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 However, clinical practice is variable, with some providers using outdated criteria and others exceeding current labeled indications.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.

Perceptions Surrounding Cochlear Implants Among At-Risk and Qualifying Older Adults in the United States.

OBJECTIVE: Limited evidence to date has examined public perceptions of cochlear implants among adult hearing aid users with moderate to profound hearing loss. The current work was conceived with the chief objective of characterizing the perceptions surrounding cochlear implants among the prospective candidate pool. STUDY DESIGN: National cross-sectional survey study. SETTING: United States. PATIENTS: Adults between 50 and 80 years of age with self-reported moderate to moderately severe (n = 200) or moderately severe to profound (n = 200) hearing loss currently using hearing aids. RESULTS: The overall survey response rate was 12%. Median age at time of survey for the 400 respondents was 66 years (interquartile range, 60-71 yr) and included 215 (54%) men. In total, 26% did not think of hearing loss as a medical condition, and another 23% were unsure. Overall, 63% of respondents had heard of cochlear implants, but only 2% indicated they were very familiar with them. Despite 52% of respondents reporting "very positive" or "somewhat positive" feelings about cochlear implants, only 9% indicated they were "very likely" to get a cochlear implant in the future, including 7% of those with estimated moderately severe to profound hearing loss at time of survey. CONCLUSIONS: Even among people with presumed qualifying levels of hearing loss, there exists a widespread lack of familiarity with cochlear implantation as a viable treatment option. This limited awareness seems influenced by a generally poor appreciation for hearing loss as a chronic disease state that warrants treatment. However, among those familiar with cochlear implants, they are generally viewed favorably.

Cochlear Implant Awareness in the United States: A National Survey of 15,138 Adults.

Hearing loss is increasingly recognized as a chronic disease that warrants treatment. Depression, social isolation, loneliness, and poorer cognitive performance have all been linked to untreated and undertreated adult-onset hearing loss. A significant subset of the patient population with hearing loss is inadequately rehabilitated by hearing aids alone and may benefit from cochlear implantation. Yet, it is estimated that less than 10% of those who qualify have received implants to date. A national survey was conducted online in November and December 2021. Subjects were identified using Dynata panelists and river sampling. Enrollment occurred on a rolling basis. Upfront sample management techniques were used to control the distribution, balancing the respondent cohort to the 2018 U.S. Census on age, household income, sex, marital status, household size, race/ethnicity, and education. Among 15,138 adult respondents with a mean (SD) age of 51 (17) years (54% female), only 10% reported being very familiar with cochlear implants, and 31% of those with hearing difficulty reported that they have "never heard" of a cochlear implant. Females were statistically significantly more likely to report some degree of familiarity with cochlear implants than men (34 vs. 26%; p < 0.01). The greatest familiarity with cochlear implants was observed among those aged 35 to 44 years (18% reporting "very familiar"), whereas only 9% of those aged 65 to 74, 10% aged 75 to 84, and 8% ≥85 reported being very familiar ( p < 0.01). Those identifying as White/Caucasian were statistically significantly more likely to report familiarity with cochlear implants than those identifying as Black/African American and Hispanic/Latino/Spanish (33 vs. 56 vs. 50% responding that they had "never heard" of cochlear implants; p < 0.01). Among adults with hearing difficulty, nearly 80% report having never talked with a medical or hearing care professional about cochlear implants. Limited cochlear implant awareness likely influences its widespread underutilization across the United States. Sex, age, and race disparities compound these issues among men, the Medicare-aged population, and those identifying as Black/African American and Hispanic/Latino/Spanish.

(Even Off-Label) Cochlear Implantation in Single-Sided Deafness and Asymmetric Hearing Loss Results in Measurable Objective and Subjective Benefit.

