National Medical Policy

Transcription

1 National Medical Policy Subject: Policy Number: Cochlear Implantation NMP298 Effective Date*: November 2006 Updated: May 2016 This National Medical Policy is subject to the terms in the IMPORTANT NOTICE at the end of this document For Medicaid Plans: Please refer to the appropriate State's Medicaid manual(s), publication(s), citations(s) and documented guidance for coverage criteria and benefit guidelines prior to applying Health Net Medical Policies The Centers for Medicare & Medicaid Services (CMS) For Medicare Advantage members please refer to the following for coverage guidelines first: Use Source Reference/Website Link X National Coverage Determination (NCD) Cochlear Implantation: National Coverage Manual Citation Local Coverage Determination (LCD) Article (Local) X Other Technology Assessment: Effectiveness of Cochlear Implants in Adults with Sensorineural Hearing Loss: Medicare Learning Matters Network Number: MM6914 Revised, Related Change Request (CR): April 30, Effective Date: January 1, 2010 Related CR Transmittal #: R695OTN Implementation Date: October 4, 2010 Available at: Education/Medicare-Learning-Network- MLN/MLNMattersArticles/Downloads/MM6914.pdf Cochlear Implants May 16 1

2 None Use Health Net Policy Instructions Medicare NCDs and National Coverage Manuals apply to ALL Medicare members in ALL regions. Medicare LCDs and Articles apply to members in specific regions. To access your specific region, select the link provided under Reference/Website and follow the search instructions. Enter the topic and your specific state to find the coverage determinations for your region If more than one source is checked, you need to access all sources as, on occasion, an LCD or article contains additional coverage information than contained in the NCD or National Coverage Manual. If there is no NCD, National Coverage Manual or region specific LCD/Article, follow the Health Net Hierarchy of Medical Resources for guidance. Current Policy Statement Health Net, Inc. considers uniaural or (monaural) or binaural (bilateral) cochlear implantation medically necessary in members 12 years of age or older, when all of the following is met: 1. Diagnosis of bilateral, pre-or-post-linguistic, sensorineural moderate-toprofound hearing loss* in individuals who demonstrate limited benefit from amplification; 2. Limited benefit from amplification is defined by test scores of less than or equal to 40% correct in the best-aided listening condition on tape- recorded tests of open-set sentence recognition; 3. Cognitive ability to use auditory clues and a willingness to undergo an extended program of rehabilitation; 4. Freedom from middle ear infection, an accessible cochlear lumen that is structurally suited to implantation, and freedom from lesions in the auditory nerve and acoustic areas of the central nervous system; 5. No contraindications to surgery; and 6. The device must be used in accordance with Food and Drug Administration (FDA)-approved labeling. *Note Moderate hearing loss is defined as a bilateral hearing threshold of decibels (db). Profound hearing loss is defined as hearing only sounds that are louder than 90 db at frequencies of 2 and 4 khz without acoustic hearing aids. Health Net Inc., considers uniaural or (monaural) or binaural (bilateral) cochlear implantation medically necessary in children 12 months of age or older with bilateral sensorineural hearing impairment who meet all of the following criteria: 1. The child has profound, bilateral sensorineural hearing loss with thresholds of 90.0 db or greater at 1000 Hz; and 2. The child derives limited benefit from appropriately fitted binaural hearing aids; and 3. For children without previous experience with hearing aids, a three to six month hearing aid trial has been attempted and failed. Cochlear Implants May 16 2

3 Not Medically Necessary Cochlear implantation* is considered not medically necessary in any of the following scenarios: 1. Deafness due to lesions of the acoustic nerve or central auditory pathways; 2. Otitis media or other active, unresolved ear problems; 3. Radiographic evidence of absent cochlear development; or 4. Inability or lack of willingness to participate in post-implantation aural rehabilitation Note*: Upgrades of an existing, functioning external system are considered not medically necessary. However, if the external system is malfunctioning, or the existing components is inadequate to the point of interfering with the individual s activities of daily living, which would include school and work, then replacement with either an updated external system or a behind the ear (BTE) model, would be considered medically necessary. Investigational Health Net Inc., considers cochlear hybrid implants (e.g. Nucleus Hybrid L24 Cochlear Implant System) investigational due to insufficient evidence in the peer review literature demonstrating the safety and efficacy of cochlear hybrid implants in the management of patients with severe hearing loss. To date, the studies have been small and lack long term results. In addition, published evidence has shown that there is a potential risk of low frequency hearing loss as a result of cochlear hybrid implant surgery. Additional larger, long term studies are needed. Codes Related To This Policy NOTE: The codes listed in this policy are for reference purposes only. Listing of a code in this policy does not imply that the service described by this code is a covered or non-covered health service. Coverage is determined by the benefit documents and medical necessity criteria. This list of codes may not be all inclusive. On October 1, 2015, the ICD-9 code sets used to report medical diagnoses and inpatient procedures have been replaced by ICD-10 code sets. ICD-9 Codes Neurofibromatosis Neurofibromatosis, type 2 (acoustic neurofribromatosis) Sensorineural hearing loss, unspecified Sensory hearing loss Neural hearing loss Central hearing loss Sensorineural hearing loss of combined types ICD-10 Codes H90.3 Sensorineural hearing loss, bilateral H90.5 Unspecified sensorineural hearing loss Q85.00 Neurofibromatosis, unspecified Q85.02 Neurofibromatosis, type 2 Cochlear Implants May 16 3

4 CPT Codes Cochlear device implantation, with or without mastoidectomy Evaluation of speech, language, voice, communication, auditory processing, and/or auditory processing (code deleted 12/2014) Diagnostic analysis of cochlear implant, patient under 7 years of age; with programming Diagnostic analysis of cochlear implant, patient under 7 years of age; subsequent programming (Do not report in addition to 92601) Diagnostic analysis of cochlear implant, age 7 years or older; with programming HCPCS Codes L8614 Cochlear device, includes all internal and external components L8615 Headset/headpiece for use with cochlear implant device, replacement L8616 Microphone for use with cochlear implant device, replacement L8617 Transmitting coil for use with cochlear implant device, replacement L8618 Transmitter cable for use with cochlear implant device, replacement L8619 Cochlear implant, external speech processor and controller, integrated system, replacement L8623 Lithium Ion Battery For Use With Cochlear Implant Device speech Processor, other than ear level replacement L8624 Lithium Ion Battery For Use With Cochlear Implant Device speech- Processor, ear level replacement, each L8627 Cochlear implant, external speech processor, component, replacement L8628 Cochlear implant, external controller component, replacement L8629 Transmitting coil and cable, integrated, for use with cochlear implant device, replacement Scientific Rationale Update May 2016 Gantz et al (2016) described the final outcomes of a multicenter longitudinal, single-subject design study of the Nucleus Hybrid S8 CI that took place between 2002 and Eighty-seven subjects received a Nucleus Hybrid S8 CI in their poorer ear. Speech perception in quiet (Consonant-Nucleus-Consonant [CNC] words) and in noise (Bamford-Kowal-Bench Sentences-In-Noise [BKB-SIN]) were collected pre- and postoperatively at 3, 6, and 12 months. Subjective questionnaire data using the Abbreviated Profile for Hearing Aid Benefit (APHAB) were also collected. Some level of hearing preservation was accomplished in 98% subjects, with 90% maintaining a functional low-frequency pure-tone average (LFPTA) at initial activation. By 12 months, five subjects had total hearing loss, and 80% of subjects maintained functional hearing. CNC words demonstrated that 82.5% and 87.5% of subjects had significant improvements in the hybrid and combined conditions, respectively. The majority had improvements with BKB-SIN. Results also indicated that as long as subjects maintained at least a severe LFPTA, there was significant improvement in speech understanding. Furthermore, all subjects reported positive improvements in hearing in three of the four subscales of the APHAB. The authors concluded the concept of hybrid speech processing has significant advantages for subjects with residual low-frequency hearing. In this study, the Nucleus Hybrid S8 provided improved word understanding in quiet and noise. Additionally, there appears to be stability of the residual hearing after initial activation of the device. Cochlear Implants May 16 4

5 Roland et al (2016) sought to evaluate the safety and efficacy of acoustic and electric sound processing for individuals with significant residual low-frequency hearing and severe-to-profound high-frequency sensorineural hearing loss in a prospective, single-arm repeated measures, single-subject design. Fifty individuals, 18 years old, with low-frequency hearing and severe high-frequency loss were implanted with the Cochlear Nucleus Hybrid L24 implant at 10 investigational sites. Preoperatively, subjects demonstrated consonant-nucleusconsonant word scores of 10% through 60% in the ear to be implanted. Subjects were assessed prospectively, preoperatively, and postoperatively on coprimary endpoints of consonant-nucleus-consonant words, AzBio sentences in noise, and self-assessment measures. Significant mean improvements were observed for coprimary endpoints: consonant-nucleus-consonant words (35.8 percentage points) and AzBio sentences in noise (32.0 percentage points), both at P< Ninety-six percent of subjects performed equal or better on speech in quiet and 90% in noise. Eighty-two percent of subjects showed improved performance on speech in quiet and 74% in noise. Self-assessments were positive, corroborating speech perception results. The authors concluded the Nucleus Hybrid System provides significant improvements in speech intelligibility in quiet and noise for individuals with severe high-frequency loss and some low-frequency hearing. This device expands indications to hearing-impaired individuals who perform poorly with amplification due to bilateral high-frequency hearing loss and who previously were not implant candidates. Erixon and Rask-Andersen (2015) sought to measure patient satisfaction and correlate to hearing results in partially deaf patients, after hearing preservation cochlear implant surgery with hybrid hearing strategy, and to evaluate the stability of residual low-frequency hearing (LFH) over time. A patient satisfaction survey and a retrospective, 2-year follow-up journal study were performed. Nineteen partially deaf patients intended for hybrid hearing responded to a questionnaire when they had used their cochlear implants for at least a year. The questionnaire consisted of the International Outcome Inventory for Hearing Aids, EuroQol Group visual analogue scale and nine questions about hybrid hearing. Pure-tone audiometry, monosyllables, and hearing in noise test results from the patients' medical records were evaluated and compared with the results from the patient satisfaction survey. All of the patients were satisfied with their cochlear implants (CIs). The mean International Outcome Inventory for Hearing Aids score was 29. The CIs provided a major contribution to the speech comprehension of these partially deaf patients. Two years after surgery, the patients' mean binaural score on tests of monosyllables was 58%, and the mean signal to noise ratio was 4.6 db. We observed ongoing deteriorations in the residual hearing of the operated ears that surpassed the deteriorations observed in the contralateral ears. One month after surgery, the LFH loss ( Hz) was 17 db, and after 2 years, this loss was 24 db compared with 5 db in the nonoperated ear. There were no significant correlations between preserved LFH and patient satisfaction or speech perception results. The authors concluded the electric stimulation provided a major contribution to speech comprehension of partially deaf patients. The gain reached in speech understanding widely exceeded the downside in losing some residual hearing. All the patients showed a high degree of satisfaction with their CIs regardless of varying hearing preservation. Several clinical trials were identified at clinicaltrials.gov website. The Nucleus Hybrid L24 Extended Duration Post Approval Study (HED) is enrolling participants by invitation only (ClinicalTrials.gov Identifier:NCT ) and seeks to Cochlear Implants May 16 5

