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Objective: To estimate costs of severe to profound hearing loss, including costs and cost-savings associated with cochlear implantation., Methods: Data was obtained from the National Health Interview Survey, the National Health and Nutrition Examination Survey and national Medicare rates. We used continuous time state transition models with individual patient simulations to estimate the costs of severe to profound hearing loss (SPHL) across the lifespan. The model included four states, normal hearing, severe to profound hearing loss, cochlear implantation, and death., Results: The estimated lifetime cost of an individual born with SPHL is $489,274 [377,518; 616,519]. Costs are lower for those who received a cochlear implant before 18 months of age $390,931 [311,976; 471,475], compared to those who are not implanted $608,167 [442,544; 791,719]. For individuals with a later onset of hearing loss (60 years old) lifetime costs were $154,536 [7,093; 302,936]. The annual societal costs for the US population were estimated to be $37 [8; 187] billion., Conclusions: SPHL is a costly condition, with the primary driver being lost productivity. Medical costs were higher for cochlear implantation, however, the higher income earnings offset the higher medical costs. Overall, early implantation substantially reduced lifetime costs. Access to hearing health care and technology is critical given the documented benefits for language, education, and quality of life. Government and insurance policies should be modified to allow for equal access and coverage for hearing technology, which will ultimately reduce lifetime and societal costs., Levels of Evidence: N/A The current study used existing nationally representative datasets. Thus, these levels of evidence do not apply. Laryngoscope, 134:4358-4365, 2024., (© 2024 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Purpose: Clinical registries have great potential for quality control of medical procedures regarding the indications, therapeutic processes and results, including their possible complications. This is particularly true when providing patients with severe hearing loss or deafness with a cochlear implant (CI). This treatment represents a lifelong care process that requires continuous quality control over time. On the initiative of the Executive Committee of the German Society of Otorhinolaryngology (Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e.V., DGHNO-KHC), a national German CI registry (Deutsches Cochlear Implant Register, DCIR) was established in January 2022. This article focuses on the first demographic and baseline data of the DCIR., Methods: The DCIR covers the complete therapeutic process from indication, surgery, fitting and lifelong aftercare in CI therapy. By the end of 2022, 75 hospitals in Germany had agreed to contribute to the DCIR., Results: During the year 2022, 63 hospitals actively contributed data to the DCIR. Pseudonymized data from 2,292 CI implantations (2,176 primary implantations, 99 explantations with immediate re-implantations and 17 re-implantations following an earlier explantation) in 2,108 patients were documented. Cochlear implantation was accomplished in 1,807 adults (≥ 18 years) and 301 children (< 18 years). Fourty patients (1,9%) were children < 1 year of age and 55 (2,6%) were patients > 85 years. From the total of 2,292 implantations, 226 (9.9%) were performed as simultaneous bilateral implantations (CI implantation in both ears of 113 patients on the same day of surgery) and 412 implantations (19.1% of 2,162 implantations with data provided on the contralateral ear's hearing status) were in patients with single sided deafness (normal hearing in the contralateral ear). In addition, the reported complications in 2022 were also evaluated. Seven reports (0.4%) of mild to moderate severe facial nerve dysfunctions were documented. No reports of severe or total facial nerve dysfunction (House-Brackmann grade V/VI), meningitis or death related to CI therapy were documented., Conclusion: Although still in the start-up phase, these initial DCIR data already provide an interesting first insight into the demographic structure and baseline data of CI therapy in Germany. The successful implementation of the DCIR represents an important step towards continuous quality control of CI care., (© 2024. The Author(s).)

Objectives: Research samples that are representative of patient populations are needed to ensure the generalizability of study findings. The primary aim was to assess the efficacy of a study design and recruitment strategy in obtaining a participant sample that was representative of the broader cochlear implant (CI) patient population at the CI center. A secondary aim was to review whether the CI recipient population was representative of the state population., Methods: Demographic variables were compared for a research participant sample (n = 79) and the CI patient population (n = 338). The participant sample was recruited from the CI patient population. The study design included visits that were at the same location and frequency as the recommended clinical follow-up intervals. The demographics for the combined group (participant sample and patient population) were then compared to the reported demographics for the population in North Carolina., Results: There were no significant differences between the participant sample and patient population for biological sex, age at implantation, racial distribution, socioeconomic position, degree of urbanization, or drive time to the CI center (p ≥ 0.086). The combined CI recipient population was significantly different from the North Carolina population for the distributions of race, ethnicity, and degree of urbanization (p < 0.001)., Conclusion: The study design and recruitment strategy allowed for recruitment of a participant sample that was representative of the CI patient population. Disparities in access to cochlear implantation persist, as supported by the significant differences in the combined CI recipient population and the population for our state., Level of Evidence: 3 Laryngoscope, 134:4101-4110, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: We assessed three cochlear implant (CI) suppliers: Advanced Bionics, Cochlear Limited, and MED-EL, for implant revision requiring reoperation after CI placement., Study Design: Retrospective cohort study of integrated-health-system database between 2010 and 2021. Separate models were created for pediatric (age <18) and adult (age ≥18) cohorts., Patients: Pediatric (age <18) and adult (age ≥18) patients undergoing cochlear implantation within our integrated healthcare system., Main Outcome Measure: Revision after CI placement. Cox proportional hazard regression was used to evaluate revision risk and adjust for confounding factors. Hazard ratios (HRs) and 95% confidence intervals (CIs) are presented., Results: A total of 2,347 patients underwent a primary CI placement, and Cochlear Limited was most implanted (51.5%), followed by Advanced Bionics (35.2%) and MED-EL (13.3%). In the pediatric cohort, the 7-year crude revision rate was 10.9% for Advanced Bionics and 4.8% for Cochlear Limited, whereas MED-EL had insufficient cases. In adults, the rates were 9.1%, 4.5%, and 3.3% for Advanced Bionics, MED-EL, and Cochlear Limited, respectively. After 2 years of postoperative follow-up, Advanced Bionics had a significantly higher revision risk (HR = 8.25, 95% CI = 2.91-23.46); MED-EL had no difference (HR = 2.07, 95% CI = 0.46-9.25)., Conclusion: We found an increased revision risk after 2 years of follow-up for adults with Advanced Bionics CI devices. Although we found no statistical difference between manufacturers in the pediatric cohort, after 2 years of follow-up, there were increasing trends in the revision probability for Advanced Bionics. Further research may determine whether patients are better suited for some CI devices., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2024, Otology & Neurotology, Inc.)