OBJECTIVE: To quantify objective and subjective outcomes in cochlear implant (CI) recipients with asymmetric hearing loss, including single-sided deafness (SSD) whose candidacy was determined on an ear-specific basis when word recognition was 50% or less. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. BACKGROUND: The effectiveness of CI in cases of SSD and asymmetric hearing loss (AHL) has been described in terms of tinnitus suppression, improved speech recognition in quiet and noise, enhanced localization ability, and improved quality of life. However, CI is not yet routinely offered as a top option or standard of care for these individuals. Recent Food and Drug Administration (FDA) labeling limits aided word recognition in the ear to be implanted to only 5% in cases of AHL/SSD, which is significantly poorer than 40 to 50%, which is often referenced in cases of bilateral hearing loss. Anecdotal experience suggests that patients with much better preoperative word recognition than 5% can benefit from CI. METHODS: We conducted a retrospective chart review of all adult CI candidates, with one ear exhibiting consonant-nucleus-consonant (CNC) word recognition scores at least 50% and one ear not meeting CI candidacy (i.e., CNC word recognition >50%). Outcome variables of interest included word and sentence recognition and subjective handicap questionnaires (hearing, tinnitus, dizziness) and the Speech Spatial Qualities questionnaire. RESULTS: Statistically and clinically significant improvement in speech understanding (word, sentence, sentence in noise) was noted for both the SSD and AHL groups in the implanted ear. There were statistically and clinically significant subjective improvements noted for both groups on the Hearing Handicap Inventory, the Tinnitus Handicap Inventory, and the Speech Spatial Qualities questionnaire by 1 month after activation. There were no significant differences between the AHL and SSD groups on either objective or subjective measures of the implanted ear. Individual word understanding improved for the majority of recipients across both groups and is not dependent on meeting the FDA criteria of less than 5%. CONCLUSIONS: Cochlear implantation is a viable option with measurable objective and perceived benefits for recipients with preoperative aided CNC word scores exceeding current FDA labeling. There is no significant difference between the AHL and SSD groups, suggesting that candidacy and outcome expectations should be set based on the ear to be implanted alone, without regard for the ability of the better hearing ear.

Hearing Health Perceptions and Literacy Among Primary Healthcare Providers in the United States: A National Cross-Sectional Survey.

OBJECTIVE: To characterize current awareness, perceptions, and literacy surrounding hearing loss among United States primary healthcare professionals. STUDY DESIGN: National cross-sectional survey study. SETTING: United States. PARTICIPANTS: Four hundred six healthcare professionals. RESULTS: Survey respondents included 205 primary care physicians and 201 nurse practitioners or physician assistants. When compared with 10 other common health conditions, only 1% of respondents ranked hearing loss as a "most important" health condition to manage. Less than half of providers reported recommending hearing testing for their patients at least once per year, whereas evaluation of blood pressure, total cholesterol, body mass index, and blood glucose levels are recommended at least annually by more than 80% of providers. Although 95% of respondents indicated that it is somewhat important or very important for patients to know the standard definition for normal hearing, only 57% of surveyed providers know of a standard definition themselves, and only 28% reported familiarity with the concept of "20/20 hearing." Conversely, more than 80% of respondents know the "normal" metric for blood pressure, total cholesterol, body mass index, blood glucose, and vision. Most respondents realize that hearing is important to overall health and hearing loss can impact personal safety, lead to social isolation, and negatively impact quality of life. Fifty-four percent also acknowledged a link between hearing loss and depression, but a majority were not very aware of the relationship of hearing loss to risk of falling and dementia, reduced income and job opportunities, and type 2 diabetes. Importantly, only 40% of providers believe hearing loss is treatable, and only 17% believe it is preventable. CONCLUSION: Despite widespread literacy of what constitutes normal blood pressure, total cholesterol, body mass index, blood glucose, and vision metrics, healthcare providers exhibit a poor understanding of normal hearing levels. Few providers prioritize hearing health or regularly recommend for annual hearing evaluation. Most providers believe that options for people with hearing loss are limited, which may have important implications for prioritizing discussion of hearing loss with patients.

Awareness, Perceptions, and Literacy Surrounding Hearing Loss and Hearing Rehabilitation Among the Adult Population in the United States.

