Holistic assessment of cochlear implant outcomes using the international classification of functioning disability and health model: data analysis of a longitudinal prospective multicenter study.

PURPOSE: To study outcome after cochlear implantation using the Cochlear Implant (CI) outcome assessment protocol based on the International Classification of Functioning, Disability and Health (ICF) model (CI-ICF). METHODS: Raw data of a prospective, longitudinal, multicenter study was analyzed. Seventy-two CI candidates were assessed preoperatively and six months postoperatively using the CI-ICF protocol. Following tools were used: (1) Work Rehabilitation Questionnaire (WORQ), (2) Abbreviated Profile of Hearing Aid Benefit (APHAB), (3) Audio Processor Satisfaction Questionnaire (APSQ), (4) Speech, Spatial, and Qualities of Hearing Scale (SSQ12), (5) Hearing Implant Sound Quality Index (HISQUI19), (6) Nijmegen CI Questionnaire (NCIQ) (7) pure tone audiometry, (8) speech audiometry, (9) sound localization. RESULTS: There was a significant improvement of speech discrimination in quiet (p = 0.015; p < 0.001) and in noise (p = 0.041; p < 0.001), sound detection (p < 0.001), tinnitus (p = 0.026), listening (p < 0.001), communicating with-receiving-spoken messages (p < 0.001), conversation (p < 0.001), family relationships (p < 0.001), community life (p = 0.019), NCIQ total score and all subdomain scores (p < 0.001). Subjective sound localization significantly improved (p < 0.001), while psychometric sound localization did not. There was no significant subjective deterioration of vestibular functioning and no substantial change in sound aversiveness. CI users reported a high level of implant satisfaction postoperatively. CONCLUSION: This study highlights the positive impact of cochlear implantation on auditory performance, communication, and subjective well-being. The CI-ICF protocol provides a holistic and comprehensive view of the evolution of CI outcomes.

Outcomes of Cochlear Implantation Using FLEX26 Electrode: Audiological Results and Quality of Life after 12 Months.

INTRODUCTION: The electrode length is one of the many factors impacted on results of cochlear implantation. Among lateral wall flexible electrode arrays the latest one is FLEX26 (MED-EL GmbH, Innsbruck, Austria). The main aim of the study was to evaluate the preservation of residual hearing, the level of speech understanding, and quality of life after cochlear implantation with FLEX26 electrode array. METHODS: The study was conducted in a tertiary referral centre. Fifty-two patients implanted unilaterally with FLEX26, including 10 EAS patients (electric acoustic stimulation) and 42 ES patients (electric stimulation). The intervention was minimally invasive cochlear implantation via the round window. Pure-tone audiometry (0.125-8 kHz) was performed preoperatively and at 1, 6, and 12 months postoperatively. Twelve-month hearing preservation was established using HEARRING group formula. Quality of life was measured with AQoL-8D (Assessment of Quality of Life-8 Dimensions) pre- and postoperatively. RESULTS: Residual hearing was preserved in 88.8% EAS patients. Quality of life was significantly better postoperatively in comparison to preoperative period (the effect size for overall quality of life was 0.49). Especially, it increased in relationships and senses dimensions (the effect sizes 0.47 and 0.44, respectively). CONCLUSION: Preservation of residual hearing can be achieved in the majority of patients implanted with FLEX26. Improvement of quality of life was also documented. FLEX26 seems to be an option for surgeons who seek an electrode providing sufficient cochlear coverage.

Can an individual with low frequency hearing in the candidate ear benefit from a cochlear implant even if they have normal hearing in the other ear?

PURPOSE: To determine hearing preservation and subjective benefit after cochlear implant (CI) surgery in patients with low frequency hearing in the ear to be implanted (i.e., they have partial deafness, PD) and close to normal hearing in the other. METHODS: There were two study groups. The test group was made up of 12 adult patients (mean age 43.4 years; SD 13.6) with normal hearing or mild hearing loss in one ear, and with PD in the ear to be implanted. The reference group consisted of 12 adult patients (mean age 44.5 years; SD 14.1) who had PD in both ears and who underwent unilateral implantation in their worse ear. Hearing preservation was assessed 1 and 14 months after CI surgery using the Skarzynski Hearing Preservation Classification System. The APHAB questionnaire was used to evaluate the benefit from the CI. RESULTS: The differences in HP% between the groups were not significant: mean hearing preservation (HP%) in the test group was 82% one month after CI surgery and 75% some 14 months after implantation; corresponding results in the reference group were 71% and 69%. However, on the APHAB background noise subscale, the benefit in the test group was significantly larger than in the reference group. CONCLUSION: To a large extent it was possible to preserve low-frequency hearing in the implanted ear. This means that individuals with low frequency hearing in the implanted ear (partial deafness) and with normal hearing in the other generally received more benefits from cochlear implantation than did patients with partial deafness in both ears. We conclude that residual low frequency hearing in the ear to be implanted should not be considered a contraindication for a CI in a patient with single-sided deafness.

