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The aim of robot-assisted cochlear implant surgery (RACIS) is to access the inner ear with minimal trauma. High-resolution imaging, empowered with a highly accurate navigation system can enable the planning of a direct keyhole drilling trajectory toward the inner ear. The time has come to (re)define the ideal trajectory into the inner ear with robot-assisted tools that can drill with the highest accuracy. The trajectories of past RACIS procedures were analysed to gain insight into how to calculate the most ideal trajectory and to determine which trajectory parameters influence the course of the procedure. Data-analysis was performed on three groups of previously performed RACIS-procedures. Group 1 included the RACIS-procedures with a round window (RW) approach. Group 2 included the RACIS-procedures with difficulty entering the inner ear. Group 3 included the converted cases to conventional CI surgery due to inadequate intra-operative safety margins. The RW diameter was significantly smaller and its orientation significantly less favourable in group 2 compared to group 1. A smaller surface size and an unfavourable orientation of the RW in relation to the drilling trajectory are thus associated with a more difficult electrode array insertion in RACIS. Both must be taken into account when planning a drilling trajectory into the inner ear., Competing Interests: Declarations Competing interests The authors declare no competing interests., (© 2024. The Author(s).)

Introduction: Robot-assisted cochlear implant surgery (RACIS) as defined by the HEARO®-procedure performs minimal invasive cochlear implant (CI) surgery by directly drilling a keyhole trajectory towards the inner ear. Hitherto, an entirely robotic automation including electrode insertion has not been described yet. The feasability of using a newly developed, dedicated motorised device for automated electrode insertion in the first clinical case of entirely robotic cochlear implant surgery was investigated., Aim: The aim is to report the first experience of entirely robotic cochlear implantation surgery., Intervention: RACIS with a straight flexible lateral wall electrode., Primary Outcome Measurements: Electrode cochlear insertion depth., Secondary Outcome Measurements: The audiological outcome in terms of mean hearing thresholds., Conclusion: Here, we report on a cochlear implant robot that performs the most complex surgical steps to place a cochlear implant array successfully in the inner ear and render similar audiological results as in conventional surgery. Robots can execute tasks beyond human dexterity and will probably pave the way to standardize residual hearing preservation and broadening the indication for electric-acoustic stimulation in the same ear with hybrid implants., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objectives: Cochlear implantation is the most effective treatment for patients with severe-to-profound sensorineural hearing loss. Much scientific work has been published since their inception. There is a need for a critical reflection on how and what we publish on cochlear implantation., Methods: All Science Citation Index Expanded featured articles between 1980 and 2022 with the word 'cochlear implants' or 'cochlear implantation' were collected from the Web of Science database. Separate characteristics, such as the publication dates, the journals, the number of citations, the countries of origin, the authors, the institutions and co-occurring keywords, were assessed., Results: 13,934 articles were included in the data analysis. The journals of of Otology and Neurotology, Ear and Hearing and of Pediatric Otorhinolaryngology represent the top three most publishing journals. Hannover Medical School, the University of Melbourne and the University of Northern Iowa represent the top three most publishing institutions., Discussion: The amount of scientific publications on cochlear implant technology has increased for the last 40 years. Besides the focus on speech perception, the research landscape on cochlear implantation is broad and diverse. The number of countries and institutions contributing to these publications is limited., Conclusion: This bibliometric analysis serves as a quantitative overview of the research landscape on cochlear implantation.

Objective: To investigate the hypothesis that day-case cochlear implantation is associated with equal quality of life, hearing benefits and complications rates, compared to inpatient cochlear implantation., Study Design: A single-center, non-blinded, randomized controlled, equivalence trial in a tertiary referral center., Methods: Thirty adult patients with post-lingual bilateral sensorineural hearing loss eligible for unilateral cochlear implantation surgery were randomly assigned to either the day-case or inpatient treatment group. The effect on general quality of life, patient satisfaction, (subjective) hearing improvement, postoperative complications and causes of crossover and/or readmission were assessed using questionnaires, auditory evaluations and patients' charts over a follow-up period of 1 year., Results: Overall quality of life measured by the HUI3 was equal between the day-case (n = 14) and inpatient group (n = 14). The overall patients' satisfaction showed a slight favor towards an inpatient approach. There was no significant difference in the subjective and objective hearing improvement between both treatment groups. During the 1-year follow-up period no major complications occurred. Minor complications occurred intraoperatively in three day-case patients resulting in three out of nine admissions of day-case patients. Other causes of admission of day-case patients were nausea and vomiting (n = 1), drowsiness (n = 1), late scheduled surgery (n = 2), social reasons (n = 1), or due to an unclear reason (n = 1). No patients required readmission., Conclusion: We found equal outcomes of QoL, patient satisfaction, objective, and subjective hearing outcomes between day-case and inpatient unilateral cochlear implantation. Nine out of 14 day-case patients were admitted for at least one night postoperatively (crossover). No major complications occurred in both groups. A day-case approach seems feasible when using specific patient selection, surgical planning and the preoperative provision of patient information into account. Besides this, the familiarity with a day-case approach of both patient and the surgical team can increase the feasibility of day-case surgery., (© 2024. The Author(s).)