6 evaluate the long term safety and efficacy of the Nucleus Hybrid L24 Implant System in a group of already implanted recipients. Another study that is enrolling participants by invitation only (ClinicalTrials.gov Identifier: NCT ), is the Hybrid Cochlear Implants in Severe to Profound Adults, Children, and Adolescents Study. The purpose of this study is to determine if adults and children with residual low-pitch hearing in the severe hearing loss range can develop improved speech perception by combining their residual acoustic hearing with electrical stimulation through a short cochlear implant. The low-pitches would be amplified with a hearing aid and the high-pitch sounds would be stimulated electrically. In addition, a study currently enrolling participants, The Hybrid L24 New Enrollment Post Approval Study (HNE), ClinicalTrials.gov Identifier: NCT , will evaluate the long term safety and effectiveness of the Nucleus Hybrid L24 Implant in a group of newly implanted adults. Scientific Rationale Update May 2015 Sensorineural hearing loss is the most common form of hearing loss and occurs when there is damage to the inner ear (cochlea). It may be caused by aging, heredity, exposure to loud noise, drugs that are toxic to the inner ear (e.g., antibiotics), and certain other illnesses. People with severe or profound sensorineural hearing loss of high-frequency sounds may have difficulty hearing faint sounds, understanding people with higher-pitched voices, hearing certain speech sounds, and, in some cases, hearing high-pitched emergency vehicle sirens or common safety alarms, such as smoke detectors. The most common treatment of severe to profound bilateral high-frequency sensorineural hearing loss with residual low-frequency hearing is the use of conventional air conduction hearing aids or, in some cases, frequency transposition hearing aids. The Nucleus Hybrid L24 Cochlear Implant System combines the functions of a cochlear implant and a hearing aid. According to the U.S.FDA approval (March 2014), the Nucleus Hybrid L24 Cochlear Implant System is is an electric-acoustic stimulation (EAS) cochlear implant system. The Hybrid L24 provides electric (cochlear implant) stimulation to the mid- to highfrequency region of the cochlea and for patients with sufficient levels of residual low-frequency hearing sensitivity postoperatively, also provides acoustic (hearing aid) amplification in low-frequency regions. It consists of both internal and external components. The electronic device consists of an external microphone and speech processor that picks up sounds from the environment and converts them into electrical impulses. The impulses are transmitted to the cochlea through a small bundle of implanted electrodes, creating a sense of sound that the user learns to associate with the mid- and high-frequency sounds they remember. The hearing aid portion of the device is inserted into the outer ear canal like a conventional hearing aid, and can amplify sounds in the lowfrequency range. The system is indicated for unilateral use in patients aged 18 years and older who have residual low-frequency hearing sensitivity and severe to profound highfrequency sensorineural hearing loss, and who obtain limited benefit from appropriately fit bilateral hearing aids. Typical preoperative hearing of candidates ranges from normal to moderate hearing loss in the low frequencies (thresholds no poorer than 60 db HL up to and including 500 Hz), with severe to profound mid- to high-frequency hearing loss (threshold average of 2000, 3000, and 4000 Hz 75 db HL) in the ear to be implanted, and moderately severe to profound Cochlear Implants May 16 6

7 mid to high-frequency hearing loss (threshold average of 2000, 3000, and 4000 Hz 60 db HL) in the contralateral ear. The Consonant-Nucleus-Consonant (CNC) word recognition score will be between 10% and 60%, inclusively, in the ear to be implanted in the preoperative aided condition and in the contralateral ear will be equal to or better than that of the ear to be implanted but not more than 80% correct. Prospective candidates should go through a suitable hearing aid trial, unless already appropriately fit with hearing aids. The device is contraindicated for individuals who have the following conditions: 1. Deafness due to lesions of the acoustic nerve or central auditory pathway 2. Active middle ear disease, with or without tympanic membrane perforation 3. Absence of cochlear development 4. A duration of severe to profound hearing loss of 30 years or greater The FDA approval was based on a clinical study involving 50 individuals with severe to profound high-frequency hearing loss who still had significant levels of low-frequency hearing. The individuals were tested before and after being implanted with the device. A majority of the patients reported statistically significant improvements in word and sentence recognition at six months after activation of the device compared to their baseline pre-implant performance using a conventional hearing aid. The device also underwent non-clinical testing, which included the electrical components, biocompatibility and durability of the device. Of the 50 individuals participating in the study, 68 percent experienced one or more anticipated adverse events, such as low-frequency hearing loss, tinnitus (ringing in the ear), electrode malfunction and dizziness. Twenty-two developed profound or total low-frequency hearing loss in the implanted ear, six of whom underwent an additional surgery to replace the Nucleus Hybrid L24 Cochlear Implant System with a standard cochlear implant. The FDA determined that while the risk of low-frequency hearing loss is of concern, the overall benefits of the device outweigh this risk for those who do not benefit from traditional hearing aids. They recommend that prospective patients should carefully discuss all benefits and risks of this new device with their physicians. The device is intended for use on one ear only. Jurawitz et al (2014) reports that it has been possible to preserve hearing after cochlear implantation in patients with significant amounts of low-frequency residual hearing. Due to the dimensions and characteristics of the cochlear implants (CIs) Nucleus Hybrid-L24 and Nucleus Freedom CI422, both can be used to preserve residual hearing. (The Freedom Hybrid Sound Processor was used in the pivotal IDE study (G100971) and is no longer being marketed or produced).the authors sought to investigate the degree and progression of hearing preservation over a longitudinal postoperative period in a large consecutive cohort of implanted patients with preoperative residual hearing who received either the Nucleus Hybrid-L24 or the Nucleus Freedom CI422 implant. The intention was to examine potential characteristics and triggers of resulting postoperative hearing loss which may support a differentiation of CI candidacy criteria for a certain implant type. A retrospective data analysis of patient files Cochlear Implants May 16 7

8 on consecutively implanted subjects presenting with a severe-to-profound sensorineural hearing loss at frequencies>1,500 Hz and substantial residual hearing at frequencies 1,500 Hz, implanted with a Nucleus Hybrid-L24 (n=97) or a CI422 implant (n=100), was undertaken. A single-subject repeated-measure design comparing the mean threshold shift for pure-tone thresholds under headphones up to 24 months after implantation was used. Hearing preservation is observed in the majority of subjects with either implant (250-1,500 Hz frequency range). Hybrid-L24 patients exhibited a median hearing loss of 10 db at initial fitting (n=97) and of 15 db after 24 months (n=51). A 14.4-dB decrease in median hearing loss at initial fitting (n=100) and a 30-dB decrease after 24 months (n=28) was observed with the CI422 electrode. At initial fitting, 54.6% of the Hybrid-L24 (n=97) and 49.0% of the CI422 (n=100) subjects showed a mean threshold shift<15 db. After 24 months, 58.8% (Hybrid-L24, n=51) and 28.6% (CI422, n=28) of the patients showed a mean threshold shift<15 db. The reviewers concluded the results indicate that residual hearing was preserved for the majority of implanted patients with the Hybrid-L24 and the CI422 implant. Patients implanted with the Hybrid-L24 implant demonstrate greater stability and less median hearing loss over time than those with the CI422 implant. Assessments of onset and stability of hearing loss prior to implantation are important factors to consider during candidacy evaluation for electrode selection to potentially maximize the performance outcome for each patient. Lenarz et al (2013) investigated the preservation of residual hearing in subjects who received the Nucleus Hybrid L24 cochlear implant. They investigated the performance benefits up to one year post-implantation in terms of speech recognition, sound quality, and quality of life in a prospective, with sequential enrolment and within-subject comparisons. Post-operative performance using a Freedom Hybrid sound processor was compared with that of pre-operative hearing aids. Sixty-six adult hearing-impaired subjects with bilateral severe-toprofound high frequency hearing los were included in the study. Group median increase in air-conduction thresholds in the implanted ear for test frequencies Hz was < 15 db across the population; both immediately and one year post-operatively. Eighty-eight percent of subjects used the Hybrid processor at one year post-op. Sixty-five percent of subjects had significant gain in speech recognition in quiet, and 73% in noise ( 20 percentage points/2 db SNR). Mean SSQ subscale scores were significantly improved (+ 1.2, + 1.3, points, p < 0.001), as was mean HUI3 score ( , p < 0.01). Combining residual hearing with CI gave %age points mean benefit in speech recognition scores over CI alone (p < 0.01). The authors concluded useful residual hearing was conserved in 88% of subjects. Speech perception was significantly improved over preoperative hearing aids, as was sound quality and quality of life. Clinical trials evaluating the Nucleus Hybrid L24 were identified on the Clinical Trial.gov website. Some are active and not recruiting participant and others are enrolling by invitation only. Scientific Rationale Update May 2014 Ching et al (2013) reported the Longitudinal Outcomes of Children with Hearing Impairment (LOCHI) study to directly compare outcomes of early- and lateidentified children. The authors investigated whether early performance measured shortly after initial amplification predicts language development at 3 years of age in a prospective, population-based study. The authors assessed outcomes at 6- and 12-months after amplification, and then at 3 and 5 years of Cochlear Implants May 16 8

9 age. Main outcome measures included directly-assessed language, receptive vocabulary, speech production; and parent-reported functional performance in everyday life. A range of demographic and audiological information was also collected at evaluation intervals. About 450 children participated, and 3-year outcomes scores were available for 356 participants. Multiple regression analysis revealed that early language scores or functional performance ratings were significant predictors of 3-year outcomes. Other significant predictors included the presence or absence of additional disabilities, severity of hearing loss, and age at cochlear implant activation. The authors concluded that early performance, either directly-assessed language ability (PLS-4) or parent-reported functional ratings (PEACH), were significant predictors of 3-year outcomes; along with presence or absence of additional disabilities, severity of hearing loss, and age at CI activation. Earlier implantation is possible with early detection of hearing loss via UNHS. Monitoring performance after initial amplification allows preventive strategies to be implemented early to improve outcomes. Hashemi et al (2013) aimed to determine the effect of cochlear implantation on the improvement of the auditory performance in 2-7 years old children. The follow-up study was conducted on 98 children between 2-7 years old who had referred to a single cochlear implantation center. The patients' information was gathered from their profiles both before and after the operation. The auditory performance score was obtained in 3 stages; 6 months, 1 year, and 2 years after the cochlear implantation through the Cap test. The data was analyzed using the nonparametric Friedman test as well as Mann-Withney, Kruskal-Wallis, and Spearman's Ranks Correlation coefficients. The mean and the median of the auditory performance score of the children who had undergone the cochlear implantation revealed a significant improvement from 6 months to 1 year, and 2 years after the implantation. It showed a significant statistical association between implantation age, type of hearing loss, regular reference, and the length of being present in the rehabilitation program with the auditory performance. It showed no significant association between sex, mother's level of education, being monolingual or bilingual, and family size with the auditory performance. The investigators concluded the study revealed that the type of hearing loss, presence in the rehabilitation program, and the age of cochlear implantation can be major prognostic factors of the response to the treatment, then the country's health policy makers and health planners must executively take into account the infants' hearing screening program during the first 6 month of age. Ikeya et al (2013) analyzed long-term postoperative complications in patients with cochlear implants with a view to improve clinical interventions and propose a consensus for reporting complications. A total of 406 cases received cochlear implants between December 1985 and April 2007 at a single center. The authors retrospectively reviewed case notes from 366 patients who had undergone cochlear implantation (215 adults and 151 children) after excluding 40 patients of re-implantation including 13 cases implanted initially at other hospitals. Lifethreatening, major and minor complications were examined retrospectively. Major complications occurred following cochlear implantation in 32 patients (8.7%) who had received their initial implant at the center.. Revision surgery was required for 30 patients. The mean age at implantation was 33 years 6 months (range, 1 year 9 months to 83 years; median, 37 years). The main etiology of deafness was unknown or progressive (113, 52.6%) in adults and congenital (132, 87.4%) in children. The cause of deafness was meningitis in 41 cases (11.2%), and 26 cases (7.1%) were diagnosed with idiopathic sudden deafness. Cochlear Implants May 16 9

