Otology Neurotology 2021-03-01

Intracanalicular Vestibular Schwannoma: A Systematic Review and Meta-analysis of Therapeutics Outcomes

Neves Cavada, Marina; Fook-Ho Lee, Michael; Jufas, Nicholas Emmanuel; Harvey, Richard John; Patel, Nirmal P.

Publication date 01-03-2021


Objective: To perform a systematic review and meta-analysis summarizing the current evidence on the management of intracanalicular vestibular schwannoma.
Data Sources: Embase (1947–), Medline (1946–), Cochrane library (1947–), Scopus (2010–), and CINAHL (1961–) were searched from 1969 to October 5, 2019 (50 years).
Study Selection: A search strategy was performed to identify patients with vestibular schwannoma confined to the internal auditory canal without extension to the cerebellopontine angle. Studies with patients aged less than 18, Neurofibromatosis type 2, revision cases, and non-English language were excluded.
Data Extraction: A standardized collection sheet was used for the extracted data and a quality assessment was performed using the Newcastle-Ottawa Scale with the comparability criterion omitted.
Data Synthesis: Seventy-one studies were included with 24 on observation, 14 on radiotherapy, and 34 on surgery. The primary outcome was serviceable hearing preservation. Secondary outcomes were preservation of facial nerve function, growth, involution, and dizziness. Sub-analysis on the type of surgery and type of radiotherapy were performed. Excel 2016 with MIX 2.0 Pro add-on package was used to analyze the data and create forest plots. Data were presented in proportion with a 95% confidence interval.
Conclusions: Serviceable hearing was observed in 31% of patients after observation, 56% after radiotherapy, and 51% after surgical treatment with mean follow-up time of 4.04 years, 4.92 years, and 2.23 years, respectively. Facial nerve function was found to be best preserved in both observation and radiotherapy groups. Vestibular schwannoma growth occurred in 33% of patients under observation. Involution occurred in 2% of patients under observation and in 38% after radiotherapy.

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Clinical Utility of Intraoperative Electrocochleography (ECochG) During Cochlear Implantation: A Systematic Review and Quantitative Analysis

Yin, Linda X.; Barnes, Jason H.; Saoji, Aniket A.; Carlson, Matthew L.

Publication date 01-03-2021


Objective: To evaluate the utility of intraoperative electrocochleography (ECochG) as a predictive tool for preservation of residual acoustic hearing after cochlear implantation.
Data Sources: A systematic review employing a multi-database search strategy (Ovid MEDLINE, Embase, EBM Cochrane, and Scopus) was conducted from inception to August 1, 2019. English language studies in humans were included.
Study Selection: All articles were independently reviewed by two authors according to Preferred Reporting Items of Systematic Reviews and Meta-analysis (PRISMA) guidelines. Studies without intraoperative ECochG obtained during cochlear implantation were excluded.
Data Extraction: Extracted variables included number of patients, ECochG recording technique, success rate of obtaining ECochG potentials, intraoperative changes in ECochG signal, and postoperative hearing preservation outcomes.
Data Synthesis: Among 537 eligible articles, 22 met inclusion criteria encompassing 498 unique patients. Ten studies featured extracochlear measurements, eight featured intracochlear measurements, and four featured both. Extracochlear ECochG had an average (SD) recording success rate of 94.9% (12.7%) while intracochlear ECochG had an average (SD) recording success rate of 91.8% (9.8%). One hundred forty five unique patients from six studies had complete intraoperative ECochG data with postoperative behavioral audiometry. After accounting for study-specific definitions of ECochG signal disturbance, worsening changes in intraoperative ECochG signal predicted postoperative hearing loss with limited sensitivity and specificity and notable heterogeneity across studies.
Conclusions: Intraoperative ECochG recordings can be obtained in over 90% of patients, but accuracy in predicting postoperative hearing loss remains limited. Standardization of intraoperative ECochG monitoring technique and data interpretation are necessary to more robustly investigate outcomes and refine technique.

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The Relationship Between Otitis Media With Effusion and Gastroesophageal Reflux Disease: A Meta-analysis

Wu, Zeng-Hong; Tang, Yun; Niu, Xun; Sun, Hai-Ying; Chen, Xiong

Publication date 01-03-2021


Objective: Recent studies have investigated the mechanism by which refluxed gastric materials reach the middle ear, to establish otitis media with effusion (OME) causal relation between them in both children and adults. Therefore, the relationship between OME and gastro-esophageal reflux disease (GERD) should be further studied extensively.
Methods: To identify eligible original articles, we searched a range of computerized databases, including Medline via Pub Med, EMBASE, CNKI, and Web of Science with a systematic searching strategy. Subgroup analysis was performed to analyze heterogeneity and Egger and Begg funnel plot to assess the publication bias of the included articles.
Results: The meta-analysis had an overall sample size of 1961. We identified a significant relationship between OME and GERD, with a pooled odds ratio (OR) of 4.52 (95% confidence interval CI: 2.42–8.44; p < 0.001). The pooled data were calculated with the random-effects model as a high significant heterogeneity was found among the studies and there was no significant publication bias observed.
Conclusions: The meta-analysis suggested that there was a significant association between otitis media with effusion and gastroesophageal reflux disease.

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Active Middle Ear Implant Patient Outcomes With Adaptive Feedback Canceller

Peixoto, Maria Conceição; Pratas, Rui; Miranda, Cristina; Bento, Mafalda; Oliveira, Susana; Correia da Silva, Victor

Publication date 01-03-2021


Objective: Evaluation of the audiological patient performance with an upgrade of the firmware from the fixed feedback canceller (FFC) to the adaptive feedback canceller (AFC) on an active middle ear implant.
Study Design: Retrospective observational nonrandomized group study.
Setting: Private hospital.
Patients/Intervention(s): From March 2018 to September 2019, 15 patients implanted with an active middle ear implant, with 6 or more months of experience with a FFC system, were upgraded to an AFC algorithm.
Main Outcome Measure(s): Functional gain, speech perception in silence and in noise, and sound localization capacities were examined. Feedback reduction was also analyzed.
Results: Thirteen patients were analyzed. Pure tone audiometric evaluation with FFC (mean value of 48.02 dB) compared with AFC at 1 (mean value of 49.12 dB) and 6 months (mean value of 42.75 dB) revealed no statistically significant differences (p = 0.889 and p = 0.358 respectively).
In speech discrimination in silence, clinically relevant improvements were observed with AFC at 1 and 6 months, with a mean value of 41.5 and 38.3 dB, respectively (p = 0.03 and p = 0.021 correspondingly). In speech discrimination in noisy environments, we observed an improvement of the different conditions tested. No differences were found in localization capacities between FFC and AFC at the two different moments of evaluation.
Conclusions: AFC is more effective than FFC in cancelling feedback and improving sound quality, allowing for better speech understanding in silence and in noise.

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Tinnitus Is Associated With Extended High-frequency Hearing Loss and Hidden High-frequency Damage in Young Patients

Song, Zijun; Wu, Yongzhen; Tang, Dongmei; Lu, Xiaoling; Qiao, Liang; Wang, Jing; Li, Huawei

Publication date 01-03-2021


Objectives: To analyze the results of extended high-frequency (EHF) and high-frequency hearing tests in young patients with tinnitus who show normal response in conventional pure-tone audiometry (PTA), and to explore the correlation between tinnitus and hearing loss (HL).
Study Design: A case–control study.
Setting: A Tertiary Eye Ear Nose & Throat Hospital of China.
Participants: Patients with tinnitus, aged 18 to 35 years old, and with normal conventional PTA (125 Hz–8 k Hz) were enrolled in the tinnitus group. Volunteers without tinnitus of the same age were enrolled in the control group.
Main Outcome Measures: The incidence of EHF-HL and the hearing thresholds at each frequency, as well as the distribution of maximum HL frequency and edge frequency in all participants were compared.
Results: In total, 28 cases (43 ears) were enrolled in the tinnitus group and 34 cases (68 ears) in the control group. The incidence of EHF-HL, average hearing threshold of each frequency ranging from 4 to 16 k Hz, and the maximum hearing threshold were significantly higher in the tinnitus group. The edge frequency in the tinnitus group was lower than that in the control group (10.4 ± 3.1 k Hz versus 12.3 ± 2.5 k Hz, p = 0.010). The dominant tinnitus pitch in cases whose EHF was impaired was positively correlated with the hearing-level loudness of tinnitus (r = 0.627, p < 0.001).
Conclusion: Patients with tinnitus and normal hearing in conventional PTA showed signs of EHF-HL and hidden damage in the high-frequencies more easily. EHF hearing tests and the follow-up of HF hearing tests are recommended to facilitate early detection of hearing impairment for timely intervention.