Purpose: The main objective of this study is to evaluate the short-term and long-term audiological outcomes in patients who underwent cochlear implantation with a robot-assisted system to enable access to the cochlea, and to compare outcomes with a matched control group of patients who underwent cochlear implantation with conventional access to the cochlea., Methods: In total, 23 patients were implanted by robot-assisted cochlear implant surgery (RACIS). To evaluate the effectiveness of robotic surgery in terms of audiological outcomes, a statistically balanced control group of conventionally implanted patients was created. Minimal outcome measures (MOM), consisting of pure-tone audiometry, speech understanding in quiet and speech understanding in noise were performed pre-operatively and at 3 months, 6 months, 12 months and 2 years post-activation of the audioprocessor., Results: There was no statistically significant difference in pure-tone audiometry, speech perception in quiet and speech perception in noise between robotically implanted and conventionally implanted patients pre-operatively, 3 months, 6 months, 12 months and 2 years post-activation. A significant improvement in pure-tone hearing thresholds, speech understanding in quiet and speech understanding in noise with the cochlear implant has been quantified as of the first measurements at 3 months and this significant improvement remained stable over a time period of 2 years for HEARO implanted patients., Conclusion: Clinical outcomes in robot-assisted cochlear implant surgery are comparable to conventional cochlear implantation. CLINICALTRAILS., Gov Trail Registration Numbers: NCT03746613 (date of registration: 19/11/2018), NCT04102215 (date of registration: 25/09/2019)., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Cochlear implantation (CI) for deafblindness may have more impact than for non-syndromic hearing loss. Deafblind patients have a double handicap in a society that is more and more empowered by fast communication. CI is a remedy for deafness, but requires revision surgery every 20 to 25 years, and thus placement should be minimally invasive. Furthermore, failed reimplantation surgery will have more impact on a deafblind person. In this context, we assessed the safety of minimally invasive robotically assisted cochlear implant surgery (RACIS) for the first time in a deafblind patient. Standard pure tone audiometry and speech audiometry were performed in a patient with deafblindness as part of this robotic-assisted CI study before and after surgery. This patient, with an optic atrophy 1 ( OPA1 ) (OMIM#165500) mutation consented to RACIS for the second (contralateral) CI. The applicability and safety of RACIS were evaluated as well as her subjective opinion on her disability. RACIS was uneventful with successful surgical and auditory outcomes in this case of deafblindness due to the OPA1 mutation. RACIS appears to be a safe and beneficial intervention to increase communication skills in the cases of deafblindness due to an OPA1 mutation. The use of RACIS use should be widespread in deafblindness as it minimizes surgical trauma and possible failures.

Aim: To report the experience of an image-guided and navigation-based robot arm as an assistive surgical tool for cochlear implantation in a case with a labyrinthitis ossificans., Patient: A 55-years-old man with a history of childhood meningitis whose hearing deteriorated progressively to bilateral profound sensorineural hearing loss., Intervention: Robotic Assisted Cochlear Implant Surgery (RACIS) with a straight flexible lateral wall electrode., Primary Outcome Measures: Electrode cochlear insertion depth with RACIS with facial recess approach and autonomous inner ear access with full electrode insertion of a flexible straight cochlear implant array., Conclusions: Intra cochlear ossifications pose a challenge for entering the cochlea and full-length insertion of a cochlear implant. RACIS has shown that computations of radiological images combined with navigation-assisted robot arm drilling can provide efficient access to the inner ear., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: To investigate the effect of frequency-to-place mismatch, i.e. the mismatch between the tonotopic frequency map in the cochlea and the frequency band that is assigned to an electrode contact of a cochlear implant (CI) at the same cochlear location on speech perception outcomes, using postoperative CT images., Study Design: Retrospective observational single-centre study., Methods: Retrospective pre- and postoperative clinical CT data of 39 CI recipients with normal cochlear anatomy were analysed in an otological surgical planning software. The tonotopic frequency at each electrode position was estimated using the Greenwood function. For each patient, frequency-to-place mismatch between the tonotopic frequency and the fitted centre frequency for each electrode contact was calculated. The influence of frequency-to-place mismatch on speech perception in noise at 6 and 12 months after CI activation was studied., Results: A significant linear correlation was found between the frequency-to-place mismatch and speech perception in noise 6 months after cochlear implantation (p < 0.05). The smaller the frequency-to-place mismatch, the better the initial speech perception in noise results of the CI recipients. The significant effect disappeared after 12 months CI experience., Conclusion: The study findings support the idea of minimizing the frequency-to-place mismatch in CI recipients in order to pursue better initial speech perception in noise. Further research is needed to investigate the prospect of tonotopic fitting strategies based upon postoperative CT images of the exact locations of the electrode contacts., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To determine the usefulness of the Health Utilities Index (HUI) in older cochlear implant (CI) recipients, the primary aims were: (1) to assess health-related quality of life (HRQoL), measured with HUI, in older CI candidates while comparing with age- and gender-matched normal-hearing controls; (2) to compare HRQoL after CI with the pre-operative situation, using HUI and the Nijmegen cochlear implant questionnaire (NCIQ). The difference between pre- and postoperative speech intelligibility in noise (SPIN) and in quiet (SPIQ) and the influence of pre-operative vestibular function on HRQoL in CI users were also studied., Methods: Twenty CI users aged 55 years and older with bilateral severe-to-profound postlingual sensorineural hearing loss and an age- and gender-matched normal-hearing control group were included. HRQoL was assessed with HUI Mark 2 (HUI2), HUI Mark 3 (HUI3) and NCIQ. The CI recipients were evaluated pre-operatively and 12 months postoperatively., Results: HUI3 Hearing (p = 0.02), SPIQ (p < 0.001), SPIN (p < 0.001) and NCIQ (p = 0.001) scores improved significantly comparing pre- and postoperative measurements in the CI group. No significant improvement was found comparing pre- and postoperative HUI3 Multi-Attribute scores (p = 0.07). The HUI3 Multi-Attribute score after CI remained significantly worse (p < 0.001) than those of the control group. Vestibular loss was significantly related to a decrease in HUI3 Multi-Attribute (p = 0.037) and HUI3 Emotion (p = 0.021) scores., Conclusion: The HUI is suitable to detect differences between normal-hearing controls and CI users, but might underestimate HRQoL changes after CI in CI users over 55., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Magnetic resonance imaging (MRI) is often used to visualize and diagnose soft tissues. Hearing implant (HI) recipients are likely to require at least one MRI scan during their lifetime. However, the MRI scanner can interact with the implant magnet, resulting in complications for the HI recipient. This survey, which was conducted in two phases, aimed to evaluate the safety and performance of MRI scans for individuals with a HI manufactured by MED-EL (MED-EL GmbH, Innsbruck, Austria)., Methods: A survey was developed and distributed in two phases to HEARRING clinics to obtain information about the use of MRI for recipients of MED-EL devices. Phase 1 focused on how often MRI is used in diagnostic imaging of the head region of the cochlear implant (CI) recipients. Phase 2 collected safety information about MRI scans performed on HI recipients., Results: 106 of the 126 MRI scans reported in this survey were performed at a field strength of 1.5 T, on HI recipients who wore the SYNCHRONY CI or SYNCHRONY ABI. The head and spine were the most frequently imaged regions. 123 of the 126 scans were performed without any complications; two HI recipients experienced discomfort/pain. One recipient required reimplantation after an MRI was performed using a scanner that had not been approved for that implant. There was only one case that required surgical removal of the implant to reduce the imaging artefact., Conclusion: Individuals with either a SYNCHRONY CI or SYNCHRONY ABI from MED-EL can safely undergo a 1.5 T MRI when it is performed according to the manufacturer's safety policies and procedures., (© 2021. The Author(s).)

