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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 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 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.)

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: 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.)

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.)

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.)

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.

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.

Hypothesis: Electrocochleography (ECochG) recorded during cochlear implant (CI) insertion from the apical electrode in conjunction with postinsertion ECochG can identify electrophysiologic differences that exist between groups with and without a translocation of the array from the scala tympani (ST) into the scala vestibuli (SV)., Background: Translocation of the CI electrode from ST into SV can limit performance postoperatively. ECochG markers of trauma may be able to aid in the ability to detect electrode array-induced trauma/scalar translocation intraoperatively., Methods: Twenty-one adult CI patients were included. Subjects were postoperatively parsed into two groups based on analysis of postoperative imaging: 1) ST (n = 14) insertion; 2) SV (n = 7) insertion, indicating translocation of the electrode. The ECochG response elicited from a 500 Hz acoustic stimulus was recorded from the lead electrode during insertion when the distal electrode marker was at the round window, and was compared to the response recorded from a basal electrode (e13) after complete insertion., Results: No statistically significant change in mean ECochG magnitude was found in either group between recording intervals. There was a mean loss of preoperative pure-tone average of 52% for the nontranslocation group and 94% for the translocation group., Conclusions: Intraoperative intracochlear ECochG through the CI array provides a unique opportunity to explore the impact of the CI electrode on the inner ear. Specifically, a translocation of the array from ST to SV does not seem to change the biomechanics of the cochlear region that lies basal to the area of translocation in the acute period.

Objectives: A mixed reality (MR) headset that enables three-dimensional (3D) visualization of interactive holograms anchored to specific points in physical space was developed for use with lateral skull base anatomy. The objectives of this study are to: 1) develop an augmented reality platform using the headset for visualization of temporal bone structures, and 2) measure the accuracy of the platform as an image guidance system., Methods: A combination of semiautomatic and manual segmentation was used to generate 3D reconstructions of soft tissue and bony anatomy of cadaver heads and temporal bones from 2D computed tomography images. A Mixed-Reality platform was developed using C# programming to generate interactive 3D holograms that could be displayed in the HoloLens headset. Accuracy of visual surface registration was determined by target registration error between seven predefined points on a 3D holographic skull and 3D printed model., Results: Interactive 3D holograms of soft tissue, bony anatomy, and internal ear structures of cadaveric models were generated and visualized in the MR headset. Software user interface was developed to allow for user control of the virtual images through gaze, voice, and gesture commands. Visual surface point matching registration was used to align and anchor holograms to physical objects. The average target registration error of our system was 5.76 mm ± 0.54., Conclusion: In this article, we demonstrate that an MR headset can be applied to display interactive 3D anatomic structures of the temporal bone that can be overlaid on physical models. This technology has the potential to be used as an image guidance tool during anatomic dissection and lateral skull base surgery.

Hypothesis: Electrocochleography (ECochG) patterns observed during cochlear implant (CI) electrode insertion may provide information about scalar location of the electrode array., Background: Conventional CI surgery is performed without actively monitoring auditory function and potential damage to intracochlear structures. The central hypothesis of this study was that ECochG obtained directly through the CI may be used to estimate intracochlear electrode position and, ultimately, residual hearing preservation., Methods: Intracochlear ECochG was performed on 32 patients across 3 different implant centers. During electrode insertion, a 50-ms tone burst stimulus (500 Hz) was delivered at 110 dB SPL. The ECochG response was monitored from the apical-most electrode. The amplitude and phase changes of the first harmonic were imported into an algorithm in an attempt to predict the intracochlear electrode location (scala tympani [ST], translocation from ST to scala vestibuli [SV], or interaction with basilar membrane). Anatomic electrode position was verified using postoperative computed tomography (CT) with image processing., Results: CT analysis confirmed 25 electrodes with ST position and 7 electrode arrays translocating from ST into SV. The ECochG algorithm correctly estimated electrode position in 26 (82%) of 32 subjects while 6 (18%) electrodes were wrongly identified as translocated (sensitivity = 100%, specificity = 77%, positive predictive value = 54%, and a negative predictive value = 100%). Greater hearing loss was observed postoperatively in participants with translocated electrode arrays (36 ± 15 dB) when compared with isolated ST insertions (28 ± 20 dB HL). This result, however, was not significant (p = 0.789)., Conclusion: Intracochlear ECochG may provide information about CI electrode location and hearing preservation.

