A cochlear implant is intended to restore a level of auditory sensation to an individual with severe to profound sensorineural hearing loss by means of electrical stimulation of the auditory nerve. 

An auditory brainstem implant is a device designed to restore some hearing in an individual with neurofibromatosis type 2 (NF-2) rendered deaf by the surgical removal of neurofibromas involving both auditory nerves. 

This document addresses cochlear implants, auditory brainstem implants, and replacement or upgrade of speech processor and controller components. This document does not address replacement parts other than as specifically described below.

Cochlear Implants

Medically Necessary:

Unilateral or bilateral implantation of a U.S. Food and Drug Administration (FDA) approved single or multi-channel cochlear implant is considered medically necessary in an individual with bilateral severe-to-profound pre- or postlingual hearing loss (sensorineural deafness), defined as a hearing threshold of 70 decibels (dB) or greater, when all of the following criteria are met:

1. The individual, including those with hearing loss due to meningitis, cannot benefit from conventional hearing devices; and
2. The individual is free from lesions in the auditory nerve and acoustic areas of the central auditory pathway (nervous system); and
3. The individual is free from otitis media or other active middle ear infections; and
4. The individual is able to participate in a post-cochlear rehabilitation program in order to achieve benefit from the cochlear implant device.

Upgrade to or replacement of an existing external speech processor, controller or speech processor and controller (integrated system) is considered medically necessary for an individual whose response to existing components is inadequate to the point of interfering with the activities of daily living or when components are no longer functional.

Not Medically Necessary:

Upgrade to or replacement of an existing external speech processor, controller or speech processor and controller (integrated system) is considered not medically necessary when the criteria specified above are not met or when requested for convenience or to upgrade to a newer technology when the current components remain functional.

Investigational and Not Medically Necessary:

A cochlear implant is considered investigational and not medically necessary for all other indications when the above criteria are not met.

Auditory Brainstem Implants

Medically Necessary:

An FDA-approved auditory brainstem implant (ABI) is considered medically necessary in an individual when all of the following criteria are met:

1. Is 12 years of age or older; and
2. Diagnosed with neurofibromatosis type 2; and
3. Is completely deaf as a result of bilateral neurofibromas of the auditory nerve.

Upgrade to or replacement of an existing external sound processor, remote assistant or both components is considered medically necessary for an individual whose response to existing components is inadequate to the point of interfering with the activities of daily living or when components are no longer functional.

Not Medically Necessary:

Upgrade to or replacement of an existing external sound processor, remote assistant, or both components is considered not medically necessary when the criteria specified above are not met or when requested for convenience or to upgrade to a newer technology when the current components remain functional.

Investigational and Not Medically Necessary:

An auditory brainstem implant is considered investigational and not medically necessary for all other indications when the above criteria are not met.

Cochlear Implants

Cochlear implants are recognized as an effective treatment of sensorineural deafness. While use of a unilateral cochlear implant in an individual with severe to profound hearing loss has become a standard clinical practice, bilateral implantation has been less common. Evolution of cochlear implant devices has focused on minimizing the internally implanted electrodes, such that one device, the Nucleus^® 24 (Cochlear Americas, Englewood, CO), received FDA approval (2000) for use in children 12 months of age and older. A review of the early peer-reviewed literature includes several reports on individuals with bilateral cochlear implants (Long, 2003; Muller, 2002; Schoen, 2005; Tyler, 2002; van Hoesel, 2002; van Hoesel, 2003). These early reports evaluated small numbers of individuals and provided limited outcome information. In these reports, most, but not all, individuals reported very slight to modest improvements in sound localization and speech intelligibility with bilateral cochlear implants, especially with noisy backgrounds, but not necessarily in quiet environments. When reported, the combined use of binaural stimulation improved hearing by only a few decibels or percentage points. This improvement appeared marginal, and may not outweigh the significant risks of a second implantation. In addition, similar binaural results can be achieved with a contralateral hearing aid, assuming the contralateral ear has speech recognition ability (Morera, 2005).