Objective: To compare the cognitive evolution of older adults with severe or profound hearing impairment after cochlear implantation with that of a matched group of older adults with severe hearing impairment who do not receive a cochlear implant (CI)., Design: In this prospective, longitudinal, controlled, and multicenter study, 24 older CI users were included in the intervention group and 24 adults without a CI in the control group. The control group matched the intervention group in terms of gender, age, formal education, cognitive functioning, and residual hearing. Assessments were made at baseline and 14 months later. Primary outcome measurements included the change in the total score on the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing impaired individuals score and on its subdomain score to assess cognitive evolution in both groups. Secondary outcome measurements included self-reported changes in sound quality (Hearing Implant Sound Quality Index), self-perceived hearing disability (Speech, Spatial, and Qualities of Hearing Scale), states of anxiety and depression (Hospital Anxiety and Depression Scale), and level of negative affectivity and social inhibition (Type D questionnaire)., Results: Improvements of the overall cognitive functioning (p = 0.05) and the subdomain "Attention" (p = 0.02) were observed after cochlear implantation in the intervention group; their scores were compared to the corresponding scores in the control group. Significant positive effects of cochlear implantation on sound quality and self-perceived hearing outcomes were found in the intervention group. Notably, 20% fewer traits of Type D personalities were measured in the intervention group after cochlear implantation. In the control group, traits of Type D personalities increased by 13%., Conclusion: Intervention with a CI improved cognitive functioning (domain Attention in particular) in older adults with severe hearing impairment compared to that of the matched controls with hearing impairment without a CI. However, older CI users did not, in terms of cognition, bridge the performance gap with adults with normal hearing after 1 year of CI use. The fact that experienced, older CI users still present subnormal cognitive functioning may highlight the need for additional cognitive rehabilitation in the long term after implantation., (Copyright © 2020 The Authors. Ear & Hearing is published on behalf of the American Auditory Society, by Wolters Kluwer Health, Inc.)

Incomplete partition type III (IP-III) is a relatively rare inner ear malformation that has been associated with a POU3F4 gene mutation. The IP-III anomaly is mainly characterized by incomplete separation of the modiolus of the cochlea from the internal auditory canal. We describe a 71-year-old woman with profound sensorineural hearing loss diagnosed with an IP-III of the cochlea that underwent cochlear implantation. Via targeted sequencing with a non-syndromic gene panel, we identified a heterozygous c.934G > C p. (Ala31Pro) pathogenic variant in the POU3F4 gene that has not been reported previously. IP-III of the cochlea is challenging for cochlear implant surgery for two main reasons: liquor cerebrospinalis gusher and electrode misplacement. Surgically, it may be better to opt for a shorter array because it is less likely for misplacement with the electrode in a false route. Secondly, the surgeon has to consider the insertion angles of cochlear access very strictly to avoid misplacement along the inner ear canal. Genetic results in well describes genotype-phenotype correlations are a strong clinical tool and as in this case guided surgical planning and robotic execution.

Background and Objectives: A rare type of nonsyndromic autosomal recessive hereditary hearing loss is caused by pathogenic mutations in the TRIOBP gene mostly involving exons 6 and 7. These mutations cause hearing loss originating from dysfunction of sensory inner ear hair cells. Of all the affected siblings, 2 brothers and 1 sister, part of an Afghan family, were referred to our clinic for diagnostic workup and candidacy selection for cochlear implantation (CI)., Methods: Molecular analysis showed a homozygous c.1342C > T p. (Arg448*) pathogenic variant in exon 7 of the TRIOBP gene (reference sequence NM&#95;001039141.2) in all 3 affected siblings. Clinical audiometry demonstrated profound sensorineural hearing loss in all 3 affected siblings (2 males and 1 female), and they were implanted unilaterally., Results: One month after activation, the pure-tone averages with the CI processor were between 30 and 23 dBHL. Ten months after the first activation of the implant, open-set speech audiometry test could be performed for the first time in the 2 younger CI recipients (S5 and S9), and they could identify up to a maximum 77% phonemes correctly. The oldest brother (S12) could not yet perform open-set speech audiometry at that moment., Conclusions: Implant outcomes are better with normal inner ear anatomy in general. The earlier congenital patients are implanted, the better their outcomes. Here, we demonstrate both statements are true in a homozygous c.1342C > T p. (Arg448*) pathogenic variant in the TRIOBP gene in all 3 affected siblings., (© 2020 S. Karger AG, Basel.)