Objective: To demonstrate the safety and effectiveness of the MED-EL Electric-Acoustic Stimulation (EAS) System, for adults with residual low-frequency hearing and severe-to-profound hearing loss in the mid to high frequencies., Study Design: Prospective, repeated measures., Setting: Multicenter, hospital., Patients: Seventy-three subjects implanted with PULSAR or SONATA cochlear implants with FLEX electrode arrays., Intervention: Subjects were fit postoperatively with an audio processor, combining electric stimulation and acoustic amplification., Main Outcome Measures: Unaided thresholds were measured preoperatively and at 3, 6, and 12 months postactivation. Speech perception was assessed at these intervals using City University of New York sentences in noise and consonant-nucleus-consonant words in quiet. Subjective benefit was assessed at these intervals via the Abbreviated Profile of Hearing Aid Benefit and Hearing Device Satisfaction Scale questionnaires., Results: Sixty-seven of 73 subjects (92%) completed outcome measures for all study intervals. Of those 67 subjects, 79% experienced less than a 30 dB HL low-frequency pure-tone average (250-1000 Hz) shift, and 97% were able to use the acoustic unit at 12 months postactivation. In the EAS condition, 94% of subjects performed similarly to or better than their preoperative performance on City University of New York sentences in noise at 12 months postactivation, with 85% demonstrating improvement. Ninety-seven percent of subjects performed similarly or better on consonant-nucleus-consonant words in quiet, with 84% demonstrating improvement., Conclusion: The MED-EL EAS System is a safe and effective treatment option for adults with normal hearing to moderate sensorineural hearing loss in the low frequencies and severe-to-profound sensorineural hearing loss in the high frequencies who do not benefit from traditional amplification.

Objective: To describe outcomes from cochlear implantation with a new, slim modiolar electrode array., Study Design: Retrospective cohort study., Setting: Tertiary referral centers., Patients: Adult cochlear implant candidates., Interventions: Cochlear implantation with CI532 (Cochlear Corp)., Main Outcome Measures: Pre- and postoperative speech perception scores, operative details, and postoperative computed tomography (CT) reconstructions of array location., Results: One hundred seventeen patients are implanted to date. There were eight tip rollovers identified with intraoperative x-ray and resolved with reinsertion. An additional rollover was identified on postoperative CT. CT reconstructions in 17 of 23 patients showed complete scala tympani placement with a wrap factor of 58% (range 53-64%) and a mean insertion angle of 406 degrees (range 360-452 degrees). Three implants demonstrated array translocation with electrodes in the scala vestibuli. Consonant-nucleus-consonant word scores improved from 10% preoperatively to 48% at 3 months postoperatively. Pure-tone thresholds were preserved postoperatively in 37 to 52% of patients across frequencies from 250 to 4000 Hz. Functional pure-tone thresholds (≤80 dB) were recorded in 9 to 25% of patients., Conclusion: CI532 array insertion results in consistent scala tympani location and provides expected audiologic performance. Initial hearing preservation results are not consistent with current electro-acoustic arrays.