Objective(s): Despite undergoing thorough cochlear implant (CI) candidacy evaluation and counseling, some patients ultimately elect against implantation. This study sought to identify patient-related and socioeconomic factors predicting CI deferral., Methods: A retrospective study of adult (≥18 years old) CI candidates presenting between 2007 and 2021 at a tertiary academic CI center was performed. The primary outcome was device implantation. Data collected included age, gender, hearing status, race, zip code of residence, median family income (MFI), distance traveled from the CI center, marital status, employment status, and insurance status. Multivariable binary logistic regression was performed to identify predictors of implantation., Results: A total of 200 patients qualifying for CI were included, encompassing 77 adults deferring surgery (CI-deferred) and 123 consecutive adults electing for surgery (CI-pursued). Age, gender, hearing status, insurance type, employment status, distance from the implant center, and MFI were comparable between the groups (p > 0.05). Compared to CI-pursued patients, CI-deferred patients were more likely to be non-Caucasian (24.7% vs. 9.8%, p = 0.015) and unmarried (55.8% vs. 38.2%, p = 0.015). On multivariable logistic regression, older age (OR 0.981, 0.964-0.998, p = 0.027), African American race (OR 0.227, 0.071-0.726, p = 0.012), and unmarried status (OR 0.505, 0.273-0.935, p = 0.030) were independent predictors of implant deferral., Conclusion: This study demonstrates that increasing age at evaluation, African American race, and unmarried status are predictors for deferring CI surgery despite being implant candidates. These patients may benefit from increased outreach in the form of counseling, education, and social support prior to undergoing CI surgery., Level of Evidence: 3 - retrospective study with internal control group Laryngoscope, 134:2857-2863, 2024., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: The newborn hearing screening (NHS) program was globally established for early hearing loss (HL) identification and intervention. Early intervention is essential to minimize or prevent the negative consequences of HL. In Saudi Arabia, the NHS was officially implemented in 2016. Currently, its impact on the timing of cochlear implantations (CIs) in Saudi Arabia remains unclear, and information on potential hospital-related delays affecting early implantation is lacking. Thus, this study aimed to evaluate the effect of implementing the NHS on age at CI in children with prelingual deafness in a CI center in Saudi Arabia, and to evaluate the hospital timing in the CI process., Methods: All pediatric CI users who presented for the first time to the CI committee (CIC) at a tertiary center and received their implants between 2015 and 2022 were enrolled in this study. Date of birth (DOB), date of presentation to the CI committee (DOCIC), and date of CI surgery (DOCIS) were retrospectively reviewed., Results: In total, 304 CI children were included in the analysis. Approximately 55 % of the children (n = 167) were screened for HL through the NHS, whereas 45 % of the children (n = 137) were born before the launch of the NHS. Both age at the presentation to the CIC (i.e. difference between DOCIC and DOB) and age at implantation (i.e. difference between DOCIS and DOB) were significantly earlier in children who were screened for HL through the NHS than those who were not screened (P < 0.0001). The time difference between the DOCIC and DOCIS was not significantly different between the screened and unscreened children (P > 0.05)., Conclusion: The implementation of the NHS in the tertiary center has a significant positive effect on age at presentation to the CIC and age at implantation, but not on the actual CI surgery. Further research is needed to reduce the hospital delays before the actual surgery in order to increase the likelihood of children receiving implantation early in their life., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Background:  Incomplete partition type II (IP-II) malformation is often accompanied by a large vestibular aqueduct (LVA). In IP anomalies, the patient's auditory rehabilitation requirements are decided according to the presence of inner ear structures and the degree of hearing loss (HL). There has been limited research on auditory rehabilitation (AR) requirement selection in patients diagnosed with IP-II and LVA. This study investigated the typical characteristics of HL and AR choices in patients diagnosed with IP-II and LVA., Methods:  Patients with IP-II and LVA (n=55; 25 women and 30 men) were identified, and audiological evaluations were performed. The patient's demographic characteristics, the type and degree of HL, the AR method, age at diagnosis, and educational status were retrospectively compared., Results:  The distribution of our 55 patients according to cochlear implants, hearing aids (HA), and bimodal applications was 29.1% (n=16), 43.6% (n=24), and 27.3% (n=15), respectively. Statistical analyses using chi-square tests found no significant differences in the incidence of dizziness/imbalance, tinnitus, HL progression, or the degree and onset of HL among the patients., Conclusion:  The data revealed different audiological characteristics among patients with IP-II and LVA, as well as different AR solutions. The most widely used AR modality was found to be HA. Prediction of sudden versus progressive HL development among patients is challenging, and the characteristics of IP-II vary. Therefore, they should be interpreted with caution.

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: This study aimed to determine if the major public awareness campaign for cochlear implants 'International Cochlear Implant Day' influenced national and international public interest as measured by internet search activity., Methods: Weekly search volume data in the United States, Canada, Australia, Germany, United Kingdom, Brazil, India, Japan, and a 'Worldwide' group for the search topic 'cochlear implant' was collected from Google Trends over a 5-year period (2017-2021). The 'Campaign' window was defined as 1 week before, the week of, and 2 weeks after International Cochlear Implant Day (February 25th). 'Non-Campaign' weeks were considered any data outside the 'Campaign' window., Results: Of the studied regions, the United States, United Kingdom, Australia, India, and 'Global' demonstrated a significant increase in internet search activity between 2017 and 2021. Although some individual years showed significant increases during the 'Campaign' period for Canada, Germany, Brazil, and Japan, none showed statistically significant increases over the 5-year period studied., Conclusion: Public awareness campaigns are recognized crucial elements to delivering effective healthcare, but their success varies worldwide. While data from Google Trends suggests that cochlear implant awareness campaigns can translate into increased internet searches, greater efforts can be made in select countries to improve public interest.

Background: Hearing loss is known to be an independent risk factor for inadequate health literacy. The objective of this study was to assess the level of health literacy among patients undergoing cochlear implantation to determine areas for improvement in delivery of patient information., Methods: A cross-sectional survey was conducted at the otology-neurotology clinic at Sunnybrook Health Sciences Centre. Patients eligible for cochlear implantation completed two health literacy screening tools: The Short Test of Functional Health Literacy in Adults (S-TOFHLA) and Brief Health Literacy Screen (BHLS)., Results: Thirty seven patients were included (41% female, 59% male, mean age: 55 years). Most patients had adequate health literacy through BHLS (76%) and S-TOFHLA (98%) scoring. Over 80% of patients were not able to correctly recount all the operative risks associated with cochlear implant surgery and one third of patients did not correctly recount any risks associated with a cochlear implant surgery. Female sex was associated with higher scores (p=0.03) and low income (<$35,000) was associated with lower scores (p=0.05)., Conclusion: Patients eligible for cochlear implants have adequate health literacy, but most are not able to recount operative risks. Educational tools are required to improve patient retention, understand, and perioperative health information delivery.

