American Cochlear Implant Alliance Task Force: Recommendations for Determining Cochlear Implant Candidacy in Adults.

The indications for cochlear implantation have expanded over time due to evidence demonstrating identification and implantation of appropriate cochlear implant (CI) candidates lead to significant improvements in speech recognition and quality of life (QoL). However, clinical practice is variable, with some providers using outdated criteria and others exceeding current labeled indications. As a results, only a fraction of those persons who could benefit from CI technology receive it. This document summarizes the current evidence for determining appropriate referrals for adults with bilateral hearing loss into CI centers for formal evaluation by stressing the importance of treating each ear individually and a "revised 60/60 rule". By mirroring contemporary clinical practice and available evidence, these recommendations will also provide a standardized testing protocol for CI candidates using a team-based approach that prioritizes individualized patient care. This manuscript was developed by the Adult Cochlear Implantation Candidacy Task Force of the American Cochlear Implant Alliance using review of the existing literature and clinical consensus. LEVEL OF EVIDENCE: N/A Laryngoscope, 134:S1-S14, 2024.

Catchment Profile of Large Cochlear Implant Centers in the United States.

OBJECTIVE: To characterize the catchment area and patient profile of large cochlear implant (CI) centers in the United States. STUDY DESIGN: Multi-institutional retrospective case series. SETTING: Tertiary referral CI centers. METHODS: Patients who underwent CI surgery at 7 participating CI centers between 2015 and 2020 were identified. Patients' residential zip codes were used to approximate travel distances and urban vs rural residential areas. RESULTS: Over the 6-year study period (2015-2020), 6313 unique CI surgical procedures occurred (4529 adult, 1784 pediatric). Between 2015 and 2019, CI procedures increased by 43%. Patients traveled a median 52 miles (interquartile range, 21-110) each way; patients treated at rural CI centers traveled greater distances vs those treated at urban centers (72 vs 46 miles, P < .001). Rural residents represented 61% of the patient population and traveled farther than urban residents (73 vs 24 miles, P < .001). Overall, 91% of patients lived within a 200-mile radius of the institution, while 71% lived within a 100-mile radius. In adults, multiple regression analysis redemonstrated an association between greater travel distances and (1) older age at the time of CI and (2) residential rural setting (both P < .001, r2 = 0.2). CONCLUSIONS: While large CI centers serve geographically dispersed populations, most patients reside within a 200-mile radius. Strategies to expand CI utilization may leverage remote programming, telemedicine, and strategic placement of new centers and satellite clinics to ameliorate travel burden.

Decoding Billing Practices in Cochlear Implant Programs.

OBJECTIVES: The purpose of this study was to examine current cochlear implant (CI) billing practices across CI audiologists in the United States, to determine if CI audiologists are following the National Correct Coding Initiative (NCCI) edits, and to assess the CI audiologist's exposure to billing education. DESIGN: A 48-question survey was electronically distributed to and completed by audiologists who bill for CI services. Demographic data including work setting, population served, years of experience, number of CI patients managed per week, and exposure to billing education were collected. Data were analyzed to identify codes and modifiers used to bill for commonly performed CI procedures such as unilateral and bilateral CI programming, preoperative and postoperative testing, and objective measures. RESULTS: Data were obtained from 96 audiologists. The majority (86.3%, n = 82) of respondents agreed or strongly agreed they understand billing and coding practices for cochlear implants and 94.7% (n = 89) rated themselves as somewhat to highly efficient when performing these practices. Only 16.8% (n = 16) of respondents reported receiving formal training for practice management, and half of the respondents (51.1%, n = 48) reported unfamiliarity with national billing guidelines. Those who received formal training reported higher billing efficiency. Wide variability was seen for various billing scenarios. Billing questions were presented, and answers were coded as correct or incorrect based on the NCCI edits. Respondents who reported higher agreement with understanding billing and who received formal training scored better on common billing questions related to the NCCI edits. CONCLUSIONS: Most CI audiologists rated themselves as efficient in billing; however, wide variance in billing practices was observed. Incorporating practice management and current billing education into daily practice and into audiology training programs is essential to clinic efficiency, practice management, and CI program viability. CI audiologists should be knowledgeable about appropriate billing practices to ensure long-term sustainability of programs.

Electrophysiology and genetic testing in the precision medicine of congenital deafness: A review.

BACKGROUND: Congenital hearing loss is remarkably heterogeneous, with over 130 deafness genes and thousands of variants, making for innumerable genotype/phenotype combinations. Understanding both the pathophysiology of hearing loss and molecular site of lesion along the auditory pathway permits for significantly individualized counseling. Electrophysiologic techniques such as electrocochleography (ECochG) and electrically-evoked compound action potentials (eCAP) are being studied to localize pathology and estimate residual cochlear vs. neural health. This review describes the expanding roles of genetic and electrophysiologic evaluation in the precision medicine of congenital hearing loss.The basics of genetic mutations in hearing loss and electrophysiologic testing (ECochG and eCAP) are reviewed, and how they complement each other in the diagnostics and prognostication of hearing outcomes. Used together, these measures improve the understanding of insults to the auditory system, allowing for individualized counseling for CI candidacy/outcomes or other habilitation strategies. CONCLUSION: Despite tremendous discovery in deafness genes, the effects of individual genes on neural function remain poorly understood. Bridging the understanding between molecular genotype and neural and functional phenotype is paramount to interpreting genetic results in clinical practice. The future hearing healthcare provider must consolidate an ever-increasing amount of genetic and phenotypic information in the precision medicine of hearing loss.

Multicenter US Clinical Trial With an Electric-Acoustic Stimulation (EAS) System in Adults: Final Outcomes.