Objective: To identify differences in outcomes and map characteristics in pediatric bilateral cochlear implants with modiolar conforming and lateral wall arrays in opposite ears., Study Design: Retrospective case series., Setting: Tertiary care pediatric referral center., Patients: Fourteen children who received a perimodiolar array in one ear and a slim straight array in the opposite ear in sequential surgeries., Interventions: None., Main Outcome Measures: Consonant-nucleus-consonant test (CNC) word recognition score, battery life, power levels, electrical compound action potential (ECAP) thresholds, and electrical threshold and comfort charge levels., Results: Speech perception outcomes were poorer in the lateral wall ears than the perimodiolar ears, and scores in the bilateral condition were better than with the lateral wall device alone. Sequential placement was a factor with differences in preoperative candidacy time correlating with greater difference in speech perception. There was no difference in charge levels between ears, in spite of higher ECAP threshold values for the lateral wall devices., Conclusion: While bilateral speech perception was good, speech perception with the lateral wall device alone was poorer. This cannot be explained solely by the device, as differences in preoperative candidacy time were a significant factor. ECAP thresholds are significantly higher for lateral wall electrodes, but that did not translate in to higher psychophysical measurements.

Hypothesis: The compound action potential (CAP) is a purely neural component of the cochlea's response to sound, and may provide information regarding the existing neural substrate in cochlear implant (CI) subjects that can help account for variance in speech perception outcomes., Background: Measurement of the "total response" (TR), or sum of the magnitudes of spectral components in the ongoing responses to tone bursts across frequencies, has been shown to account for 40 to 50% of variance in speech perception outcomes. The ongoing response is composed of both hair cell and neural components. This correlation may be improved with the addition of the CAP., Methods: Intraoperative round window electrocochleography (ECochG) was performed in adult and pediatric CI subjects (n = 238). Stimuli were tones of different frequencies (250 Hz-4 kHz) at 90 dB nHL. The CAP was assessed in two ways, as an amplitude and with a scaling factor derived from a function fitted to the response. The results were correlated with consonant-nucleus-consonant (CNC) word scores at 6 months post-implantation (n = 51)., Results: Only about half of the subjects had a measurable CAP at any frequency. The CNC word scores correlated weakly with both amplitude (r = 0.20, p < 0.001) and scaling factor (r = 0.25, p < 0.01). In contrast, the TR alone accounted for 43% of the variance, and addition of either CAP measurement in multiple regression did not account for additional variance., Conclusions: The underlying pathology in CI patients causes the CAP to be often absent and highly variable when present. The TR is a better predictor of speech perception outcomes than the CAP.

Background: Access to cochlear implantation varies greatly around the world. It is affected by factors that are specific to each country's health care system, by awareness, and by societal attitudes regarding deafness., Methods: Cochlear implant clinicians and researchers from six countries explored and discussed these variations and their likely causes: Robert Briggs from Australia; Wolfe-Dieter Baumgartner from Austria; Thomas Lenarz from Germany; Eva Koltharp from Sweden; Christopher Raine from the United Kingdom, and Craig Buchman, Donna Sorkin, and Christine Yoshinago from the United States., Results: Utilization rates are quite different for the pediatric and adult demographics in all six countries. Pediatric utilization ranges in the six countries (all in the developed world) ranged from a low of 50% in the United States to a high of 97% in Australia. Adult utilization is less than 10% everywhere in the world., Conclusions: Pediatric access to care was excellent for children with the exception of Germany and the United States where there is an inadequate referral system. Adult utilization was low everywhere because of the lack of screening for adults and the fact that primary care physicians and even audiologists are unfamiliar with CI candidacy criteria and outcomes, and hence typically do not make patient referrals.