10 Flap-related problems (including middle ear infection and/or flap necrosis) developed in 13 cases (3.6%), with 12 cases (7 adults, 5 children) requiring reimplantation. Electrode slip-out occurred in 8 patients (7 adults, 1 child). All adult cases in whom electrodes slipped out underwent implantation before 1994, while the child (1 pediatric case) was operated in All cases required reimplantation and most cochlear implantations were performed using the modified split-bridge technique after Six patients (4 adults, 2 children) experienced device failure. Four patients experienced electrode problems. Non-surgical major complications included 1 patient with permanent facial nerve paralysis as a result of thermal injury in The total number of minor medical and surgical complications was 27, representing 7.4% of all operations. Authors concluded many cases of major complications, including electrode problems and facial paralysis, excluding traumatic device failure were considered avoidable by strict operative and postoperative procedures. Some cases of flap infection and traumatic device failure may not be able to be avoided completely, and every possible care should be taken by implant patients and others involved. Scientific Rationale Update May 2013 Tobey et al (2013) examined specific spoken language abilities of 160 children with severe-to-profound sensorineural hearing loss followed prospectively 4, 5, or 6 years after cochlear implantation. Ninety-eight children received implants before 2.5 years, and 62 children received implants between 2.5 and 5 years of age. Language was assessed using four subtests of the Comprehensive Assessment of Spoken Language (CASL). Standard scores were evaluated by contrasting age of implantation and follow-up test time. Children implanted under 2.5 years of age achieved higher standard scores than children with older ages of implantation for expressive vocabulary, expressive syntax, and pragmatic judgments. However, in both groups, some children performed more than two standard deviations below the standardization group mean, while some scored at or well above the mean. Investigators concluded younger ages of implantation are associated with higher levels of performance, while later ages of implantation are associated with higher probabilities of continued language delays, particularly within subdomains of grammar and pragmatics. Longitudinal data from this cohort study demonstrate that after 6 years of implant experience, there is large variability in language outcomes associated with modifiers of rates of language learning that differ as children with implants age. Gaylor et al (2013) sought to evaluate the communication-related outcomes and health-related QOL outcomes after unilateral or bilateral cochlear implantation in adults with sensorineural hearing loss. Published studies of adult patients undergoing unilateral or bilateral procedures with multichannel cochlear implants and assessments using open-set sentence tests, multisyllable word tests, or QOL measures. Five researchers extracted information on population characteristics, outcomes of interest, and study design and assessed the studies for risk of bias. Discrepancies were resolved by consensus. A total of 42 studies met the inclusion criteria. Most unilateral implant studies showed a statistically significant improvement in mean speech scores as measured by open-set sentence or multisyllable word tests; meta-analysis revealed a significant improvement in QOL after unilateral implantation. Results from studies assessing bilateral implantation showed improvement in communication-related outcomes compared with unilateral implantation and additional improvements in sound localization compared with unilateral device use or implantation only. Based on a few studies, the QOL outcomes varied across tests after bilateral implantation. The reviewers Cochlear Implants May 16 10

11 concluded unilateral cochlear implants provide improved hearing and significantly improve QOL, and improvements in sound localization are noted for bilateral implantation. Future studies of longer duration, higher-quality reporting, and large databases or registries of patients with long-term follow-up data are needed to yield stronger evidence. DiNardo et al (2013) sought to evaluate the benefits of unilateral cochlear implant (CI) in patients over 60 on speech perception and quality of life, comparing the results obtained with a control group of younger CI recipients. Twenty CI users (mean age 72 years), postlingually deafened, were included in this study. Audiological performance was evaluated using bisyllabic words and sentences recognition tests in a quiet and a noise environment. Moreover, we administered two questionnaires to evaluate the health status (SF-36), CI-related effects on daily activities and personal satisfaction (Questionnaire for selfevaluation of CI benefit with SADL scale modification). Performance measures of the geriatric population showed a significant benefit on speech recognition tests compared to pre-implantation condition, even if younger CI users scored significantly better in both bisyllabic words and sentences recognition test. All study patients reported being able to have a normal conversation with an acquaintance. No significant difference was found between the study and control group in physical and mental health status, conversation with an outsider, use of TV and phone. A significant difference (p < 0.05) was noticed, instead, between elderly and younger adult patients about the overall satisfaction derived from CI. Our findings confirm the indisputable utility of CI and provide evidence that elderly patients derive a substantial benefit from it on quality of life, as demonstrated by health status, success in the common activities of daily living and perceived satisfaction after this procedure. Scientific Rationale Update June 2010 (2009) The National Institute of Health and Clinical Effectiveness (NICE) has listed technology appraisal guidance, number 166, for cochlear implants for children and adults with severe to profound deafness. Their recommendations are as follows: Unilateral cochlear implantation is recommended as an option for people with severe to profound deafness who do not receive adequate benefit from acoustic hearing aids; Simultaneous bilateral cochlear implantation is recommended as an option for the following groups of people with severe to profound deafness who do not receive adequate benefit from acoustic hearing aids: Children or adults who are blind or who have other disabilities that increase their reliance on auditory stimuli as a primary sensory mechanism for spatial awareness. Sequential bilateral cochlear implantation is not recommended as an option for people with severe to profound deafness; People who had a unilateral implant before publication of this guidance, and who fall into one of the categories described in the 2 nd bullet noted above, should have the option of an additional contralateral implant only if this is considered to provide sufficient benefit by the responsible clinician after an informed discussion with the individual person and their caregivers; For the purposes of this guidance, severe to profound deafness is defined as hearing only sounds that are louder than 90 db HL at frequencies of 2 and 4 Cochlear Implants May 16 11

12 khz without acoustic hearing aids. Adequate benefit from acoustic hearing aids is defined for this guidance as: For adults, a score of 50% or greater on Bamford Kowal Bench (BKB) sentence testing at a sound intensity of 70 db SPL For children, speech, language and listening skills appropriate to age, developmental stage and cognitive ability. Cochlear implantation should be considered for children and adults only after an assessment by a multidisciplinary team. As part of the assessment children and adults should also have had a valid trial of an acoustic hearing aid for at least 3 months (unless contraindicated or inappropriate); When considering the assessment of adequacy of acoustic hearing aids, the multidisciplinary team should be mindful of the need to ensure equality of access. Tests should take into account a person s disabilities (such as physical and cognitive impairments), or linguistic or other communication difficulties, and may need to be adapted. If it is not possible to administer tests in a language in which a person is sufficiently fluent for the tests to be appropriate, other methods of assessment should be considered. The NICE Committee considered the evidence for the clinical effectiveness of bilateral cochlear implants. The Committee considered that the additional benefits of bilateral cochlear implantation were less certain than the benefits of unilateral cochlear implantation. This was because of the limitations of the evidence base owing to the small number of studies and the small numbers of participants. However, the Committee considered that the studies had shown additional benefits to having a second cochlear implant in relation to speech perception in noisy situations and directional perception of sound. The Committee heard from patient experts that they considered that there were other benefits from bilateral cochlear implantation. These benefits included easier, less exhausting communication (for example, determining the direction of the sound in group conversations without unnecessary head movement). The Committee concluded that there were additional benefits of bilateral cochlear implants that had not been adequately evaluated in the published studies, although these may vary among individuals. The guidance on this technology will be considered for review in February 2011 to enable further research to be carried out to assess the benefits of bilateral cochlear implantation. The U.S. Food and Drug Administration (FDA) notes 510K approval with K of the BAHA CORDELLE II COCHLEAR BONE ANCHORED SYSTEMS AB on 4/10/2008. (2009) The American Academy of Audiology recognizes multichannel cochlear implants as sensory aid options for children with profound hearing impairments who demonstrate limited or no functional benefit from conventional hearing aid amplification. Multichannel cochlear implants are appropriate for children with prelingual or postlingual deafness. The Academy further recognizes that parents (or legal guardian) have the right to choose a cochlear implant if they decide that it is the most appropriate option for their child. Medicare Members Effective for services performed on or after April 4, 2005, cochlear implantation may be covered for individuals meeting the selection guidelines above and with hearing test scores of greater than 40% and less than or equal to 60% only when the provider is participating in, and patients are enrolled in, either an FDA- Cochlear Implants May 16 12

13 approved category B investigational device exemption clinical trial as defined at 42 CFR , a trial under the Centers for Medicare & Medicaid (CMS) Clinical Trial Policy as defined at section of the National Coverage Determinations Manual, or a prospective, controlled comparative trial approved by CMS as consistent with the evidentiary requirements for National Coverage Analyses and meeting specific quality standards. Scientific Rationale Update June 2008 Cochlear implantation offers sound perception to individuals who are unable to achieve adequate help with conventional hearing aids. This is particularly beneficial for individuals with binaural deafness. The lack of binaural hearing makes it impossible for the individual to localize sound and interferes with speech discrimination. Hearing and speech intelligibility are of critical importance during early childhood and adolescent development. Even moderate unilateral hearing loss during this time has lasting effects on speech, language, and intellectual development. The additional benefits of improved sound localization and also improved hearing in background noise are well supported by substantial literature and patient experience. Additional evidence demonstrates patients who undergo both monaural and binaural implantation at an earlier age (<3.5 yrs) appear to have more normal auditory development. The hardware and software advances during this time have yielded progressively better and more predictable results. Building upon the experience gained from the use of binaural conventional amplification, binaural cochlear implantation has become more common in an effort to achieve maximal improvement in binaurally deaf individuals, both those who are congenitally deaf and those deafened postlingually. Cochlear implantation is routinely and reliably performed in both the pediatric and adult populations. The US Food and Drug Administration (FDA) has granted final approval to cochlear implantation, and has approved multiple devices produced by several manufacturers. The FDA has issued a letter from Elisa D. Harvey, DVM, PhD, Director Investigational Device Exemption and Humanitarian Device Exemption Programs, Office of Device Evaluation, Center for Devices and Radiological Health, Food and Drug Administration. In this letter, dated May 12, 2006, the Director states: From the Agency s perspective the bilateral use of cochlear implants falls under the approved marketing label for these devices. There are no warnings, precautions, or contraindications to bilateral use in the labeling. This letter indicates the FDA s approval of bilateral implantation of these devices. As a consequence of this position, binaural cochlear implantation cannot be considered off-label. Additionally, the final approval by the FDA attests to the safety of cochlear implantation, monaural and binaural. There is absolutely no indication of a maximum limit (or dose ) to the implanted materials comprising the currently marketed cochlear implants. Multiple studies have reviewed over 1,312 patients and have established beyond any doubt the safety of cochlear implantation, monaurally and binaurally, sequentially or simultaneously implanted, in pediatric and adult patients. The rates of major and minor complications in these studies are low and acceptable. Sequential implantation does not expose the subject patients to a significant increase in risk as is clearly demonstrated by this extensive experience. The peer-reviewed published literature has demonstrated the benefits of binaural implantation on sound localization and speech perception in complex listening Cochlear Implants May 16 13