Subsequently, a number of prospective case series designed to assess whether bilateral cochlear implantation (CI) could provide some of the benefits of binaural hearing were published in the peer-reviewed literature. These studies assessed the benefits of bilateral CI on sound localization and speech perception in both adults (Gantz, 2002; Laszig, 2004; Litovsky, 2004; Schoen, 2005; Verschuur, 2005), and, to a lesser extent, in children (Kuhn-Inacker, 2004; Litovsky, 2006). The largest and most complete of these case studies included a case series of 30 U.S. children with sequentially placed bilateral CI (Peters, 2007).

Bond and colleagues (2009) authored a technology assessment in the U.K. to investigate the clinical and cost-effectiveness of unilateral CI (using or not using hearing aids), and bilateral CI with a single CI (unilateral or unilateral plus hearing aid) for severely to profoundly deaf children and adults. The clinical effectiveness review included 33 papers, of which two were randomized controlled trials (deaf children [n= 1,513] and adults [n= 1,379]). They used 62 different outcome measures and overall evidence was of moderate to poor quality. All studies in children comparing one CI with non-technological support or an acoustic hearing aid reported gains on all outcome measures. Weak evidence shows greater gain from earlier implantation (prior to starting school). The strongest evidence for an advantage from bilateral over unilateral implantation was for understanding speech in noisy conditions. The comparison of bilateral with unilateral CI plus an acoustic hearing aid was limited by small sample sizes and poor reporting. The authors concluded, "Unilateral cochlear implantation is safe and effective for adults and children and likely to be cost-effective in profoundly deaf adults and profoundly and prelingually deaf children. There are likely to be overall additional benefits from bilateral implantation, enabling children and adults to hold conversations more easily in social situations."

Cochlear Implantation in Adults

In 2002, Gantz and colleagues evaluated bilateral CI in ten adults with severe to profound, postlingual hearing loss. These adults were evaluated for monaural CI, found to have different duration of deafness and tone thresholds in each ear, and received bilateral CI in a single surgical procedure. Age of onset of deafness was not reported.  Follow-up at one year showed significantly better performance under the "binaural condition" versus either monaural condition for sound localization in all the adults. Five of the ten adults showed better performance in speech perception in quiet and in noise. These findings, without a study control group, provided an initial indication that compared with unilateral CI, bilateral CI may allow significant improvement in sound localization and speech perception. 

Following the Gantz study, Litovsky and colleagues (2004) examined 14 adults with postlingual deafness and three adults with prelingual deafness who received bilateral CI. Sound localization and speech perception tests were completed three months after bilateral CI activation, with sound localization reported as significantly improved in 15 adults (88%) compared with monaural testing. Speech perception was improved in six adults (43%). Limitations of this study include the small sample size and duration of follow-up of only three months, which may have been insufficient to identify the scope and degree of benefit. It is not reported as to how the adults were selected for participation in this case series. Cause of deafness and exclusion criteria were not reported. In a multi-center trial, Laszig and colleagues (2004) studied 37 postlingually deafened adults, 22 with simultaneous bilateral CI and 15 with sequentially implanted CI. Serial auditory tests were carried out at one, three, and six months with each adult serving as his or her own control. Serial assessments of unilateral and bilateral listening performance on tests of speech comprehension and sound localization were performed. Mean speech perception performance scores at six months with the speech signal closest to the better ear and the noise signal closest to the poorer ear resulted in a mean unilateral (better ear) score of 62% and mean bilateral score of 70%. Although modest (8%), this bilateral listening advantage did reach statistical significance. However, during testing in a quiet environment, there was no difference in mean speech perception scores between the best ear monaural condition and binaural listening condition. The binaural localization ability was superior to monaural localization ability for all but one adult. Binaural localization was significantly superior to the monaural condition (p<0.0005), with localization error reduced by 38 degrees compared with unilateral stimulation (88 degrees compared to 50 degrees). The methods by which these adults were selected from tertiary referral centers with cochlear implant programs were not reported. All of the adults received the Nucleus^® 24 cochlear implant, but there was variation in the type of speech processor and speech coding strategy.

In 2005, Schoen and colleagues retrospectively studied 12 adults with postlingual hearing loss who received bilateral CI in a single surgical procedure (n=3) or in two sequential procedures (n=9). Eleven of the adults were tested for sound localization at three months, with the location of sound source significantly improved to near normal accuracy. The study subjects were selected from a group of 14 adults bilaterally implanted in Wuerzburg, Germany prior to 2002. No other information is provided with regard to participant selection criteria for this case study.