Background: Cochlear implantation in children with sensorineural hearing loss is preferably performed at youngest age because early auditory input is essential to prevent neural plasticity decline. In contrast, the rate of anesthetic adverse events is increased during infancy. Therefore, to provide recommendations regarding an optimal pediatric implantation age, these possible anesthetic risks in infants need to be taken into account., Aims: This study aimed at assessing the relation between the age at cochlear implant surgery and anesthetic and surgical adverse events. Secondary aims were to evaluate anesthetic and surgical adverse events in relation to (a) the number of preoperative anesthesia-related procedures for cochlear implant candidacy assessment and (b) the anesthetic maintenance agent (total intravenous anesthesia versus inhalation anesthesia) during implantation., Methods: We executed a retrospective cohort study to evaluate cochlear implantation performed in infants and toddlers between January 2008 and July 2015 in a tertiary pediatric center. We compared anesthetic and surgical adverse events between age-at-implantation (0-12 and 12-24 months of age) groups. Furthermore, we assessed whether anesthetic adverse events occurred during preoperative anesthesia-related procedures for cochlear implant candidacy assessment., Results: Forty-six cochlear implantations were performed in 43 patients requiring 42 preoperative anesthesia-related procedures. Nineteen cochlear implantations (41.3%) were performed during infancy. During implantation, the maintenance agent was either sevoflurane (n = 22) or propofol (n = 24). None of the patients encountered major anesthetic adverse events, whereas minor adverse events occurred during 34 cochlear implantations. Those attributed to surgery occurred following six implantations. Neither the age at implantation nor the anesthetic maintenance agent was significantly related to the occurrence of both types of adverse events., Conclusions: Adverse events occur independent of the age at implantation, the number of anesthetic preoperative procedures, and the type of anesthetic maintenance agent in patients who received a cochlear implant before 24 months of age., (© 2020 The Authors. Pediatric Anesthesia published by John Wiley & Sons Ltd.)

Objectives: The ideal outcome of cochlear implant surgery involves the insertion of the array inside the scala tympani of the cochlea with the least mechanical trauma. Recently, round window insertion and the direction in which the cochlea is approached have gained attention in this respect. The Angles of Cochlear Approach (ACA) can be defined with a plane in the plane of the basal turn, termed the in-plane angle, and the plane orthogonal to this plane, termed the out-plane angle. The aim of this study was to compare the trajectory angles for different surgical techniques of Veria, suprameatal, pericanal, and multiple posterior tympanotomy (PT) approaches, including an optimal trajectory that is simulated for robotic surgery., Materials and Methods: The trajectories of these surgical techniques were simulated on the same high-resolution computed tomography scan. The simulated trajectory angles were analyzed with dedicated software for medical images, defining the ACA and distances to critical otological structures., Results: The ACA are the smallest for surgical techniques that pass thought the PT. However, performing a surgical PT can include variability in the ACA, ranging from almost 0° to 20.8° in an out-plane angle, depending on how close a surgeon would approach the facial nerve. The Veria, Suprameatal approach (SMA), and peri-canal approaches have larger ACA and minimal distances to the ossicular chain and the ear canal. The maximum distance to the facial nerve and the widest out-plane angle is observe with a pericanal approach. The optimal PT approach refers to the trajectory without collisions and with the best possible ACA that can be planned., Conclusion: Different surgical approaches yield important differences in the ACA. PT allows better ACA with maximum distances to the critical structures. However, the optimal PT trajectory simulated for robotic keyhole surgery is a further improvement on the trajectories through the facial recess.

Importance: Although current guidelines recommend cochlear implantation only for children with profound hearing impairment (HI) (>90 decibel [dB] hearing level [HL]), studies show that children with severe hearing impairment (>70-90 dB HL) could also benefit from cochlear implantation., Objective: To perform a systematic review to identify audiologic thresholds (in dB HL) that could serve as an audiologic candidacy criterion for pediatric cochlear implantation using 4 domains of speech and language development as independent outcome measures (speech production, speech perception, receptive language, and auditory performance)., Evidence Review: PubMed and Embase databases were searched up to June 28, 2017, to identify studies comparing speech and language development between children who were profoundly deaf using cochlear implants and children with severe hearing loss using hearing aids, because no studies are available directly comparing children with severe HI in both groups. If cochlear implant users with profound HI score better on speech and language tests than those with severe HI who use hearing aids, this outcome could support adjusting cochlear implantation candidacy criteria to lower audiologic thresholds. Literature search, screening, and article selection were performed using a predefined strategy. Article screening was executed independently by 4 authors in 2 pairs; consensus on article inclusion was reached by discussion between these 4 authors. This study is reported according to the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) statement., Findings: Title and abstract screening of 2822 articles resulted in selection of 130 articles for full-text review. Twenty-one studies were selected for critical appraisal, resulting in selection of 10 articles for data extraction. Two studies formulated audiologic thresholds (in dB HLs) at which children could qualify for cochlear implantation: (1) at 4-frequency pure-tone average (PTA) thresholds of 80 dB HL or greater based on speech perception and auditory performance subtests and (2) at PTA thresholds of 88 and 96 dB HL based on a speech perception subtest. In 8 of the 18 outcome measures, children with profound HI using cochlear implants performed similarly to children with severe HI using hearing aids. Better performance of cochlear implant users was shown with a picture-naming test and a speech perception in noise test. Owing to large heterogeneity in study population and selected tests, it was not possible to conduct a meta-analysis., Conclusions and Relevance: Studies indicate that lower audiologic thresholds (≥80 dB HL) than are advised in current national and manufacturer guidelines would be appropriate as audiologic candidacy criteria for pediatric cochlear implantation.