14 environments, which has been proven in significant numbers of patients. While sound localization may be primarily related to head-shadow effect (interaural level differences or ILD) rather than interaural time differences (ITD), the effect is nonetheless real, statistically significant, and repeatable for all age groups and temporal implantation scenarios. These are findings that have been established reliably in multiple patient populations, in multiple clinical and research facilities internationally. Similarly, multiple peer-reviewed published studies have reliably and repeatedly demonstrated a statistically significant advantage for speech perception in noise for binaurally implanted individuals versus those tested in the monaural condition, or monaural implant + contralateral hearing aid. These studies do not duplicate patients and utilize internal controls (same individual in the monaurally activated condition). Frequently the issues of proven efficacy and those of quality of life are confused. In determining whether binaural cochlear implantation is effective, and not investigational, intellectual and academic honesty dictates reliance on objective measures of performance in the binaural versus monaural condition. The studies described above, and cited below, conclusively demonstrate objective improvements resulting from binaural cochlear implantation. These benefits are confirmed in studies in which the patients themselves serve as the perfect controls, matching subjects in the monaural condition with those in the binaural condition identically. The lack of an appropriate control population cannot be argued. The facts speak for themselves: well-designed and controlled studies, using established testing methods and adequate numbers of subjects, performed internationally, independent of industry influence, published in the peer-reviewed literature, demonstrate identical findings of statistical significance that are reliable and reproducible. This is the gold-standard of clinical efficacy and without exception establishes that binaural cochlear implantation is not investigational and that bilateral cochlear implantation is effective for the treatment of severe sensorineural hearing loss. The short and long-term complications of a second cochlear implant in the contralateral ear are no different from implantation in the first ear. Each ear and implantation may be taken in isolation in this consideration. Candidacy for a second implant should be the same in terms of hearing function as for the initial implant. (2007) The American Academy of Otolaryngology-Head and Neck Surgery, Inc. (AAO-HNS) considers cochlear implantation an appropriate treatment for adults and children with severe to profound hearing loss. Based on extensive literature demonstrating that clinically selected adults and children can perform significantly better with two cochlear implants than one, bilateral cochlear implantation is an accepted medical practice. (2007) The National Institute for Health and Clinical Excellence (NICE) technology appraisal recommended simultaneous bilateral cochlear implantation as an option for persons with severe to profound deafness who are at risk for ossification of the cochlea (e.g., after meningitis). The Committee heard from clinical specialists that ossification of the cochlea could preclude successful re-implantation if the first implanted device failed. Cochlear Implants May 16 14

15 Bilateral Cochlear Implants in Children (NICE) The Committee recommended that a randomized controlled trial should be carried out to examine the benefit of bilateral cochlear implantation compared with unilateral cochlear implantation in postlingual children with severe to profound deafness. It is proposed that the guidance on this technology is considered for review in February 2011 to enable further research to be carried out to assess the benefits of bilateral cochlear implantation. (December 2007) There is a currently a Clinical Trial recruiting participants on Bilateral Cochlear Implant Benefit in Young Children. The ClinicalTrials.gov Identifier is NCT The primary purpose of this study is to track patient outcomes for bilateral cochlear implant recipients in a cohort of 60 children, ages 12 to 36 months at time of surgery, who receive two implants in the same operation or in two different surgeries with the initial fitting of the devices separated by no more than six months. (2007) American Speech-Language-Hearing Association (ASHA) - Bilateral implantation is currently being studied in a limited number of cochlear implant recipients with mixed results. In some cases, recipients experience enhanced speech understanding, especially in noise; in other users the improvement in speech understanding compared with unilateral performance is minimal or absent and the primary advantage of binaural implantation is sound localization. Bilateral implantation outcomes to date are encouraging but inconclusive due to the limited number of participants and the scope of the projects. There is a clear need for further exploration of the many variables that can affect the performance of people with binaural implants before widespread use is warranted. The long-term benefit and identification of factors that contribute to the acquisition of binaural hearing with bilateral implants in children need further investigation. Systematic study of children who receive bilateral cochlear implants will provide for more informed recommendations concerning if, and when, to implant the contralateral ear. (2007) Research funded by the National Institute on Deafness and other Communication Disorders (NIDCD) suggests that children who are deaf and have a cochlear implant in each ear more accurately locate sounds when they use both implants instead of one. Children with two implants also become more skilled at localizing sound over time. Litovsky et al., (2006) The Canadian Association of Speech-Language Pathologists and Audiologists (CASLPA) Research in the area of bilateral cochlear implantation continues to show promise particularly for listening in noise and for directionality. Electroacoustic stimulation is becoming more of a possibility as less invasive surgical techniques are explored and improved electrode arrays are designed to preserve residual low frequency hearing. Future developments in cochlear implants can be expected to include investigations of fully implantable devices. Presently, all cochlear implants have been shown to be effective in improving auditory-only speech understanding despite variability in patient outcomes. There is a need for ongoing research and exploration of the effectiveness of different rehabilitation and educational strategies with children with cochlear implants. Long-term follow up is essential to evaluate the impact of cochlear implants on the lives of children with significant hearing loss and their families. Cochlear Implants May 16 15

16 (2006) The Swedish Council on Health Technology Assessment (SBU), a leading international technology assessment agency, conducted a comprehensive assessment of current evidence for bilateral cochlear implantation in children. The assessment concluded: "Scientific documentation on the benefits of bilateral cochlear implantation in children is insufficient. Well-designed, scientific studies are needed to determine whether the method yields positive effects that outweigh the increased risk for complications. The amount of auditory stimulation in each ear needed for the development of binaural hearing in children is unknown. In addition, the potential benefit or loss of benefit related to the timing of a second implant is not clear. While bilateral cochlear implants could strengthen the bilateral pathways during development and allow for improved binaural hearing, it is likely that there is a group of children at risk for poor benefit from a second cochlear implant. Despite the theoretical advantages of binaural hearing, there could be peripheral or central degeneration that results in binaural interference rather than binaural improvements. The long-term benefit and identification of factors that contribute to the acquisition of binaural hearing with bilateral implants in children need further investigation. Systematic study of children who receive bilateral CIs will provide for more informed recommendations concerning if, and when, to implant the contralateral ear. More studies need to be done to elicit the effects of age at time of implants (1st and 2nd) and the effects of sequential versus simultaneous implantation. As more information about the results of bilateral implantation becomes available, candidacy decisions will include comparisons with children who have received bilateral cochlear implants. A critical component in the assessment of bilateral performance appears to be the evaluation of speech recognition under more real life listening conditions. Binaural implantation in infants less than 12 months of age is not yet the standard of care in the Neurotologic community. Scientific Rationale Initial A cochlear implant is a small, surgically implanted electronic hearing device, designed to provide a sense of sound to children or to adults who are profoundly deaf or severely hard-of-hearing. The cochlea is the part of the inner ear that processes sound. Sensory receptor cells (commonly called hair cells) are located in the inner ear. The function of the hair cells is to change the sound energy into electrochemical signals that are recognized by the auditory nerve. When hair cells are damaged or dead, parts of the signal may be distorted, or may not be sent to the hearing nerve at all. Because hair cell damage is by far the most common cause of hearing loss, Cochlear Implants bypass the damaged hair cells and replace their function by converting sound energy into electrical energy that can stimulate the auditory nerve directly. The nerve recognizes this stimulation in much the same way normal sound is recognized, and the information is sent along the nerve to the brain where meaning is attached. An implant does not restore normal hearing but it can give a deaf person a useful representation of sounds in the environment and help him or her to understand speech. This allows many people to recognize warning signals and enjoy a conversation in person or by telephone. Cochlear Implants May 16 16

17 The implant consists of an external portion that sits behind the ear and a second portion that is surgically placed under the skin. An implant has four basic parts: A microphone, worn behind the ear, which picks up sound from the environment. A speech processor, worn on the body, (some types may be worn behind the ear), which selects and arranges sounds picked up by the microphone. A transmitter and receiver/stimulator, which receive signals from the speech processor and convert them into electric impulses. And electrodes that collect the impulses from the stimulator and sends them to the auditory nerve. Current devices have a magnet that holds the external system in place next to the implanted internal system. The external system may be worn entirely behind the ear or its parts may be worn in a pocket, belt pouch, or harness. Cochlear implants are recognized by the American Medical Association (AMA) and the American Academy of Otolaryngology - Head and Neck Surgery (AAO- HNS) as an approved medical procedure for adults and children. They were approved by the Food and Drug Administration (FDA) in the mid-1980s and are covered by Medicare, Medicaid and Vocational Rehabilitation. According to the Food and Drug Administration s (FDA s) 2005 data, nearly 100,000 people worldwide have received implants. In the United States, roughly 22,000 adults and nearly 15,000 children have received them. The first step in the work-up of patients who have hearing loss is an audiogram. This battery of tests includes pure tone thresholds from 250 to 8000 Hz, word recognition tests, speech reception thresholds, acoustic reflexes, and tympanometry. Pre- operative evaluation includes the following: High-resolution CT scan or MRI of the temporal bone to evaluate the patency of the cochlea; Assessment for evidence of prior ear surgery and active mastoiditis; Identification of congenital malformations and assessment of the surgical anatomy; No medical contraindications to general anesthesia; No contraindications to surgical intervention or postoperative follow up; Patient must demonstrate psychological stability and suitable motivation with realistic expectations for outcomes; Use of a cochlear implant requires both a surgical procedure and significant therapy to learn or relearn the sense of hearing. The decision to receive an implant should involve discussions with medical specialists, including an experienced cochlear-implant surgeon. Surgical implantations are almost always safe, although complications are a risk factor, just as with any kind of surgery. An additional consideration is learning to interpret the sounds created by an implant, which takes time and practice. Speech-language pathologists and audiologists are frequently involved in this learning process. Prior to implantation, all of these factors need to be considered. Cochlear Implants in Adults Adult candidates for cochlear implants who are post lingual (had speech and language skills before losing their hearing) can benefit from cochlear implants. Cochlear Implants May 16 17