New technology can benefit established middle ear implant users: Samba 2 vs previous models of audio processors for Vibrant Soundbridge.

INTRODUCTION: The Vibrant Soundbridge (VSB) is a semi-implantable hearing aid for patients with various types of hearing loss and has been available for over 25 years. Recently, new audio processors with advanced signal processing, noise reduction, and multi-microphone technology have appeared. The aim of this study is to compare the benefits of using the newest Samba 2 processor to the previous generation processors in a group of experienced VSB users. METHODS: There were 22 experienced VSB users (mean time of using VSB was 9 years, SD = 2) who had their processor (D404 or Amadé) upgraded to the newest model (Samba 2). The mean age of the subjects was 56 years (SD = 20). Assessments were made by free-field audiometry, speech reception in quiet and noise, and Patient-Reported Outcome Measures (PROMs). RESULTS: Hearing tests in free field showed statistically significant improvements in hearing sensitivity and speech discrimination in quiet and noise with the Samba 2 audio processor compared to the earlier technology. PROMs confirmed the benefits of using the newest audio processor and there was more satisfaction in terms of usability. CONCLUSIONS: Access to modern technology for VSB patients provides measurable benefits.

Towards a Consensus on an ICF-Based Classification System for Horizontal Sound-Source Localization.

The study aimed to develop a consensus classification system for the reporting of sound localization testing results, especially in the field of cochlear implantation. Against the background of an overview of the wide variations present in localization testing procedures and reporting metrics, a novel classification system was proposed to report localization errors according to the widely accepted International Classification of Functioning, Disability and Health (ICF) framework. The obtained HEARRING_LOC_ICF scale includes the ICF graded scale: 0 (no impairment), 1 (mild impairment), 2 (moderate impairment), 3 (severe impairment), and 4 (complete impairment). Improvement of comparability of localization results across institutes, localization testing setups, and listeners was demonstrated by applying the classification system retrospectively to data obtained from cohorts of normal-hearing and cochlear implant listeners at our institutes. The application of our classification system will help to facilitate multi-center studies, as well as allowing better meta-analyses of data, resulting in improved evidence-based practice in the field.

The Clinical Effect of Steroids for Hearing Preservation in Cochlear Implantation: Conclusions Based on Three Cochlear Implant Systems and Two Administration Regimes.

The main aim of this study was to assess the clinical effect of steroids (dexamethasone and prednisone) on hearing preservation in patients who underwent cochlear implantation with different cochlear implant systems (Oticon®, Advanced Bionics®, Med-El®). 147 adult patients met the inclusion criteria and were enrolled to the study and divided into three groups depending on the brand of cochlear implant they received and participated in all follow-up visits regularly. They were also randomly divided into three subgroups depending on the steroid administration regime: (1) intravenous dexamethasone (0.1 mg/kg body weight twice a day for three days); (2) combined intravenous and oral steroids (dexamethasone 0.1 mg/kg body weight twice a day plus prednisone 1 mg/kg weight once a day); and (3) no steroids (control group). The results were measured by pure tone audiometry (PTA) at three time points: (i) before implantation, (ii) at processor activation, and (iii) 12 months after activation. A hearing preservation (HP) figure was also calculated by comparing the preoperative results and the results after 12 months. Further measures collected were electrode impedance and hearing threshold in the non-operated ear. The highest HP measures (partial and complete) were obtained in the subgroups who were given steroids. Of the 102 patients given steroids, HP was partial or complete in 63 of them (62%). In comparison, partial or complete HP was achieved in only 15 patients out of 45 (33%) who were not given steroids. There were differences between the three cochlear implant groups, with the Med-El and Advanced Bionics groups performing better than the Oticon group (45% and 43% of the former two groups achieved partial or complete HP compared to 20% in the latter). Hearing thresholds in the non-operated ear were stable over 12 months. Generally, impedance was slightly lower in the 12 month follow-up in comparison with the activation period, with the exception of the Oticon group. (4) Conclusions: Pharmacological treatment with steroids in patients undergoing cochlear implantation helps to preserve residual hearing.