OBJECTIVE: To characterize current awareness, perceptions, and literacy surrounding hearing loss among the adult population in the United States. STUDY DESIGN: National cross-sectional survey study. SETTING: United States. PATIENTS: Adults between 50 and 80 years of age in the United States. RESULTS: Survey respondents included 1,250 adults between the ages of 50 and 80 years, including 500 who indicated at least moderate hearing loss and were using hearing aids and 750 who denied having hearing loss and were not using hearing aids.Only 9% of patients were able to correctly identify what constitutes a "normal" or "average" range for hearing. By comparison, a "normal" range of values for vision, blood pressure, and total cholesterol were identified correctly by 93%, 85%, and 52% of the 1,250 surveyed adults, respectively. When asked to rank the importance of addressing hearing loss within the context of 10 other common health conditions, hearing loss was ranked third to least important. When considering annual health maintenance, respondents indicated they were "very likely" to have an annual physical exam (72%), a cholesterol test (70%), an eye exam (66%), and bring their pet to a veterinarian (59%) over twice as frequently as having their hearing evaluated (27%).When evaluating awareness surrounding associations between hearing loss and other health and social issues, less than one-fourth indicated strong awareness about links between hearing loss and depression, employability and income, fall risk, dementia, and type 2 diabetes. While most patients acknowledge the potential impacts of hearing loss on safety, quality of life, and health, less than half believe that hearing loss is treatable and less than 20% believe that hearing loss is preventable. CONCLUSION: Despite widespread literacy of what constitutes normal vision, blood pressure, and total cholesterol levels, respondents exhibit substantially poorer understanding of "normal" hearing levels. Most adults believe that few treatment options exist for the management of hearing loss. Underlying the uniformly limited literacy surrounding hearing loss and its treatment options is the adult population's lack of appreciation for the long-term health sequelae of untreated hearing loss.

Pediatric Bilateral Sensorineural Hearing Loss: Minimum Test Battery and Referral Criteria for Cochlear Implant Candidacy Evaluation.

Among the various cochlear implant systems approved by the Food and Drug Administration, current labeling for pediatric usage encompasses (1) bilateral profound bilateral sensorineural hearing loss in children aged 9 to 24 months and bilateral severe to profound sensorineural hearing loss in children older than 2 years; (2) use of appropriately fitted hearing aids for 3 months (this can be waived if there is evidence of ossification); and (3) demonstration of limited progress with auditory, speech, and language development. Pediatric guidelines require children to have significantly worse speech understanding before qualifying for cochlear implantation. The early years of life have been shown to be critical for speech and language development, and auditory deprivation is especially detrimental during this crucial time.Level of evidence: 2.

Functional Assessment of Hearing Aid Benefit: Incorporating Verification and Aided Speech Recognition Testing into Routine Practice.

Potential cochlear implant (CI) candidates arrive to the clinic with a variety of hearing loss configurations, hearing aid history, and aided capabilities. CI candidacy is primarily determined based on aided speech recognition capability, which relies on benefit derived from use of hearing aids. Therefore, contemporary evaluation for CI candidacy should incorporate a battery of testing to determine abilities and limitations and must be predicated on appropriate verification of the hearing aid fitting. However, recent reports, including a retrospective chart review of patients presenting to Cleveland Clinic for CI evaluation, suggest that a significant subset of patients may be using inappropriately fit or programmed amplification. Thus, a combination of simulated real-ear measurements and aided speech recognition testing is essential for fully assessing the effect of amplification and ultimately determination of CI candidacy. Furthermore, waiting to incorporate these tools until CI candidacy is suspected may delay timely identification of problems or need to change technology. Utilization of evidence-based decision drivers ultimately leads clinicians to timely patient-specific interventions which may include surgical intervention or other amplification options. As audiology moves into a healthcare era in which payers consider the benefit of our services to overall health and well-being, demonstrating timely, optimal outcomes using thorough, multifactorial evaluation is essential.

Maximizing Cochlear Implant Outcomes with Short-Term Aural Rehabilitation.

