remain unclear. In order to adequately study the attributes of endolymphatic hydrops, such as the origins of low-frequency hearing loss, a reliable model is needed. The guinea pig is a good model because it hears in the low-frequency regions that are putatively affected by endolymphatic hydrops. Previous research has demonstrated that endolymphatic hydrops can be induced surgically via intradural or extradural approaches that involve drilling on the endolymphatic duct and sac. However, whether it was possible to create an endolymphatic hydrops model using an extradural approach that avoided dangerous drilling on the endolymphatic duct and sac was unknown. The objective of this study was to demonstrate a revised extradural approach to induce experimental endolymphatic hydrops at 30 days post-operatively by obliterating the endolymphatic sac and injuring the endolymphatic duct with a fine pick. The sample size consisted of seven guinea pigs. Functional measurements of hearing were made and temporal bones were subsequently harvested for histologic analysis. The approach had a success rate of 86% in achieving endolymphatic hydrops. The risk of cerebrospinal fluid leak was minimal. No perioperative deaths or injuries to the posterior semicircular canal occurred in the sample. The presented method demonstrates a safe and reliable way to induce endolymphatic hydrops at a relatively quick time point of 30 days. The clinical implications are that the presented method provides a reliable model to further explore the origins of low-frequency hearing loss that can be associated endolymphatic hydrops.

Objective: To explore the immediate and 1-year outcomes of patients who underwent implantation with the slim modiolar electrode (SME)., Study Design: Consecutive case series with chart review., Setting: Tertiary referral academic center., Subject and Methods: Between May 2016 and August 2018, a total of 326 cochlear implantations (CIs) were performed. Intraoperative x-rays were performed in all cases to identify tip rollovers. Scalar location was identified for 76 CIs that had postoperative computed tomography reconstructions. Speech outcomes were measured at 3, 6, and 12 months with consonant-nucleus-consonant word and AzBio sentences in quiet and noise (+10-dB signal-to-noise ratio). Preservation of hearing was defined as maintaining a low-frequency pure tone average ≤80 dB at 250 and 500 Hz., Results: Among 326 CIs, 23 (7%) had tip rollovers. Postoperative reconstructions revealed 5 of 76 (6.6%) scalar translocations. A subset of 177 cases met criteria for evaluation of speech perception scores. The marginal mean differences between presurgery and 12 months for speech tests were as follows: consonant-nucleus-consonant, 43.7 (95% CI, 39.8-47.6); AzBio in quiet, 49.7 (95% CI, 44.9-54.4); and AzBio in noise, 29.9 (95% CI, 25.2-34.7). Sixty-one patients were identified with preservable hearing (low-frequency pure tone average ≤80 dB), and 12 of 61 (20%) preserved hearing at 1 year., Conclusion: CI with SME provides reliable scala tympani insertion in a consistent perimodiolar position. An initially increased tip rollover rate improved with case volume and sheath design improvement. For long-term outcomes, speech performance was comparable to that of other cochlear implants. While hearing preservation for the SME may be better than prior perimodiolar electrodes, consistent outcomes are unlikely.

Endolymphatic hydrops is associated with low-frequency sensorineural hearing loss, with a large body of research dedicated to examining its putative causal role in low-frequency hearing loss. Investigations have been thwarted by the fact that hearing loss is measured in intact ears, but gold standard assessments of endolymphatic hydrops are made postmortem only; and that no objective low-frequency hearing measure has existed. Yet the association of endolymphatic hydrops with low-frequency hearing loss is so strong that it has been established as one of the important defining features for Ménière's disease, rendering it critical to detect endolymphatic hydrops early, regardless of whether it serves a causal role or is the result of other disease mechanisms. We surgically induced endolymphatic hydrops in guinea pigs and employed our recently developed objective neural measure of low-frequency hearing, the Auditory Nerve Overlapped Waveform (ANOW). Hearing loss and endolymphatic hydrops were assessed at various time points after surgery. The ANOW detected low-frequency hearing loss as early as the first day after surgery, well before endolymphatic hydrops was found histologically. The ANOW detected low-frequency hearing loss with perfect sensitivity and specificity in all ears after endolymphatic hydrops developed, where there was a strong linear relationship between degree of endolymphatic hydrops and severity of low-frequency hearing loss. Further, histological data demonstrated that endolymphatic hydrops is seen first in the high-frequency cochlear base, though the ANOW demonstrated that dysfunction begins in the low-frequency apical cochlear half. The results lay the groundwork for future investigations of the causal role of endolymphatic hydrops in low-frequency hearing loss., (Copyright © 2019 IBRO. Published by Elsevier Ltd. All rights reserved.)