American Cochlear Implant Alliance Task Force Guidelines for Determining Cochlear Implant Candidacy in Children

Ear & Hearing

Warner-Czyz, A. D., Roland, J. T., Jr., et al. (2022).

Ear & Hearing, 43(2), 268-282.

This guideline provides recommendations regarding the clinical protocols and candidacy recommendations for cochlear implantation in the United States. The target audiences of this guideline are professionals working with children with hearing loss and consumers.

American Cochlear Implant Alliance Task Force

Hearing screening should be performed for all infants prior to 1 month of chronological age, with a referral for comprehensive audiological and medical evaluation as needed by 2 to 3 months of chronological age.

Amplification fitting should be performed following confirmed diagnosis of hearing loss within 1 month of identification. Enrollment in appropriate therapeutic intervention should occur by 3 to 6 months chronological age.

Cochlear implant (CI) candidacy should consider audiometric criteria (e.g. speech recognition and functional assessment), insufficient benefit from appropriately fit amplification, and quality of life concerns. Assessment of functional outcomes through parent and clinician feedback and HL-specific questionnaires regarding the child’s auditory awareness, auditory responsiveness, and progress in acquiring language skills is essential. Pediatric patients should be referred for CI evaluation if they have appropriately selected word recognition scores of less than 50% correct, unaided pure-tone thresholds of greater than 70 dB HL, poor functional performance, limited progress in language or auditory development, or poor quality of life. "Nonaudiologic factors such as the child and family’s quality of life, availability of resources, history of family involvement, assurance of consistent device use during all waking hours, and realistic expectations afford a well-rounded perspective of a child’s prognosis for CI outcomes" (p 270).  "Implementation of and commitment to an aural (re) habilitation program is necessary and required" (p. 277).

"Continued monitoring of hearing status and outcomes from amplification is essential to provide the child the best (re)habilitation. This point is particularly cogent for genetic and acquired causes of HL, which can result in progressive losses that may require a shift in auditory technology" (p. 270). Input from the parents, caregivers, and professionals involved in the child's care should inform these decisions based upon a holistic approach. Additional auditory technology should be considered to allow the child to gain full access to auditory information.

The cochlear implant (CI) team should include at least seven different professionals, with more as needed to meet the unique needs of each individual child. Team members may include but are not limited to physicians, audiologists, speech-language pathologists, teachers of the deaf, early intervention specialists in children who are Deaf or Hard of Hearing, and counselors, psychologists, and social workers. The CI team should provide timely and well-coordinated care. Families and caregivers are essential members of the CI team.

Hearing health history should include onset of hearing loss (HL), duration of profound HL, duration and retention of hearing aid use, and etiology of HL. "Assessment of residual hearing in the ear under consideration and the contralateral ear (should hearing sensitivity differ between ears) affords information important for device and electrode selection as well as possible bimodal hearing solutions" (p 272). Diagnostic hearing assessments should include age-appropriate measures to characterize HL, objective electrophysiological measures using frequency-specific stimuli (e.g. tone burst auditory brainstem response or auditory steady state response), behavioral audiogram, tympanometry to rule out middle ear dysfunction, acoustic reflex testing, otoacoustic emissions, parental questionnaires regarding auditory skills, ear-specific unaided air and bone conduction threshold testing, and documentation of insurance authorization. Evidence suggests ear-specific consideration in cases of asymmetric HL. " When an individual is determined to be a CI candidate, consideration should also be given to the nonimplanted ear to allow for appropriate recommendations for hearing technology for optimization of overall hearing and use of compatible accessories" (p. 277).

Use of a uniform test battery for children with hearing loss can aid in the facilitation of continuity of care, assist in clinical decision-making, and allow clinicians and researchers to define benchmarks. The Pediatric Minimum Speech Test Battery (PMSTB) is one such battery and includes a “hierarchical organization of perceptual tasks ranging from prelexical instruments (e.g., parental questionnaires and speech discrimination testing) to lexically-based word and sentence tests administered in quiet and/or noise” based upon chronologic age and language age and provides guidance for when to move from one test to another. “Ideally, each testing session should yield a measure of word recognition, sentence recognition in quiet, and sentence recognition in noise. However, clinicians must be realistic in assessing a child’s ability to complete a variety of tasks within a single session and recognize the need to schedule a follow-up testing session” (p. 272).

Functional listening assessment should be completed by a professional (e.g. audiologist, speech-language pathologist, or early intervention specialist) with experience in treating children with hearing loss. Functional listening assessments may include the analysis of: suprasegmental features (e.g., duration, intensity, pitch), the child's ability to hear distinctive features necessary to identify vowels and consonants or to identify speech sounds, words, or sentences, and how the child's listening changes with noise and distance.