18 They often can associate the sounds made through an implant with sounds they remember. This may help them to understand speech without visual cues or systems such as lip reading or sign language. Adults typically perceive more of a mechanical sound after implantation; sound becomes more natural after four to eight weeks. Dr. Niparko et al (2005) conducted a research study to help clarify the effect of age on outcome of cochlear implantation. Investigators assessed 749 people who had undergone this procedure and who ranged in age from 14 to 91 years. They used multivariate regression to analyze a number of pre- and post-operative variables. Although those 65 years of age and older had a 4.6% lower score on postoperative monosyllabic word recognition tests, this difference was not significant, the researchers note, and may not be "clinically detectable. This study indicates that early language learning and residual hearing accounts for older implant recipients (including those who had been deaf for longer than 25 years), scoring higher than younger patients. "A foundation of central auditory processing in the older individual may actually reduce the disadvantage of advanced age at implantation, build on adaptive skills, and help explain the encouraging results of this study." Elderly patients, they conclude, "Should therefore not be discriminated against in assessments for cochlear implant candidacy." Arch Otolaryngology Head Neck Surgery (2005; 131: ). Tyler et al (2003) performed a study to evaluate the efficacy of bilateral cochlear implants. Although single cochlear implants (CI) are well accepted, the implantation of a single device does not provide normal (binaural) hearing to a patient with severe bilateral hearing loss. Some adults are now benefiting from bilateral implants, which allow them to hear better in conditions with background noise (such as restaurants), localize sound, and hear sound coming from either side without having to turn one's head. With two ears, the brain uses localization cues to separate sounds coming from different locations. Optimal sound localization requires the ability to detect differences in time and amplitude between signals reaching both ears. In conclusion, although there are favorable results from this study, additional research is still necessary to address the safety, efficacy and the long-term effects of bilateral cochlear implants. Das et al (2002) from The Head and Neck Surgical Division of the Department of Otolaryngology at the University of North Carolina at Chapel Hill completed an additional study on the advantage of bilateral cochlear implantation over unilateral implantation. The documented benefits include improved speech perception in noisy environments and improved sound localization. Despite technological improvements in speech processing strategies, measured intraaural time differences in bilateral cochlear implant recipients remain considerably greater than those with normal hearing. Programming challenges persist to optimize sound processing with bilateral implants. Vestibular effects of bilateral cochlear implantation appear safe but need further study. Important considerations including the duration of implant function, long-term complication rate, and improvements in implant technology will continue to strongly influence the role of bilateral cochlear implantation. At this time, bilateral cochlear implantation provides advantages over unilateral implantation including improved speech perception in noise and improved sound localization. However, further research is needed to define the optimal indications, efficacy and to maximize the benefit of bilateral implantation. Cochlear Implants May 16 18

19 Cochlear Implants in Children Cochlear implants, coupled with intensive post-implantation therapy, can help young children to acquire speech, language, developmental, and social skills. Most children who receive implants are between two and six years old. Early implantation provides exposure to sounds that can be helpful during the critical period when children learn speech and language skills. In 2000, the FDA lowered the age of eligibility to 12 months for one type of cochlear implant. The vast majority of candidates for implants are congenitally deaf; over 90% of them have normally hearing parents who want their children to hear and speak. Parents who think that deafness is a way of life and not a disability are unlikely to consider implantation. The evaluation process should encompass a child's social, domestic, psychological, and educational needs. No child should be considered too young or too disabled to be evaluated for cochlear implantation. The delivery of a high quality service for children thus requires multidisciplinary teams, capable of making complex assessments. Important prerequisites include access to an education program that stresses auditory and verbal skills and highly motivated parents who have realistic expectations. Parents who are considering a cochlear implant for their child often have a choice of cochlear implant clinicians in more than one center for pre-operative testing, implantation, and post-operative rehabilitation. There are a number of considerations that parents should consider when selecting an implant center based on the individual needs and circumstances of the child. This should include the financial aspects as well as the distance of the facility. The child must return to the implant center periodically for monitoring and program adjustments to the speech processor. Cheng et al (2000) from Johns Hopkins University in Baltimore examined data from surveys of 78 parents whose profoundly deaf children had received cochlear implants. The researchers found that on average the quality of life scores improved and estimated the costs and savings associated with the child living life with the implant. They estimate that the net benefit (savings with direct and indirect medical costs subtracted) to society per child who received a cochlear implant would be $53,198 per child. The expected lifetime cost to a society for a child with pre-lingual onset of profound deafness exceeds $1 million, largely because of special education and reduced work productivity," the authors note, citing a previous study. "Cochlear implantation may result in a net savings to society if benefits translate into education costs and increased earnings." Cheng et al (2000) The average age of the children in this study who received cochlear implants was 6 years old. They were estimated to live an average life expectancy of 78 years (72 years with implant). Citing other sources, the authors note that: "Impairment of hair cell function [cells in the inner ear] induces profound deafness in approximately 0.3 percent of children younger than 5 years. Cochlear implants may affect the auditory rehabilitation of an estimated 200,000 U.S. children with profound deafness who fail to benefit from conventional hearing aids." Weber et al (2006) compared the language achievement of 29 pre-lingually deaf children three or more years after cochlear implantation with the achievement of 29 pre-lingually deaf children who were treated with hearing aids. The children with cochlear implants had better language comprehension and production skills. Cochlear Implants May 16 19

20 Sanford et al (2006) researched children whose deafness occurs after age two years and who are deaf for short periods of time have the best language outcome. As an example, the speech perception and production skills of three groups of children were analyzed after cochlear implantation: 70 children who were congenitally deaf, 22 children who were deafened by meningitis before two years of age, and 14 children who were deafened by meningitis after two years of age. The speech perception skills of the groups of children who were deafened after age two were better than those of the other two groups; no difference was found in speech perception or production skills between the two groups of children whose deafness began before age two years. Sanford et al (2006) Speech perception was measured three, four, and five years after cochlear implantation in forty children who were born deaf or who became deaf before three years of age and who had no measurable speech perception before implantation with the most powerful hearing aids. Speech perception improved with time (27, 35, and 45 mean words per minute at three, four, and five years of follow-up, respectively). Improvement in speech perception was greater for children who were younger at the time of implantation and who used oral rather than total communication (E.g., including signing). Weber et al (2006) researched the risk of bacterial meningitis among cochlear implant recipients. A study among 4,264 children who had received cochlear implants in the United States between January 1997 and August 2002 and were younger than six years at the time of implantation showed that the incidence of pneumococcal meningitis was 30 times higher than in children of the same age in the general population. The use of a positioner (a small rubber wedge that helps the surgeon position the implant), radiographic malformation of the inner ear, and cerebrospinal fluid (CSF) leak were risk factors for the development of meningitis in these patients. The study was not designed to evaluate the contribution of other pre-existing risk factors for meningitis including a prior history of meningitis, recurrent otitis media, immune deficiency, or occult CSF leak. Positioners have not been used since July The information from the follow-up study highlights the importance of close monitoring of all children with cochlear implants, particularly those with positioners, for early signs of meningitis, and of prompt treatment of middle ear infections. Worldwide, there are over 90 known reports of people getting meningitis post cochlear implant, but this is out of approximately 60,000 people who have had this procedure. (Bacterial meningitis is the commonest cause of acquired severe deafness in early childhood. Meningitis may cause new bone formation in the cochlea, which could technically compromise the insertion of a cochlear implant and such children should be evaluated with some urgency). Pneumococcal Meningitis In 2002, the Centers for Disease Control and Prevention (CDC) and the FDA, in partnership with state health departments completed an investigation that found children with cochlear implants are more likely to get bacterial meningitis than children without cochlear implants. Because people with cochlear implants are at increased risk for pneumococcal meningitis, CDC recommends that people with cochlear implants follow recommendations for pneumococcal vaccinations that apply to members of other groups at high risk for invasive pneumococcal disease. This would include the following: Cochlear Implants May 16 20

21 A child with cochlear implants less than aged 2 years should receive pneumococcal conjugate vaccine (PCV-7) (Prevnar) as is recommended for all children. Children with cochlear implants aged 2 years and older who have completed the PCV-7 series should receive one dose of the pneumococcal polysaccharide vaccine (PPV-23) (Pneumovax 23). If they have just received PCV-7, they should wait at least two months before receiving PPV-23. Children with cochlear implants between 24 and 59 months of age who have never received either PCV-7 or PPV-23 should receive two doses of PCV-7 two or more months apart and then receive one dose of PPV-23 at least two months later. Persons aged 5 years and older with cochlear implants should receive one dose of PPV-23. Revaccination is not indicated. Pneumococcal vaccination should be complete by two weeks before surgery, if possible, for patients who are scheduled to receive cochlear implants. Surgical Issues Cochlear implantation includes risks common to most surgical procedures, e.g., general anesthetic exposure, as well as unique risks that are influenced by device design, individual anatomy and pathology, and surgical technique. The complication rate in pediatric implantation is less than that currently seen in adults. Overall, the complication rate compares favorably to the 10 percent rate seen with pacemaker/defibrillator implantation. Major complications, i.e., those requiring revision surgeries, include flap problems, device migration or extrusion, and device failure. This would cause reimplantation in approximately 5 percent of cases. In general, reimplantation in the same ear is usually possible, and individual auditory performance after reimplantation equals or exceeds that seen with the original implant. Particularly in the child, the potential consequences of otitis media have been of concern, but as the implanted electrode becomes enclosed in a fibrous envelope, it appears protected from the consequences of local infection. Facial palsy is also considered a major complication but is distinctly uncommon and rarely permanent. No mortalities have been attributed to cochlear implantation. All implants are potentially prone to failure, either because of manufacturing defects or use - related trauma. For the most commonly implanted device, 95 percent of implants are still functioning after 9 years. Most current implants with transcutaneous connectors do not provide self-test capability for the implanted portion, making it cumbersome to test for simple electrode failure, such as open and short circuits. Device manufacturers should include self-test circuity in future implant designs. Minor complications are those that resolve without surgical intervention. The most common is unwanted facial nerve stimulation with electrode activation, which is readily rectified by device reprogramming. Summary Unilateral cochlear implants have been a recognized effective treatment of bilateral sensorineural deafness. The literature assessing the safety and efficacy of bilateral cochlear implants in children has developed more fully as has the treating community s use of, and experience with, bilateral implants. However, Cochlear Implants May 16 21

22 larger, more randomized studies analyzing the effectiveness and safety of bilateral implants are necessary before this procedure is recognized as standard. Literature and research suggests that bilateral cochlear implantation may have the potential to improve sound localization and speech perception in many patients, compared with unilateral CI. Gantz et al (2002) analyzed head shadow (the barrier the head creates between sounds emitted from one direction and the contralateral ear) that dampens noise reaching the contralateral ear and delivers to that ear a better signal to noise (SNR) or speech-to-noise ratio. This shadowing or decreasing effect works best for high frequency sounds. Although research studies have indicated that bilateral cochlear implantation appears to be a promising treatment for the future, programming challenges persist and vestibular effects appear safe but need further study. Important considerations including the duration of implant function, long-term complication rate, and improvements in implant technology will continue to strongly influence the role of bilateral cochlear implantation. Additional research is needed to define the optimal indications and to maximize the benefit of bilateral implantation. At present, no published peer-reviewed article has reported the results of clinical trials analyzing the effectiveness of bilateral implants; instead, the majority of the published literature consists of relatively small case studies. In addition, further study is needed to determine if recently developed (but not FDA approved) CI devices capable of stimulating electrodes implanted in both ears but using a single microphone and speech processor/transmitter prove superior to currently available bilateral implants with independent speech processors. Finally, additional larger, controlled studies may well provide greater insight into the nature and extent of any benefits offered by bilateral CI, particularly if those studies are well designed and involve more significant patient populations than those addressed in the case studies published to date. Review History November 2006 June 2008 January 2010 June 2010 June 2011 May 2012 May 2013 May 2014 May 2015 Medical Advisory Council initial approval Updated. Codes updated. Added criteria to consider bilateral cochlear implants as medically necessary. Revised policy statement to note that if the external cochlear system is malfunctioning, beyond normal lifespan, or the existing components is inadequate to the point of interfering with the individual s activities of daily living, which would include school and work, then replacement with either an updated external system or a behind the ear (BTE) model, would be considered medically necessary. Update. Added additional information in policy statement regarding Medicare coverage, for those enrolled in, either an FDA-approved category B investigational device exemption clinical trial or a prospective, controlled comparative trial approved by CMS. Codes updated. Update. Medicare Table added with links to NCD and other Medicare publications. No revisions. Update. No revisions Update no revisions. Code updates Update no revisions Update- added cochlear hybrid implants as investigational. Code updates Cochlear Implants May 16 22