Objective: To assess the distance burden for access to cochlear implant (CI)-related services and to assess whether socioeconomic disadvantage or level of education and occupation influenced uptake of CIs., Study Design: Retrospective case review., Setting: A CI services provider operating across multiple centers., Patients: All patients undergoing CI surgery in a 2-year period between March 2018 and February 2020., Interventions: Diagnosis of hearing loss, CI surgery, and subsequent habilitation and mapping., Main Outcome Measures: Distance traveled by patients to their audiological diagnostic, CI surgery hospital, and habilitation sites; subjects' index of relative socioeconomic advantage and disadvantage (IRSAD) and index of education and occupation (IEO)., Results: n = 201 children and n = 623 adults. There was a significant difference across IRSAD domains for children (p < 0.0001) and adults (p < 0.0001), and IEO in children (p = 0.015) and adults (p < 0.0001) when tested for equal proportions. The median driving distance from home to the diagnostic audiological site for children was 20 km (mean, 69 km; range, 1-1184 km; upper quartile, 79 km; lower quartile, 8 km). There was no significant difference between the driving distances from home to the CI surgery hospital site, or the mapping/habilitation sites between children and adults. There was no correlation for age at first surgery and either IRSAD/IEO., Conclusions: The burden of distance for access to CI in Australia is significant for the upper quartile who may not live within the large city centers. Greater consideration needs to be given regarding barriers to CI for those in lower socioeconomic and educational groups to ensure equity of access across different socioeconomic and educational level backgrounds., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Introduction: Cochlear Implantation (CI) surgery has long been used as an effective treatment for children with bilateral profound sensorineural hearing loss who failed to benefit from the use of hearing aids. Among other factors, the age at which implantation is done is thought to have an effect on the speech progression outcome of patients postoperatively., Objectives: The Jordanian CI Program has limited resources, and Jordan has an underdeveloped early screening and intervention program for deafness. In this study, we aimed to explore the effect of age at time of implantation on receptive and expressive language outcomes in Jordan, to guide funding and focus efforts on patient groups who would benefit most, thus reducing unnecessary longterm morbidity and disability, and improving cost efficiency., Methods: Data was gathered from all major sectors in Jordan on patients who underwent CI from 2006 to 2018 (a total of 1815 patients). We compared the language outcome 2 to 4 years after implantation for patients aged below 3 years, 3 to under 6, 6 to under 9 years, and 9 and older at the time of implantation., Results and Conclusion: We found a statistically significant difference in language outcomes between patients aged below 6 years vs those 6 and older, with better receptive and expressive language outcomes in the younger age groups.

Objectives: Pediatric cochlear implantation (CI) is a multistep process, which exposes a healthcare system's potential weaknesses in ability to deliver timely care to deaf children. The current systematic review aims to determine the sociodemographic disparities that predict pediatric CI access and use among CI candidates and recipients across the world. We hypothesize that sociodemographic factors independently influence CI access and use within a given country., Study Design: Systematic review., Methods: A qualitative systematic review of PubMed, Scopus, Web of Science, and Embase databases was conducted for studies investigating the association of sociodemographic factors such as race, income, or insurance status with measures of pediatric CI access, such as age at CI or CI rate., Results: Out of 807 unique abstracts initially retrieved, 39 papers were included in the final qualitative systematic review. Twenty-seven thousand seven hundred and fifty-one CI-candidate children (6,623 CI recipients) were studied in 14 countries, with 21 studies conducted in the United States of America, published within the years of 1993 to 2020., Conclusion: Some measures of CI access, such as age at CI and rates of CI, are consistently reported in the CI disparities literature while others such as access to rehabilitation services, willingness to undergo CI, and daily CI use are rarely measured. There are persistently reported disparities in a few key measures of CI access in a few populations, while there are some populations with a paucity of data. Future studies should delineate the nuances in the mechanisms of disparities by conducting multivariable analysis of representative sample data. Laryngoscope, 132:670-686, 2022., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

Importance: Earlier cochlear implantation among children with bilateral severe to profound sensorineural hearing loss is associated with improved language outcomes. More work is necessary to identify patients at risk for delayed cochlear implantation and understand targets for interventions to improve cochlear implantation rates among children., Objective: To describe the demographics among children receiving cochlear implantations and variability in implantation rates in California and to investigate sociodemographic and parental factors associated with early pediatric cochlear implantation., Design, Setting, and Participants: This retrospective cross-sectional study was conducted using data from the Healthcare Cost and Utilization Project California State Ambulatory Surgery Database in calendar year 2018. Included patients were children aged 9 years old or younger undergoing cochlear implantation. Sociodemographic factors, location of treatment, and parental factors were collected. Data were analyzed from March through August 2021., Main Outcomes and Measures: Binary logistic regression was performed to investigate sociodemographic factors associated with early cochlear implantation (ie, before age 2 years). Geographic variability in pediatric cochlear implantation across hospital referral regions in California was described, and various parental factors associated with implantation before age 2 years were analyzed., Results: Among 182 children receiving cochlear implantations, the median (IQR) age was 3 (1-5) years and 58 children (31.9%) received implantations at ages 2 years or younger. There were 90 girls (49.5%) and 92 boys (50.5%), and among 170 children with race and ethnicity data, there were 27 Asian or Pacific Islander children (15.9%), 63 Hispanic children (37.1%), and 55 White children (32.4%). The risk of CI was significantly decreased among Black children compared with Asian or Pacific Islander children (relative risk [RR], 0.18 [95% CI, 0.07-0.47]; P = .001) and White children (RR, 0.24 [95% CI, 0.10-0.59]; P = .002) and among Hispanic children compared with Asian or Pacific Islander children (RR, 0.32 [95% CI, 0.21-0.50]; P < .001) and White children (RR, 0.42 [95% CI, 0.29-0.59; P < .001). Compared with private insurance, Medicaid insurance was associated with decreased odds of implantation at ages 2 years or younger (odds ratio [OR], 0.19 [95% CI, 0.06-0.64]; P = .007), and every 1 percentage point increase in maternal high school completion percentage in a given California hospital referral region was correlated with a 5-percentage point increase in percentage of cochlear implants performed at age 2 years or younger (b = 5.18 [95% CI, 1.34-9.02]; P = .008). There were no significant differences in rates of early implantation by race or ethnicity., Conclusions and Relevance: This study found significant variability in pediatric cochlear implantation rates in California. These findings suggest that socioeconomic and parental factors may be associated with differences in access to early cochlear implantation and suggest the need to invest in initiatives to address barriers to appropriate and timely access to care.

Objective: To conduct a longitudinal national survey of cochlear implantation (CI) in Japan from 1985 through 2017., Methods: A retrospective analysis of adult and pediatric CI cases from 1985 to 2017 using the Oto-Rhino-Laryngological Society of Japan CI registration database was conducted. Surgical statistics were obtained, including number of surgeries, age, implanted side, facilities, and preoperative threshold levels for CI., Results: Since 1985, more than 11,100 CI surgeries have been conducted in Japan. The number exceeded 500 in 2005 and 1,000 in 2015. Since 2007, pediatric cases consistently surpassed adult cases until 2016. More facilities meet criteria for conducting CI as determined by Ministry of Health, Labor and Welfare and have now reached more than 100. Adult patients over 70 years old have become proportionally higher. Age at implantation steadily reduced in accordance with modifications of Japanese CI guidelines for children but is still not comparable to that in other developed countries. Although the number of CI surgeries for patients with moderate to severe hearing loss increased in the past ten years, the proportion was small., Conclusions: The CI database in Japan is quite unique in that it documents almost all the CI surgeries in Japan, allowing us to summarize the present situation as follows: fewer CI surgeries occur than are necessary, and children receiving implants are older than those in other developed countries. Continuous improvement of CI awareness is needed in Japan., (Copyright © 2020. Published by Elsevier B.V.)