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Mapping Cochlear Duct Length to Electrically Evoked Compound Action Potentials in Cochlear Implantation

Mlynski, Robert; Lüsebrink, Adele; Oberhoffner, Tobias; Langner, Soenke; Weiss, Nora M.

Publication date 01-03-2021


Objective: Objective measurements may assist in indicating cochlear implants and in predicting outcomes of cochlear implantation surgery. Using electrically evoked compound action potentials (ECAP), information about the function of the auditory nerve can be obtained by analyzing responses to electrical stimulation transmitted and derived by the recording electrode. The aim of this study was to determine whether ECAP characteristics differ depending on the stimulated intracochlear region and the size of the cochlea.
Study Design: Retrospective cohort study.
Setting: University Medical center, tertiary academic referral center.
Patients: Patients undergoing cochlear implant surgery between 2015 and 2018.
Intervention: Cochlear implantation with FLEXsoft electrode arrays (length 31.5 mm, 12 stimulating channels).
Main Outcome Measures: The cochlear duct length (CDL) and the cochlear coverage (CC) were measured using a new computed tomography-based software and correlated to the postoperative speech performance. Additionally, ECAP were measured and associated to the CDL.
Results: A total of 59 ears of 53 cochlear implant users with a mean age of 63.6 (SD 14.9) years were included. The mean estimated CDL was 35.0 (SD 2.2) mm. The mean CC was 90.3% (SD 5.5%). A total of 4,873 ECAP were measured. A statistically significant, moderate, negative correlation between the ECAP slope and the site of stimulation was found (r = −0.29, 95% confidence interval: −0.32 to −0.27, p < 0.0001). No correlation between the CC and the speech performance was found (r = −0.08, 95% confidence interval: −0.33 to 0.18 p = 0.52).
Conclusion: ECAP slopes seem to be a reliable tool to identify the electrodes position inside the cochlea and also showed correlations to the anatomy of the patient. A combination of objective measurements such as anatomical parameters and ECAPs are helpful to assist the postoperative fitting and are promising tools to improve patient care.

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Reliability of Primary Health Care Audiograms by Non-qualified Examiners—An Analysis of 1,224 Cases

Kokkonen, Jukka; Varonen, Sini

Publication date 01-03-2021


Objective: To evaluate the accuracy of primary health care audiograms conducted by non-qualified examiners in a non-standard acoustic environment.
Study Design: Retrospective chart review.
Setting: Referring primary health care institutions and hospital hearing center.
Patients: One thousand two hundred twenty four adult patients evaluated for hearing aid fitting at North Karelia Central hospital in years 2017 and 2018.
Main Outcome Measures: Intraclass correlation coefficient (ICC), mean threshold differences, and mean absolute errors were used to assess agreement between primary health care and hospital audiograms. Primary health care audiometry sensitivity, specificity and positive (PPV) and negative (NPV) predictive values were calculated for hearing aid candidacy in general and open ear mold candidacy in particular.
Results: ICC for both better ear hearing level (BEHL0.5,1,2,4  k Hz) and pure-tone average (PTA0.5,1,2,4  k Hz) in frequencies 0.5, 1, 2, and 4 k Hz was 0.82, and in individual full octave frequencies from 0.125 to 8 k Hz ranged from 0.70 to 0.83. Mean threshold differences in BEHL0.5,1,2,4  k Hz and PTA0.5,1,2,4  k Hz were 1.8 and 1.6 dB and mean absolute errors 4.9 and 5.3 dB, respectively. Sensitivity for hearing aid candidacy was 0.97, specificity 0.58, PPV 0.92, and NPV 0.79.
Conclusions: Primary health care audiometry is reasonably accurate, allowing preselection of patients to adequate hearing care pathways.

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Attitudes of Potential Participants Towards Potential Gene Therapy Trials in Autosomal Dominant Progressive Sensorineural Hearing Loss

Levie, Camille; Moyaert, Julie; Janssens de Varebeke, Sebastien; Verdoodt, Dorien; Vanderveken, Olivier M.; Topsakal, Vedat; Van Wijk, Erwin; de Vrieze, Erik; Pennings, Ronald; Van de Berg, Raymond; Van Camp, Guy; Ponsaerts, Peter; Van Rompaey, Vincent

Publication date 01-03-2021


Background: Advances in gene therapeutic approaches to treat sensorineural hearing loss (SNHL) confront us with future challenges of translating these animal studies into clinical trials. Little is known on patient attitudes towards future innovative therapies.
Objective: We aimed to better understand the willingness of patients with progressive SNHL and vestibular function loss of autosomal dominant (AD) inheritance to participate in potential gene therapy trials to prevent, stabilize, or slow down hearing loss.
Methods: A survey was performed in carriers of the P51S and G88E pathogenic variant in the COCH gene (DFNA9). Various hypothetical scenarios were presented while using a Likert scale.
Results: Fifty three participants were included, incl. 49 symptomatic patients, one presymptomatic patient, and three participants at risk. Their attitude towards potential trials studying innovative therapies was overall affirmative, even if the treatment would only slow down the decline of hearing and vestibular function, rather than cure the disease. Among the different potential scenarios, the less invasive and less frequent treatments increased the likelihood to enroll. Daily oral medication and annual intravenous infusion were awarded the highest scores. The more invasive, more frequent, and more at-risk treatments were still likely to be accepted but decreased the willingness to participate. The presence of a placebo arm was met with the lowest scores of willingness to participate.
Conclusions: Overall, most symptomatic DFNA9 patients would likely consider participation in future innovative inner ear therapy trials, even if it would only slow down the decline of hearing and vestibular function.

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Clinical Feature and Prognosis of Sudden Sensorineural Hearing Loss With Rheumatoid Arthritis

Li, Hui; Zhang, Mingjie; Wang, Mengjun; Zhang, Shuhan; Ma, Shiyin; Wang, Xiaomin

Publication date 01-03-2021


Objectives: Recent publications reported that patients with rheumatoid arthritis (RA) experience an increased risk of suffering sudden sensorineural hearing loss (SSNHL), however, these cases are poorly understood. This study aims to explore the clinical presentations and hearing recovery of SSNHL patients with RA.
Study Design: Retrospective study.
Setting: Tertiary medical center.
Patients: Forty-seven SSNHL patients with RA (RA group) and 431 SSNHL patients without RA (non-RA group) were recruited between April 2015 and June 2019.
Interventions: In the non-RA group, all patients were administrated with oral steroids. In the RA group, 21 patients were treated with oral steroid (OS group) and 26 patients were administrated with intratympanic steroids plus oral steroids (IS+OS group).
Main Outcome Measures: We explored the clinical features and hearing recovery of SSNHL patients with RA in comparison with patients without RA, we also evaluated the therapeutic effect of combined steroids in SSNHL patients with RA.
Results: RA group had higher initial hearing loss level (mean 68.5 dB), final hearing threshold (mean 52.3 dB), the rates of profound hearing loss (48.9%), and no recovery (48.9%) than non-RA group (mean 55.1 dB, mean 34.8 dB, 32.9 and 27.6%, all p < 0.05), however, had lower hearing gains (mean 16.1 dB) and the rate of partial recovery (12.8%) than non-RA group (mean 20.3 dB and 28.8%, all p < 0.05). Furthermore, IS+OS group had higher hearing gains (mean 21.1 dB) and lower rate of no recovery (30.8%) than in the OS group (mean 10.0 dB and 71.4%, all p < 0.05).
Conclusions: SSNHL patients with RA experienced severe hearing loss and had poor hearing prognosis. IS+OS provide better hearing recovery than OS for this population.

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Prognostic Factors for Communication Improvement in Young Children After Cochlear Implant Surgery

Yang, Fang-Jane; Yeh, Li-Li; Wu, Che-Ming

Publication date 01-03-2021


Objective: Our objective was to examine the prognostic factors for post-cochlear implant (CI) communication improvement and determine whether the otologists and speech-language pathologists (SLPs) recommendations exhibited the same predictive performance regarding childrens post-CI verbal expression acquisition.
Study Design: Retrospective, diagnostic study.
Setting: Hospital.
Patients: The study reviewed 339 medical charts of preschool children who underwent CI surgery before the age of 6 years during 1999 to 2016. After these medical charts were assessed, the cohort comprised of 79 preschool children with pre- and post-CI surgery speech-language assessment reports and medical records.
Intervention: A senior SLP and an otologist conducted blind testing of CI candidacy according to the 79 childrens pre-CI reports.
Main Outcome Measures: Spoken mean length of utterance (MLU).
Results: Children with superior skills in comprehending facial and gesture expressions or using gestures or sounds for expression were significantly more likely to receive the SLPs recommendation for CI surgery. The SLPs CI candidacy recommendation, based on background information, communication skill assessments, and magnetic resonance imaging (provided by an otologist), had significant predictive effects. Children recommended by the SLP exhibited a 20 times higher probability of benefiting from CI surgery; such a benefit would be a longer MLU after 2 years.
Conclusion: CI candidacy testing for young children with prelingual hearing impairment based on a SLPs recommendations provided significant predictive effects.
Level of Evidence: 4 (Evidence from well-designed case–control or cohort studies).