Systematic Literature Review of Hearing Preservation Rates in Cochlear Implantation Associated With Medium- and Longer-Length Flexible Lateral Wall Electrode Arrays.

Background: The last two decades have demonstrated that preoperative functional acoustic hearing (residual hearing) can be preserved during cochlear implant (CI) surgery. However, the relationship between the electrode array length and postoperative hearing preservation (HP) with lateral wall flexible electrode variants is still under debate. Aims/Objectives: This is a systematic literature review that aims to analyze the HP rates of patients with residual hearing for medium-length and longer-length lateral wall electrodes. Method: A systematic literature review methodology was applied following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) recommendations to evaluate the HP rates of medium-length and longer-length lateral wall electrodes from one CI manufacturer (medium length FLEX 24, longer length FLEX 28 and FLEX SOFT, MED-EL, Innsbruck, Austria). A search using search engine PubMed (https://www.ncbi.nlm.nih.gov/pubmed/) was performed using the search terms "hearing preservation" or "residual hearing" and "cochlear implant" in "All fields." Articles published only in English between January 01, 2009 and December 31, 2020 were included in the search. Results: The HP rate was similar between medium-length (93.4%-93.5%) and longer (92.1%-86.8%) electrodes at 4 months (p = 0.689) and 12 months (p = 0.219). In the medium-length electrode group, patients under the age of 45 years had better HP than patients above the age of 45 years. Conclusions: Both medium-length and longer electrode arrays showed high hearing preservation rates. Considering the hearing deterioration over time, implanting a longer electrode at primary surgery should be considered, thus preventing the need for future reimplantation.

Multi-Frequency Intraoperative Monitoring of Hearing Preservation during Cochlear Implantation.

(1) Background: Current indications for cochlear implants (CIs) have expanded to include patients with appreciable low-frequency hearing. However, longitudinal results indicate that only one-third of these recipients retain full hearing preservation. In another words, the remaining two-thirds lose this facility either partially or fully. This points to the need to better understand the impact of cochlear implantation on cochlear integrity. Intracochlear electrocochleography (ECochG) involves the recording of electrical potentials generated in the inner ear in response to acoustic stimuli, and previous studies have shown that these potentials give an indication of residual inner ear function. Aim of the research: The aim is to monitor intracochlear ECochG during CI surgery and gain a better understanding of how the implant impacted inner ear function. A newly developed SPL Chirp was used for stimulation. (2) Methods: Intracochlear ECochG signals were measured in a subject with residual preoperative low-frequency hearing, while an electrode array was introduced into the cochlea and was continued until the round window was sealed. Afterwards, surgical events were reviewed with the surgeon; preoperative and postoperative radiological data and hearing thresholds were also evaluated. (3) Conclusions: Real-time intraoperative monitoring, with multifrequency evaluation and video recording, has the potential to allow surgeons and audiologists to continuously assess cochlear function. ECochG monitoring may be a useful tool during cochlear implantation to gain frequency-specific information on the status of the patient's hearing, assisting surgeons to lower hearing trauma during the operation.

The Clinical Effect of Steroid Therapy on Preserving Residual Hearing after Cochlear Implantation with the Advanced Bionics HiRes Ultra 3D Cochlear Implant System.

(1) Background: The main aim of this study was to assess the clinical effectiveness of two different schemes of administration of steroids ((1) dexamethasone administered intravenously in comparison with (2) combination of steroid treatments: orally administered prednisone and intravenously administered dexamethasone) in comparison with a control group (no steroid administration) on hearing preservation (HP) in patients who underwent an Advanced Bionics cochlear implantation. (2) Methods: Thirty-five adult patients met the inclusion criteria. All patients were randomly divided into three subgroups depending on the scheme of steroid administration: (1) the first subgroup with only intravenously administered dexamethasone (0.1 mg per kg body weight twice a day for three days), (2) the second subgroup with a combination of methods of administration of steroids (intravenous and oral steroid therapy (dexamethasone, 0.1 mg/kg body weight twice a day plus prednisone, 1 mg/kg weight once a day for three days before surgery and after administration of dexamethasone (4th, 5th, 6th day) and after this time the dose of prednisone was reduced)) and (3) the third subgroup without steroid therapy (control group). The results were measured by pure tone audiometry (PTA) in three periods: (1) before implantation, (2) during activation of the processor (one month after implantation), and (3) 12 months after activation. Patients' hearing thresholds before implantation were on average 82 dB HL, 77 dB HL, and 88 dB HL, respectively. (3) Results: The majority of the patients from the first subgroup had hearing preserved partially (77.8%). A similar result was observed in the second study group (oral + i.v.) (partial hearing preservation was found in 61.5% of the participants). The opposite was true in the control group; a plurality of control patients (38.5%) had no measurable hearing 12 months after the activation of the processor. (4) Conclusions: Pharmacological treatment consisting of the administration of steroids in patients who had undergone cochlear implantation with the Advanced Bionics HiRes Ultra 3D cochlear implant system may be beneficial for preserving residual hearing in patients.