BACKGROUND: Increasing numbers of adults are receiving cochlear implants (CIs) and many achieve high levels of speech perception and improved quality of life. However, a proportion of implant recipients still struggle due to limited speech recognition and/or greater communication demands in their daily lives. For these individuals a program of aural rehabilitation (AR) has the potential to improve outcomes. PURPOSE: The study investigated the effects of a short-term AR intervention on speech recognition, functional communication, and psychosocial outcomes in post lingually deafened adult CI users. RESEARCH DESIGN: The experimental design was a multisite clinical study with participants randomized to either an AR treatment or active control group. Each group completed 6 weekly 90-minute individual treatment sessions. Assessments were completed pretreatment, 1 week and 2 months post-treatment. STUDY SAMPLE: Twenty-five post lingually deafened adult CI recipients participated. AR group: mean age 66.2 (48-80); nine females, four males; months postactivation 7.7 (3-16); mean years severe to profound deafness 18.4 (2-40). Active control group: mean age 62.8 (47-85); eight females, four males; months postactivation 7.0 (3-13); mean years severe to profound deafness 18.8 (1-55). INTERVENTION: The AR protocol consisted of auditory training (words, sentences, speech tracking), and psychosocial counseling (informational and communication strategies). Active control group participants engaged in cognitive stimulation activities (e.g., crosswords, sudoku, etc.). DATA COLLECTION AND ANALYSIS: Repeated measures ANOVA or analysis of variance, MANOVA or multivariate analysis of variance, and planned contrasts were used to compare group performance on the following measures: CasperSent; Hearing Handicap Inventory; Nijmegen Cochlear Implant Questionnaire; Client Oriented Scale of Improvement; Glasgow Benefit Inventory. RESULTS: The AR group showed statistically significant improvements on speech recognition performance, psychosocial function, and communication goals with no significant improvement seen in the control group. The two groups were statistically equivalent on all outcome measures at preassessment. The robust improvements for the AR group were maintained at 2 months post-treatment. CONCLUSION: Results of this clinical study provide evidence that a short-term AR intervention protocol can maximize outcomes for adult post lingually deafened CI users. The impact of this brief multidimensional AR intervention to extend CI benefit is compelling, and may serve as a template for best practices with adult CI users.

Auditory phenotype of Smith-Lemli-Opitz syndrome.

Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive multiple congenital malformation and intellectual disability syndrome resulting from variants in DHCR7. Auditory characteristics of persons with SLOS have been described in limited case reports but have not been systematically evaluated. The objective of this study is to describe the auditory phenotype in SLOS. Age- and ability-appropriate hearing evaluations were conducted on 32 patients with SLOS. A subset of 21 had auditory brainstem response testing, from which an auditory neural phenotype is described. Peripheral or retrocochlear auditory dysfunction was observed in at least one ear of 65.6% (21) of the patients in our SLOS cohort. The audiometric phenotype was heterogeneous and included conductive, mixed, and sensorineural hearing loss. The most common presentation was a slight to mild conductive hearing loss, although profound sensorineural hearing loss was also observed. Abnormal auditory brainstem responses indicative of retrocochlear dysfunction were identified in 21.9% of the patients. Many were difficult to test behaviorally and required objective assessment methods to estimate hearing sensitivity. Individuals with SLOS are likely to have hearing loss that may impact communication, including speech and language development. Routine audiologic surveillance should be conducted to ensure prompt management of hearing loss.

Evolving Criteria for Adult and Pediatric Cochlear Implantation.

The indications for cochlear implantation have gradually expanded as advancements in technology have evolved, resulting in improved audiologic outcomes for both adult and children. There remains a significant underutilization of cochlear implant technology in the United States, and recognition of the potential benefits of cochlear implantation for non-traditional indications is critical for encouraging the evolution of candidacy criteria. Adult cochlear implantation candidacy has progressed from patients with bilateral profound sensorineural hearing loss (SNHL) to include patients with greater degrees of residual hearing, single-sided deafness and asymmetric hearing, and atypical etiologies of hearing loss (eg, vestibular schwannoma, Ménière's disease, and otosclerosis). Indications for pediatric cochlear implantation have similarly evolved from children with bilateral severe to profound SNHL to implanting children at a younger age, including those with residual hearing, asymmetric hearing loss, inner ear malformations, as well as cochlear nerve deficiency. In this editorial, the literature investigating cochlear implantation for nontraditional indications is reviewed with an aim to use the best available evidence to encourage the evolution of candidacy criteria.