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Cochlear Implantation and Electric Acoustic Stimulation in Children With TMPRSS3 Genetic Mutation

Holder, Jourdan T.; Morrel, William; Rivas, Alejandro; Labadie, Robert F.; Gifford, René H.

Publication date 01-03-2021


Background: Mutations in the TMPRSS3 gene, although rare, can cause high frequency hearing loss with residual hearing at low frequencies. Several previous studies have reported cochlear implant (CI) outcomes for adults with TMPRSS3 mutation with mixed results. Although some studies have suggested that TMPRSS3 is expressed in spiral ganglion cells, it remains unclear if previously reported poor CI outcomes in this population were secondary to long durations of deafness or to the effects of the TMPRSS3 mutation. To date, no studies in the literature have reported CI outcomes for children with TMPRSS3 mutation treated with CI.
Objective: The current case series aimed to describe outcomes for three children with sloping hearing loss caused by TMPRSS3 mutation who underwent bilateral CI.
Study Design: Case series.
Setting: Academic medical center.
Patients: Three children (3–4 yr) with TMPRSS3 mutation and normal sloping to profound high frequency hearing loss.
Interventions: CI and electric acoustic stimulation (EAS).
Main Outcome Measures: Outcome measures were residual hearing thresholds, speech recognition scores, and electrode placement determined via intraoperative CT imaging.
Results: All three children maintained residual acoustic hearing and received benefit from EAS. Mean change in low-frequency pure-tone average was 17 dB. Mean postoperative word and sentence recognition scores in the bilateral EAS condition were 80 and 75%, respectively.
Conclusions: Results indicate that CI with EAS is an appropriate treatment for children with TMPRSS3 genetic mutation. Pediatric results from this case series show more favorable CI outcomes than are currently reported for adults with TMPRSS3 mutation suggesting that the intervention may be time sensitive.

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Streamlining the Cochlear Implant Evaluation: Utility of Community Audiometry in Cochlear Implant Candidacy Assessment

Shah, Ravi R.; Jeon, Jun W.; Naples, James G.; Hwa, Tiffany P.; Davis, Sherrie; Eliades, Steven J.; Brant, Jason A.; Bigelow, Douglas C.; Ruckenstein, Michael J.

Publication date 01-03-2021


Objective: To identify redundancy in the cochlear implant candidacy evaluation and assess its financial impact.
Study design: Retrospective chart review.
Setting: Tertiary care academic cochlear implant center.
Patients: One hundred thirty-five patients referred for cochlear implant candidacy evaluation from 2004 through 2019.
Intervention: Community and academic audiometry were compared in a matched-pair analysis.
Main outcome measures: Pure-tone audiometry and word recognition scores (WRS) were compared using the Wilcoxon signed-rank test. Cost of repeated audiometry was estimated using the Medicare Provider Utilization and Payment data.
Results: The majority of pure-tone thresholds (PTT) and pure-tone averages (PTA) had no statistically significant differences between community and academic centers. Only air PTT at 2000 Hz on the right and air PTA on the right demonstrated differences with α = 0.05 after Bonferroni correction. Despite statistical differences, mean differences in PTT and PTA were all under 3.5 dB. WRS were on average lower at the academic center, by 14.7% on the right (p < 0.001) and 10.6% on the left (p = 0.003). Repeating initial audiometry costs patients up to $60.58 and costs the healthcare system up to $42.94 per patient.
Conclusions: Pure-tone audiometry between community and academic centers did not demonstrate clinically significant differences. Lower academic WRS implies that patients identified as potential cochlear implant candidates based on community WRS are likely suitable to proceed to sentence testing without repeating audiometry, saving patients and the healthcare system time and resources.

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Failure and Revision Surgery After Cochlear Implantation in the Adult Population: A 10-year Single-institution Retrospective and Systematic Review of the Literature

Layfield, Eleanor; Hwa, Tiffany Peng; Naples, James; Maina, Ivy; Brant, Jason A.; Eliades, Steven J.; Bigelow, Douglas C.; Ruckenstein, Michael J.

Publication date 01-03-2021


Objective: To characterize failure rate and etiology after cochlear implantation; to identify predictors and describe outcomes after implant failure.
Study Design: Retrospective chart review and systematic review of the literature using Pub Med and Embase.
Setting: Academic Cochlear Implant Center.
Subject Population: Four hundred ninety-eight devices in 439 distinct adult patients.
Interventions: Unilateral or bilateral cochlear implantation.
Main Outcome Measures: Implant failure rate and etiology.
Results: A total of 32 devices (5.9%) failed in 31 patients encompassing the following failure types in accordance with the European Consensus Statement of Cochlear Implants: 17 device failures (53.1%), 11 failures due to performance decrement/adverse reactions (34.4%), and 4 medical reasons (12.9%). There was no significant difference in age, sex, or manufacturer between patients with and without failures. Twenty-five percent of patients with failure leading to explantation had childhood onset of deafness compared to 12.1% of patients with adult-onset hearing loss (OR = 2.42; p = 0.04). Performance decrement/adverse reaction patients had an older average age at implantation compared to device failure patients (mean 68.5 yr 95% CI: 59.9–77.1 vs mean 47.6 yr, CI: 39.9–55.3, p < 0.01). There was no significant difference in time to failure, sex, or device manufacturer between the different types of failures. Twenty-nine patients who experienced CI failure underwent a revision surgery, while the remaining two opted for explantation without reimplantation. One patient who underwent revision surgery subsequently presented with a second failure and underwent a second revision, which was successful.
In our systematic review, 815 citations were reviewed, and 9 studies were selected for inclusion. Overall failure rate across all studies was 5.5%. Device failure was the leading cause of failure in the majority (6/9) of studies, accounting for 40.8% of all failures. Medical reasons were the second leading cause at 33.6%, followed by performance decrement/adverse reaction (20.9%) and other (4.8%).
Conclusions: Cochlear implant failure is a rare phenomenon. Childhood-onset of hearing loss appears to be associated with an increased risk of overall failure. Older patients are at increased risk for performance decrement/adverse reaction. Revision surgery success rates remain very high and patients with failure of any cause should be offered explantation with concurrent reimplantation.

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Cochlear Implantation in Adults With Single-sided Deafness: Outcomes and Device Use

Deep, Nicholas L.; Spitzer, Emily R.; Shapiro, William H.; Waltzman, Susan B.; Roland, J. Thomas Jr; Friedmann, David R.

Publication date 01-03-2021


Objective: To describe our experience with adults undergoing cochlear implantation (CI) for treatment of single-sided deafness (SSD).
Study Design: Retrospective case review.
Setting: Tertiary referral center.
Patients: Fifty-three adults with SSD.
Intervention(s): Unilateral CI.
Main Outcome Measure(s): Speech perception testing in quiet and noise, tinnitus suppression, and device usage from datalogs.
Results: The mean age at CI was 53.2 years (SD 11.9). The mean duration of deafness was 4.0 years (SD 7.8). The most common etiology was idiopathic sudden SNHL (50%). Word recognition improved from 8.7% (SD 15) preoperatively to 61.8% (SD 20) at a mean follow-up of 3.3 years (SD 1.8) (p < 0.0001). Adaptive speech recognition testing in the “binaural with CI” condition (speech directed toward the front and noise toward the normal hearing ear) revealed a significant improvement by 2.6-dB SNR compared to the preoperative unaided condition (p = 0.0002) and by 3.6-dB SNR compared to when a device to route sound to the contralateral side was used (p < 0.0001). Tinnitus suppression was reported to be complete in 23 patients (43%) and improved in 20 patients (38%) while the device was on. The addition of the CI did not lead to a decrement in hearing performance in any spatial configuration. Device usage averaged 8.7 (SD 3.7) hours/day.
Conclusions: Cochlear implantation in adult SSD patients can suppress tinnitus and achieve speech perception outcomes comparable with CI in conventional candidates. Modest improvements in spatial hearing were also observed and primarily attributable to the head shadow effect. Careful patient selection and counseling regarding potential benefits are important to optimize outcomes.