23 May 2016 Update no revisions This policy is based on the following evidenced-based guidelines: 1. The National Deaf Children s Society (NDCS). Cochlear implants for children and young people. Quality Standards and good practice guidelines in cochlear implants for children and young people - a new publication from The National Deaf Children s Society. May NIH Consensus Conference: Cochlear Implants in adults and children; May Journal of the American Medical Association (JAMA), National Institute of Hearing (NIH) consensus conference. Cochlear implants in adults and children. Vol. 274 No. 24, December 27, American Academy of Audiology (AAA). Position statement. Pediatric rehabilitation & hearing aids. Cochlear implants in children. 2008a. Accessed Mar 25, American Academy of Otolaryngology Head and Neck Surgery (AAO-HNS). Cochlear implants. 2008b. Updated 7/2014. Available at: 6. National Institute for Health and Clinical Excellence (NICE). Cochlear implants for children and adults with severe to profound deafness. Appraisal Consultation Document. London, UK: NICE; December Hayes Medical Technology Directory. Cochlear Implantation. September 22, National Institute for Health and Clinical Excellence (NICE). Cochlear implants for children and adults with severe to profound deafness. NICE Technology Appraisal Guidance 166. London, UK: NICE; January Available at: 9. Hayes Medical Technology Dirctory. Bilateral Cochlear Implantation in Children. July Update July Update July Hayes Medical Technology Dirctory. Bilateral Cochlear Implantation in Adults. Jyly Update July Update July Hayes News Government. FDA Approves First Implantable Device for Sensorineural Hearing Loss. Mar FDA Summary of Safety and Effectiveness Data. Nucleus Hybrid L24 Cochlear Implant System. March Available at: Hayes Health Technology Brief. Nucleus Hybrid L24 Cochlear Implant System (Cochlear Limited) for Hearing Loss. July References Update May Brown CJ, Jeon EK, Chiou LK, et al. Cortical Auditory Evoked Potentials Recorded From Nucleus Hybrid Cochlear Implant Users. Ear Hear Nov-Dec;36(6): Erixon E, Rask-Andersen H. Hearing and Patient Satisfaction Among 19 Patients Who Received Implants Intended for Hybrid Hearing: A Two-Year Follow-Up. Ear Hear Sep-Oct;36(5):e Friedmann DR, Peng R, Fang Y, et al. Effects of loss of residual hearing on speech performance with the CI422 and the Hybrid-L electrode. Cochlear Implants Int Sep;16(5): Gantz BJ, Dunn C, Oleson J, et al. Multicenter clinical trial of the Nucleus Hybrid S8 cochlear implant: Final outcomes. Laryngoscope Apr;126(4): Cochlear Implants May 16 23

24 5. Plant K, Babic L. Utility of bilateral acoustic hearing in combination with electrical stimulation provided by the cochlear implant. Int J Audiol Mar 17: Roland JT Jr, Gantz BJ, Waltzman SB, et al. United States multicenter clinical trial of the cochlear nucleus hybrid implant system. Laryngoscope Jan;126(1): References Update May Jurawitz MC, Büchner A, Harpel T, et al. Hearing preservation outcomes with different cochlear implant electrodes: Nucleus HybridL24 and Nucleus Freedom CI422. Audiol Neurootol. 2014;19(5): Langereis M, Vermeulen A. School performance and wellbeing of children with CI in different communicative-educational environments. Int J Pediatr Otorhinolaryngol Mar Lenarz T, James C, Cuda D, et al. European multi-centre study of the Nucleus Hybrid L24 cochlear implant. Int J Audiol Dec;52(12): Marnane V, Ching TY. Hearing aid and cochlear implant use in children with hearing loss at three years of age: Predictors of use and predictors of changes in use. Int J Audiol Mar 30:1-8 References Update May Ching TY, Day J, Seeto M et al. Predicting 3-year outcomes of earlyidentified children with hearing impairment. B-ENT. 2013;Suppl 21: Hashemi SB, Rajaeefard A, Norouzpour H, et al. The Effect of Cochlear Implantation on the Improvement of the Auditory Performance in 2-7 Years old Children, Shiraz Iran Red Crescent Med J Mar;15(3): Humphriss R, Hall A, Maddocks J, et al. Does cochlear implantation improve speech recognition in children with auditory neuropathy spectrum disorder? A systematic review. Int J Audiol Jul;52(7): Ikeya J, Kawano A, Nishiyama N, et al. Long-term complications after cochlear implantation. Auris Nasus Larynx Dec;40(6): References Update May Blamey P, Artieres F, Başkent D, et al. Factors affecting auditory performance of postlinguistically deaf adults using cochlear implants: an update with 2251 patients. Audiol Neurootol. 2013;18(1): Di Nardo W, Anzivino R, Giannantonio S, et al. The effects of cochlear implantation on quality of life in the elderly. Eur Arch Otorhinolaryngol Feb Fitzgerald MB, Green JE, Fang Y, Waltzman SB. Factors influencing consistent device use in pediatric recipients of bilateral cochlear implants. Cochlear Implants Int Mar Gaylor JM, Raman G, Chung Met al. Cochlear Implantation in Adults: A Systematic Review and Meta-analysis. JAMA Otolaryngol Head Neck Surg Mar 1;139(3): Gifford RH, Dorman MF, Skarzynski H, et al. Cochlear Implantation With Hearing Preservation Yields Significant Benefit for Speech Recognition in Complex Listening Environments. Ear Hear Feb Holman MA, Carlson ML, Driscoll CL, et al. Cochlear implantation in children 12 months of age and younger. Otol Neurotol Feb;34(2): Cochlear Implants May 16 24

25 7. Leigh J, Dettman S, Dowell R, Briggs R. Communication development in children who receive a cochlear implant by 12 months of age. Otol Neurotol Apr;34(3): Nichani J, Bruce IA, Mawman D, et al. Cochlear implantation in patients deafened by ototoxic drugs. Cochlear Implants Int Mar Orhan KS, Celik M, Başaran B, et al. Revision surgery in cochlear implantation. Kulak Burun Bogaz Ihtis Derg Nov-Dec;22(6): Park GY, Moon IJ, Kim EY, et al. Auditory and speech performance in deaf children with deaf parents after cochlear implant. Otol Neurotol Feb;34(2): Peixoto MC, Spratley J, Oliveira G et al. Effectiveness of cochlear implants in children: Long term results. Int J Pediatr Otorhinolaryngol Apr;77(4): Percy-Smith L, Busch G, Sandahl M, et al. Language understanding and vocabulary of early cochlear implanted children. Int J Pediatr Otorhinolaryngol Feb;77(2): Ramos A, Guerra-Jiménez G, Rodriguez C, et al. Cochlear implants in adults over 60: a study of communicative benefits and the impact on quality of life. Cochlear Implants Int Mar Santa Maria PL, Domville-Lewis C, Sucher CM, et al. Hearing preservation surgery for cochlear implantation-hearing and quality of life after 2 years. 15. Otol Neurotol Apr;34(3): Tarkan O, Tuncer U, Ozdemir S, et al. Surgical and medical management for complications in 475 consecutive pediatric cochlear implantations. Int J Pediatr Otorhinolaryngol Apr;77(4): Távora-Vieira D, Marino R, Krishnaswamy J, et al. Cochlear implantation for unilateral deafness with and without tinnitus: A case series. Laryngoscope Apr Tobey EA, Thal D, Niparko JK, et al. Influence of implantation age on schoolage language performance in pediatric cochlear implant users. Int J Audiol Apr;52(4): Toe DM, Paatsch LE. The conversational skills of school-aged children with cochlear implants. Cochlear Implants Int Mar;14(2): Vallés-Varela H, Royo-López J, Carmen-Sampériz L, et al. The cochlear implant as a tinnitus treatment. Acta Otorrinolaringol Esp Mar van Schoonhoven J, Sparreboom M, van Zanten BG, et al. The effectiveness of bilateral cochlear implants for severe-to-profound deafness in adults: a systematic review. Otol Neurotol Feb;34(2): Warner-Czyz AD, Loy B, Roland PS, Tobey EA. A comparative study of psychosocial development in children who receive cochlear implants. Cochlear Implants Int Jan 18. References Update May Boons T, Brokx JP, Frijns JH, et al. Effect of pediatric bilateral cochlear implantation on language development. Arch Pediatr Adolesc Med Jan;166(1): Smith RJH. Gooi A. Treatment of hearing impairment in children. UpToDate. March 14, Zeitler DM, Anwar A, Green JE, et al. Cochlear implantation in prelingually deafened adolescents. Arch Pediatr Adolesc Med Jan;166(1): Cochlear Implants May 16 25

26 References Update June National Institute on Deafness and Other Communication Disorders. Cochlear Implants Available at: 2. Isaacson B. Cochlear Implants, Indications. January 25, Available at: References Update June Baldassari CM, Schmidt C, Schubert CM, et al. Receptive language outcomes in children after cochlear implantation. Otolaryngol Head Neck Surg. 2009; 140(1): Elvsåshagen T, Solyga V, Bakke SJ, et al. Neurofibromatosis type 2 and auditory brainstem implantation. Tidsskr Nor Laegeforen. 2009; 129(15): Bond M, Elston J, Mealing S, et al. Effectiveness of multi-channel unilateral cochlear implants for profoundly deaf children: A systematic review. Clin Otolaryngol. 2009; 34(3): Bond M, Mealing S, Anderson R, et al. The effectiveness and costeffectiveness of cochlear implants for severe to profound deafness in children and adults: A systematic review and economic model. Health Technol Assess. 2009; 13(44): Food and Drug Administration (FDA). Center for Devices and Radiological Health (CDRH). Cochlear Implants. Updated April 30, 2009a. Available at: 6. Food and Drug Administration (FDA). Center for Devices and Radiological Health (CDRH). Devices Database. Cochlear implants. Updated September 8, 2009b. Available at: 7. Jacot E, Van Den Abbeele T, Debre HR, et al. Vestibular impairments preand post-cochlear implant in children. Int J Pediatr Otorhinolaryngol. 2009; 73(2): American Academy of Audiology (AAA). Cochlear implants in children Available at: n.aspx References Update June Flipsen P Jr. Intelligibility of spontaneous conversational speech produced by children with cochlear implants: A review. Int J Pediatr Otorhinolaryngol Mar Jöhr M, Ho A, Wagner CS, Linder T. Ear Surgery in Infants Under One Year of Age: Its Risks and Implications for Cochlear Implant Surgery. Otol Neurotol Apr; 29(3): Gordon KA, Valero J, van Hoesel R, Papsin BC. Abnormal timing delays in auditory brainstem responses evoked by bilateral cochlear implant use in children. Otol Neurotol Feb; 29(2): Holt RF, Svirsky MA. An Exploratory Look at Pediatric Cochlear Implantation: Is Earliest Always Best? Ear Hear Mar Kirk KI, Firszt JB, Hood LJ, et al. New Directions in Pediatric Cochlear Implantation: Effects on Candidacy. American Speech-Language-Hearing Association (ASLHA) Available at: Cochlear Implants May 16 26