Hypothesis: Electrocauterization and subsequent transection of the cochlear nerve induce greater injury to the cochlear nucleus than sharp transection alone., Background: Some studies show that neurofibromatosis Type 2 (NF2) patients fit with auditory brainstem implants (ABIs) fail to achieve speech perception abilities similar to ABI recipients without NF2. Reasons for these differences remain speculative. One hypothesis posits poorer performance to surgically induced trauma to the cochlear nucleus from electrocautery. Sustained electrosurgical depolarization of the cochlear nerve may cause excitotoxic-induced postsynaptic nuclear injury. Equally plausible is that cautery in the vicinity of the cochlear nucleus induces necrosis., Methods: The cochlear nerve was transected in anesthetized adult gerbils sharply with or without bipolar electrocautery at varying intensities. Gerbils were perfused at 1, 3, 5, and 7 days postoperatively; their brainstem and cochleas were embedded in paraffin and sectioned at 10 μm. Alternate sections were stained with flourescent markers for neuronal injury or Nissl substance. In additional experiments, anterograde tracers were applied directly to a sectioned eighth nerve to verify that fluorescent-labeled profiles seen were terminating auditory nerve fibers., Results: Cochlear nerve injury was observed from 72 hours postoperatively and was identical across cases regardless of surgical technique. Postsynaptic cochlear nucleus injury was not seen after distal transection of the nerve. By contrast, proximal transection was associated with trauma to the cochlear nucleus., Conclusion: Distal application of bipolar electrocautery seems safe for the cochlear nucleus. Application near the root entry zone must be used cautiously because this may compromise nuclear viability needed to support ABI stimulation.

Objective: The purpose of the present study was to assess the influence of electrode insertion length on cochlear implant (CI) performance., Study Design: Prospective randomized allocation of CI patients to receive either a standard (26.4 mm)- or medium (20.9 mm)-length electrode array. The processing strategy and electrode insertion number were held constant. The postoperative testing audiologist was blinded to the map details and array., Setting: Tertiary referral center., Patients: Thirteen adult CI candidates randomized to receive the standard- (n = 7) or medium-length (n = 6) electrode array., Intervention(s): Unilateral CI using standard- or medium-length array from the same implant system., Main Outcome Measures(s): Speech perception was assessed with HINT sentences in quiet and steady-state noise (SNR, +10) and CNC words in quiet at defined intervals. Quality of life was assessed using the Hearing Device Satisfaction Survey and the Abbreviated Profile of Hearing Aid Benefit (APHAB). Music perception was assessed using the Musical Sounds In Cochlear implants (MuSIC) test. Postoperative electrode insertion angle was assessed using reconstructed computed tomographic images., Results: Interim analysis necessitated discontinuation of subject enrollment by the institutional review board. There was a trend (p = 0.07) for improved speech perception performance among standard array patients. This difference was significant when the standard array group was increased retrospectively. Quality of life and music perception differences were not apparent between groups., Conclusion: Longer electrode insertions (and greater insertion angles) appear to offer better speech perception performance in the early postactivation period when using the same implant system.

Hypothesis: Intraoperative round window (RW) electrocochleography (ECoG) can help predict speech perception outcomes in adult cochlear implant (CI) recipients., Background: Speech perception outcomes using CIs are highly variable. Recent data demonstrated that intraoperative ECoG could account for nearly half the variance in postoperative word scores. The present study seeks to update this correlation with a larger sample size and determine if addition of clinical variables improves the prediction., Methods: Intraoperative RW ECoG was performed in adult subjects undergoing CI. Amplitudes of the ongoing response to tone bursts of multiple frequencies at 85 to 95 dB HL were summed to obtain the total response (ECoG-TR). ECoG-TR was correlated with postoperative speech perception scores. Multiple linear regression was used to combine clinical factors with the ECoG-TR., Results: The ECoG-TR accounted for 40% of the variance in CNC word scores (n = 32). The preoperative pure tone average (PTA) was the only clinical factor with a significant correlation (r² = 20%). The ability to predict word scores using ECoG-TR and PTA, or after addition of age and duration of hearing loss, was not significantly different from using ECoG-TR alone. For 2 outliers, ECoG-TR predicted a better word score than obtained., Conclusions: The measurement of cochlear physiology before CI, reduced to a single variable, is a better predictor of postoperative speech perception than common clinical factors. Additional analysis of the outliers showed that waveform morphology can provide distinct information in individual cases.