BACKGROUND Indications for cochlear implantation (CI) are constantly being updated, and with them, the audiometric results achieved by patients. Patient satisfaction should always be considered, even in patients with lower audiological results. The aim of the present study was to compare quality of life (QoL), self-perceived hearing benefit, and audiometric results between prelingually and postlingually deafened patients, with and without sound deprivation, after CI. MATERIAL AND METHODS The sample included 46 patients with bilateral sensorineural hearing loss: 22 postlingually deafened and 24 prelingually deafened, further subdivided into sound-deprived (n=10) and non-sound-deprived (n=14). Auditory performance was evaluated with pure tone audiometry, speech recognition scores (SRS), and self-perceived hearing benefit, whereas QoL was evaluated with 2 self-reported questionnaires (Comprehensive Cochlear Implant Questionnaire and World Health Organization Quality of Life-BREF). RESULTS Audiometric results were worse in the prelingually deafened than in the postlingually deafened group, and worse in the prelingually deafened patients with sound deprivation. There was no marked difference in perceived CI benefit or QoL between the 2 groups or within the 2 prelingually deafened subgroups. No correlation was found between SRS and duration of CI use or between QoL and SRS in the prelingually and postlingually deafened groups. CONCLUSIONS Our findings demonstrate better auditory performance for the postlingually deafened group and no differences in perceived QoL or benefit of CI between the groups. The sound-deprived patients had equal scores on the perceived QoL questionnaire. These analyses suggest that sound-deprived, prelingually deafened patients may benefit from CI.

Objective: To assess whether listening with two cochlear implants (bilateral) offers significant benefits in terms of speech perception over listening with one cochlear implant and one hearing aid (bimodal)., Methods: Retrospective review of bilateral cochlear implant recipients (24 pediatric and 26 adult). Bimodal listening was compared to bilateral listening in terms of speech perception performance at 1-year post second implant under three listening conditions: 50 dBHL, 35 dBHL, and 50 dBHL+5 SNR. Changes in speech performance from bimodal (before second implant) to bilateral (after second implant) listening were determined within subjects and compared to a separate control group of bimodal users matched for age of first implantation who never received a second implant (10 pediatric and 20 adult)., Results: In the pediatric group, compared to bimodal listening prior to a second implant, speech perception scores with bilateral implants increased significantly when measured at 50 dBHL, 35 dBHL, and 50 dBHL+5 SNR. By contrast, pediatric bimodal controls who never received a second implant failed to demonstrate similar improvement over 1 year's time. In the adult group, compared to bimodal listening prior to a second implant, speech perception scores with bilateral implants increased when measured at 50 dBHL, but were not significantly different at 35 dBHL and 50 dBHL + 5 SNR. Adult bimodal controls who never received a second implant failed to demonstrate significant improvement in all conditions over 1 year's time., Conclusion: Bilateral listening with two cochlear implants improved speech perception performance relative to bimodal listening in the pediatric population. Improvement in the adult population was not as significant., Level of Evidence: 4, Retrospective Chart Review. Laryngoscope, 131:E1322-E1327, 2021., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Objective: To evaluate the association between race/ethnicity and insurance status on the access to early cochlear implantation., Study Design: Population-based retrospective analysis of pediatric cochlear implantation procedures., Setting: State Ambulatory Surgery and Services Databases of Florida from 2005 to 2017., Methods: All children aged 18 years or younger in the state of Florida undergoing cochlear implantation were identified. The outcome measures were access to early cochlear implantation (before 1 and 2 years of age). Descriptive and multivariate logistic regression analyses were conducted., Results: Among 1511 pediatric cochlear implantation procedures with complete data, 65 (4.3%) procedures were performed by 1 year of age and 348 (23.0%) by 2 years of age. Black children (odds ratio [OR], 0.44; 95% CI, 0.28-0.70), Hispanic children (OR, 0.70; 95% CI, 0.52-0.94), and children with Medicaid (OR, 0.64; 95% CI, 0.48-0.84) were significantly less likely to be implanted before 2 years of age. Even when insured by private insurance, black and Hispanic children were still less likely to be implanted before 2 years of age compared to white children with private insurance. Greater racial and insurance disparities existed in access to cochlear implantation before 1 year of age compared to implantation before 2 years of age., Conclusion: Racial/ethnic and insurance disparities in pediatric cochlear implantation can be observed at the population level. To address these racial and insurance inequalities, a multidisciplinary care team is needed and priorities should be given to research endeavors and policy interventions that target these disparities.

Objectives: To demonstrate the feasibility of continuing cochlear implantation during the coronavirus disease 2019 crisis and to report on trends of referrals via the neonatal hearing screening programme., Methods: A prospective case series was conducted on children who underwent cochlear implantation during the coronavirus disease 2019 crisis in the UK and a sample of referrals via the neonatal hearing screening programme. A step-by-step description of peri-operative management is included., Results: Regionally, between February and May 2020, 106 babies were referred via the neonatal hearing screening programme to paediatric audiology. Eleven children were operated on during the coronavirus disease 2019 study period. None of the 11 children developed coronavirus symptoms., Discussion: It is widely recognised that the demands of managing the current pandemic may compromise screening, clinical assessment and elective surgery. Time-sensitive issues such as cancer management have gained prominence, but a similar need exists for timely paediatric cochlear implantation., Conclusion: Implantation in the paediatric population during the coronavirus disease 2019 pandemic is feasible with careful planning.

Objectives: Cochlear implants have a 10-year manufacturer's warranty and may remain functional for more than 25 years after implantation. With the increasing number of users, the number of explantations is set to increase in the future. The main objective of the present study was to determine reasons for cochlear explantation in adults and the proportion carried out during the warranty period., Material and Methods: A single-centre retrospective multidisciplinary study of cochlear explantation in adults was performed over a 33-month period. Explantation reasons, interval since implantation and warranty application were analysed., Results: 54% of the 71 explantations were for medical reasons, 45% due to failure (35% "hard failure", 10% "soft failure") and 1% could not be classified; 69% were followed by reimplantation. Failure was confirmed before explantation in 66% of cases. Manufacturer warranties were applicable in 70% of explantations for breakdown, for a total of €335,500., Conclusions: This analysis provides decision support for reimplantation and allows better patient information. Follow-up is essential to optimise warranty application and to promote the proper implant use., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Introduction: While cochlear implants (CI) prior to 12 months of age have become common, the prevalence and impact of issues that either arise or were not evident prior to implantation is unknown. Methods: Retrospective chart review of children implanted under 12 months of age with minimum 3 years follow up. The children were divided into three groups: those with no identified additional disabilities, those with no known disabilities at time of implantation but diagnosed with additional disabilities following implantation, and those that had known anticipated additional disabilities at time of implantation. Results: 108 children under the age of 12 months were implanted at our Center between 2000 and 2013 with an average age of 9 months at time of implantation and n  = 93 met inclusion criteria. In 79.6% (74/93) of children, there were no additional issues detected. In 11.8% (11/93), additional issues were known at the time of implantation while in 8.6% (8/93) of the children were diagnosed with additional issues that were not evident prior to implantation. The auditory and linguistic benefits vary commensurate with the severity of their disabilities. Those with anticipated issues preoperatively did not perform as well. Conclusions: Children implanted below one year of age but diagnosed with additional disabilities following implantation obtained substantial though varying degrees of benefit. In none of these cases would knowledge of the disability have altered the decision to offer early CI. It is important to address these potential issues when counseling families about outcomes.