In Vivo Basilar Membrane Time Delays in Humans.

To date, objective measurements and psychophysical experiments have been used to measure frequency dependent basilar membrane (BM) delays in humans; however, in vivo measurements have not been made. This study aimed to measure BM delays by performing intracochlear electrocochleography in cochlear implant recipients. Sixteen subjects with various degrees of hearing abilities were selected. Postoperative Computer Tomography was performed to determine electrode locations. Electrical potentials in response to acoustic tone pips at 0.25, 0.5, 1, 2, and 4 kHz and clicks were recorded with electrodes at the frequency specific region. The electrode array was inserted up to the characteristic cochlear frequency region of 250 Hz for 6 subjects. Furthermore, the array was inserted in the region of 500 Hz for 15 subjects, and 1, 2, and 4 kHz were reached in all subjects. Intracochlear electrocochleography for each frequency-specific tone pip and clicks showed detectable responses in all subjects. The latencies differed among the cochlear location and the cochlear microphonic (CM) onset latency increased with decreasing frequency and were consistent with click derived band technique. Accordingly, BM delays in humans could be derived. The BM delays increased systematically along the cochlea from basal to apical end and were in accordance with Ruggero and Temchin, 2007.

Evaluating the Revised Work Rehabilitation Questionnaire in Cochlear Implant Users Cochlear Implant Outcome Assessment Based on the International Classification of Functioning, Disability, and Health (ICF).

OBJECTIVES: The 59-item Work Rehabilitation Questionnaire (WORQ) was developed based on the International Classification of Functioning, Disability and Health (ICF) core set for vocational rehabilitation to assess work related functioning. It was revised to include 17 questions, assigned to 14 ICF categories relevant to cochlear implant (CI) users. This cross-sectional multicenter study aimed to evaluate CI users' responses on the WORQ questions to describe and generate ICF qualifiers for the revised WORQ in CI users, forming part of a broader framework of CI outcome measures linked to the ICF. METHODS: One hundred seventy-seven adults over the age of 18 years with a minimum of one year's device experience were included in the analysis. The WORQ was completed by the participants at a routine visit to the clinic, via email, or via post. RESULTS: Most of the CI users perceived no problem on the WORQ questions (53.7%-91%), finished secondary school (54.2%) or obtained a college or university degree (32.8%) and are either employed (41.2%) or retired (34.5%). CI users that are currently working mostly have a full-time position (34.5%). Subjects reported no problem (91%) with sensation of falling, while handling communication devices and techniques (10.9%) and tinnitus (9.6%) showed the highest number of subjects reporting a complete problem. CONCLUSIONS: Overall, most of the CI users experienced no impairment, restriction or limitation on the WORQ questions and their assigned ICF categories. Their education level resembles the education level of the general population and they seem to integrate or reintegrate well in professional life postoperatively.

The COVID-19 pandemic and upgrades of CI speech processors for children: part II-hearing outcomes.

PURPOSE: To gauge the benefits to children of upgrading speech processors during the COVID-19 pandemic. METHODS: The study involved 297 children, aged from 7.3 to 18.0 years, whose processors were upgraded to either Nucleus 7 or Kanso 2, or to Sonnet 2 or Rondo 3. To document the benefits of the upgrades, a speech-in-noise discrimination test and Patient Reported Outcome Measures (PROMs) were used. RESULTS: There was a significant benefit from the newer processors in terms of speech discrimination in noise. Patient Reported Outcome Measures (PROMs) indicated less hearing disability, a higher level of functioning in everyday life situations, and more satisfaction with the new speech processor in social situations. CONCLUSION: There is a measurable improvement in performance when the devices are upgraded to the new technology.

