Post-operative patient perception of decisional regret in cochlear implant recipients.

IMPORTANCE: Decision regret post-surgery has has been linked to health outcomes for a number of elective procedures but is understudied in cochlear implantation satisfaction. Theunpredictability in outcomes may lead to unmet expectations by the recipient. This study is the first study to investigate the decision regret concept in cochlear implant recipients. OBJECTIVE: Tto investigate post-operative decision regret in (CI) recipients. DESIGN: This was a prospective cohort study using the validated Ottawa Decision Regret Scale, and whether the CI met the patient's expectations. Variables potentially associated with decision regret including patient demographics, post-operative speech perception scores, duration of deafness, duration of CI use and age were analyzed using the logistic regression model. SETTING: This was a multi-center study. Participants were recruited and enrolled from the University of Miami and the University of Kansas in an outpatient setting. PARTICIPANTS: Adult, English-speaking CI recipients with at least 6 months of listening experience with their implant. RESULTS: Out of 173 58% reported no regret, 27% reported mild, and 15% reported moderate-to-strong regret. Expectations were met in 77% while not meeting expectations in 14%. The remaining 8% were neutral. Decisional regret was significantly associated (p = 0.02) with poor post-operative speech perception. No other variables were associated with regret. CONCLUSIONS: Post-operative decision regret was reported by 42% of CI recipients. Poor speech perception abilities were associated with increased risk of regret. Further research is required to identify regret risks and provide resources to mitigate regret in CI recipients.

The natural history and genotype-phenotype correlations of TMPRSS3 hearing loss: an international, multi-center, cohort analysis.

TMPRSS3-related hearing loss presents challenges in correlating genotypic variants with clinical phenotypes due to the small sample sizes of previous studies. We conducted a cross-sectional genomics study coupled with retrospective clinical phenotype analysis on 127 individuals. These individuals were from 16 academic medical centers across 6 countries. Key findings revealed 47 unique TMPRSS3 variants with significant differences in hearing thresholds between those with missense variants versus those with loss-of-function genotypes. The hearing loss progression rate for the DFNB8 subtype was 0.3 dB/year. Post-cochlear implantation, an average word recognition score of 76% was observed. Of the 51 individuals with two missense variants, 10 had DFNB10 with profound hearing loss. These 10 all had at least one of 4 TMPRSS3 variants predicted by computational modeling to be damaging to TMPRSS3 structure and function. To our knowledge, this is the largest study of TMPRSS3 genotype-phenotype correlations. We find significant differences in hearing thresholds, hearing loss progression, and age of presentation, by TMPRSS3 genotype and protein domain affected. Most individuals with TMPRSS3 variants perform well on speech recognition tests after cochlear implant, however increased age at implant is associated with worse outcomes. These findings provide insight for genetic counseling and the on-going design of novel therapeutic approaches.

Initial hearing preservation outcomes of cochlear implantation with a slim perimodiolar electrode array.

OBJECTIVE: To assess the slim modiolar array as a hearing preservation electrode. METHODS: Retrospective chart review of adult, post-lingual CI recipients implanted with slim modiolar array Sept 2016 to July 2017 in a tertiary referral center. Baseline audiograms were obtained within six months of initial CI evaluation. Patients with low frequency pure tone average (LFPTA) (125, 250, 500 Hz) <80 dB were considered HP candidates. Postoperative audiograms were obtained within 48 h before activation. Successful HP was considered as (1) retention of LFPTA threshold <80 dB and (2) change in threshold from pre- to post-operative. RESULTS: Sixty-three patients received the slim perimodiolar array and 42 were HP candidates. Post-operative audiograms were obtained for 39 of 42 patients an average of 28.92 days after surgery. 56.4% of HP candidates retainedLFPTA <80 dB. Mean ΔLFPTA was 24.15 dB (±16.14; p < 0.001). 56.4% of HP candidates experienced Δ LFPTA <20 dB; 69.2% <30 dB. Functional hearing preservation was more successful in lower frequencies where starting thresholds were better - 78% with LFPTA <50 dB retained serviceable hearing at activation. The postoperative change was similar in each low frequency (Δ125 Hz: mean 21.25 +/- 14.76 (N = 28); Δ250 Hz: 26.28 +/- 19.29 (N = 39); Δ500 Hz: 25.00 +/- 17.73 (N = 39)). CONCLUSIONS: The slim perimodiolar array is moderately effective at immediate hearing preservation. In subjects with preoperative audiometric profiles similar to those in prior EAS trials, immediate HP is comparable.