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Word and Nonword Reading Efficiency in Postlingually Deafened Adult Cochlear Implant Users

Tamati, Terrin N.; Vasil, Kara J.; Kronenberger, William G.; Pisoni, David B.; Moberly, Aaron C.; Ray, Christin

Publication date 01-03-2021


Hypothesis: This study tested the hypotheses that 1) experienced adult cochlear implants (CI) users demonstrate poorer reading efficiency relative to normal-hearing controls, 2) reading efficiency reflects basic, underlying neurocognitive skills, and 3) reading efficiency relates to speech recognition outcomes in CI users.
Background: Weak phonological processing skills have been associated with poor speech recognition outcomes in postlingually deaf adult CI users. Phonological processing can be captured in nonauditory measures of reading efficiency, which may have wide use in patients with hearing loss. This study examined reading efficiency in adults CI users, and its relation to speech recognition outcomes.
Methods: Forty-eight experienced, postlingually deaf adult CI users (ECIs) and 43 older age-matched peers with age-normal hearing (ONHs) completed the Test of Word Reading Efficiency (TOWRE-2), which measures word and nonword reading efficiency. Participants also completed a battery of nonauditory neurocognitive measures and auditory sentence recognition tasks.
Results: ECIs and ONHs did not differ in word (ECIs: M = 78.2, SD = 11.4; ONHs: M = 83.3, SD = 10.2) or nonword reading efficiency (ECIs: M = 42.0, SD = 11.2; ONHs: M = 43.7, SD = 10.3). For ECIs, both scores were related to untimed word reading with moderate to strong effect sizes (r = 0.43–0.69), but demonstrated differing relations with other nonauditory neurocognitive measures with weak to moderate effect sizes (word: r = 0.11–0.44; nonword: r = (−)0.15 to (−)0.42). Word reading efficiency was moderately related to sentence recognition outcomes in ECIs (r = 0.36–0.40).
Conclusion: Findings suggest that postlingually deaf adult CI users demonstrate neither impaired word nor nonword reading efficiency, and these measures reflect different underlying mechanisms involved in language processing. The relation between sentence recognition and word reading efficiency, a measure of lexical access speed, suggests that this measure may be useful for explaining outcome variability in adult CI users.

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Factors That Influence Second-side Cochlear Implant Speech Recognition Outcomes

Dornhoffer, James; Liu, Yuan F.; Zhao, Elise E.; Camposeo, Elizabeth L.; Meyer, Ted A.; McRackan, Theodore R.

Publication date 01-03-2021


Objective: Assess relationships between patient, hearing, and cochlear implant (CI)-related factors and second-side CI speech recognition outcomes in adults who are bilaterally implanted.
Study Design: Retrospective review of a prospectively maintained CI database.
Setting: Tertiary academic center.
Patients: One hundred two adults receiving bilateral sequential or simultaneous CIs.
Outcome Measures: Postimplantation consonant-nucleus-consonant (CNC) word and Az Bio sentence scores at ≥12 months.
Results: Of patient, hearing and CI-specific, factors examined only postimplantation speech recognition scores of the first CI were independently associated with speech recognition performance of the second CI on multivariable regression analysis (CNC: ß = 0.4710.298, 0.644; Az Bio: ß = 0.6020.417, 0.769). First-side postoperative CNC scores explained 24.3% of variation in second CI postoperative CNC scores, while change in first CI Az Bio scores explained 40.3% of variation in second CI Az Bio scores. Based on established 95% confidence intervals, 75.2% (CNC) and 65.9% (Az Bio) of patients score equivalent or better with their second CI compared to first CI performance. Age at implantation, duration of hearing loss, receiving simultaneous versus sequential CIs, and preoperative residual hearing (measured by pure-tone average and aided speech recognition scores) were not associated with 12 month speech recognition scores at 12 months.
Conclusions: The degree of improvement in speech recognition from first CI may predict speech recognition with a second CI. This provides preliminary evidence-based expectations for patients considering a second CI. Counseling should be guarded given the remaining unexplained variability in outcomes. Nonetheless, these data may assist decision making when considering a second CI versus continued use of a hearing aid for an unimplanted ear.
Level of Evidence: III

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Effect of Cochlear Implantation on the Endocochlear Potential and Stria Vascularis

McClellan, Joseph; He, Wenxuan; Raja, Joseline; Stark, Gemaine; Ren, Tianying; Reiss, Lina

Publication date 01-03-2021


Hypothesis: Animals with cochlear implantation-induced hearing loss will have a lower endocochlear potential (EP) and decreased strial vascular density.
Background: The cause of residual hearing loss following cochlear implantation remains poorly understood. Recent work from our lab has shown a correlation between vascular changes in the cochlear lateral wall and postimplantation hearing loss, suggesting a role of the stria vascularis and EP.
Methods: Fourteen young, normal-hearing male albino guinea pigs underwent cochlear implantation using either a cochleostomy (CI-c, n = 9) or an extended round window (CI-eRW, n = 5) approach. Hearing sensitivity was assessed pre- and postoperatively using auditory brainstem response thresholds. Three weeks after implantation, EP measurements were obtained from the first and second turns. Hair cell counts and stria vascularis capillary density measurements were also obtained.
Results: The implanted group experienced significant threshold elevations at 8 to 24 k Hz (mean threshold shift 9.1 ± 1.1 dB), with a more robust threshold shift observed in the CI-eRW group compared to the CI-c group. Implanted animals had a significantly lower first turn EP (81.4 ± 5.1 mV) compared with controls (87.9 ± 6.1 mV). No differences were observed in the second turn (75.8 ± 12.0 mV for implanted animals compared to 76.5 ± 7.0 mV for controls). There were no significant correlations between turn-specific threshold shifts, EP measurements, or strial blood vessel density.
Conclusions: Reliable EP measurements can be obtained in chronically implanted guinea pigs. Hearing loss after implantation is not explained by changes in strial vascular density or reductions in EP.

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Precise Evaluation of the Cochlear Duct Length by Flat-panel Volume Computed Tomography (fpVCT)—Implication of Secondary Reconstructions

Schendzielorz, Philipp; Ilgen, Lukas; Mueller-Graff, Tassilo; Noyalet, Laurent; Völker, Johannes; Taeger, Johannes; Hagen, Rudolf; Neun, Tilmann; Zabler, Simon; Althoff, Daniel; Rak, Kristen

Publication date 01-03-2021


Hypothesis: Flat-panel volume computed tomography (fpVCT) and secondary reconstruction allow for more accurate measurements of two-turn length (2TL), cochlear duct length (CDL), and angular length (AL).
Background: Cochlear geometry is a controversially debated topic. In the meantime, there are many different studies partly reporting highly divergent values. Our aim is to discuss the differences and to propose a radiological possibility to improve cochlear measurements using 3D-curved multiplanar reconstruction and fpVCT.
Methods: Performing different image modalities and settings, we tried to find a clinically usable option that allows for a high degree of accuracy. Therefore, we tested them against reference values of high-definition micro-computed tomography.
Results: Comparison of 99 μm slice thickness secondary reconstruction of fpVCT and reference showed no significant differences for 2TL and CDL (p ≥ 0.05). Accordingly, ICC (intraclass correlation) values were excellent (ICC ≥ 0.75; lower limit of confidence interval CI ≥ 0.75; Cronbachs alpha α ≥ 0.9). Evaluating AL, there was a significant difference (difference: −17.27°; p = 0.002). The lower limit of the CI of the ICC was unacceptable (ICC = 0.944; lower limit of CI = 0.248; α = 0.990). Regarding the Bland-Altman plots, there were no clinically unacceptable errors, but a systematic underestimation of AL.
Conclusion: Secondary reconstruction is a suitable tool for producing reliable data that allow the accurate measurement of 2TL and CDL. The option of generating these reconstructions from raw data limits the need for higher radiation doses. Nevertheless, there is an underestimation of AL using secondary reconstructions.

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Audiometric Outcomes Following Transmastoid and Middle Cranial Fossa Approaches for Repair of Cerebrospinal Fluid Otorrhea

Ren, Yin; Tawfik, Kareem O.; Cueva, Roberto A.