Objectives: International guidelines indicate that children with profound hearing loss should receive a cochlear implant (CI) soon after diagnosis in order to optimize speech and language rehabilitation. Although prompt rehabilitation is encouraged by current guidelines, delays in cochlear implantation are still present. This study investigated whether European countries establish timely pediatric CI care based on epidemiological, commercial, and clinical data., Methods: An estimation of the number of pediatric CI candidates in European countries was performed and compared to epidemiological (Euro-CIU), commercial (Cochlear ® ), and clinical (institutional) age-at-implantation data. The ages at implantation of pediatric patients in eight countries (the Netherlands, Belgium, Germany, the United Kingdom, France, Turkey, Portugal, and Italy) between 2005 and 2015 were evaluated., Results: From 2010 onwards, over 30% of the pediatric CI candidates were implanted before 24 months of age. Northern European institutions implanted children on average around 12 months of age, whereas southern European institutions implanted children after 18 months of age. The Netherlands and Germany implanted earliest (between 6 and 11 months)., Discussion: Implemented newborn hearing screening programs and reimbursement rates of CIs vary greatly within Europe due to local, social, financial, and political differences. However, internationally accepted recommendations are applicable to this heterogeneous European CI practice. Although consensus on early pediatric cochlear implantation exists, this study identified marked delays in European care., Conclusion: Regardless of the great heterogeneity in European practice, reasons for latency should be identified on a national level and possibilities to prevent avoidable future implantation delays should be explored to provide national recommendations.

Objectives/hypothesis: To investigate hearing capabilities and self-reported benefits of simultaneous bilateral cochlear implantation (BiCI) compared with unilateral cochlear implantation (UCI) after a 2-year follow-up and to evaluate the learning effect of cochlear implantees over time., Study Design: Multicenter randomized controlled trial., Methods: Thirty-eight postlingually deafened adults were included in this study and randomly allocated to either UCI or simultaneous BiCI. Our primary outcome was speech intelligibility in noise, with speech and noise coming from straight ahead (Utrecht-Sentence Test with Adaptive Randomized Roving levels). Secondary outcomes were speech intelligibility in noise with spatially separated sources, speech intelligibility in silence (Dutch phoneme test), localization capabilities and self-reported benefits assessed with different quality of hearing and quality of life (QoL) questionnaires. This article describes the results after 2 years of follow-up., Results: We found comparable results for the UCI and simultaneous BiCI group, when speech and noise were both presented from straight ahead. Patients in the BiCI group performed significantly better than patients in the UCI group, when speech and noise came from different directions (P = .01). Furthermore, their localization capabilities were significantly better. These results were consistent with patients' self-reported hearing capabilities, but not with the questionnaires regarding QoL. We found no significant differences on any of the subjective and objective reported outcomes between the 1-year and 2-year follow-up., Conclusions: This study demonstrates important benefits of simultaneous BiCI compared with UCI that remain stable over time. Bilaterally implanted patients benefit significantly in difficult everyday listening situations such as when speech and noise come from different directions. Furthermore, bilaterally implanted patients are able to localize sounds, which is impossible for unilaterally implanted patients., Level of Evidence: 1b Laryngoscope, 127:1161-1168, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Introduction: Cochlear implantation is an increasingly common procedure in the treatment of severe to profound sensorineural hearing loss (SNHL) in children and adults. It is often performed as a day-case procedure. The major drive towards day-case surgery has been from a logistical, economical and societal perspective, but we also speculate that the patient's quality of life (QoL) is at least equal to inpatient surgery if not increased as a result of rapid discharge and rehabilitation. Even though cochlear implantation seems well suited to a day-case approach and this even seems to be common practice in some countries, evidence is scarce and of low quality to guide us towards the preferred treatment option., Methods and Analysis: A single-centre, non-blinded, randomised, controlled trial was designed to (primarily) investigate the effect on general QoL of day-case cochlear implantation compared to inpatient cochlear implantation and (secondarily) the effect of both methods on (subjective) hearing improvement, disease-specific QoL, tinnitus, vertigo and cost-effectiveness. 30 adult patients with severe to profound bilateral postlingual SNHL who are eligible for unilateral cochlear implantation will be randomly assigned to either the day-case or inpatient treatment group. The outcome measures will be assessed using auditory evaluations, questionnaires (preoperatively, at 1-week, 3-week, 3-month and 1-year follow-up) and costs diaries (weekly during the first month postoperatively, after which once in a month until 1-year follow-up). Preoperative and postoperative outcomes will be compared. The difference in costs and benefit will be represented using the incremental cost utility/effectiveness ratio. The analyses will be carried out on an intention-to-treat basis., Ethics and Dissemination: This research protocol was approved by the Institutional Review Board of the UMC Utrecht (NL45590.041.13; V.5, November 2015). The trial results will be disseminated through peer-reviewed medical journals and presented at scientific conferences., Trial Registration Number: NTR4464; Pre-results., Competing Interests: Conflicts of Interest: None declared., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Objectives/hypothesis: To determine the effect of cochlear implantation on tinnitus perception in patients with severe bilateral postlingual sensorineural hearing loss and to demonstrate possible differences between unilateral and bilateral cochlear implantation., Study Design: Prospective study., Methods: Thirty-eight adult patients were included in this prospective study, as part of a multicenter randomized controlled trial investigating the benefits of bilateral cochlear implantation versus unilateral cochlear implantation. Pre- and postoperative tinnitus perception scores were evaluated, before and 1 year after implantation on three tinnitus questionnaires; the Tinnitus Handicap Inventory (THI), the Tinnitus Questionnaire (TQ), and a visual analogue scale for tinnitus burden., Results: Before implantation, the tinnitus prevalence was 42.1% (16 of 38) in the whole study group. One year after implantation, the tinnitus questionnaire scores had decreased in 71.4% according to the TQ and 80.0% according to the THI. Tinnitus was induced after cochlear implantation in six patients, five in the bilateral and one in the unilateral group., Conclusions: Our study shows that cochlear implantation is effective in the reduction of tinnitus in patients with bilateral sensorineural hearing loss who suffered from preoperative tinnitus. Conversely, tinnitus may also increase or even be induced by the cochlear implantation itself. Cochlear implant candidates should be well informed about these possible consequences before undergoing surgery., Level of Evidence: 2b., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: The mastoidectomy with facial recess approach (MFRA) is considered the reference standard for cochlear implantation. The suprameatal approach (SMA) was developed more recently and does not require mastoidectomy, which could influence postoperative outcomes. We aim to identify the optimal operative approach for cochlear implantation based on postoperative complications and hearing preservation in children and adults., Data Sources: PubMed, EMBASE, Scopus, and Google Scholar., Review Methods: Studies comparing MFRA and SMA in children and adults were eligible for inclusion. Original reports with moderate relevance and validity were included. Relevance and validity were assessed with a self-modified critical appraisal tool. This review was reported in accordance to PRISMA guidelines., Results: We retrieved 294 citations. Only retrospective nonrandomized studies were identified (level III evidence). Six articles were selected for full-text inclusion and 4 articles for data extraction. No article found a significant difference between MFRA and SMA with respect to postoperative complications in children and adults. One study found a significantly (P < .023) higher pediatric MFRA mastoiditis rate; however, meta-analysis did not indicate an overall effect. Hearing preservation was reported only in adults, and outcomes between techniques did not differ., Conclusion: No evidence was noted for lower complication rates or improved hearing preservation between the MFRA and SMA for cochlear implantation in children and adults. Pediatric data were available for children implanted above the age of 24 months only. Level I evidence is needed to resolve the uncertainty regarding differences in postoperative outcomes of pediatric and adult MFRA and SMA., (© American Academy of Otolaryngology—Head and Neck Surgery Foundation 2016.)