Successful cochlear implantation in a case of Vogt-Koyanagi-Harada disease.

OBJECTIVE: To characterize the auditory phenotype of Vogt-Koyanagi-Harada disease (VKH) and highlight the potential for successful cochlear implantation (CI). PATIENTS: Single case report INTERVENTION(S): Therapeutic MAIN OUTCOME MEASURE(S): AzBio Sentences in quiet and noise, CNC Words, BKB-SIN RESULTS: VKH is a systemic autoimmune disorder characterized by granulomatous uveitis accompanied by neurologic and auditory-vestibular symptoms. Development is attributed to an autoimmune response against pigmented cells, with destruction of melanocytes by T lymphocytes directed against an unidentified antigen; accordingly, the disease typically affects races with darkly pigmented skin. VKH presents as blurring of vision and eye inflammation, severe headaches, and meningismis. Auditory symptoms include sensorineural hearing loss (SNHL), tinnitus, and imbalance. SNHL can persist despite immunosuppressant therapy.This report describes successful cochlear implantation in a 26-year-old female patient with VKH who presented with complaints of blurred vision and bilateral sudden SNHL. Oral steroids initially restored hearing, but benefit ceased upon discontinuation of oral steroids and was not restored following a series of intratympanic steroid injections. The patient underwent bilateral CI, which was fast-tracked because of the autoimmune nature of her hearing loss. After implantation, her speech understanding in both quiet and noise far exceeds her pre-implant scores. CONCLUSION: This unique case is the first to report successful CI in VKH. It is important for hearing health professionals to be aware of the signs and symptoms of this disease as patients may present initially with hearing loss, tinnitus, or imbalance, and may benefit from CI in cases of persistent, steroid-unresponsive, severe-to-profound SNHL.

Auditory profile in superficial siderosis of the central nervous system: a prospective study.

OBJECTIVE: To identify auditory pathology resulting from superficial siderosis of the central nervous system (SSCN), auditory site of lesion, and a clinical profile for differential diagnosis and development of recommendations. STUDY DESIGN: Prospective study. SETTING: Academic clinical center. PATIENTS: Ten participants with SSCN (the largest prospective evaluation of audiologic status reported to date). INTERVENTION(S): Demographics, clinical characteristics and history, audiometric evaluation, and Tinnitus Handicap Inventory (THI). MAIN OUTCOME MEASURE(S): Type and degree of hearing loss, relationship to clinical course of SSCN, and expected results based on age and sex. RESULTS: Sensorineural hearing loss (SNHL) is the most common symptom in SSCN (100%). Tinnitus (100%), imbalance (80%), and gait disorder (80%) were also frequently reported. Hearing loss is typically bilateral, asymmetric, progressive, sloping, and exceeds expected hearing loss related to age and sex. Hearing loss may be cochlear and/or retrocochlear in origin. Decreased word recognition is possible, and traditional amplification may offer limited benefit. CONCLUSION: We observed significant audiometric findings in all participants. SSCN variably and pathologically targets the auditory system without regard for duration of disorder. A long asymptomatic phase and lack of predictive relationship between duration and severity of hearing loss makes suspicion of SSCN based solely on audiometric battery difficult; however, asymmetric hearing loss exceeding expectations, particularly with history of head trauma or previous neurosurgical procedure, should raise a red flag and trigger further medical evaluation including MRI. Diagnosis of SSCN may alter expectations for audiologic prognosis and is a critical component for comprehensive management of SSCN patients.

Superficial siderosis of the central nervous system: phenotype and implications for audiology and otology.