27 f061128a.htm 6. Connell SS, Balkany TJ, Hodges AV, et al. Electrode migration after cochlear implantation. Otol Neurotol Feb; 29(2): Shafer DN. Study Researches Bilateral Cochlear Implants. NIDCD Demonstrates Bilateral Implants Benefit Children Who Are Deaf Available at: 8. Use of Meningitis Vaccine in Persons with Cochlear Implants. Centers for Disease Control and Prevention. June 4, Available at: 9. Centers for Medicare & Medicaid Services (CMS). NCD for Cochlear Implant. (50.3)Updated August 20, Available at: ket=ncd%3a50%2e3%3a2%3acochlear+implantation 10. National Institute on Deafness and Other Communication Disorders (NIDCD). Cochlear implants. May Accessed Mar 25, Available at: Hopfenspirger MT, Levine SC, Rimell FL. Infectious complications in pediatric cochlear implants. Laryngoscope Oct; 117(10): Marschark M, Rhoten C, Fabich M. Effects of cochlear implants on children's reading and academic achievement. J Deaf Stud Deaf Educ Summer; 12(3): Epub 2007 May Côté M, Ferron P, Bergeron F, et al. Cochlear reimplantation: causes of failure, outcomes, and audiologic performance. Laryngoscope Jul; 117(7): Cullen RD, Fayad JN, Luxford Wm, et al. Revision cochlear implant surgery in children. Otol Neurotol Feb; 29(2): Neuman AC, Haravon A, Sislian N, et al. Sound-direction identification with bilateral cochlear implants. Ear Hear. 2007; 28(1): Colletti L. Beneficial auditory and cognitive effects of auditory brainstem implantation in children. Acta Otolaryngol Sep; 127(9): Wackym PA, Runge-Samuelson CL, Firszt JB, et al. More challenging speechperception tasks demonstrate binaural benefit in bilateral cochlear implant users. Ear Hear. 2007; 28(2 Suppl):80S-85S. 19. Peters BR, Litovcsky R, Parkinson A, et al. Importance of age and postimplantation experience on speech perception measures in children with sequential bilateral cochlear implants. Otol Neurotol. 2007; 28(5): Beijen JW, Snik AF, Mylanus EA. Sound localization ability of young children with bilateral cochlear implants. Otol Neurotol. 2007; 28(4): Tyler RS, Dunn CC, Witt SA, et al. Speech perception and localization with adults with bilateral sequential cochlear implants. Ear Hear. 2007; 28(2 Suppl):86S-90S. 22. Wolfe J, Baker S, Caraway T, et al. 1-year postactivation results for sequentially implanted bilateral cochlear implant users. Otol Neurotol. 2007; 28(5): Portmann D, Felix F, Negrevergne M, et al. Bilateral cochlear implantation in a patient with long-term deafness. Rev Laryngol Otol Rhinol (Bord). 2007; 128(1-2): Galvin KL, Mok M, Dowell RC. Perceptual benefit and functional outcomes for children using sequential bilateral cochlear implants. Ear Hear. 2007; 28(4): Cochlear Implants May 16 27

28 25. Grantham DW, Ashmead DH, Ricketts TA, et al. Horizontal-plane localization of noise and speech signals by postlingually deafened adults fitted with bilateral cochlear implants. Ear Hear. 2007; 28(4): Smith ZM, Delgutte B. Sensitivity to interaural time differences in the inferior colliculus with bilateral cochlear implants. J Neurosci. 2007; 27(25): Gordon KA, Valero J, Papsin BC. Binaural processing in children using bilateral cochlear implants. Neuroreport. 2007; 18(6): Murphy J, O'Donoghue G. Bilateral cochlear implantation: An evidence-based medicine evaluation. Laryngoscope. 2007; 117: Ching TY, van Wanrooy E, Dillon H. Binaural-bimodal fitting or bilateral implantation for managing severe to profound deafness: A review. Trends Amplif. 2007; 11(3): U.S. Food and Drug Administration (FDA). FDA public health notification: Importance of vaccination in cochlear implant recipients. Rockville, MD: FDA; October 10, Available at: cochlear.html. 31. Ali W, O'Connell R. The effectiveness of early cochlear implantation for infants and young children with hearing loss. New Zealand Health Technology Assessment (NZHTA); Battmer RD, O'Donoghue GM, Lenarz T. A multicenter study of device failure in European cochlear implant centers. Ear Hear Apr; 28(2 Suppl):95S- 99S. 33. Black IM, Bailey CM, Albert Dm, et al. The Great Ormond Street Hospital paediatric cochlear implant programme A review of surgical complications. Cochlear Implants Int Jun; 8(2): Brown KD, Balkany TJ. Benefits of bilateral cochlear implantation: a review. Curr Opin Otolaryngol Head Neck Surg Oct; 15(5): Buss E, Pillsbury HC, Buchman CA, et al. Multicenter U.S. bilateral MED-EL cochlear implantation study: speech perception over the first year of use. Ear Hear Jan; 29(1): Dettman SJ, Pinder D, Briggs RJ, Dowell RC, Leigh JR. Communication development in children who receive the cochlear implant younger than 12 months: risks versus benefits. Ear Hear Apr; 28(2 Suppl):11S-18S. 37. Dodson KM, Maiberger PG, Sismanis A... Intracranial complications of cochlear implantation. Otol Neurotol Jun; 28(4): Ertmer DJ, Young NM, Nathani S. Profiles of vocal development in young cochlear implant recipients. J Speech Lang Hear Res Apr; 50(2): Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol Apr; 6(4): Fink NE, Wang NY, Visaya J, Niparko JK, Quittner A, Eisenberg LS, Tobey EA; CDACI Investigative Team. Childhood Development after Cochlear Implantation (CDaCI) study: design and baseline characteristics. Cochlear Implants Int Jun; 8(2): References - Initial 1. Centers for Medicare and Medicaid Services. National Coverage Determination for Cochlear Implantation. NCD #50.3. Effective April 4, Accessed at: or Accessed August Cochlear Implants May 16 28

29 2. Litovsky R, Johnston PM, Godar S, et al. Bilateral cochlear implants in children: localization acuity measured with minimum audible angle. Ear Hear. 2006; 27 (1) Swedish Council on Technology Assessment in Health Care (SBU). Bilateral cochlear implantation (CI) in children (ALERT). Available at: Accessed August Biernath KR, Reefhuis J, Whitney CG, et al. Bacterial meningitis among children with cochlear implants beyond 24 months after implantation. Pediatrics 2006; 117: Wilson-Clark, SD, Squires, S, Deeks, S. Bacterial meningitis among cochlear implant recipients--canada, MMWR Morb Mortal Wkly Rep 2006; 55 Suppl 1: FDA Public Health Notification: Continued risk of bacterial meningitis in children with cochlear implants with a positioner beyond twenty-four months post-implantation, February 6, Available at: Accessed August Hayes Inc. Hayes Medical Technology Directory. Cochlear Implantation. Lansdale, PA: Hayes, Inc.; September Schoen F, Mueller J, Helms J, et al. Sound Localization and Sensitivity to Interaural Cues in Bilateral Users of the Med-El Combi 40/40+ Cochlear Implant System, Otology & Neurootology.2005; 26(3): Verschuur CA, Lutman ME, Ramsden R., et al. Auditory localization abilities in bilateral cocklear implant recipients. OtolNeurotol.2005; 26(5): Centers for Medicare and Medicaid Services (CMS). Decision memo for cochlear implantation (CAG-00107N). National Coverage Analyses. Baltimore, MD: CMS; April 4, Available at: Accessed August Ramsden R. Prognosis after cochlear implantation. BMJ 2004; 328: (21 February, 2004), doi: /bmj Summerfield AQ, Marshall DH. United Kingdom Cochlear Implant Study Group. (2004). Criteria of candidacy for unilateral cochlear implantation in post-lingually deafened adults III: Prospective evaluation of an actuarial approach to defining a criterion. Ear and Hearing, 25: Copeland BJ, Pillsbury HC 3rd. Cochlear implantation for the treatment of deafness. Annual Rev Med. 2004; 55: Kuhn-Inacker H, Shehata-Dieler W, Muller J, et al. Bilateral cochlear implants: a way to optimize auditory perception abilities in deaf children? Int Jl Pediatr Otorhinolaryngol.2004; 68(10): Laszig R, Aschendorff A, Stecker M, et al. Benefits of bilateral electrical stimulation with the nucleus cochlear implant in adults: 6-month postoperative results. Otol Neurotol. 2004; 25(6): Centers for Disease Control and Prevention (CDC). Use of vaccines for the prevention of meningitis in persons with cochlear implants. Fact Sheet for Health Care Professionals. Atlanta, GA: CDC; July 31, 2003 (previously published October 2002). Available at: Accessed August Van Hoesel RJ. Exploring the benefits of bilateral cochlear implants. Audiol Neurootol. 2004; 9(4): Chatelin V, Kim EJ, Driscoll C, et al. Cochlear implant outcomes in the elderly. Otol Neurotol (2004) 25: pp Cochlear Implants May 16 29