Importance: The cost of bilateral cochlear implantation (BCI) is usually not reimbursed by insurance companies because of a lack of well-designed studies reporting the benefits of a second cochlear implant., Objective: To determine the benefits of simultaneous BCI compared with unilateral cochlear implantation (UCI) in adults with postlingual deafness., Design, Setting, and Participants: A multicenter randomized clinical trial was performed. The study took place in 5 Dutch tertiary referral centers: the University Medical Centers of Utrecht, Maastricht, Groningen, Leiden, and Nijmegen. Forty patients eligible for cochlear implantation met the study criteria and were included from January 12, 2010, through November 2, 2012. The main inclusion criteria were postlingual onset of hearing loss, age of 18 to 70 years, duration of hearing loss of less than 20 years, and a marginal hearing aid benefit. Two participants withdrew from the study before implantation. Nineteen participants were randomized to undergo UCI and 19 to undergo BCI., Interventions: The BCI group received 2 cochlear implants during 1 surgery. The UCI group received 1 cochlear implant., Main Outcomes and Measures: The primary outcome was the Utrecht Sentence Test with Adaptive Randomized Roving levels (speech in noise, both presented from straight ahead). Secondary outcomes were consonant-vowel-consonant words in silence, speech-intelligibility test with spatially separated sources (speech in noise from different directions), sound localization, and quality of hearing questionnaires. Before any data were collected, the hypothesis was that the BCI group would perform better on the objective and subjective tests that concerned speech intelligibility in noise and spatial hearing., Results: Thirty-eight patients were included in the study. Fifteen patients in the BCI group used hearing aids before implantation compared with 19 in the UCI group. Otherwise, there were no significant differences between the groups' baseline characteristics. At 1-year follow-up, there were no significant differences between groups on the Utrecht Sentence Test with Adaptive Randomized Roving levels (9.1 dB, UCI group; 8.2 dB, BCI group; P = .39) or the consonant-vowel-consonant test (median percentage correct score 85.0% in the UCI group and 86.8% in the BCI group; P = .21). The BCI group performed significantly better than the UCI group when noise came from different directions (median speech reception threshold in noise, 14.4 dB, BCI group; 5.6 dB, BCI group; P <.001). The BCI group was better able to localize sounds (median correct score of 50.0% at 60°, UCI group; 96.7%, BCI group; P <.001). These results were consistent with the patients' self-reported hearing capabilities., Conclusions and Relevance: This randomized clinical trial demonstrates a significant benefit of simultaneous BCI above UCI in daily listening situations for adults with postlingual deafness., Trial Registration: trialregister.nl Identifier: NTR1722.