Similar results were reported in a case series (n=20) of post-lingually deafened adults who participated in a United Kingdom (U.K.) multi-center trial (Verschuur, 2005). The adults were recruited from a group using either the Nucleus 24M^® or 24K^® cochlear implant with at least nine months experience with using the second device. Additional criteria for inclusion in the study were duration of severe deafness no longer than 15 years in either ear, 30% speech recognition in the first ear, minimal benefit from a conventional hearing aid in the non-implanted ear, and a "high level of motivation for bilateral implantation." Mean duration of deafness was nine years; mean gap between the first and second CI was 35 months. The adults were tested between three and nine months after initial tuning of the second device. This study suggested that bilateral CI provides significant improvement in sound localization ability compared with unilateral CI in all subjects regardless of sound source location. Mean localization error with bilateral implants was 24 degrees compared with 67 degrees for monaural CI and two degrees for normal hearing controls. In this study, binaural sound localization performance was better than monaural performance for all subjects, for all stimulus types, and for different sound sources.

A multicenter prospective study by Litovsky, Parkinson, and colleagues (2006) reported on 37 adults with post-linguistic bilateral hearing loss. Bilateral benefit (speech understanding in quiet and noise) was seen in 32 of the 34 individuals. The authors suggested that the three dB improvement in signal to noise ratio noted in the study would result in an average improvement of 28% in speech understanding and that this improvement could be crucial. Questionnaire data indicated that bilateral users perceived their performance to be better than when using a single device. Ricketts and colleagues (2006) reported on 16 adults with similar postlinguistic bilateral hearing loss. They found a small but significant advantage with bilateral implants for speech recognition in noise. While a training effect was noted over time for a subset of adults followed up to 17 months, a consistent bilateral advantage was noted. Ramsden and colleagues (2005) studied 30 adults in England who received their second implant a mean of three years after the first. At nine months, a significant (12.6%, p< 0.001) binaural advantage was seen for speech and noise from the front. They were not able to predict if the second ear would be the better performer. Sequential implantation with long delays between ears resulted in poor second ear performance for some of their subjects. When viewed together, these small retrospective case studies of post-lingually deafened adults suggest that bilateral CI may have the potential to improve sound localization in most adults and may improve speech perception in others when compared with unilateral CI. 

Cochlear Implantation in Children Twelve Months of Age and Older

Until recently, the peer-published evidence was limited, to a greater extent than for adults, concerning the use of bilateral CI in children. It has been demonstrated that children with single-sided deafness experience far greater difficulty in school, are ten times more likely to fail a grade or need tutoring, and are twice as likely to exhibit behavioral difficulties in the classroom (Bess, 1986). These children have difficulty with speech recognition in a noise environment. A child with mild to moderate hearing loss needs speech to be 20 to 30 dB louder than background noise (signal-to noise ratio of plus 20 to plus 30 dB) for optimal speech understanding (Gengel, 1971) while the typical classroom has a signal to noise ratio of only plus five dB. It has been theorized that bilaterally implanted children with the benefits of binaural hearing would perform better in noise environments.

The first significant study of bilateral CI in children was reported by Kuhn-Inacker and colleagues (2004). This study described the auditory skills in a heterogeneous group of 32 children enrolled in a German cochlear rehabilitation program following the placement of bilateral CI. Age at first implant ranged from eight months to 16 years, with age at second implant ranging from one year to 16 years. Ten children had their second implant within one year following the first CI. Four children had simultaneous implants. Sixteen children were postlingually implanted with the second ear after acquiring basic speech perception. Two children were prelingually deafened due to meningitis and 14 had limited speech perception when they received their second CI. Eighteen children were tested with speech audiometry. The participant selection criteria applied to form this study group were not reported. Results suggested that children reached a higher word discrimination score (WDS percent) in quiet with the bilateral CI condition than with monaural CI. WDS for bilateral CI was 86%, left CI 75% and right CI 72% (p< 0.05). There was significant improvement in speech discrimination in noise with bilateral CI compared to unilateral CI. The WDS difference between binaural and monaural conditions in speech discrimination in noise WDS was 18% during the first test session and 14% during second test session (p<0.001). Using linear regressions analysis, the time gap between both implants and the age at first implantation did not affect speech discrimination in noise test results.