The COVID-19 pandemic and upgrades of CI speech processors for children: part I-procedure of speech processor upgrade.

PURPOSE: To demonstrate the feasibility of upgrading speech processors during the COVID-19 pandemic. METHODS: Adopting concepts from "lean thinking", we optimized hospital workflows to allow speech processors to be upgraded despite the obstacles arising from national guidelines for COVID-19 prevention. The study involved 297 children, aged from 7.3 to 18.0 years, whose processors were upgraded on five consecutive Saturdays during a time of peak COVID-19 in Poland. RESULTS: The optimized workflow allowed us to conduct speech processor upgrades during a time of peak COVID-19 in Poland. The upgrades were conducted as scheduled, patient flow was smooth, appropriate social distancing was kept, and no reports of COVID-19 infection in our patients in the 2 weeks after their visit were received. CONCLUSION: Upgrading of speech processors in children is still feasible under coronavirus conditions.

Validation of AQoL-8D: a health-related quality of life questionnaire for adult patients referred for otolaryngology.

PURPOSE: The purpose of the study was to validate the AQoL-8D questionnaire in the adult population of patients referred to an otolaryngology clinic. METHODS: AQoL-8D was translated into Polish. 463 patients (age18-80 years) with otolaryngological conditions were assessed with the AQoL-8D, SF-6D, and SWLS questionnaires. We investigated the item content-relevance, factor structure by means of Confirmatory Factor Analysis, corrected item-total correlations, Cronbach's alpha, Pearson correlation of the AQoL-8D scores with results from SF-6D and from the SWLS questionnaires. Finally, ANOVA was used to test the AQoL-8D ability to group the HRQoL of patients in terms of their otolaryngological management type. RESULTS: The median score of item content-relevance was 5.0 for all AQoL-8D items. Confirmatory Factor Analysis revealed the following fit indices: Comparative Fit Index = 0.81; Tucker-Lewis Index = 0.80; and Root Mean Square Error of Approximation = 0.07. Cronbach's alpha for AQoL-8D dimensions ranged from 0.48 to 0.79. Mean item-total correlations over all dimensions, super dimensions, and the instrument overall were higher than 0.3. There was a significant Pearson correlation between the results obtained with AQoL-8D and SF-6D (r = 0.68), and with AQoL-8D and SWLS (r = 0.43). A one-way ANOVA showed a significant effect of management type on HRQoL as measured by AQoL-8D [F(4,458) = 6.12, p < 0.001] CONCLUSION: AQoL-8D provides valid and reliable measures of HRQoL in patients undergoing otolaryngological treatment. Because it is a generic questionnaire, it is possible to make general comparisons of otolaryngology outcomes with those from other subspecialties.

Resumption of Cochlear Implant Surgery under COVID-19 Pandemic Conditions.

(1) Background: The novel coronavirus COVID-19 has been recognized by the World Health Organization as a public health emergency of international concern and has caused cancellation of elective cochlear implantation in many countries. This article sets out our experience with resuming cochlear implant (CI) surgery under COVID-19 conditions over a period of 3 months. In addition, early results of hearing preservation (HP) after CI surgery are presented; (2) Methods: We adopted epidemic management policies and procedures according to the National Consultant for Infectious Diseases recommendations. During preoperative visits, all patients were tested for COVID-19 with a RT-PCR test. One month postoperatively, HP values in the Partial Deafness Treatment (PDT) group of patients was established using the HEARRING group formula; (3) Results: Between January and March 2021, we performed 312 CI procedures in adult and pediatric patients. Of these, none were subsequently re-admitted to hospital and found to be COVID-19 positive. Postoperative audiometric results showed that complete or partial HP was achieved in more than half the PDT patients; (4) Conclusion: Cochlear implantation during the coronavirus disease pandemic is essential and, with careful planning, is perfectly feasible.

Cost-Utility Analysis of Bilateral Cochlear Implantation in Adults With Severe to Profound Sensorineural Hearing Loss in Poland.