OBJECTIVE: Superficial siderosis of the central nervous system (SSCN) results after chronic subarachnoid hemorrhage. Consequent demyelination, particularly of the cochleovestibular nerve and cerebellum, causes auditory-vestibular dysfunction. Predominant symptoms include progressive sensorineural hearing loss, imbalance, and ataxia. Despite characteristic auditory-vestibular involvement, SSCN is not well known among the hearing health community. STUDY DESIGN: Clinical records of 49 patients diagnosed with SSCN were reviewed. Analysis included review of demographic, audiometric, and vestibular data of the largest sample to date and comparison to 31 audiovestibular case reports in the literature. RESULTS: Hearing loss and disordered balance were reported by 92% and 67% of patients, respectively. Results suggest variable but substantial auditory-vestibular involvement related to SSCN. Hearing loss is typically progressive, sloping, and asymmetric and exceeds hearing loss expected based on age or sex. Decreased word recognition is possible and traditional amplification may fail to provide benefit. CONCLUSION: SSCN is a destructive disorder affecting the auditory-vestibular system. Although not a common diagnosis, SSCN may be more prevalent than clinicians realize. Site of lesion may be anywhere within the auditory-vestibular system from the inner ear to the cortex, although the cochleovestibular nerve and cerebellum are particularly vulnerable. The progressive retrocochlear nature of the disorder makes differential diagnosis difficult and development of effective treatment options challenging. It is essential that audiologists and otologists recognize this uncommon cause of sensorineural hearing loss and balance disorder and the implications for evaluation, treatment, and counseling.

Evidence for the expansion of adult cochlear implant candidacy.

OBJECTIVES: The primary objective of this study was to determine whether a revision and/or expansion of current audiologic cochlear implant candidacy criteria is warranted. DESIGN: The study design was a retrospective review of postoperative speech perception performance for 22 adult cochlear implant recipients who demonstrated preoperative Consonant Nucleus Consonant word recognition scores of 30% or higher in the best-aided condition. This criterion was chosen to exceed that specified by the North American clinical trial of the Nucleus Freedom cochlear implant system. RESULTS: The mean preoperative best-aided monosyllabic word score for the 22 patients was 41% correct. The degree of postoperative benefit for the best postoperative condition (electric only or bimodal) ranged from 10 to 68 percentage points with a mean benefit of 27 percentage points for the electric-only condition and 40 percentage points for the bimodal condition. Statistical analyses revealed highly significant differences between preoperative-aided, implant-only, and bimodal performance on Consonant Nucleus Consonant monosyllabic word recognition performance. That is, both postoperative scores--electric only and bimodal--were significantly different from one another and from the preoperative best-aided performance. CONCLUSIONS: The current results suggest that a large-scale reassessment of manufacturer and Medicare preoperative audiologic candidacy criteria for adults is warranted to allow more hearing-impaired individuals to take advantage of the benefits offered by cochlear implantation.

Negative effect of deep cochlear implant electrode insertion on speech perception.

This case study details the evaluation, explantation, and subsequent reimplantation of a cochlear implant (CI) recipient with an unusually deep electrode array insertion. Although the positive value of sufficiently deep insertion and the effect of insertion variability have been researched, there are few data available that illustrate the detrimental effects on speech recognition when deep insertion corrupts optimal use of the CI. This unique case report challenges the assumption that deeper insertion will result in improved speech understanding and demonstrates the importance of fully evaluating recipients' complaints and recognizing the impact of frequency-to-place mismatch.

Cochlear implant patients with superficial siderosis.

BACKGROUND: Considered a rare disorder, superficial siderosis of the central nervous system (SSCN) has become more frequently diagnosed in recent years. As it is characterized by progressive sensorineural hearing loss, patients' needs may surpass the capability of hearing aid technology. Despite the retrocochlear nature of the disorder, patients have undergone cochlear implantation (CI) with varying success. PURPOSE: To summarize the issues surrounding cochlear implant candidates with SSCN as well as highlight trends in performance postimplantation. RESEARCH DESIGN: Retrospective case reports of seven cochlear implant candidates detail the symptoms, typical audiologic presentation, and array of clinical issues for patients with this progressive and potentially fatal disease. RESULTS: Despite the retrocochlear component of a hearing loss caused by SSCN, cochlear implantation may be a viable option. CONCLUSIONS: It is essential that the CI audiologist not only be aware of the disorder but also be well versed in the resulting implications for the cochlear implant process. A more thorough case history, an expanded candidacy test battery, and knowledge of the typical presentation of SSCN are critical. The diagnosis of SSCN will impact expectations for success with the cochlear implant, and counseling should be adjusted accordingly.