OBJECTIVE: To demonstrate the safety and effectiveness of the MED-EL Electric-Acoustic Stimulation (EAS) System, for adults with residual low-frequency hearing and severe-to-profound hearing loss in the mid to high frequencies. STUDY DESIGN: Prospective, repeated measures. SETTING: Multicenter, hospital. PATIENTS: Seventy-three subjects implanted with PULSAR or SONATA cochlear implants with FLEX electrode arrays. INTERVENTION: Subjects were fit postoperatively with an audio processor, combining electric stimulation and acoustic amplification. MAIN OUTCOME MEASURES: Unaided thresholds were measured preoperatively and at 3, 6, and 12 months postactivation. Speech perception was assessed at these intervals using City University of New York sentences in noise and consonant-nucleus-consonant words in quiet. Subjective benefit was assessed at these intervals via the Abbreviated Profile of Hearing Aid Benefit and Hearing Device Satisfaction Scale questionnaires. RESULTS: Sixty-seven of 73 subjects (92%) completed outcome measures for all study intervals. Of those 67 subjects, 79% experienced less than a 30 dB HL low-frequency pure-tone average (250-1000 Hz) shift, and 97% were able to use the acoustic unit at 12 months postactivation. In the EAS condition, 94% of subjects performed similarly to or better than their preoperative performance on City University of New York sentences in noise at 12 months postactivation, with 85% demonstrating improvement. Ninety-seven percent of subjects performed similarly or better on consonant-nucleus-consonant words in quiet, with 84% demonstrating improvement. CONCLUSION: The MED-EL EAS System is a safe and effective treatment option for adults with normal hearing to moderate sensorineural hearing loss in the low frequencies and severe-to-profound sensorineural hearing loss in the high frequencies who do not benefit from traditional amplification.

Temporal and spectral contributions to musical instrument identification and discrimination among cochlear implant users.

OBJECTIVE: To investigate the contributions of envelope and fine-structure to the perception of timbre by cochlear implant (CI) users as compared to normal hearing (NH) listeners. METHODS: This was a prospective cohort comparison study. Normal hearing and cochlear implant patients were tested. Three experiments were performed in sound field using musical notes altered to affect the characteristic pitch of an instrument and the acoustic envelope. Experiment 1 assessed the ability to identify the instrument playing each note, while experiments 2 and 3 assessed the ability to discriminate the different stimuli. RESULTS: Normal hearing subjects performed better than CI subjects in all instrument identification tasks, reaching statistical significance for 4 of 5 stimulus conditions. Within the CI population, acoustic envelope modifications did not significantly affect instrument identification or discrimination. With envelope and pitch cues removed, fine structure discrimination performance was similar between normal hearing and CI users for the majority of conditions, but some specific instrument comparisons were significantly more challenging for CI users. CONCLUSIONS: Cochlear implant users perform significantly worse than normal hearing listeners on tasks of instrument identification. However, cochlear implant listeners can discriminate differences in envelope and some fine structure components of musical instrument sounds as well as normal hearing listeners. The results indicated that certain fine structure cues are important for cochlear implant users to make discrimination judgments, and therefore may affect interpretation toward associating with a specific instrument for identification.

Differences in Perception of Musical Stimuli among Acoustic, Electric, and Combined Modality Listeners.

BACKGROUND: Cochlear implants have shown vast improvements in speech understanding for those with severe to profound hearing loss; however, music perception remains a challenge for electric hearing. It is unclear whether the difficulties arise from limitations of sound processing, the nature of a damaged auditory system, or a combination of both. PURPOSE: To examine music perception performance with different acoustic and electric hearing configurations. RESEARCH DESIGN: Chord discrimination and timbre perception were tested in subjects representing four daily-use listening configurations: unilateral cochlear implant (CI), contralateral bimodal (CIHA), bilateral hearing aid (HAHA) and normal-hearing (NH) listeners. A same-different task was used for discrimination of two chords played on piano. Timbre perception was assessed using a 10-instrument forced-choice identification task. STUDY SAMPLE: Fourteen adults were included in each group, none of whom were professional musicians. DATA COLLECTION AND ANALYSIS: The number of correct responses was divided by the total number of presentations to calculate scores in percent correct. Data analyses were performed with Kruskal-Wallis one-way analysis of variance and linear regression. RESULTS: Chord discrimination showed a narrow range of performance across groups, with mean scores ranging between 72.5% (CI) and 88.9% (NH). Significant differences were seen between the NH and all hearing-impaired groups. Both the HAHA and CIHA groups performed significantly better than the CI groups, and no significant differences were observed between the HAHA and CIHA groups. Timbre perception was significantly poorer for the hearing-impaired groups (mean scores ranged from 50.3-73.9%) compared to NH (95.2%). Significantly better performance was observed in the HAHA group as compared to both groups with electric hearing (CI and CIHA). There was no significant difference in performance between the CIHA and CI groups. Timbre perception was a significantly more difficult task than chord discrimination for both the CI and CIHA groups, yet the easier task for the NH group. A significant difference between the two tasks was not seen in the HAHA group. CONCLUSIONS: Having impaired hearing decreases performance compared to NH across both chord discrimination and timbre perception tasks. For chord discrimination, having acoustic hearing improved performance compared to electric hearing only. Timbre perception distinguished those with acoustic hearing from those with electric hearing. Those with bilateral acoustic hearing, even if damaged, performed significantly better on this task than those requiring electrical stimulation, which may indicate that CI sound processing fails to capture and deliver the necessary acoustic cues for timbre perception. Further analysis of timbre characteristics in electric hearing may contribute to advancements in programming strategies to obtain optimal hearing outcomes.