OBJECTIVE: The aim of the study was to develop a Markov model and apply it for the evaluation of three different treatment scenarios for adult patients with severe to profound bilateral sensorineural hearing loss. STUDY DESIGN: Prospective Observational Study. SETTINGS: Hospital. PATIENTS: A clinical group of 22 adult patients (59.1% men, 40.9% women) aged from 59.13 ±â€Š8.9 years were included in the study. The study comprised two arms: patients in group 1 received the second cochlear implant one to three months after the first implant; while patients in group 2 got the second cochlear implant approximately one year after the first implant. MAIN OUTCOME MEASURES: All participants were first asked to complete an AQoL-8D questionnaire. For the cost-effectiveness analyses, a Markov model analyzed as microsimulation was developed to compare the different treatment options. RESULTS: The analyses show that bilateral cochlear implantation strategies are cost-effective compared to the 'no treatment' alternative when having a 10-year model time horizon. When all three model scenarios are compared, the bilateral simultaneous cochlear implantation strategy (Scenario 3) compared to the 'no treatment' option is even more cost-effective than the Scenarios 1 and 2, compared with the 'no treatment' alternative. CONCLUSIONS: The model results summarize that bilateral (sequential and simultaneous) cochlear implantation that are represented in the model scenarios, are cost-effective strategies for Polish adult patients with bilateral severe to profound sensorineural hearing loss.

Benefits of Binaural Integration in Cochlear Implant Patients with Single-Sided Deafness and Residual Hearing in the Implanted Ear.

The purpose of the study is to gauge the benefits of binaural integration effects (redundancy and squelch) due to preserved low-frequency residual hearing in the implanted ear of cochlear implant users with single-sided deafness. There were 11 cochlear implant users (age 18-61 years old) who had preserved low-frequency hearing in the implanted ear; they had a normal hearing or mild hearing loss in the contralateral ear. Patients were tested with monosyllabic words, under different spatial locations of speech and noise and with the cochlear implant activated and deactivated, in two listening configurations-one in which low frequencies in the implanted ear were masked and another in which they were unmasked. We also investigated how cochlear implant benefit due to binaural integration depended on unaided sound localization ability. Patients benefited from the binaural integration effects of redundancy and squelch only in the unmasked condition. Pearson correlations between binaural integration effects and unaided sound localization error showed significance only for squelch (r = -0.67; p = 0.02). Hearing preservation after cochlear implantation has considerable benefits because the preserved low-frequency hearing in the implanted ear contributes to binaural integration, presumably through the preserved temporal fine structure.

Electrode estimation in the acoustic region of the human Cochlea.

Background: In this study, a method to estimate number of electrodes in the acoustic region of Electric Acoustic Stimulation (EAS) subjects was proposed. Aims/Objectives: To develop and validate an anatomy-based method for EAS subjects to estimate the number of electrodes within the acoustic region.Material and methods: The postoperative CTs of adults with various degree of hearing implanted with lateral wall electrodes with mean insertion depth of 23.9 mm (18.0-28.2 mm) and mean insertion angle of 505° (355-695°) were evaluated.Results: The difference between the estimated and measured angle varied between -18 and 25°, with a mean of 0.9°. For the insertion angle of 230° and higher, the maximum difference was 24°. Taking this uncertainty into account, all electrodes in the acoustic region were predicted correctly.Conclusions and significance: The method decides on non-overlapping acoustic and electric stimulation in terms of place in the cochlea. With the accuracy of 0.84 mm for the electrode arrays inserted for more than 230°, the method was sufficient to estimate the exact number of electrodes in the acoustic region of cochlear implantees. The benefit of this method may be in fitting of EAS subjects with some portion of the electrode array in the acoustic region.

Cochlear Implantation Outcome in Children with DFNB1 locus Pathogenic Variants.

Almost 60% of children with profound prelingual hearing loss (HL) have a genetic determinant of deafness, most frequently two DFNB1 locus (GJB2/GJB6 genes) recessive pathogenic variants. Only few studies combine HL etiology with cochlear implantation (CI) outcome. Patients with profound prelingual HL who received a cochlear implant before 24 months of age and had completed DFNB1 genetic testing were enrolled in the study (n = 196). LittlEARS questionnaire scores were used to assess auditory development. Our data show that children with DFNB1-related HL (n = 149) had good outcome from the CI (6.85, 22.24, and 28 scores at 0, 5, and 9 months post-CI, respectively). A better auditory development was achieved in patients who receive cochlear implants before 12 months of age. Children without residual hearing presented a higher rate of auditory development than children with responses in hearing aids over a wide frequency range prior to CI, but both groups reached a similar level of auditory development after 9 months post-CI. Our data shed light upon the benefits of CI in the homogenous group of patients with HL due to DFNB1 locus pathogenic variants and clearly demonstrate that very early CI is the most effective treatment method in this group of patients.