Introduction: Nowadays cochlear implantation (CI) is the treatment of choice in adults in case conventional hearing devices fail. Besides speech perception, an improvement in quality of life and in cognitive performance has been reported. Thereby, the study focused on the impact of age., Participants and Methods: Thirty middle-aged (MA) between 50 and 64 years and 41 older subjects (OA) aged 65 and older with bilateral severe hearing loss performed a comprehensive computer-based neurocognitive test battery (ALAcog) pre- and 12 months post-implantation. Besides, monosyllabic speech perception in quiet (Freiburg monosyllabic speech test), health-related quality of life (HR-QoL, Nijmegen Cochlear Implant Questionnaire) and depressive symptoms (GDS-15) have been assessed., Results: Both age groups significantly improved in all three categories after 12 months. No differences were evaluated between MA and OA regarding speech perception and HR-QoL pre- and post-operatively. In contrast, cognitive performance differed between the age groups: pre-operatively OA performed worse in most neurocognitive subdomains like working memory (p=0.04), inhibition (p=0.004), processing speed (p=0.003) and mental flexibility (p=0.01), post-operatively MA outperformed OA only in inhibition (p=0.01). Age only slightly influenced cognitive performance in MA, whereas in OA age per se tremendously impacted on working memory (p=0.04), inhibition (p=0.02), memory (p=0.04) and mental flexibility (p=0.01). Educational level also affected processing speed, mental flexibility (p=0.01) and working memory (p=0.01). This was more pronounced in OA. In both age groups, hearing status had a strong effect on attentional tasks (p=0.01). In MA, depressive symptoms were more influential on cognitive functioning and on HR-QoL than in OA. Improvement in quality of life (p=0.0002) and working memory (p=0.001) was greater for those with a higher pre-operative depression score., Conclusion: Speech perception and HR-QoL improved in hearing impaired, independently of age. Pre-operative differences in cognitive performance between OA and MA clearly attenuated 12 months after CI. Impact of comorbidities differed between age groups., Competing Interests: The Department of Otorhinolaryngology, Head and Neck Surgery at the Katholisches Klinikum in Bochum, Ruhr-University of Bochum, has received unrelated third-party funds from MED-EL. Christiane Völter, Jan Peter Thomas, Stefan Dazert have received travel expense support from MED-EL. The authors declare that the research was conducted in the absence of any commercial or financial relationships and report no other conflicts of interest in this work., (© 2020 Völter et al.)

This study aims to determine the frequency and causes of cochlear explants with re-implantation (ERI) after 5 years' follow up of the patients included in the French national EPIIC (étude post-inscription des implants cochléaires) registry tracking patients with cochlear implantation. This multicenter, descriptive prospective study was conducted on 5051 patients enrolled in the EPIIC database between January 2012 and December 2016. Ninety-five patients (1.9%) received a primary implant and an ERI during the study. Of these, four benefitted from two ERIs. The number of ERIs was significantly higher in the pediatric population than among adults. The explantation and reimplantation were performed simultaneously in 86% of cases. The reasons for explantation were: in 46.4% of cases linked to a malfunction of the implant, and in 39.3% of cases for medical or surgical reasons. The number of electrodes inserted was significantly higher after the ERI than after the first implantation. There was just one post-ERI infection for these 95 explanted and re-implanted patients. As well as explantation with reimplantation rarely being necessary, it generally presents no major surgical difficulty and in most cases it allows a better integration than in the first implantation., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objective: Evaluate in France the outcomes of cochlear implantation outside the selection criteria, off-label., Material and Methods: This is a prospective cohort study including adults and children having received a cochlear implant (CI) in an off-label indication, that is outside the criteria established by the "Haute Autorité de santé (HAS)" in 2012. The data was collected from the "EPIIC" registry on recipients who received CIs in France between 2011 and 2014. Speech audiometry was performed at 60dB preimplantation and after one year of CI use, as well as an evaluation of the scores of the quality of life with the APHAB questionnaire, the scores for CAP and the professional/academic status in pre- and post-implantation conditions. Major and minor complications at surgery have been recorded., Results: In total, 590 patients (447 adults and 143 children) with an off-label indication for CIs were included in this study from the EPIIC registry (11.7% of the whole cohort of EPIIC). For adults, the median percentage of comprehension using monosyllabic word lists was 41% in preimplantation condition versus 53% after one year of CI use (P<0.001) and 60% versus 71% in dissyllabic word lists (P<0.001). The CAP scores were 5 versus 6 in pre- and post-implantation conditions respectively (P<0.001) and the APHAB scores were statistically lower after implantation (P<0.001). In the children cohort, the median percentage of comprehension using monosyllabic word lists was 51% in preimplantation condition and 65% after CI (P<0.001), and 48% versus 82% (P<0.001) for dissyllabic word lists. The CAP scores were 5 versus 7 respectively in pre- and post-CI conditions (P<0.001). Thirty-two minor complications (5.4%) and 17 major complications (2.8%) were reported in our panel of off-label indication patients., Conclusion: These results suggest that a revision of the cochlear implantation candidacy criteria is necessary to allow more patients with severe or asymmetric hearing loss to benefit from a CI when there is an impact on quality of life despite the use of an optimal hearing aid., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

This study concerns the results of cochlear implantation in children and adults from French cochlear implantation centers, monitored at one, two and three years by the Cochlear Implant French Registry EPIIC. This multicenter study enrolled 2603 subjects (1667 adults and 936 children) implanted in one ear. The following parameters were studied: hearing overall performances, monosyllabic or dissyllabic word perception, speech intelligibility, self-assessment questionnaire of Cochlear Implant (CI) benefits (Abbreviated profile of Hearing aid Benefit); professional activity and schooling. This study confirms the ceiling effect in adults' performances after the 1 st year and the progressive growth in children's performances. It also shows that the contralateral hearing aid enhances performances compared to the CI alone condition, in all follow-up sessions. The French register of CIs is the only worldwide register of systematic follow-up on a period of three years and more of all adults and children implanted in a country., (Copyright © 2020. Published by Elsevier Masson SAS.)