Objective: To study the cost-utility of simultaneous bilateral cochlear implantation (CI) versus unilateral CI., Study Design: Randomized controlled trial (RCT)., Setting: Five tertiary referral centers., Patients: Thirty-eight postlingually deafened adults eligible for cochlear implantation., Interventions: A cost-utility analysis was performed from a health insurance perspective., Main Outcome Measures: Utility was assessed using the HUI3, TTO, VAS on hearing, VAS on general health and EQ-5D. We modeled the incremental cost per quality-adjusted life year (QALY) of unilateral versus bilateral CI over periods of 2, 5, 10, 25 years, and actual life-expectancy., Results: Direct costs for unilateral and bilateral CI were €43,883 ± €11,513(SD) and €87,765 ± €23,027(SD) respectively. Annual costs from the second year onward were €3,435 ± €1,085(SD) and €6,871 ± €2,169(SD), respectively. A cost-utility analysis revealed that a second implant became cost-effective after a 5- to 10-year period, based on the HUI3, TTO, and VAS on hearing., Conclusion: This is the first study that describes a cost-utility analysis to compare unilateral with simultaneous bilateral CI in postlingually deafened adults, using a multicenter RCT. Compared with accepted societal willingness-to-pay thresholds, simultaneous bilateral CI is a cost-effective treatment for patients with a life expectancy of 5-10 years or longer.

Objective: This review aimed to evaluate the additional benefit of pediatric cochlear implantation before 12 months of age considering improved speech and language development and auditory performance., Materials and Methods: We conducted a search in PubMed, EMBASE and CINAHL databases and included studies comparing groups with different ages at implantation and assessing speech perception and speech production, receptive language and/or auditory performance. We included studies with a high directness of evidence (DoE)., Results: We retrieved 3,360 articles. Ten studies with a high DoE were included. Four articles with medium DoE were discussed in addition. Six studies compared infants implanted before 12 months with children implanted between 12 and 24 months. Follow-up ranged from 6 months to 9 years. Cochlear implantation before the age of 2 years is beneficial according to one speech perception score (phonetically balanced kindergarten combined with consonant-nucleus-consonant) but not on Glendonald auditory screening procedure scores. Implantation before 12 months resulted in better speech production (diagnostic evaluation of articulation and phonology and infant-toddler meaningful auditory integration scale), auditory performance (Categories of Auditory Performance-II score) and receptive language scores (2 out of 5; Preschool Language Scale combined with oral and written language skills and Peabody Picture Vocabulary Test)., Conclusions: The current best evidence lacks level 1 evidence studies and consists mainly of cohort studies with a moderate to high risk of bias. Included studies showed consistent evidence that cochlear implantation should be performed early in life, but evidence is inconsistent on all speech and language outcome measures regarding the additional benefit of implantation before the age of 12 months. Long-term follow-up studies are necessary to provide insight on additional benefits of early pediatric cochlear implantation., (© 2016 S. Karger AG, Basel.)

Objectives/hypothesis: To evaluate the influence of the introduction of newborn hearing screening programs on the age at cochlear implantation in children., Study Design: Retrospective, multicenter cohort study., Methods: All 1,299 pediatric cochlear implant users who received their implants before the age of 5 years between 1995 and 2011 in the Medical University Hannover, Germany and University Medical Center Utrecht, the Netherlands were enrolled in this study. Age at implantation and the number of children implanted within the first year of life was assessed for each center., Results: Age at cochlear implantation gradually declined over the years in both centers. The introduction of the screening resulted in significant decline in the age at implantation in the Netherlands; simultaneously, the number of children implanted within their first year of life increased significantly. Comparing 4-year epochs immediately before and after introduction of the screening, the mean age decreased from 2.4 to 1.2 years, and the percentage of early implanted children increased from 9% to 37%. In the German population, a similar effect of the introduction of the hearing screening program was absent., Conclusions: The introduction of the national newborn hearing screening program has reduced the age at cochlear implantation in young children in the Netherlands but not in Germany. Correspondingly, it resulted in an increase in the number of children implanted early in life. The difference between the Dutch and German population might be due to differences in the follow-up and referral after the hearing screening., (© 2014 The American Laryngological, Rhinological and Otological Society, Inc.)

Image-guided and robot-assisted surgeries have found their applications in skullbase surgery. Technological improvements in terms of accuracy also opened new opportunities for robotically-assisted cochlear implantation surgery (RACIS). The HEARO® robotic system is an otological next-generation surgical robot to assist the surgeon. It first provides software-defined spatial boundaries for orientation and reference information to anatomical structures during otological and neurosurgical procedures. Second, it executes a preplanned drill trajectory through the temporal bone. Here, we report how safe the HEARO procedure can provide an autonomous minimally invasive inner ear access and the efficiency of this access to subsequently insert the electrode array during cochlear implantation. In 22 out of 25 included patients, the surgeon was able to complete the HEARO® procedure. The dedicated planning software (OTOPLAN®) allowed the surgeon to reconstruct a three-dimensional representation of all the relevant anatomical structures, designate the target on the cochlea, i.e., the round window, and plan the safest trajectory to reach it. This trajectory accommodated the safety distance to the critical structures while minimizing the insertion angles. A minimal distance of 0.4 and 0.3 mm was planned to facial nerve and chorda tympani, respectively. Intraoperative cone-beam CT supported safe passage for the 22 HEARO® procedures. The intraoperative accuracy analysis reported the following mean errors: 0.182 mm to target, 0.117 mm to facial nerve, and 0.107 mm to chorda tympani. This study demonstrates that microsurgical robotic technology can be used in different anatomical variations, even including a case of inner ear anomalies, with the geometrically correct keyhole to access to the inner ear. Future perspectives in RACIS may focus on improving intraoperative imaging, automated segmentation and trajectory, robotic insertion with controlled speed, and haptic feedback. This study [Experimental Antwerp robotic research otological surgery (EAR2OS) and Antwerp Robotic cochlear implantation (25 refers to 25 cases) (ARCI25)] was registered at clinicalTrials.gov under identifier NCT03746613 and NCT04102215. Clinical Trial Registration: https://www.clinicaltrials.gov, Identifier: NCT04102215. [ABSTRACT FROM AUTHOR]

