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Purpose: This study is a systematic review of the literature which seeks to evaluate auditory and quality of life (QOL) outcomes of cochlear implantation in patients with Usher syndrome. Methods: Systematic review of studies indexed in Medline via PubMed, Ovid EMBASE, Web of Science, CENTRAL and clinicaltrials.gov was performed up to March 9th 2022, conducted in accordance with the PRISMA statement. Patient demographics, comorbidity, details of cochlear implantation, auditory, and QOL outcomes were extracted and summarized. Results: 33 studies reported over 217 cochlear implants in 187 patients with Usher syndrome, comprising subtypes 1 (56 patients), 2 (9 patients), 3 (23 patients), and not specified (99 patients). Auditory outcomes included improved sound detection, speech perception, and speech intelligibility. QOL outcomes were reported for 75 patients, with benefit reported in the majority. Conclusions: Many patients with Usher syndrome develop improved auditory outcomes after cochlear implantation with early implantation being an important factor. [ABSTRACT FROM AUTHOR]

Rehabilitation options for conductive and mixed hearing loss are continually expanding, but without standard outcome measures comparison between different treatments is difficult. To meaningfully inform clinicians and patients core outcome sets (COS), determined via a recognised methodology, are needed. Following our previous work that identified hearing, physical, economic and psychosocial as core areas of a future COS, the AURONET group reviewed hearing outcome measures used in existing literature and assigned them into different domains within the hearing core area. Scoping review. Literature including hearing outcome measurements for the treatment of conductive and/or mixed hearing loss. The literature search identified 1434 studies, with 278 subsequently selected for inclusion. A total of 837 hearing outcome measures were reported and grouped into nine domains. The largest domain constituted pure-tone threshold measurements accounting for 65% of the total outcome measures extracted, followed by the domains of speech testing (20%) and questionnaires (9%). Studies of hearing implants more commonly included speech tests or hearing questionnaires compared with studies of middle ear surgery. A wide range of outcome measures are currently used, highlighting the importance of developing a COS to inform individual practice and reporting in trials/research. [ABSTRACT FROM AUTHOR]

Objectives: The new CI600 series cochlear implants (Nucleus® Profile™ Plus Series; CI) are more MRI (magnetic resonance imaging) compatible. The magnet's attraction force is lower, possibly posing a problem in patients needing a higher strength magnet due to increased skin thickness. This study aims to investigate the relationship between skin thickness and magnet strength in patients undergoing cochlear implantation. Methods: This cross-sectional study used data from 46 patients with cochlear implants. Data collected included age, body mass index, magnet strength used, pre-operative and post-operative imaging and skin thickness. Results: There was a weak, positive correlation between magnet strength and skin thickness, which was not statistically significant (r(46) = 0.149, p = 0.324). There was a weak, positive correlation between magnet strength and body mass index, which was not statistically significant (r(46) = 0.113, p = 0.456). There was a moderate, positive correlation between body mass index and skin thickness, which was statistically significant (r(46) = 0.362, p = 0.012). Discussion: The increased skin thickness in patients with higher body mass indexes, might mean that these patients will require a stronger magnet. Conclusion: There are concerns that the lower attraction force in the CI600 (Nucleus® Profile™ Plus Series) may pose a retention problem for these patients. This might mean that discussions about skin flap reduction surgery are needed. [ABSTRACT FROM AUTHOR]

Objectives: To compare the hearing performance of patients with conductive and mild mixed hearing loss and single‐sided sensorineural deafness provided with a new transcutaneous bone conduction hearing implant (the Baha Attract System) with unaided hearing as well as aided with a sound processor on a softband. Furthermore, to evaluate safety and subjective benefit before and after implantation of the test device. Participants: Fifty‐four adult patients in five participating centres were enrolled in this prospective study. Baseline data were collected during a pre‐operative visit, and after a softband trial, all patients were implanted unilaterally. Follow‐up visits were scheduled at 10 days, 4, 6, 12 weeks and 6 months. Main outcome measures. Free‐field hearing thresholds pure‐tone average (PTA4 in dB HL; mean threshold at 500, 1000, 2000, 4000 Hz; primary outcome measure). Individual free‐field hearing thresholds, speech recognition in quiet and in noise, soft tissue status during follow‐up and subjective benefit as measured with the Abbreviated Profile of Hearing Aid Benefit (APHAB), Speech, Spatial and Qualities of Hearing Scale (SSQ) and Health Utilities Index (HUI) questionnaires. Results: Implantation of the Baha Attract System resulted in favourable audiological outcomes compared to unaided conditions. On the primary outcome parameter, a statistically significant improvement was observed compared to unaided hearing for the patients with conductive/mixed hearing loss (mean PTA4 difference −20.8 dB HL, SD 9.8; P < 0.0001) and for the patients with single‐sided sensorineural deafness (SSD) (mean PTA4 difference −21.6 dB HL, SD 12.2; P < 0.0001). During all audiology tests, the non‐test ear was blocked. Statistically significant improvements were also recorded in speech tests in quiet and noise compared to unaided hearing for the conductive/mixed hearing loss group and for speech in quiet in the SSD group. Compared to the pre‐operative measurement with softband, no significant differences were recorded in the PTA4 free‐field hearing threshold or the other audiological outcomes in either of the groups (P > 0.05). Soft tissue‐related issues observed during follow‐up included numbness, pain/discomfort at the implant site and to a lesser extent pressure‐related skin complications. A declining trend was noted in the rate of these complications during follow‐up. Approximately 20% of patients reported some degree of numbness and 38% (slight) pain/discomfort at final follow‐up of 6 months. Good results on the subjective benefit questionnaires were observed, with statistically significant improvements on APHAB and SSQ questionnaires, and on the hearing attribute of HUI3. Conclusions: The Baha Attract System provided a significant improvement in hearing performance and subjective benefit compared to the pre‐operative unaided condition (with the non‐test ear blocked). Hearing performance of the Baha Attract was similar to a test situation with the same sound processor on a softband. A proportion of the patients reported numbness and pain/discomfort at the implant site during follow‐up, especially during the first post‐operative weeks. Based on the results of the current multicentre study, the Baha Attract can be considered as a treatment option for patients with the aforementioned hearing losses. Especially in the SSD patients, a careful selection procedure is warranted. Therefore, a pre‐operative trial should be part of the decision‐making process before fitting a patient with the Baha Attract System. [ABSTRACT FROM AUTHOR]

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