Objective: Multi-centre study of the National French Registry (EPIIC) of patients with cochlear implants, focusing on infants who were operated-on under the age of 24 months between 2012 and 2016., Patients and Methods: A total of 615 profoundly deaf infants, who received cochlear implants (CIs) before their second birthday, were included in the registry by different CI centers. Epidemiological, surgical, speech therapy and school, follow-up data were included in the registry, 12, 24, 36 and 48 months thereafter. The following parameters were studied: type of implantation (uni- or bilateral), complications, cause of deafness, category of auditory perception (CAP), Open-set word recognition score (OSW), speech intelligibility rating, lexical comprehension with EVIP (Peabody), communication mode and type of schooling. Bilateral simultaneous CI (BiCI) and unilateral CI (UniCI) groups were compared., Results: There were 744 implantations. The explantation-reimplantation rate, within the four-year follow-up, was just 3.6%. Mean implantation age was 16.0 months, and similar in the two groups (BiCI/UniCI). A total of 51% of children had their first implant between 12 and 18 months, and 15% before 12 months. Implantation was unilateral in 52% of cases. Fifty-six percent of the bilateral procedures were sequential, with a mean delay of 16.8 months for the second implantation. The cause of deafness was unknown in 52% of cases. Of the 48% (297/615) of attributed cases, 32% had clear genetic causes. The remaining deafness was due to cytomegalovirus (CMV, 8%), inner-ear malformation (5%) and meningitis (3%). The main complications were from infections (47%) and internal device failure (25%). Four years post-operation, 84% of the UniCI and 75% of BiCl groups had a CAP≥5, and 83% of UniCl and 100% BiCI had OSW≥80%. Furthermore 74% of UniCI and 77% of BiCI communicated orally and 85% of UniCI and 90% of BiCI integrated into mainstream schooling., Conclusion: The French Registry of cochlear implants (EPIIC) is the only such national registry in the world. Our analysis illustrates the immediate benefits of, either single or double, cochlear implantation for language, perception skills and schooling., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objectives: To analyze the performance of cochlear implants in French patients aged 65 and over, implanted between 2012 and 2016, using data from the French national registry for cochlear implants (EPIIC)., Materials and Methods: The French national registry incorporates patient data from before implantation and for three years after implantation, stratified in different age groups (18-39, 40-64years, 65-74years and>75years). Here, we assessed the latter two categories. Hearing was assessed using mono- and disyllabic words in a silent background. The Category of Auditory Performance (CAP) scale was also implemented and subjects took the Abbreviated Profile of Hearing Aid Benefit (Aphab) questionnaire., Results: The population aged over 65 accounted for 38% (n=1193) of the 3178 adult implanted patients. The performance for mono- and disyllabic words in silence, the CAP scores and the APHAB questionnaire answers for ease of communication, background noise and reverberation were dramatically improved at one year post-implantation (P<0.0001 for each score) and remained stable between one and three years thereafter. The percentage improvement was similar across all age groups. The scores for loud-noise intolerance did not change after cochlear implantation in any age group., Conclusion: Cochlear implants improve hearing and communication in subjects aged 65 and over, with comparable efficiency to that achieved in younger subjects. Cochlear implantation should thus be proposed whenever hearing aids provide only limited benefit. However, between 2012 and 2016, cochlear implantation was given to less than 1% of the French population aged 65 and over with profound deafness., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objectives: Assessment of the incidence and results of bilateral cochlear implantation in adults and children in France., Materials and Methods: Multicenter retrospective study of data in the French national registry of cochlear implantations from January 1st 2012 to December 31st 2016. Functional results from CAP (Category of Auditory Performance) questionnaires and speech audiometry tests, with mono- and di-syllabic word-lists, were compared before and after implantation. Speech audiometry tests were carried out against a noisy background, except before simultaneous implantations., Results: Nine hundred and forty two bilateral cochlear implantations were performed during this period, that is, 16.4% of all cochlear implantations. Five hundred and eighty eight bilateral implantations were performed sequentially. 59% of the bilateral implantations were performed in children. Bilateral implants significantly improved CAP scores in all cases (P<0.001). Auditory performance, with the two types of word-list, were significantly improved after simultaneous implantation (P<0.01). After sequential implantation, the speech discrimination score, already very good with the first implant, reached 63±26% [0-100] with monosyllabic word lists, and 72±28% [0-100] with dissyllabic words. There were more complications due to surgery in bilateral cases than in the entire population of cochlear recipients (9.1% vs 6.4%, P<0.02)., Conclusion: Hearing is significantly improved by simultaneous cochlear implantation. For sequential implantation, at one year, when auditory results were already excellent from the first implant, in the bimodal condition CAP scores were significantly improved, although there was no further change in speech audiometry in noise., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Cochlear and brainstem implants have been included on the list of reimbursable products (LPPR) in France since March of 2009. The implants were initially inscribed for 5 years, after which an application for renewal with the French National Commission for the Evaluation of Medical Devices and Health Technologies (Commission Nationale d'évaluation des dispositifs médicaux et des technologies de santé - CNEDiMTS) was required [Haute Autorité de santé, 2009]. Upon registration to the list of reimbursable products, the companies and the reference centers for cochlear and brainstem implants were asked to set up a post-registration registry called EPIIC. This article reports the evolution in the EPIIC registry of the general indicators for 5051 patients over the five years from 2012-2016., (Copyright © 2020. Published by Elsevier Masson SAS.)

Objectives/hypothesis: To outline a possible decision-making process for sporadic vestibular schwannoma (VS) with contralateral nonserviceable hearing at diagnosis., Study Design: Retrospective case series., Methods: Diagnosed VS was studied in a tertiary referral center from 1995 to 2013., Results: Twenty-eight patients were included, with a mean follow-up of 6.9 years (range = 0.5-20 years). Ten were stage 1, 10 were stage 2, five were stage 3, and three were stage 4. Ipsilateral hearing levels were A (n = 3), B (n = 10), C (n = 7) and D (n = 8) American Academy of Otolaryngology-Head and Neck Surgery classification. Contralateral hearing levels were C (n = 11) and D (n = 17). Initial VS management included surveillance (n = 12) or surgery (n = 16), and four patients were later treated with surgery (n = 3) or stereotactic irradiation (n = 1). Hearing was improved by hearing aids and/or etiological treatment (n = 8), restored by contralateral (n = 15) or ipsilateral (n = 4) cochlear implants (CIs), or ipsilateral auditory brainstem implants (ABIs) (n = 3). Finally, 18 CIs were active daily; 14 of them presented high or moderate benefit with mean open-set dissyllabic word scores (WRS) of 58.1% and sentence recognition scores (SRS) of 69.7%, but only one ABI was still active (WRS of 70% and SRS of 87% with lip reading)., Conclusions: When early removal of VS was not necessary, contralateral CI or etiological treatment for hearing loss might be recommended initially. Ipsilateral CI is proposed, whereas VS should be operated on if previous hearing restoration was not successful. ABI should be reserved for the rare cases where a contralateral CI could not be implanted or the cochlear nerve was sectioned during VS removal., Level of Evidence: 3 Laryngoscope, 130:E407-E415, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: Programming the cochlear implant's speech processor with subjective methods in young, uncooperative children is difficult. Since young children cannot provide adequate feedback to the clinician, objective methods which do not require patient's response were often used. Electrical Stapedius Reflex Test is one of the most common procedures used. The goal of this study was to investigate the relationship between electricalstapedius reflex thresholds and behaviorally most comfortable levels in experienced cochlear implant users., Materials and Methods: The patients implanted with two brands of cochlear implants were compared in terms of electrical stapedius reflex thresholds versus most comfortable levels. Speech tests results were also compared between the two groups. 46 cochlear implanted patients who had at least 1 year of cochlear implant experience were included in the study. 28 patients were implanted with Nucleus, 18 patients with Med-El devices., Results: Moderate correlations were obtained between Electrical Stapedius Reflex (ESR) thresholds and C levels in Nucleus users; higher correlations were obtained for Med-El patients. ESR thresholds were present at higher levels than MCL/C levels in both Nucleus and Med-El users. No significant difference was obtained between the two groups in terms of speech tests., Conclusion: ESR test can be very informative for programming young and uncooperative patients.

Purpose The aims of this study were to (a) determine a metric for describing full-time use (FTU), (b) establish whether age at FTU in children with cochlear implants (CIs) predicts language at 3 years of age better than age at surgery, and (c) describe the extent of FTU and length of time it took to establish FTU in this population. Method This retrospective analysis examined receptive and expressive language outcomes at 3 years of age for 40 children with CIs. Multiple linear regression analyses were run with age at surgery and age at FTU as predictor variables. FTU definitions included 8 hr of device use and 80% of average waking hours for a typically developing child. Descriptive statistics were used to describe the establishment and degree of FTU. Results Although 8 hr of daily wear is typically considered FTU in the literature, the 80% hearing hours percentage metric accounts for more variability in outcomes. For both receptive and expressive language, age at FTU was found to be a better predictor of outcomes than age at surgery. It took an average of 17 months for children in this cohort to establish FTU, and only 52.5% reached this milestone by the time they were 3 years old. Conclusions Children with normal hearing can access spoken language whenever they are awake, and the amount of time young children are awake increases with age. A metric that incorporates the percentage of time that children with CIs have access to sound as compared to their same-aged peers with normal hearing accounts for more variability in outcomes than using an arbitrary number of hours. Although early FTU is not possible without surgery occurring at a young age, device placement does not guarantee use and does not predict language outcomes as well as age at FTU.