A number of studies relevant to the benefits and outcomes of bilateral CI in children have been published since 2004. Litovsky, Johnstone, and colleagues (2006) reported that nine of 13 (70%) children with bilateral CI discriminated source separations of equal to or less than 20 degrees and seven of nine children performed better when using bilateral (vs. unilateral) devices. Sharma and Dorman (2005) reported on auditory development in 23 children with unilateral or bilateral devices. In one child who received a bilateral device with later (after age seven) implantation of the second ear, the auditory responses in the second device were similar to that seen in "late-implanted" children. Sharma and Dorman (2006) subsequently studied congenitally deaf children to establish the existence and time limits of a sensitive period for the development of central auditory pathways in humans. The investigators reported that central auditory pathways are "maximally plastic" for a period of about three and one-half years in children with cochlear implants. Stimulation delivered within this timeframe results in auditory evoked potentials that reach normal values in three to six months. However, when stimulation occurs after seven years, changes occur within one month, but then have little to no subsequent change.

Peters and colleagues (2007) presented significant study results of bilateral CI in children at an annual meeting of the American Otological Society. Thirty children, ages three years to 13 years, were bilaterally implanted with sequential surgeries at least six months apart. All children received their first cochlear implant prior to five years of age and "possessed the necessary speech and cognitive skills to complete the speech tests." Other requirements for enrollment were based on speech measures obtained while the children were using the single implant. These included the Multi-syllabic Lexical Neighborhood Test (MLNT in children less than five years) or the Lexical Neighborhood Test (LNT in children greater than five years) score greater than 30% at 70 dB in the first implanted ear, severe to profound hearing loss in the second ear, participation in a rehabilitation program, and normal cochlear anatomy determined by radiographic measures. Children acquired speech perception in the second ear within six months. However, children under eight years of age acquired speech perception in the second ear more rapidly and ultimately gained a higher level of speech understanding than older children. These findings were believed to support the premise of a more "plastic" auditory system at a younger age. Speech perception testing in quiet and noise was performed preoperatively and again post activation at three, six, and twelve months in both the unilateral and bilateral conditions. Some older children were also tested at 24 months. The authors reported that sequentially implanted children in all age groups showed better mean speech perception scores in background noise with bilateral CI than with a single implant. Speech performance in quiet was improved to a lesser degree, but this difference did not meet a level of statistical significance. In younger children, speech perception scores improved for 12 months following the second implant while scores for older children plateaued at six months. The rate of improvement in speech perception scores in the second ear was inversely related to the child's age at the time of the second implant.

Other studies have reported benefits for both adults and children with a unilateral CI with hearing aid in the opposite ear. Ching and colleagues (2004) reported on 21 adults who used unilateral cochlear implant and a contralateral hearing aid. Binaural benefits were seen for at least one measure for these adults; measures included speech recognition, sound localization, and functional performance. A subsequent study by Ching and colleagues (2006) reported on 29 children and 21 adults with unilateral CI and a contralateral hearing aid. The authors noted that both children and adults derived binaural advantages related to sentence perception in noise and localized sound that was better with bilateral CI. In another report, Holt and colleagues (2005) concluded that children with severe hearing loss who used a unilateral CI benefited from wearing an appropriately-fitted hearing aid in the nonimplanted ear to maximally benefit from bilateral stimulation.

Cochlear Implantation in Children Younger than Twelve Months of Age

Recent studies and developments in clinical practice have occurred with the advent of universal neonatal hearing screening in some countries and the availability of screening programs for at-risk infants in other countries, including earlier referral, diagnosis, and intervention for infants with hearing loss. Improvements in device technology, over two decades of pediatric clinical experience, and a growing recognition of the efficacy of CI have led to increasing numbers of young children receiving CI. There are multiple small retrospective studies and small and large case series which suggest improved health outcomes by several objective measures in children implanted before 12 months of age. There is some data to suggest that earlier implantation leads to improved language acquisition during this critical period of development of spoken and aural language skills; it has been shown that the age at the time of auditory stimulation is a strong predictor of auditory and language outcomes (Harrison, 2005; Miyamoto, 2005; Nicholas, 2006; Sharma, 2005; Tomblin, 2005). Cumulatively, these reports and an increase in recent evidence suggest that CI can be performed safely and successfully without serious complications in select children younger than 12 months of age with profound bilateral sensorineural deafness (Colletti, 2005b; Colletti, 2009; Dettman, 2007; Holt and Svirsky, 2008; Loundon, 2010; Roland, 2009; Tait, 2007; Valencia, 2008; Vlastarakos, 2010b; Waltzman and Roland, 2005).  