Objective: Assess surgical complications, postoperative residual hearing, and speech perception outcomes of placement of a middle ear implant on the round window in conductive and mixed hearing loss cases., Study Design: Single-subject, repeated-measures design where each subject served as his or her own control., Setting: Tertiary referral medical systems., Subjects: Eighteen subjects with either conductive or mixed hearing loss who could not benefit from conventional amplification were enrolled in a clinical trial investigating vibratory stimulation of the round window., Intervention: The floating mass transducer (FMT) was positioned in the round window niche., Main Outcome Measures: Unaided residual hearing, and aided sound field thresholds and speech perception abilities were evaluated preoperatively, and at 1, 3, 6, and 10 months post-activation of the external speech processor., Results: Six subjects experienced complications that either required further medical management or resolved on their own. There was no difference in residual bone conduction thresholds or unaided word discrimination over time. All subjects experienced a significant improvement in aided speech perception abilities as compared to preoperative performance., Conclusion: Subjects with conductive and mixed hearing loss with placement of the FMT in the round window niche experienced improved sound field thresholds and speech perception, without compromising residual hearing thresholds. Vibratory stimulation of the round window via a middle ear implant may be an appropriate treatment option for patients with conductive and mixed hearing loss. Additional research is needed on the preferred placement of the FMT, improvement of functional gain, and methods to limit postoperative complications and need for revision surgery.

Objective: Electrophysiologic responses to acoustic stimuli are present in nearly all cochlear implant recipients when measured at the round window (RW). Intracochlear recording sites might provide an even larger signal and improve the sensitivity and the potential clinical utility of electrocochleography (ECoG). Thus, the goal of this study is to compare RW to intracochlear recording sites and to determine if such recordings can be used to monitor cochlear function during insertion of a cochlear implant., Methods: Intraoperative ECoG recordings were obtained in subjects receiving a cochlear implant from the RW and from just inside scala tympani (n = 26). Stimuli were tones at high levels (80-100 dB HL). Further recordings were obtained during insertions of a temporary lateral cochlear wall electrode (n = 8). Response magnitudes were determined as the sum of the first and second harmonics amplitudes., Results: All subjects had measurable extracochlear responses at the RW. Twenty cases (78%) showed a larger intracochlear response, compared with three (11%) that had a smaller response and three that were unchanged. On average, signal amplitudes increased with increasing electrode insertion depths, with the largest increase between 15 and 20 mm from the RW., Conclusion: ECoG to acoustic stimuli via an intracochlear electrode is feasible in standard cochlear implant recipients. The increased signal can improve the speed and efficiency of data collection. The growth of response magnitudes with deeper intrascalar electrode positions could be explained by closer proximity or favorable geometry with respect to residual apical signal generators. Reductions in magnitude may represent unfavorable geometry or cochlear trauma.

Objective: To assess the potential influence of 2 different surgical access routes to scala tympani for hearing preservation cochlear implantation on outcomes., Study Design: Retrospective review., Setting: Tertiary care academic center., Patients: Twenty adult subjects participating in a prospective clinical trial on electric-acoustic stimulation. Subjects underwent cochlear implantation with attempted hearing preservation and subsequent ipsilateral electric-acoustic stimulation of the auditory system., Interventions: Eight subjects (40%) were implanted using a round window-related cochleostomy and 12 subjects (60%) via a round window approach., Main Outcome Measures: Postoperative acoustic hearing preservation and speech perception measures were obtained at defined intervals and compared for both groups with and without the use of the external speech processor., Results: The data demonstrate no statistically significant differences in postoperative outcomes for both preservation of residual hearing and unaided and aided speech perception between the cochleostomy and round window groups., Conclusion: The results of this investigation suggest that hearing preservation cochlear implantation can be performed either via a round window-related cochleostomy as well as via the round window membrane itself with similar outcomes in terms of both hearing preservation rates as well as speech perception measures.