Verification of hearing aid fittings should be conducted via objective measures (e.g. real ear measurements or real ear-to-coupler difference measurements) and subjective measures (e.g. cost and benefit analysis and quality of life). For older children, this may include aided testing, aided speech awareness, and recognition testing in the best-aided condition and in the ear to be implanted. This testing should occur after optimization of hearing aid fitting and confirmation of full-time device use. For younger children, this may include parent/caregiver questionnaires. Children who do not make the expected progress with hearing aids to achieve auditory, speech, or language milestones, despite full-time use and participation in the intervention, should be referred for evaluation.

A determination of pediatric cochlear implant (CI) candidacy should include frequent speech and language pathology (SLP) assessments to determine if a child is meeting expected milestones. For children with single-sided deafness, progressive, steeply sloping, or later onset hearing loss (HL), “the CI team should consider signs of skill regression, the risk for future delays in speech and language, cognitive or listening fatigue, and the added time needed for processing auditory information through an impaired auditory system” (p. 274). SLP testing should be conducted by a professional with working with children with HL and CIs. If the goal is age-appropriate speech and spoken language, providers should use tests standardized on children without HL. Assessment batteries should include speech, language, and functional listening assessments. Speech assessments may include the analysis of suprasegmental features (duration, intensity, and pitch), articulation, fluency, resonance, and voice quality. Language assessments may include analysis of babbling milestones, receptive and expressive vocabulary, grammar, pragmatics, and figurative language skills.

The parents' and/or caregivers' goals regarding their child and goals regarding cochlear implantation for their child should drive much of the team's decision making and counseling. Professionals across the multidisciplinary team should provide education regarding realistic expectations regarding CI use, the benefit of appropriate amplification during all waking hours, the range of options available for cochlear implant devices (e.g. speech processors, manufacturers, accessories, and connectivity) and options for varying modalities of communication. When possible, parents and caregivers should be introduced to deaf mentors or families successfully using a variety of communication modalities. Parents should be informed that the child’s communication modality may shift over time due to progress or preference.

Nonverbal, psychological factors, and social factors should be considered and provide a framework for intervention. In cases of low socioeconomic status, maternal education, or caregiver support, additional support should be provided to minimize barriers. Each family’s home language and culture should be considered and respected (e.g., multilingualism, hearing and deaf community involvement). Use of a language other than English in the home is never a reason to exclude a child from CI candidacy. Families should be engaged with a professional trained in counseling or connecting families with appropriate public and private resources.

Speech, language, and listening treatments are essential pre- and post-implantation (re)habilitation components and should be provided by a professional with experience working with children with hearing loss and cochlear implants (CIs). The professional should provide services in a specific child's or family's preferred mode of communication. Speech and language services should be provided prior to implantation with appropriately fitted hearing devices to determine potential candidacy for CI. Therapy should continue while the child is awaiting implantation to ensure the development of auditory, listening, speech, and language skills. Caregivers should be involved in therapy sessions to assist in carryover and skill development.

For older children, professionals should discuss realistic expectations for educational environments, with assessment of the child's educational needs in their own environment, at home, and at school as well as available services in the child’s school district (e.g., auditory technology, sign language interpreter, teacher of the deaf). Assistance in obtaining necessary services should be provided, as needed.

Due to variable outcomes for children with hearing loss with comorbidities, professionals and parents may need to utilize non-traditional factors to define progress relative to speech, language, and hearing milestones. Discussion regarding the family’s reasons, expectations, and goals for their child’s future with a cochlear implant is critical. For children with auditory neuropathy spectrum disorder (ANSD) specifically, it can be difficult to determine adequacy of hearing aid and/or CI fitting. Child with ANSD should be evaluated as potential CI candidates based on their speech perception scores, parent questionnaires, and therapist reports even if audiometric threshold results do not meet typical candidacy guidelines. “While medical, demographic, and otologic history deserve consideration in determining candidacy, they are rarely absolute contraindications” (p. 277).

Cochlear implantation should not be a last resort. Earlier implantation leads to better outcomes in children.

"Residual hearing in both the implanted and nonimplanted ear can lead to better speech understanding in noise, music appreciation, and sound quality. The presence of residual hearing should not be a deterrent to implantation, but should influence surgical technique, electrode choice, and consideration of acoustic plus electric hearing in the implanted ear" (p. 277).

The "[Food and Drug Administration] and Medicaid guidelines do not necessarily align with best clinical practice. The CI team should use evidence-based practice to guide clinical decision-making and should not rely on payer policies to determine candidacy. Rather, consideration of coverage should be included in counseling to supplement clinical recommendations" (p. 277).

“There are no inappropriate referrals for a CI. If a patient does not meet candidacy criteria, the evaluation will provide an opportunity for counseling and a baseline for monitoring progression. Early intervention in the form of implantation or improved amplification is paramount, but missing an opportunity to improve a patient’s auditory access and quality of life can be extremely detrimental to their long-term hearing health” (p. 277).