Publication date 01-03-2021


Objective: Describe audiometric outcomes following transmastoid and middle cranial fossa (MCF) approaches for repair of cerebrospinal fluid (CSF) otorhinorrhea.
Study design: Retrospective case series.
Setting: Tertiary skull base referral center.
Patients: Adult patients presenting with CSF otorhinorrhea undergoing operative repair between January 2009 and July 2019.
Intervention: Transmastoid repair, MCF repair, or a combined approach.
Main outcome measures: Primary outcome measures included preoperative and postoperative four-frequency pure-tone average (PTA), air-bone gap (ABG) and word recognition score. Secondary outcomes included success of repair, recurrence of CSF leak, and length of stay.
Results: Twenty-nine patients underwent 32 operations (mean age 52 yr, 75.9% female). Twenty (62.5%) patients underwent transmastoid repair, while 8 (25%) underwent an MCF approach. Patients had significant postoperative improvement in both PTA (34.8 dB preop vs. 24.5 dB postop, p = 0.003) and ABG (20.2 dB preop vs. 8.6 dB postop, p  = 0.0001). CSF leak recurred in 3 patients (9.4%) over 17-month follow-up. Compared to MCF or combined approaches, transmastoid repair was associated with greater improvement in PTA (15.6 vs. 3.0 dB, p = 0.001) and shorter length of stay (0.3 vs. 1.2 days, p = 0.005). On subset analysis, patients with spontaneous CSF leaks, a single skull base defect, or meningoencephaloceles demonstrated significant audiometric improvements.
Conclusions: The transmastoid approach for repair of CSF otorhinorrhea is effective, safe, and can be done on an outpatient basis. Patients with spontaneous CSF leaks, a single skull base defect, and associated encephaloceles may have better audiometric outcomes.

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New Compartmental Reading Method for MRI Enables Accurate Localization of Cholesteatomas With High Sensitivity and Specificity

Meranger, Antoine; Venkatasamy, Aïna; Charpiot, Anne; Tavernier, Laurent; Veillon, Françis

Publication date 01-03-2021


Objectives: Cholesteatoma is an inflammatory disease, frequently observed in childrens and young adults, with a risk of relapse or recurrence. The few studies which analyzed cholesteatoma localization on magnetic resonance imaging (MRI) usually merged CT-MR images or relied on their authors’ anatomical knowledge. We propose a compartmental reading method of the compartments of the middle ear cavity for an accurate localization of cholesteatomas on MR images alone.
Material and Methods: Our method uses easily recognizable anatomical landmarks, seen on both computed tomography (CT) and MRI, to delimit the middle ear compartments (epitympanum, mesotympanum, hypotympanum, retrotympanum, protympanum, antrum-mastoid cavity). We first tested it on 50 patients on non-enhanced temporal bone CT. Then, we evaluated its performances for the localization of cholesteatomas on MRI, compared with surgery on 31 patients (validation cohort).
Results: The selected anatomical landmarks that delimited the middle ear compartments were applicable in 98 to 100% of the cases. In the validation cohort, we were able to accurately localize the cholesteatoma on MRI in 83% of the cases (n = 26) with high sensitivity (95.7%) and specificity (98.6%).
Conclusion: With our compartmental reading method, based on the recognition of well-known anatomical landmarks to differentiate the compartments of the middle ear cavity on MRI, we were able to accurately localize the cholesteatoma with high (>90%) sensitivity and specificity. Such landmarks are widely applicable and only require limited learning time based on key images. Accurate localization of the cholesteatoma is useful for the choice of surgical approach.

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Measurement Properties of the Hearing Environments and Reflection of Quality of Life (HEAR-QL) 28-item Questionnaire in Cholesteatoma

Leonard, Colin G.; Dixon, Peter R.; Cushing, Sharon; Papsin, Blake C.; Gordon, Karen A.; James, Adrian L.

Publication date 01-03-2021


Objective: We sought to assess the reliability and construct validity of the Hearing Environments and Reflection on Quality of Life Adolescent (HEAR-QL 28) quality of life measure (QoL) in cholesteatoma.
Study Design: Observational.
Setting: Tertiary referral center.
Patients: One hundred seventeen patients with a diagnosis or history of cholesteatoma completed HEAR-QL 28. In addition to patients within the age range recommended for HEAR-QL 28 (13–18 yr), patients under 13 years old who were able to complete HEAR-QL 28 without parental assistance were included.
Intervention (s): Completion of HEAR-QL 28 QoL measure.
Main Outcome Measure (s): HEAR-QL 28 score, four tone average pure tone audiogram hearing threshold and categorical classification of hearing loss as mild, moderate, and severe.
Results: HEAR-QL 28 did not demonstrate discriminative ability on the basis of audiometric threshold, but did discriminate between participants hearing normally (four tone average pure tone audiogram <30 dB HL) (HEAR-QL 86/100) after cholesteatoma surgery from those with unilateral hearing loss (HEAR-QL 73/100) (p < 0.001). Those with unilateral loss could in turn be differentiated from those with bilateral loss (HEAR-QL 60/100) (p < 0.006).
Conclusion: HEAR-QL 28 is valid measure of QoL in cholesteatoma with no evidence of redundancy and excellent internal consistency. The importance of considering QoL impact of cholesteatoma is highlighted by 17% of participants reporting the normality or abnormality of their hearing differently from their audiometric threshold. The HEAR-QL 28 provides insight into the ability to cope with their hearing environment in a specific environment.

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Management of Intractable Petrous Bone Cholesteatoma With a Combined Translabyrinthine–Transsphenoidal Approach

Morino, Tsunetaro; Yamamoto, Yutaka; Yamamoto, Kazuhisa; Komori, Manabu; Asaka, Daiya; Kojima, Hiromi

Publication date 01-03-2021


Objective: To evaluate whether a combined translabyrinthine–transsphenoidal approach can be used to achieve adequate surgical resection of an extensive petrous bone cholesteatoma and create a debris drainage route for the residual cholesteatoma that is maintained long-term.
Patient: A 71-year-old man with residual petrous temporal bone cholesteatoma that had spread extensively to the internal carotid artery and posterior cranial fossa.
Intervention: Surgical resection of the cholesteatoma via a translabyrinthine approach and creation of a debris drainage route into the nasopharynx via a transsphenoidal approach.
Main Outcome Measures: Control of unresectable petrous temporal bone cholesteatoma and occurrence of cholesteatoma- or surgery-related complications.
Results: Although complete removal of the cholesteatoma was attempted via a translabyrinthine approach, this was not possible because the epithelium of the cholesteatoma was strongly attached to the internal carotid artery and posterior cranial fossa. A debris drainage route leading to the nasopharynx was created by drilling the clivus on the side of the lesion via a transsphenoidal approach. The patient has had no complications since surgery. The drainage route remains open, and the cholesteatoma has been controlled for 57 months.
Conclusions: A markedly advanced petrous temporal bone cholesteatoma can be managed safety and reliably by combining a translabyrinthine approach with a transsphenoidal approach. Creation of a debris drainage route into the nasopharynx can prevent isolation of the cholesteatoma and mastoid cavity problems after surgery. This one-stage surgery may be a suitable method for keeping cholesteatoma under control in patients with unresectable petrous bone cholesteatoma.

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Projected Cost Simulation in Chronic Ear Surgery: Cost in Chronic Ear Surgery

Tadokoro, Kent; Bacalao, Emily; Wozniak, Amy; Kazemian-Marvi, Elycia; Kircher, Matthew L.

Publication date 01-03-2021


Objective: To examine the projected healthcare costs of intact canal wall (ICW) and canal wall down (CWD) tympanomastoidectomy surgery and associated clinic follow-up visits in the management of chronic ear disease.
Study Design: A review of literature was performed to analyze the cholesteatoma recidivism rates of ICW and CWD tympanomastoidectomy. Projected Medicare cost simulation applied to representative samples of patients undergoing otologic surgery and follow-up clinic appointments projected over 15 years with an annual 25% cholesteatoma recidivism rate.
Setting: Tertiary academic center.
Patients: Simulated patient population status postchronic ear surgery and representative follow-up clinic visits with or without in-office mastoid debridement procedures.
Interventions: Therapeutic and rehabilitative.
Main Outcome Measures: Healthcare cost.
Results: Simulation of cost for chronic ear surgery with follow-up visits produced a higher mean cost for the ICW group compared with the CWD group at year 1 ($9112.06 versus $4954.16). However, CWD patients accumulate cost over time due to routine mastoid debridements. The accumulated costs for the CWD group surpassed the ICW group at 12.54 (CI: 12.02–13.11) years.
Conclusions: Using a projected cost analysis model, intact canal wall tympanomastoidectomy incurs a larger upfront cost than the canal wall down technique. However, long-term mastoid cavity maintenance costs accumulate to surpass the intact canal wall group at 12.54 years.

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Effect of Graft Size on Air-bone Gap Closure in Cartilage Button Tympanoplasty

Buck, Lauren S.; Carter, Ellen B.; Carron, Christopher J.; Carron, Jeffrey D.