Hypothesis: Flexible electrode interaction with intracochlear structures in a noise-damaged region of the cochlea can lead to measureable electrophysiologic changes., Background: An emerging goal in cochlear implantation is preservation of residual hearing subsequently allowing for combined electric and acoustic stimulation (EAS). However, residual hearing is at least partially lost in most patients as a result of electrode insertion. A gerbil model was used to examine changes to acoustically evoked cochlear potentials during simulated cochlear implantation., Methods: Gerbils were partially deafened by noise exposure to mimic residual hearing in human cochlear implant candidates. After 1 month, round window and intracochlear recordings during flexible electrode insertion were made in response to 1 kHz tone burst stimuli at 80 dB SPL. After the insertion, the cochleas were histologically examined for hair cell loss because of the noise exposure and trauma because of the electrode insertion., Results: Anatomic damage from the flexible electrode was not observable in most cases. However, insertions caused response declines that were, on average, greater than the controls, although some losses were similar to the controls. The CM was more sensitive than the CAP for detecting cochlear disturbance., Conclusion: Because response reductions occurred in the absence of anatomic damage, disturbances in the fluid at the base appear to affect responses from the apex. The losses were less than in previous experiments where the basilar membrane was penetrated.

Hypotheses: Electrocochleography (ECoG) to acoustic stimuli can differentiate relative degrees of cochlear responsiveness across the population of cochlear implant recipients. The magnitude of the ongoing portion of the ECoG, which includes both hair cell and neural contributions, will correlate with speech outcomes as measured by results on CNC word score tests., Background: Postoperative speech outcomes with cochlear implants vary from almost no benefit to near normal comprehension. A factor expected to have a high predictive value is the degree of neural survival. However, speech performance with the implant does not correlate with the number and distribution of surviving ganglion cells when measured postmortem. We will investigate whether ECoG can provide an estimate of cochlear function that helps predict postoperative speech outcomes., Methods: An electrode was placed at the round window of the ear about to be implanted during implant surgery. Tone bursts were delivered through an insert earphone. Subjects included children (n = 52, 1-18 yr) and postlingually hearing impaired adults (n = 32). Word scores at 6 months were available from 21 adult subjects., Results: Significant responses to sound were recorded from almost all subjects (80/84 or 95%). The ECoG magnitudes spanned more than 50 dB in both children and adults. The distributions of ECoG magnitudes and frequencies were similar between children and adults. The correlation between the ECoG magnitude and word score accounted for 47% of the variance., Conclusion: ECoGs with high signal-to-noise ratios can be recorded from almost all implant candidates, including both adult and pediatric populations. In postlingual adults, the ECoG magnitude is more predictive of implant outcomes than other nonsurgical variables such as duration of deafness or degree of residual hearing.

Objective: Estimate the available direct cost of cholesteatoma care in a university practice., Study Design: Retrospective review of both physician and hospital financial data during a recent 3-year period., Setting: University-based tertiary referral medical system., Patients: Adults (≥ 18 yr old) with cholesteatoma., Intervention(s): Financial information associated with both physician and hospital encounters were analyzed in a deidentified manner., Main Outcome Measure(s): Frequency and type of encounter, charges, collections, and payers were tabulated., Results: Approximately 949 physician encounters (817 clinic, 130 surgical, and 2 inpatient) among 344 patients resulted in greater than $700, 000 in charges and greater than $211,000 in receipts (≈ 30% rate of collection). The average physician charge per patient per year was approximately $1,600. About 259 hospital encounters among 171 patients resulted in greater than $1.8 million in charges and greater than $520,000 in receipts (≈ 28% collection rate). The average hospital charge per patient per year was ∼$10,000. For physician encounters, managed care (37%) and Medicare (25%) were the most common payers, whereas 17% were uninsured. For hospital encounters, managed care (28%) and Medicare (14%) were the most common payers, whereas 24% were uninsured., Conclusion: The direct cost of care for patients with cholesteatoma is significant. The current treatment paradigm for this chronic disorder results in repeated health care system access and associated direct (and unmeasured indirect) expenses. Future treatment paradigms should be designed to improve disease-specific quality of life while mitigating this financial impact.