Purpose: To assess preoperative features that could predict the audiological outcome after cochlear implantation in the elderly, in terms of pure tone audiometry, speech audiometry, and speech perception performance., Methods: All available records of patients with cochlear implants aged 65 or more at the time of their implantation at our Institution were reviewed (50 patients, mean age 70.76 ± 4.03 years), recording preoperative clinical features. Pure tone audiometry, speech audiometry, and speech perception performance 1 year after cochlear implant activation and fitting were used as outcome measures., Results: No statistically significant association emerged between clinical features and pure tone audiometry. On univariate analysis, progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech audiometry and speech perception performance (p = 0.035 and p = 0.033, respectively). On multivariate analysis, progressive sensorineural hearing loss retained its independent prognostic significance in terms of speech perception performance (p = 0.042). The discriminatory power of a two-variable panel (age and etiology of hearing loss) featured an AUC (ROC) of 0.738 (an acceptable discriminatory power according to the Hosmer-Lemeshow scale)., Conclusions: A progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech perception in the elderly in our case study. Further features that can predict audiological outcome achievable with cochlear implants in the elderly are desirable to perform adequate counselling and rehabilitation programs.

Background: Congenital sensorineural hearing loss (SNHL) is a common disability in children. It can affect normal language development and educational achievement. Today, the time to cochlear implant is delayed for many children, which in turn delays intervention and impacts outcomes. Lack of knowledge and experience with congenital SNHL in the family are critical factors that can delay identification and intervention., Objectives: Compare treatment seeking behavior in families for a first and second congenitally deaf child., Design: Analytical, cross-sectional using medical record data., Setting: Ear specialist hospital in Riyadh., Subjects and Methods: All patients who presented to the cochlear implant committee from March 2016 to March 2018 and met criteria were included in the study. Data on when the subjects presented to hospital and were approved for cochlear implant were retrieved from the patient files and through phone calls to the family. The age of first suspicion, audiological testing, diagnosis, hearing aid fitting, and the decision for cochlear implant were compared between the first and second child in families with multiple children with congenital SNHL., Main Outcome Measures: The timing difference between the first and second deaf child in seeking treatment., Sample Size: 116 (58 pairs)., Results: The second child was suspected to have hearing loss 13.6 months earlier than the first child and presented to the cochlear implant committee for final decision 16.7 months earlier than his\her sibling. Differences in the mean ages at suspicion of hearing loss, presentation to the hospital for audiological evaluation, hearing aid fitting, diagnosis, and decision for cochlear implant by cochlear implant committee were statistically significant ( P<.001)., Conclusion: Experience and knowledge has a major effect on early identification. We need to implement educational programs for the public to increase awareness of how to recognize a deaf child and what steps to take., Limitations: Single-centered., Conflict of Interest: None.

Introduction: Although it is recommended to perform cochlear implantation in both ears at the same time for management of profound hearing loss in children, many centers prefer to perform sequential implantation. There are many reasons as to why a simultaneous bilateral implantation is not commonly accepted and performed. The major risk is the possibility of bilateral vestibular organ impairment. However, it is beyond doubt that children who received the first implant should be given a chance for binaural hearing and associated benefits. In the literature, there are no homogenous criteria for bilateral implantation, and it is hard to find uniform and convincing algorithms for second cochlear implantation. The aim of this study is an attempt to identify a safe way of qualifying for second cochlear implantation in children., Material and Methods: Forty children with one cochlear implant were qualified for the second implantation. During qualification, the following were taken into account: time of the first implantation, audiometry results, use of the hearing aid in the ear without an implant and benefit of the device, speech and hearing development, and vestibular organ function. R esults: Fifteen out of forty children (38%) were qualified for the second implantation. In 35% of children, the decision was delayed with possible second implantation in the future. Eleven children (27%) were disqualified from the second surgery., Discussion: During evaluation according to the protocol presented in our study, 38% of children with a single cochlear implant were qualified for the second implantation with a chance for an optimal development and effective use of the second cochlear implant. We are convinced that sequential implantation with a short interval between surgeries and with an examination of the vestibular organ, hearing and speech development as well as an assessment of potential benefits from the second implant (bimodal stimulation) before the second implantation is the safest and most beneficial solution for children with severe hearing loss.

The association between deafness and visual disorders is frequent. These disorders range from simple refractive disorder to severe disease that can lead to disability. Hence the interest of early screening. This study aims to highlight the importance of multidisciplinary management and of the need for ophthalmological examination in each deaf child. We conducted a prospective monocentric data collection from medical records of 200 children followed for hypoacousia from January 2014 to January 2015. Each child underwent complete ophthalmological examination, ENT examination and clinical examination. Data from 155 medical records were collected. Ocular involvement was found in 47 patients, reflecting a rate of 30.4%. Bilateral involvement was found in 45 patients. The main syndromic causes were: Usher syndrome (8 casess), Waardenbourg syndrome (5 cases), Alport syndrome (3 cases), Wolfram syndrome (2 cases), Goldenhar syndrome (3 cases), Cogan syndrome (3 cases), Franceschetti-Kleinsyndrome (1 case), Charge syndrome(1 case), otomandibular syndrome (1 case), Stickler syndrome(1 case), Alström syndrome (1 case), Refsum disease (1 case), Susac syndrome (1 case) and KID Syndrome (1 case). Screening for ocular involvement allowed to shorten the average length of cochlear implantation from 9 months to 3 months. There are numerous ocular and auditory involvements because of the embryological and cellular similarities of these two organs, including the retina and the inner ear. The diagnosis of these involvements is facilitated by the presence of facial dysmorphism; on the other hand, diagnosis is difficult when there are visual and auditory sensorineural involvements. Early diagnosis of ocular and auditory involvements allows for best psychomotor development and optimal social inclusion. Therefore multidisciplinary management is necessary to allow for the best psychomotor, orthophonic and visual rehabilitation., Competing Interests: Les auteurs ne déclarent aucun conflit d’intérêts., (© Belghmaidi Sarah et al.)

Background: In Sweden, an estimated prevalence of adult patients with severe-to-profound hearing loss is 0.2%, which corresponds to roughly 20,000. We know little about the use of cochlear implants (CIs) in this population and why not most of them are not offered CI. Objectives: To investigate the reasons for no rehabilitation with CI among this patient group. Materials and methods: Data were collected from 1076 patients in the Swedish Quality Register of Otorhinolaryngology. A baseline questionnaire and the reason for no CI, was evaluated. Results: Only 14.5% of the patients started a CI investigation, and 8.5% were rehabilitated with CI. Significantly more women (56.5%) than men received CI. The most common reasons for not receiving CI, were hearing reason (30.5%), indicating satisfaction with technical equipment, and unknown reason (25%). The oldest patient group (81-100 years old) had the highest risk for unknown reasons . Patients receiving extended audiological rehabilitation (53.5%) had a significantly lower risk for unknown reasons. Conclusions: It is worrying that the oldest patient group (81-100 years old) seemed to have fewer chances to start a CI investigation. An extended audiological rehabilitation increased the chances that professionals would discuss CI. Significance: This study shows that surprisingly few patients are offered CI despite their severe-to-profound hearing loss.