Publication date 01-03-2021


Objective: To study the effect of graft size on postoperative air-bone gap in children undergoing butterfly inlay cartilage tympanoplasty using circular punch grafts.
Study Design: Retrospective case review.
Setting: Tertiary, academic childrens hospital.
Patients: Children less than 16 years old undergoing circular butterfly inlay tympanoplasty using 4, 5, or 6 mm round grafts.
Intervention: Butterfly inlay tympanoplasty using circular punch graft harvest technique.
Main Outcome Measures: Postoperative pure-tone average and air-bone gap.
Results: Fifty-two children were included in the analysis: 18 in the 4 mm group, 28 in the 5 mm group, and 6 in the 6 mm group. There was no significant difference in either postoperative pure-tone average or air-bone gap among the three groups. Closure rates for the 4, 5, and 6 mm graft groups were 94, 96, and 67%, respectively, for an overall rate of 92%.
Conclusions: Cartilage button butterfly inlay tympanoplasty with punch graft is an effective method for tympanic membrane repair with similar hearing results among various graft diameters but may have diminished success with perforations requiring grafts larger than 5 mm. Larger case series are necessary to determine if larger defects are best managed with other repair techniques.

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Evaluation of a New Material From an Allogenic Collagen Scaffold as a Suitable Coupling Option for Round Window Vibroplasty

Wolber, Philipp; Stürmer, Konrad; Pazen, David; Cimen, Tim; Klußmann, Jens Peter; Lüers, Jan-Christoffer; Schwarz, David

Publication date 01-03-2021


Introduction: In round window vibroplasty the most efficient coupling technique for contact of the floating mass transducer (FMT) to the round window membrane (RWM) is yet to be determined. Various materials placed between the FMT and the RWM have been proposed to enable better stimulation of the cochlea. Collagenous scaffolds derived from decellularized extracellular cartilage matrices as a commercially available biomaterial are already used for other applications in ear surgery. We aimed to examine the coupling properties of collagen scaffolds compared with commonly used other materials (round window soft coupler, porcine perichondrium, and cartilage) as interponate between the FMT and the RWM in vibroplasty.
Methods: A well-established in vitro temporal bone model was used. Volume velocities were measured with collagen scaffold compared with different interponates (round window soft coupler, porcine perichondrium, and cartilage) at 800, 1000, 1250, 1600, 2000, 2500, 3150, and 4000 Hz levels.
Results: Statistical analysis revealed no superiority of commonly used materials compared with collagen scaffolds at all tested volume velocities (p > 0.05).
Discussion: We could demonstrate that collagenous scaffolds of decellularized extracellular cartilage matrices have similar vibrational properties as conventional coupling materials of the FMT to the RWM in vibroplasty. Therefore, as a commercially available new material they display a suitable coupling option for round window vibroplasty.

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Hearing Outcomes With a Novel Total Ossicular Replacement Prosthesis

Lin, Kenny F.; Bojrab, Dennis I. II; Fritz, Christian G.; Schutt, Christopher A.; Hong, Robert S.; Babu, Seilesh C.

Publication date 01-03-2021


Introduction: A total ossicular replacement prosthesis (TORP) is used to reconstruct the ossicular chain in the absence of the stapes suprastructure. The Wildcat prosthesis is a novel TORP that eliminates the need for a separate footplate shoe prosthesis and aims to improve ease-of-use and stability. This study evaluates hearing outcomes using the Wildcat prosthesis.
Study Design: Case series with chart review.
Setting: Tertiary neurotology referral center.
Methods: Retrospective chart review of 64 patients undergoing ossicular chain reconstruction using the Wildcat TORP. Hearing outcomes after surgery were assessed with air conduction pure-tone average, bone conduction pure-tone average, air-bone gap (ABG), speech recognition threshold , and word recognition score as primary outcome measures. The stability of hearing outcomes was evaluated on subsequent long-term follow-up.
Results: At mean short-term follow-up of 4.4 ± 2.7 months, ABG improved from 31.0 ± 13.0 dB preoperatively to 22.5 ± 10.0 dB (p < 0.001) with 51.6% achieving ABG less than 20 dB. No significant difference in any primary outcome measures was found when analyzing outcomes by initial versus revision surgery, use of cartilage graft, or type of mastoidectomy. The only exception was a smaller reduction in ABG of 4.2 dB for patients with canal wall down mastoidectomy compared with a 13.7 dB ABG closure in patients with canal wall up mastoidectomy (p = 0.039).
Conclusion: Total ossicular chain reconstruction using the Wildcat demonstrates versatility in challenging cases to provide hearing outcomes that are comparable to published data using TORPs.

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Subjective Visual Vertical Evaluation by a Smartphone-based Test—Taking the Phone Out of the Bucket

Wengier, Anat; Ungar, Omer J.; Handzel, Ophir; Cavel, Oren; Oron, Yahav

Publication date 01-03-2021


Objective: To measure the subjective visual vertical (SVV) in patients suffering from peripheral vestibular disorders versus controls, using a smartphone-based test designed to simulate the bucket test, in order to validate it as an available tool for the clinician.
Study design: Prospective cohort study.
Setting: Academic tertiary medical center.
Patients: Forty-five adult patients were recruited to the study, 25 had vestibular disorders, and 20 did not (controls).
Intervention: All patients underwent conventional bucket-SVV (b-SVV) and smartphone-based SVV (s-SVV) testing.
Main outcome measures: Correlation and agreement of b-SVV and s-SVV scores in patients with peripheral vestibular disorders compared to controls.
Results: SVV score in the vestibular disorders group was significantly higher compared to controls in both testing methods (p < 0.001 for b-SVV and p  = 0.01 for s-SVV, effect size d = 1.7 for both testing methods). Intragroup correlation was excellent within the study group. Spearmans rank correlation coefficient between b-SVV and s-SVV was 0.902 (p  = 0.01). Agreement measurements suggested a greater sensitivity for the b-SVV test, showing a mean difference of 1.088 degree (SD ± 1.77); directionality, however, was preserved.
Conclusion: The smartphone-based test is a valid, simple, and efficient in-office screening tool for assisting in the diagnosis of vestibular disorders.

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Migraine Features in Patients With Recurrent Benign Paroxysmal Positional Vertigo

Bruss, David; Abouzari, Mehdi; Sarna, Brooke; Goshtasbi, Khodayar; Lee, Ariel; Birkenbeuel, Jack; Djalilian, Hamid R.

Publication date 01-03-2021


Objectives: To identify migraine features present in a cohort of patients with recurrent benign paroxysmal positional vertigo (BPPV).
Methods: Patients presenting with recurrent BPPV were surveyed. Recurrent BPPV was defined as three episodes or greater in 6 months before presentation, with resolution of symptoms after Epley maneuver. Current or past migraine headache (MH) diagnosis was made according to the International Headache Society guidelines.
Results: Fifty-eight patients with recurrent BPPV with a mean age of 53.8 ± 17.4 years were included. Half (29 patients) fulfilled criteria for MH and half (29 patients) did not meet the criteria for MH (non-MH). No statistically significant difference was found in a majority of migraine-related symptoms between the MH and non-MH cohorts with recurrent BPPV. History of migraine medication usage (p = 0.008), presence of a weekly headache (p = 0.01), and duration of dizziness after positional vertigo (p = 0.01) were the only variables that were different on multivariate analysis between the MH and non-MH cohorts.
Conclusions: Half of recurrent BPPV patients suffer from migraine headaches. The other half presented with migraine-related symptoms, but do not meet criteria for MH. The high comorbidity of MH in our recurrent BPPV cohort as well as the absence of a statistically significant difference in a majority of migraine-related features among patients who did and did not fulfill criteria for MH may suggest that recurrent BPPV has a relationship with migraine. Recurrent BPPV may potentially be a manifestation of migraine in the inner ear, which we term otologic migraine including cochlear, vestibular, or cochleovestibular symptoms.

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"Does Cognitive Dysfunction Correlate With Dizziness Severity in Meniéres Disease Patients"

Dornhoffer, James R.; Liu, Yuan F.; Zhao, Elise E.; Rizk, Habib G.

Publication date 01-03-2021


Objective: Characterize the relationship between cognitive dysfunction and the dizziness severity in Meniéres disease (MD) patients.
Study Design: Retrospective review.
Setting: University-based tertiary medical center.
Patients: Three hundred patients were evaluated for MD from 2015 to 2019. Excluding comorbid or alternative vestibular disorders, 29 patients with definite MD and available pre- and postintervention data were included for analysis.
Interventions: A progressive protocol of salt restriction, diuretics, steroid and/or gentamycin injection, and endolymphatic sac decompression for those refractory to medical therapy.
Main Outcome Measures: Quality of life measured with the Dizziness Handicap Inventory (DHI) and cognitive function measured with the Cognitive Failures Questionnaire (CFQ). Pre- and posttreatment DHI and DHI subscale scores and change in these scores were correlated with pre- and posttreatment CFQ scores and change in CFQ with therapy.
Results: Analysis showed a number of limited associations between improvement in DHI and improvement in CFQ. Total DHI scores failed to correlate scores or variation in scores with change in CFQ (p = 0.091 and p = 0.085, respectively). Improvement in the CFQ false-triggering domain was significantly associated with improvement in the DHI physical subscale (r = 0.491, p = 0.007) and was nonsignificantly associated with improvement in total DHI and DHI emotional subscale scores (r = 0.422, p = 0.016 and r = 0.399, p = 0.032).
Conclusions: The DHI correlates with several pre- and posttreatment measures of cognitive dysfunction (CFQ) in MD patients. However, change in DHI and CFQ with therapy correlate poorly. Overall, the commonly used DHI may fail to adequately assess cognitive dysfunction in MD patients possibly due to factors not directly implicated by measures of vestibular dysfunction, such as central nervous system or cognitive dysfunction; however, the specific physical and emotional subscales may offer helpful insight into cognitive dysfunction change/improvement with treatment.