Objectives: Cochlear implants are known to significantly improve the quality of life (QoL) of implanted children. However, variability exists between self-reported outcomes and parental-reported QoL questionnaires. We evaluated the QoL agreement between children and their parents following cochlear implantation and determined which factors lead to increased agreement. Methods: A cross-sectional study was performed including pediatric cochlear implant recipients and their parents. We evaluated postoperative QoL using The Pediatric Quality of Life Inventory (PedsQL). To assess agreement between pediatric and parental QoL perception, PedsQL intra-class correlations (ICCs) were calculated. Results: Thirty-five children and their parents completed QoL questionnaires. Children who were evaluated between 8-12 years of age reported highest absolute total and subscale PedsQL scores. Highest agreement was found amongst parents and children when: (1) children were between 8-12 years at QoL assessment (ICC: between 0.917[95%CI: 0.676-0.981] and 0.972[95%CI: 0.882-0.994]), and (2) when evaluating the physical health QoL domain (ICC: 0.964[95%CI: 0.849-0.992]). Conclusions: This study demonstrates high agreement (ICC > 0.8) between pediatric and parental QoL report in children aged between 8-12 years at QoL assessment. Therefore, results confirm that: (1) QoL agreement between pediatric self-report and parent proxy-report is high in chronically ill children and (2) children using cochlear implants can reliably report QoL between 8-12 years. [ABSTRACT FROM AUTHOR]

Objective: To compare speech perception between children with a different age at cochlear implantation.Design: We evaluated speech perception by comparing consonant–vowel–consonant (auditory) (CVC(A)) scores at five-year follow-up of children implanted between 1997 and 2010. The proportion of children from each age-at-implantation group reaching the 95%CI of CVC(A) ceiling scores (>95%) was calculated to identify speech perception differences masked by ceiling effects.Study sample: 54 children implanted between 8 and 36 months.Results: Although ceiling effects occurred, a CVC(A) score difference between age-at-implantation groups was confirmed (H(4) = 30.36;p < 0.001). Outperformance of early (<18 months) compared to later implanted children was demonstrated (p <0.001). A larger proportion of children implanted before 13 months compared to children implanted between 13 and 18 months reached ceiling scores. Logistic regression confirmed that age at implantation predicted whether a child reached a ceiling score.Conclusions: Ceiling effects can mask thorough delineation of speech perception. However, this study showed long-term speech perception outperformance of early implanted children (<18 months) either including or not accounting for ceiling effects during analysis. Development of long-term assessment tools not affected by ceiling effects is essential to maintain adequate assessment of young implanted infants. [ABSTRACT FROM PUBLISHER]

Objectives/hypothesis: To determine the effect of cochlear implantation on tinnitus perception in patients with severe bilateral postlingual sensorineural hearing loss and to demonstrate possible differences between unilateral and bilateral cochlear implantation.Study Design: Prospective study.Methods: Thirty-eight adult patients were included in this prospective study, as part of a multicenter randomized controlled trial investigating the benefits of bilateral cochlear implantation versus unilateral cochlear implantation. Pre- and postoperative tinnitus perception scores were evaluated, before and 1 year after implantation on three tinnitus questionnaires; the Tinnitus Handicap Inventory (THI), the Tinnitus Questionnaire (TQ), and a visual analogue scale for tinnitus burden.Results: Before implantation, the tinnitus prevalence was 42.1% (16 of 38) in the whole study group. One year after implantation, the tinnitus questionnaire scores had decreased in 71.4% according to the TQ and 80.0% according to the THI. Tinnitus was induced after cochlear implantation in six patients, five in the bilateral and one in the unilateral group.Conclusions: Our study shows that cochlear implantation is effective in the reduction of tinnitus in patients with bilateral sensorineural hearing loss who suffered from preoperative tinnitus. Conversely, tinnitus may also increase or even be induced by the cochlear implantation itself. Cochlear implant candidates should be well informed about these possible consequences before undergoing surgery.Level Of Evidence: 2b. [ABSTRACT FROM AUTHOR]

Background MYH9-related disease (MYH9-RD) is a rare syndromic disorder deriving from mutations in MYH9, the gene for the heavy chain of non-muscle myosin IIA. Patients present with congenital thrombocytopenia and giant platelets and have a variable risk of developing sensorineural deafness, kidney damage, presenile cataract, and liver abnormalities. Almost all MYH9-RD patients develop the hearing defect, which, in many individuals, progresses to severe to profound deafness with high impact on quality of life. These patients are potential candidates for cochlear implantation (CI), however, no consistent data are available about the risk to benefit ratio of CI in MYH9-RD. The only reported patient who received CI experienced perisurgery complications that have been attributed to concurrent platelet defects and/or MYH9 protein dysfunction. Methods By international co-operative study, we report the clinical outcome of 10 patients with MYH9-RD and severe to profound deafness who received a CI at 8 institutions. Results Nine patients benefited from CI: in particular, eight of them obtained excellent performances with restoration of a practically normal hearing function and verbal communication abilities. One patient had a slightly worse performance that could be explained by the very long duration of severe deafness before CI. Finally, one patient did not significantly benefit from CI. No adverse events attributable to MYH9-RD syndrome were observed, in particular no perisurgery bleeding complications due to the platelet defects were seen. Patients' perioperative management is described and discussed. Conclusions CI is safe and effective in most patients with MYH9-RD and severe to profound deafness and should be offered to these subjects, possibly as soon as they develop the criteria for candidacy. [ABSTRACT FROM AUTHOR]