Purpose of Review: Over a short period, China has adopted cochlear implants and emerged as a burgeoning market. This represents a valuable case study for emerging countries in terms of planning, initiating, and growing cochlear implant programs., Recent Findings: Although many challenges such as funding, establishing infrastructure, and recipient community support have been addressed, many more remain. Consistent rapid escalation in numbers has been driven by push-and-pull factors. Federal, state, and private funding have all played a role., Summary: The review highlights the massive need for hearing rehabilitation that currently exists in China. The shortfall can only be addressed by a purposeful and coordinated approach involving government policy, The China Disabled Persons Federation, the industry partnering with hearing and medical professionals and the deaf community.

Objective: To propose a new classification of inner-ear anomalies that is more clinically oriented and surgically relevant: the SMS (Sawai Man Singh) classification of cochleovestibular malformations., Methods: A retrospective multicentric study was conducted of 436 cochlear implantations carried out in 3 Indian tertiary care institutes. Patients with anomalous anatomy were included and classified, as per the new SMS classification, into cochleovestibular malformation types I, II, III and IV, based on cochlear morphology, modiolus and lamina cribrosa., Results: There were 19, 23, 8 and 4 patients with cochleovestibular malformation types I, II, III and IV, respectively. Two-year post-operative Meaningful Auditory Integration Scale scores were statistically analysed., Conclusion: This new classification for inner-ear anomalies is a simpler, more practical, outcome-oriented classification that can be used to better plan the surgery. These merits make it a more uniform classification for recording results.

Cochlear implants (CIs) are a routine treatment for children identified with a qualifying hearing loss. The CI, however, must be accompanied by a long-term and intense auditory training regimen in order to possibly acquire spoken language with the device. This research investigates families' experiences when they opted for the CI and undertook the task of auditory training, but the child failed to achieve what might be clinically considered "success" - the ability to function solely using spoken language. Using a science and technology studies informed approach that places the CI within a complex sociotechnical system, this research shows the uncertain trajectory of the CI, as well as the contingency of the very notions of success and failure. To do so, data from in-depth interviews with a diverse sample of parents (n = 11) were collected. Results show the shifting definitions of failure and success within families, as well as suggest areas for further exploration regarding clinical practice and pediatric CIs. First, professionals' messaging often conveyed to parents a belief in the infallibility of the CI, this potentially caused "soft failure" to go undetected and unmitigated. Second, speech assessments used in clinical measurements of outcomes did not capture a holistic understanding of a child's identity and social integration, leaving out an important component for consideration of what a 'good outcome' is. Third, minority parents experience structural racism and clinical attitudes that may render "failure" more likely to be identified and expected in these children, an individualizing process that allows structural failures to go uncritiqued., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Purpose: Bilateral cochlear implant (CI) provision is now widely regarded as the most beneficial hearing intervention for acceptable candidates. This study sought to determine if a number of well-regarded hearing professionals at highly reputable clinics shared similar practices and beliefs regarding bilateral CI provision, use, and rehabilitation in children and adults., Methods: An 11-question online questionnaire was created and distributed to all 27 clinics in the HEARRING group. Questions 1-5 asked for facts; questions 6-11 asked for opinions., Results: 20 completed questionnaires were returned. All 20 respondents reported that their clinics perform bilateral cochlear implantation in children; 18 do so in adults. Regarding the fact-based questions, bilateral CI provision is more commonly performed and more likely to be reimbursed in children than in adults. Children are also much more likely to be implanted simultaneously than are adults. Regarding the opinion-based questions, respondents gave broadly similar answers. Communication between the CIs and speech coding strategies specifically developed for bilateral CI users were regarded as the two future technologies that would most enhance the benefit of bilateral CI use., Conclusions: Most clinics in the HEARRING group are very familiar with bilateral CI provision and hold similar opinions on its results and benefits. Hopefully the results described herein will lead to a greater acceptance and regular reimbursement of bilateral CI provision, especially in adults.

Objectives: Auditory neuropathy spectrum disorder (ANSD) is an audiological diagnosis characterised by hearing dysfunction in the presence of intact outer hair cell function in the cochlea. ANSD is thought to account for 7-10% of all childhood permanent hearing impairment, and can result from a range of pathological processes. This paper describes the rationale, methods and findings from the aetiological investigation of ANSD., Methods: Retrospective audit of four cochlear implant programmes., Results: 97 patients were identified. 79% of patients were identified before the age of one. Prematurity and jaundice were the most frequently identified aetiological factors. 33 patients had cochlear nerve deficiency on imaging. Genetic diagnoses identified included otoferlin, SX010 gene, connexin 26 and A1FM1 gene mutations. ANSD was seen in conjunction with syndromes including Kallman syndrome, CHARGE syndrome, X-linked deafness, SOTOS syndrome, Brown Vieletto Van Laere syndrome, and CAPOS syndrome., Discussion: We present a two-level system of aetiological investigation that is clinically practical. Patients with ANSD sufficiently severe to consider cochlear implantation are generally identified at an early age. Aetiological investigation is important to guide prognosis and identify comorbidity., Conclusion: Prematurity and jaundice are the most commonly identified aetiological factors in ANSD. Imaging findings identify crucial factors in a significant minority. An important minority may have genetic and syndromic diagnoses that require further management., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objective: Cochlear Implant (CI) users often suffer difficulties in perceiving speech in noisy environments that could be attributed to reduced Auditory Stream Segregation (ASS) ability. ASS is the process used to separate a complex sound into different perceptual streams. The evidence that CI listeners routinely experience stream segregation skill is limited and equivocal. The present study was aimed to investigate the effects of temporal cues on ASS performance in postlingually deaf listeners with CI., Methods: Nineteen (age range: 28-64 years old) monaurally cochlear implanted listener participated in this study. They were presented with 30-s sequences of alternating stimuli in a repeating A-B-A-A-B-A…sequence, where "tone A" corresponds to a stimulus applied to electrode 11, and "tone B" to a stimulus on one of the other electrode. To investigate the effect of temporal cues on ASS, four different tone repetition times (TRTs) were utilized: 50, 100, 150, and 200 ms. Speech discrimination scores in noise were also recorded for every CI recipients., Results: Only 6 (32%) CI users demonstrated ASS pattern similar to the normal hearing subjects, while the majority of the users (n=13) possessed poorer ASS skills. An analysis of variance showed a significant effect of electrode separation (p<0.001) and TRT (p=0.041), but there was no significant interaction between electrode separation and TRT variables. The best ASS performance was obtained when TRT was 200 ms, and there was no significant effect for other TRT conditions. Moderate, significant correlations between streaming and speech discrimination measurement in noise was also observed (r=0.62), with better stream segregation associated with better understanding of speech in noise., Conclusion: ASS is a contributing factor in the ability to perceive speech in background noise. The inability of some CI recipients to perform stream segregation may therefore contribute to their difficulties in noisy backgrounds. Furthermore, stream segregation ability is related to the tone repetition time between the sounds.