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The Pathology of the Vestibular System in CANVAS

Ishai, Reuven; Seyyedi, Mohammad; Chancellor, Andrew M.; McLean, Catriona A.; Rodriguez, Michael L.; Halmagyi, Gabor Michael; Nadol, Joseph B. Jr.; Szmulewicz, David J.; Quesnel, Alicia M.

Publication date 01-03-2021


Objective: To describe the site of lesion responsible for the severe, bilateral, symmetrical, selective loss of vestibular function in Cerebellar Ataxia with Neuronopathy and Vestibular Areflexia Syndrome (CANVAS), an adult-onset recessively-inherited ataxia, characterized by progressive imbalance due to a combination of cerebellar, somatosensory, and selective vestibular impairment with normal hearing.
Methods: Histologic examination of five temporal bones and the brainstems from four CANVAS patients and the brainstem only from one more, each diagnosed and followed from diagnosis to death by one of the clinician authors.
Results: All five temporal bones showed severe loss of vestibular ganglion cells (cell counts 3–16% of normal), and atrophy of the vestibular nerves, whereas vestibular receptor hair cells and the vestibular nuclei were preserved. In contrast, auditory receptor hair cells, the auditory ganglia (cell counts 51–100% of normal), and the auditory nerves were relatively preserved. In addition, the cranial sensory ganglia (geniculate and trigeminal), present in two temporal bones, also showed severe degeneration.
Conclusions: In CANVAS there is a severe cranial sensory ganglionopathy neuronopathy (ganglionopathy) involving the vestibular, facial, and trigeminal ganglia but sparing the auditory ganglia. These observations, when coupled with the known spinal dorsal root ganglionopathy in CANVAS, indicate a shared pathogenesis of its somatosensory and cranial nerve manifestations. This is the first published account of both the otopathology and neuropathology of CANVAS, a disease that involves the central as well as the peripheral nervous system.

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Sémont Maneuver for Benign Paroxysmal Positional Vertigo Treatment: Moving in the Correct Plane Matters

Gebhart, Isabel; Götting, Carina; Hool, Sara-Lynn; Morrison, Miranda; Korda, Athanasia; Caversaccio, Marco; Obrist, Dominik; Mantokoudis, Georgios

Publication date 01-03-2021


Hypothesis: We aimed to investigate the effect of the head excursion angle on the success of the Sémont Maneuver (SM).
Background: SM is performed with the head turned by 45 degrees toward the unaffected ear. In clinical routine, it is unlikely that physicians can turn the head to a position of exactly 45 degrees. Moreover, it is unclear how possible deviations from 45 degrees would affect the outcome with SM.
Methods: We used an in vitro model (upscaled by ×5) of a posterior semicircular canal with canalithiasis to study head excursion angles (0–75 degrees) and minimum waiting times in SM. Additionally, we measured actual head excursion angles performed by trained physicians during SM on a healthy subject.
Results: Successful canalith repositioning to the utricle was possible at head excursion angles between 21 and 67 degrees. Waiting time increased from 16 to 30 seconds with increasing deviation from 45 degrees. Angles larger than 67 degrees or smaller than 21 degrees did not lead to successful repositioning even after a waiting period of 5 minutes. Physicians set head excursion angles of 50 degrees ±SD 4.8 degrees while performing the SM.
Conclusion: Angular deviations up to ±20 degrees from the ideal SCC plane (45 degrees) still allows for successful SM. Although the tested physicians tended to underestimate the actual head excursion angle by 5 degrees (and more), the success of SM will not be affected provided that the waiting time is sufficiently long. Further, the results suggest that the Brandt-Daroff maneuver is a form of habituation training rather than a liberatory maneuver.

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Prediction of Vestibular Schwannoma Enlargement After Radiosurgery Using Tumor Shape and MRI Texture Features

George-Jones, Nicholas A.; Wang, Kai; Wang, Jing; Hunter, Jacob B.

Publication date 01-03-2021


Objective: Determine if vestibular schwannoma (VS) shape and MRI texture features predict significant enlargement after stereotactic radiosurgery (SRS).
Study Design: Retrospective case review.
Setting: Tertiary referral center.
Patients: Fifty-three patients were selected who underwent SRS and had a contrast-enhanced T1 sequence planning MRI scan and a follow-up contrast enhanced T1 MRI available for review. Median follow-up of 6.5 months (interquartile range/IQR, 5.9–7.4). Median pretreatment tumor volume was 1,006 mm3 (IQR, 465–1,794).
Intervention(s): Stereotactic radiosurgery.
Main Outcome Measure(s): Texture and shape features from the SRS planning scans were extracted and used to train a linear support vector machine binary classifier to predict post-SRS enlargement >20% of the pretreatment volume. Sensitivity, specificity, area under the receiver-operating-characteristic curve (AUC), and positive likelihood ratio were computed. A stratified analysis based on pretreatment tumor volume greater or less than the median volume was also performed.
Results: The model had a sensitivity of 92%, specificity of 65%, AUC of 0.75, and a positive likelihood ratio of 2.6 (95% CI 1.4–5.0) for predicting post-SRS enlargement of >20%. In the larger tumor subgroup, the model had a sensitivity of 87%, specificity of 73%, AUC of 0.76, and a positive likelihood ratio of 3.2 (95% CI 1.2–8.5). In the smaller tumor subgroup, the model had a sensitivity of 95%, specificity of 50%, AUC of 0.65, and a positive likelihood ratio of 1.9 (95% CI 0.8–4.3).
Conclusions: VS shape and texture features may be useful inputs for machine learning models that predict VS enlargement after SRS.

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Classification of the Chorda Tympani: An Endoscopic Study

Uranaka, Tsukasa; Matsumoto, Yu; Hoshi, Yujiro; Iwasaki, Shinichi; Kakigi, Akinobu; Yamasoba, Tatsuya

Publication date 01-03-2021


Objectives: To determine the ability of preoperative computed tomography (CT) to predict the variable surgical anatomy of the chorda tympani nerve (CTN) based on endoscopic tympanotomy.
Study Design: Retrospective case review.
Setting: Tertiary referral center.
Patients: We identified 192 ears of 162 patients who underwent transcanal endoscopic ear surgery from August 2013 to June 2018. Patients with middle ear malformations, revision surgeries, myringoplasty, and cholesteatoma involving the CTN were excluded.
Intervention(s): An intraoperative endoscopic image depicting the chorda tympani was selected for each patient and classified into one of five types. Preoperative CT images were analyzed to match the pictorial classification.
Main Outcome Measure(s): The visible tympanic segment of the chorda tympani was classified into the following five types: external auditory canal (EAC), detached, attached long, attached short, and ultrashort.
Results: A total of 128 ears from 101 patients ranging in age from 2 to 81 years were enrolled. The EAC, detached, attached long, attached short, and ultrashort types of CTN were found in 7 (5.5%), 6 (4.7%), 84 (65.6%), 18 (14.0%), and 13 (10.2%) patients, respectively. The presence of the EAC type could be predicted by preoperative CT while the other four types could be predicted by binning into two groups, with a sensitivity of 0.61 and specificity of 0.72.
Conclusion: The variable anatomy of the chorda tympani nerve can be classified into five major groups based on endoscopic tympanotomy.

