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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.

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.

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.

Animals with cochlear implantation-induced hearing loss will have a lower endocochlear potential (EP) and decreased strial vascular density.

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.

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.

The implanted group experienced significant threshold elevations at 8 to 24 kHz (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.

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.

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.

Assess relationships between patient, hearing, and cochlear implant (CI)-related factors and second-side CI speech recognition outcomes in adults who are bilaterally implanted.

Retrospective review of a prospectively maintained CI database.

Tertiary academic center.

One hundred two adults receiving bilateral sequential or simultaneous CIs.

Postimplantation consonant-nucleus-consonant (CNC) word and AzBio sentence scores at ≥12 months.

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 = 0.471[0.298, 0.644]; AzBio ß = 0.602[0.417, 0.769]). First-side postoperative CNC scores explained 24.3% of variation in second CI postoperative CNC scores, while change in first CI AzBio scores explained 40.3% of variation in second CI AzBio scores. Based on established 95% confidence intervals, 75.2% (CNC) and 65.9% (AzBio) 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.

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.

III.

III.

To describe our experience with adults undergoing cochlear implantation (CI) for treatment of single-sided deafness (SSD).

Retrospective case review.

Tertiary referral center.

Fifty-three adults with SSD.

Unilateral CI.

Speech perception testing in quiet and noise, tinnitus suppression, and device usage from datalogs.

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.

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.

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.

To identify redundancy in the cochlear implant candidacy evaluation and assess its financial impact.

Retrospective chart review.

Tertiary care academic cochlear implant center.

One hundred thirty-five patients referred for cochlear implant candidacy evaluation from 2004 through 2019.

Community and academic audiometry were compared in a matched-pair analysis.

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.

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.

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.

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.

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.

The current case series aimed to describe outcomes for three children with sloping hearing loss caused by TMPRSS3 mutation who underwent bilateral CI.

Case series.

Academic medical center.

Three children (3-4 yr) with TMPRSS3 mutation and normal sloping to profound high frequency hearing loss.

CI and electric acoustic stimulation (EAS).

Outcome measures were residual hearing thresholds, speech recognition scores, and electrode placement determined via intraoperative CT imaging.

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.

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.

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.

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.

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.

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.

Fifty three participants were included, incl. see more 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.

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