30 19. McConkey RA, Burton KD, Osberger MJ, et.al. Effect of Age at Cochlear Implantation on Auditory Skill Development in Infants and Toddlers. Archives of Otolaryngol Head Neck Surg. 2004; 130: Cruickshanks KJ, Tweed TS, Wiley TL, et al. The 5-year incidence and progression of hearing loss. Arch Otolaryngol Head Neck Surg 2003; 129: Tyler R, Dunn CC, Witt SA, et al. Update on bilateral cochlear implantation. Curr Opin Otolaryngol Head Neck Surg. 2003; 11(5): Clopton BM, Spelman FA. Technology and the future of cochlear implants. Ann Otol Rhinol Laryngol Suppl. 2003; 191: Reefhuis J, Honein MA, Whitney CH, et al. Risk of bacterial meningitis in children with cochlear implants. NEJM. 2003; 349(5): Wilson BS, Lawson DT, Muller JM, et al. Cochlear implants: Some likely next steps. Annu Rev Biomed Eng. 2003;5: De Vries CS. Cochlear implants in adults. Bazian, Ltd, eds. London, UK: Wessex Institute for Health Research and Development, University of Southampton; 2003: Pichon RA, Augustovski F, Cernadas C, et al. Safety and efficacy of cochlear implants. Technology Assessment. Buenas Aires, Argentina: Institute for Clinical Effectiveness and Health Policy (IECS); September Long CJ, Eddington DK, Colburn HS, et al. Binaural sensitivity as a function of interaural electrode position with a bilateral cochlear implant user. J Acoust Soc Am 2003; 114(3): Au DK, Hui Y, Wei WI. Superiority of bilateral cochlear implantation over unilateral cochlear implantation in tone discrimination in Chinese patients. Am J Otolaryngol 2003; 24(1): Vermeire K, Brokx JP, Van de Heyning PH, et al. Bilateral cochlear implantation in children. Int J Pediatr Otorhinolaryngol 2003; 67(1): Van Hoesel RJ, Tyler RS. Speech perception, localization, and lateralization with bilateral cochlear implants. J Acoust Soc Am 2003; 113(3): Pneumococcal vaccination for cochlear implant candidates and recipients: updated recommendations of the Advisory Committee on Immunization Practices. MMWR Morb Mortal Wkly Rep 2003; 52: Yueh B, Shapiro N, MacLean CH, et al. Screening and management of adult hearing loss in primary care. JAMA 2003; 289: COCHLEAR IMPLANTS Tyler RS, Gantz BJ, Rubinstein JT, et al. Three-month results with bilateral cochlear implants. Ear Hear. 2002; 23(1 Suppl):80S-89S. 34. Van Hoesel R, Ramsden R, Odriscoll M. Sound-direction identification, interaural time delay discrimination, and speech intelligibility advantages in noise for a bilateral cochlear implant user. Ear Hear. 2002; 23(2): Sargent EW. Cochlear implant: Indications. emedicine J. 2002; 3(6). Available at: Accessed August Smosky WJ. Speech audiometry. emedicine J. 2001:2(7). Available at: Accessed August, Thai-Van H, Gallego S, Truy E et al. Electrophysiological findings in two bilateral cochlear implant cases: does the duration of deafness affect electrically evoked auditory brain stem responses? Ann Otol Rhinol Laryngol 2002; 111(11): Schon F, Muller J, Helms J. Speech reception thresholds obtained in a symmetrical four-loudspeaker arrangement from bilateral users of MED-EL cochlear implants. Otol Neurotol 2002; 23(5): Cochlear Implants May 16 30

31 39. Muller J, Schon F, Helms J. Speech understanding in quiet and noise in bilateral users of the MED-EL COMBI 40/40+ cochlear implant system. Ear Hear 2002; 23(3): Gantz BJ, Tyler RS, Rubinstein JT, et al. Binaural cochlear implants placed during the same operation. Otol Neurotol 2002; 23(2): Truy E, Ionescu E, Ceruse P, et al. The binaural digisonic cochlear implant: surgical technique. Otol Neurotol 2002; 23(5): Balkany TJ., Hodges AV, Eshraghi AA, et al. Cochlear implants in children a review. Acta Otolaryngol (2002) 122: pp Pneumococcal vaccination for cochlear implant recipients. MMWR Morb Mortal Wkly Rep 2002; 51:931. Yueh B. Souza P.E. McDowell J.A. et al. Randomized trial of amplification strategies. Arch Otolaryngol Head Neck Surg (2001) 127: pp Di Nardo W, Fetoni A, Buldrini S, et al. Auditory brainstem and cochlear implants: functional results obtained after one year of rehabilitation. Eur Arch Otorhinolaryngol. 2001; 258(1): Krabbe PF, Hinderink JB, van den Broek P. The effect of cochlear implant use in postlingually deaf adults. Int J Technol Assess Health Care. 2000; 16(3): Faber CE, Grontved AM. Cochlear implantation and change in quality of life. Acta Otolaryngol Suppl. 2000; 543: Waltzman SB, Scalchunes V, Cohen NL. Performance of multiply handicapped children using cochlear implants. Am J Otol. 2000; 21(3): Mitchell TE, Psarros C, Pegg P, et al. Performance after cochlear implantation: a comparison of children deafened by meningitis and congenitally deaf children. J Laryngol Otol 2000; 114: Mawman DJ, Ramsden RT, O'Driscoll M, et al. Bilateral cochlear implantation-- a case report. Adv Otorhinolaryngol 2000; 57: O'Donoghue GM, Nikolopoulos TP, Archbold SM. Determinants of speech perception in children after cochlear implantation. Lancet 2000; 356: Niparko JK, Kirk KI, Mellon NK, et al. Cochlear implant principles and practice. Philadelphia: Lippincott Williams and Wilkins, Odabasi O, Mobley SR, Bolanos RA, et al. Cochlear implantation in patients with compromised healing. Otolaryngol Head Neck Surg (2000) 123: pp Lantsov A, et al. Rehabilitation and assessment of aural-oral speech development in children with cochlear implants. Vestn Otorinolaringol (3):6-12, Cheng A.K. et al. Cochlear Implants for Deaf Children Improve Quality of Life and are Cost Effective. Journal of the American Medical Association. August 16, Rizer FM, Burkey JM, Cochlear implantation in the very young child. Otolaryngol Clin North Am. 1999; 32(6): Balkany T.J., Hodges A.V., Gomez-Marin O.et al. Cochlear reimplantation. laryngoscope (1999) 106: pp Lawson DT, Wilson BS, Zerbi M et al. Bilateral cochlear implants controlled by a single speech processor. Am J Otol 1998; 19(6): Archbold S, O'Donoghue G, Nikolopoulos T. Cochlear implants in children: an analysis of use over a three year period. Am J Otol 1998; 19: ? 59. Fryauf-Bertschy H, Tyler R.S. Kelsay DM, et al. Cochlear Implant Use by Prelingually Deafened Children. The Influences of Age at Implant and Length of Device Use. Journal of Speech, Language, and Hearing Research Vol February Cochlear Implants May 16 31

32 60. Gates GA, Daly K, and Dichtal WJ, et al. National Institutes of Health: Consensus Development Conference statement: cochlear implants in adults and children. Bethesda (MD), May 15 17, Important Notice General Purpose. Health Net's National Medical Policies (the "Policies") are developed to assist Health Net in administering plan benefits and determining whether a particular procedure, drug, service or supply is medically necessary. The Policies are based upon a review of the available clinical information including clinical outcome studies in the peer-reviewed published medical literature, regulatory status of the drug or device, evidence-based guidelines of governmental bodies, and evidence-based guidelines and positions of select national health professional organizations. Coverage determinations are made on a case-by-case basis and are subject to all of the terms, conditions, limitations, and exclusions of the member's contract, including medical necessity requirements. Health Net may use the Policies to determine whether under the facts and circumstances of a particular case, the proposed procedure, drug, service or supply is medically necessary. The conclusion that a procedure, drug, service or supply is medically necessary does not constitute coverage. The member's contract defines which procedure, drug, service or supply is covered, excluded, limited, or subject to dollar caps. The policy provides for clearly written, reasonable and current criteria that have been approved by Health Net s National Medical Advisory Council (MAC). The clinical criteria and medical policies provide guidelines for determining the medical necessity criteria for specific procedures, equipment, and services. In order to be eligible, all services must be medically necessary and otherwise defined in the member's benefits contract as described this "Important Notice" disclaimer. In all cases, final benefit determinations are based on the applicable contract language. To the extent there are any conflicts between medical policy guidelines and applicable contract language, the contract language prevails. Medical policy is not intended to override the policy that defines the member s benefits, nor is it intended to dictate to providers how to practice medicine. Policy Effective Date and Defined Terms. The date of posting is not the effective date of the Policy. The Policy is effective as of the date determined by Health Net. All policies are subject to applicable legal and regulatory mandates and requirements for prior notification. If there is a discrepancy between the policy effective date and legal mandates and regulatory requirements, the requirements of law and regulation shall govern. * In some states, prior notice or posting on the website is required before a policy is deemed effective. For information regarding the effective dates of Policies, contact your provider representative. The Policies do not include definitions. All terms are defined by Health Net. For information regarding the definitions of terms used in the Policies, contact your provider representative. Policy Amendment without Notice. Health Net reserves the right to amend the Policies without notice to providers or Members. In some states, prior notice or website posting is required before an amendment is deemed effective. No Medical Advice. The Policies do not constitute medical advice. Health Net does not provide or recommend treatment to members. Members should consult with their treating physician in connection with diagnosis and treatment decisions. No Authorization or Guarantee of Coverage. The Policies do not constitute authorization or guarantee of coverage of particular procedure, drug, service or supply. Members and providers should refer to the Member contract to determine if exclusions, limitations, and dollar caps apply to a particular procedure, drug, service or supply. Policy Limitation: Member s Contract Controls Coverage Determinations. Statutory Notice to Members: The materials provided to you are guidelines used by this plan to authorize, modify, or deny care for persons with similar illnesses or conditions. Specific care and treatment may vary depending on individual need and the benefits covered under your contract. The determination of coverage for a particular procedure, drug, service or supply is not based upon the Policies, but rather is subject to the facts of the individual clinical case, terms and conditions of the member s contract, and requirements of applicable laws and regulations. The contract language contains specific terms and conditions, including pre-existing conditions, limitations, exclusions, benefit maximums, eligibility, and other relevant terms and conditions of coverage. In the event the Member s contract (also known as the benefit contract, coverage document, or evidence of coverage) conflicts with the Policies, the Member s contract shall govern. The Policies do not replace or amend the Member s contract. Cochlear Implants May 16 32

33 Policy Limitation: Legal and Regulatory Mandates and Requirements The determinations of coverage for a particular procedure, drug, service or supply is subject to applicable legal and regulatory mandates and requirements. If there is a discrepancy between the Policies and legal mandates and regulatory requirements, the requirements of law and regulation shall govern. Reconstructive Surgery CA Health and Safety Code requires health care service plans to cover reconstructive surgery. Reconstructive surgery means surgery performed to correct or repair abnormal structures of the body caused by congenital defects, developmental abnormalities, trauma, infection, tumors, or disease to do either of the following: (1) To improve function or (2) To create a normal appearance, to the extent possible. Reconstructive surgery does not mean cosmetic surgery," which is surgery performed to alter or reshape normal structures of the body in order to improve appearance. Requests for reconstructive surgery may be denied, if the proposed procedure offers only a minimal improvement in the appearance of the enrollee, in accordance with the standard of care as practiced by physicians specializing in reconstructive surgery. Reconstructive Surgery after Mastectomy California Health and Safety Code requires treatment for breast cancer to cover prosthetic devices or reconstructive surgery to restore and achieve symmetry for the patient incident to a mastectomy. Coverage for prosthetic devices and reconstructive surgery shall be subject to the copayment, or deductible and coinsurance conditions, that are applicable to the mastectomy and all other terms and conditions applicable to other benefits. "Mastectomy" means the removal of all or part of the breast for medically necessary reasons, as determined by a licensed physician and surgeon. Policy Limitations: Medicare and Medicaid Policies specifically developed to assist Health Net in administering Medicare or Medicaid plan benefits and determining coverage for a particular procedure, drug, service or supply for Medicare or Medicaid members shall not be construed to apply to any other Health Net plans and members. The Policies shall not be interpreted to limit the benefits afforded Medicare and Medicaid members by law and regulation. Cochlear Implants May 16 33