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Cool OtOprotective Ear Lumen (COOL) Therapy for Cisplatin-induced Hearing Loss

Stanford, James K.; Morgan, Drew S.; Bosworth, Nicholas A.; Proctor, Georgio; Chen, Tianwen; Palmer, Trace T.; Thapa, Punam; Walters, Bradley J.; Vetter, Douglas E.; Black, Robert D.; Rogers, Lesco L.; Spankovich, Christopher

Publication date 01-03-2021


Hypothesis: Localized cooling of the external ear has a protective effect on the susceptibility to cisplatin-induced hearing loss.
Background: We previously demonstrated significant protection from cisplatin-induced hearing loss using cool water ear canal irrigation. However, the study was limited to a single bolus injection of cisplatin and an acute time period. Here, we examined the application of localized cooling of the ear canal with repeated doses of cisplatin, over an expanded period of time, and using two methods of cooling.
Methods: Twenty-four guinea pigs (12 male and 12 female) underwent auditory physiological testing (auditory brainstem response and distortion product otoacoustic emissions at 8–32 k Hz) and pre/postadministration of cisplatin. Cisplatin (4 mg/kg i.p.) was administered in 3 weekly single injections for a total of 12 mg/kg. While anesthetized, the left ears of the guinea pigs were exposed to either cool water (22°C; ICS Water Caloric Irrigator), a cool ear bar (15°C, cooled by a Peltier device; TNM, Scion Neuro Stim), or left uncooled as a sham control. The animals were tested 3 days post each dosage and 1 month post the final dose. At the end of the experiment the animals were euthanized for histological evaluation.
Results: We found that hearing loss was significantly reduced, and hair cell survival greatly improved, in animals that received cooling treatments compared to cisplatin-only control animals. No significant difference was observed between the two methods of cooling.
Conclusion: Localized cooling of the ear canal during administration of cisplatin mitigated loss of auditory function and loss of hair cells.

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Effect of Repetitive Administration of a Next-generation Sensory Organization Test in Adults With and Without Vestibular Dysfunction

Grove, Colin R.; Whitney, Susan L.; Hetzel, Scott J.; Heiderscheit, Bryan C.; Pyle, G. Mark

Publication date 01-03-2021


Hypothesis: The overall aim was to assess the effects of repetitive testing on performance on a next-generation Sensory Organization Test (NG-SOT).
Background: The psychometric properties of sensory organization assessment with a next-generation posturography device are not fully characterized. Thus, a better understanding of the reliability of the NG-SOT is required to support its use as an outcome measure.
Methods: Forty adults (20 vestibular-impaired) participated. Test–retest reliability was determined using the interclass correlation coefficient ICC (3,1) for the composite, somatosensory, vision, vestibular, and visual preference scores. Learning effects were assessed by analyzing the change in the composite score over time.
Results: Analyzing the NG-SOT scores across all participants produced the following interclass correlation coefficients 95% confidence interval (CI): composite = 0.95 (0.92, 0.97), somatosensory = 0.20 (−0.06, 0.44), vision = 0.93 (0.88, 0.96), vestibular = 0.91 (0.85, 0.94), and visual preference = 0.19 (−0.07, 0.43). The mean differences (95% CI; p value) for the composite score between administrations were: 2.7 (1.0, 4.4; 0.002) for visits 1 and 2; 1.4 (−0.3, 3.1; 0.099) for visits 2 and 3; 0.7 (−1.0, 2.4; 0.403) for visits 3 and 4; and 0.4 (−1.3, 2.1; 0.653) for visits 4 and 5.
Conclusions: Test–retest reliability was excellent for the composite, vision, and vestibular scores, but it was poor for the somatosensory and visual preference scores. A learning effect for the composite score was identified, but performance was stable after two administrations. Changes in the composite score that are greater than 6.5 points exceed the measurement error for the test.

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Prognostic Factors for the Outcome of Translabyrinthine Surgery for Vestibular Schwannomas

de Boer, Nick P.; Koot, Radboud W.; Jansen, Jeroen C.; Böhringer, Stefan; Crouzen, Jeroen A.; van der Mey, Andel G. L.; Malessy, Martijn J. A.; Hensen, Erik F.

Publication date 01-03-2021


Objective: To identify predictors of tumor recurrence and postoperative facial nerve function after translabyrinthine surgery for unilateral vestibular schwannomas.
Study Design: Retrospective study.
Setting: Tertiary referral center.
Patients: Between 1996 and 2017 a total of 596 patients with unilateral vestibular schwannoma underwent translabyrinthine surgery. Pre- and postoperative clinical status, radiological, and surgical findings were evaluated.
Intervention(s): Translabyrinthine surgery.
Main Outcome Measure(s): Potential predictors for tumor recurrence and facial nerve outcome were analyzed using Cox regression and ordinal logistic regression, respectively.
Results: The extent of tumor removal was total in 32%, near-total in 58%, and subtotal in 10%. In 5.5% (33/596) of patients the tumor recurred. Subtotal tumor resection (p = 0.004, hazard ratios HR = 10.66), a young age (p = 0.008, HR = 0.96), and tumor progression preoperatively (p = 0.042, HR = 2.32) significantly increased the risk of recurrence, whereas tumor size or histologic composition did not. A good postoperative facial nerve function (House–Brackmann grade 1–2) was achieved in 85%. The risk of postoperative facial nerve paresis or paralysis increased with tumor size (p < 0.001, OR = 1.52), but was not associated with the extent of tumor removal, histologic composition, or patient demographics.
Conclusions: Translabyrinthine surgery is an effective treatment for vestibular schwannoma, with a good local control rate and facial nerve outcome. The extent of tumor removal is a clinically relevant predictor for tumor recurrence, as are young patient age and preoperative tumor progression. A large preoperative tumor size is associated with a higher risk of postoperative facial nerve paresis or paralysis.

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Access to the Apical Cochlear Modiolus for Possible Stem Cell-based and Gene Therapy of the Auditory Nerve

Wrobel, Christian; Bevis, Nicholas F.; Meyer, Alexander C.; Beutner, Dirk

Publication date 01-03-2021


Objective: Loss of spiral ganglion neurons (SGN) is permanent and responsible for a substantial number of patients suffering from hearing impairment. It can derive from the degeneration of SGNs due to the death of sensory hair cells as well as from auditory neuropathy. Utilizing stem cells to recover lost SGNs increasingly emerges as a possible therapeutic option, but access to human SGNs is difficult due to their protected location within the bony impacted cochlea. Aim of this study was to establish a reliable and practicable approach to access SGNs in the human temporal bone for possible stem cell and gene therapies.
Methods: In seven human temporal bone specimen a transcanal approach was used to carefully drill a cochleostomy in the lateral second turn followed by insertion of a tungsten needle into the apical modiolus to indicate the spot for intramodiolar injections. Subsequent cone beam computed tomography (CBCT) served as evaluation for positioning of the marker and cochleostomy size.
Results: The apical modiolus could be exposed in all cases by a cochleostomy (1.6 mm2, standard deviation ±0.23 mm2) in the lateral second turn. 3D reconstructions and analysis of CBCT revealed reliable positioning of the marker in the apical modiolus, deviating on average 0.9 mm (standard deviation ±0.49 mm) from the targeted center of the second cochlear turn.
Conclusion: We established a reliable, minimally invasive, transcanal surgical approach to the apical cochlear modiolus in the human temporal bone in foresight to stem cell-based and gene therapy of the auditory nerve.

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"EFFECT OF INTRATYMPANIC DEXAMETHAZONE ON BELLS PALSY: LETTER TO THE EDITOR"

Ansari, Reza; Heidari, Farrokh

Publication date 01-03-2021


No abstract available

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"RESPONSE TO LETTER TO THE EDITOR: “EFFECT OF INTRATYMPANIC DEXAMETHASONE ON BELLS PALSY: LETTER TO THE EDITOR”"

Inagaki, Akira; Minakata, Toshiya; Katsumi, Sachiyo; Murakami, Shingo

Publication date 01-03-2021


No abstract available

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Exoscope-assisted Otologic Surgery During the COVID-19 Pandemic

Tu, Nathan; Bojrab, Dennis II; Sioshansi, Pedrom; Lin, Kenny; Hong, Robert; Bojrab, Dennis; Babu, Seilesh

Publication date 01-03-2021


Objective: The recent COVID-19 pandemic has required careful reconsideration of safe operating room practices. We describe our initial experiences performing otologic surgery with the exoscope during the COVID-19 pandemic.
Method: The exoscope was used for several semiurgent otologic surgeries in combination with complete eye protection, a “tent” drape, a smoke evacuator with ultra-low particulate air filter, and betadine irrigation. These techniques are demonstrated in the accompanying video. This was compared with our experiences using the microscope.
Results: The described modified goggles allowed complete eye protection while providing a fully three-dimensional view of the surgical site. The other safety measures described are simple and efficient techniques which can easily be adopted for otologic surgery using the microscope.
Conclusion: Use of the exoscope for otologic surgery during the COVID-19 pandemic allows full three-dimensional visualization of the surgical field while simultaneously providing complete eye protection. Use of the “tent” drape, ultra-low particulate air filter, and betadine irrigation are also options that otologic surgeons may consider for additional safety.

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American Otological Society: 154th Annual Meeting

Publication date 01-03-2021


No abstract available

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American Neurotology Society: 56th Annual Spring Meeting

Publication date 01-03-2021


No abstract available

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