In addition to information about children with congenital deafness, there are several small prospective and retrospective case reviews and a matched pairs analysis suggesting that children with profound bilateral sensorineural deafness secondary to bacterial meningitis may benefit from early CI (El-Kashlan, 2003; Hehar, 2002; Johnson, 1995; Young, 2000). Philippon and colleagues (2010) performed a descriptive analysis of data, including the cause of meningitis, preoperative imaging evaluation, age at implantation, time lapse between meningitis and implantation, and relevant surgical findings, in an attempt to propose guidelines for the management of profound bilateral sensorineural hearing loss after bacterial meningitis. The mean age at implantation was three years, eight months; the mean time delay between meningitis and surgery was two years, one month for the children. Eighteen children (67%) were implanted within a year. Labyrinthitis ossificans (LO) was evidenced at the time of surgery in 62% of the total study subjects (children and adults). Open-set speech discrimination was achieved by 37% of the children (10 of 27). The authors recommended early CI for individuals with bilateral profound deafness secondary to bacterial meningitis as an interventionist approach to avoid complications presented by LO and to optimize hearing outcomes.

Peters and colleagues (2010) recently reported on worldwide trends with bilateral CI from data collected via a 59 question online electronic survey of 25 clinics (considered as centers of excellence) representing experience with CI in over 23,000 users. Cumulatively to the end of 2007, 70% of all bilateral CI surgeries occurred in children, representing 26% under three years of age. Children less than three years of age also represented the only age group of all individuals in which simultaneous surgeries predominate (58%).

Vlastarakos and colleagues (2010a) conducted a meta-analysis of prospective controlled studies, prospective and retrospective cohort studies, clinical guidelines, and review articles reporting the diagnostic challenges and safety considerations in CI in 125 children younger than 12 months of age. Overall, no major anesthetic complication was reported; the rate of surgical complications was reported at 8.8% (3.2% major complications), similar to the respective complication rates in older implanted children (major complications ranging from 2.3 to 4.1%). The authors summarized that CI can be performed in otherwise healthy infants, provided that the attending pediatric anesthesiologist is considerably experienced and appropriate facilities of pediatric perioperative care are readily available. A number of concerns, with regard to anatomic constraints, existing co-morbidities or additional disorders, tuning difficulties, and special phases of the developing child should be also taken into account. The meta-analysis did not find an increased rate of anesthetic or surgical complications in implanted infants, although it identified a lack of studies with long-term follow-up reporting improved health outcomes.

In summary, given the strong evidence of benefit in children with profound hearing loss when provided with a CI after the age of 12 months, the additional evidence suggesting further benefit at ages less than 12 months, and the lack of evidence that performing CI at an earlier age is harmful, it is reasonable to perform CI in children under 12 months of age.

Auditory Brainstem Implants (ABI)

The FDA approval of the Nucleus^® 24 Auditory Brain Stem Implant System (Cochlear Americas, Englewood, CO) was based on results of a case series of 90 individuals (Donaldson, 2001). Of the 90 individuals evaluated, 28 complications occurred in 26 individuals; 26 of these complications resolved without surgical or extensive medical intervention. Two individuals had infections of the postoperative flap requiring explantation of the device. Effectiveness outcomes were evaluated in 60 individuals with a minimum experience of three to six months with the device. Device benefit was defined as a significant enhancement of lip-reading or an above-chance improvement on sound-alone tests. Based on this definition, a total of 95% (57 of 60) derived benefit from the device. While the use of an ABI is associated with a modest improvement in hearing, this level of improvement is considered significant in this group of individuals with no other treatment options. Among the 90 individuals receiving the implant, 16 did not receive auditory stimulation from the device postoperatively, either due to migration of the implanted electrodes or surgical misplacement. To place the electrode array on the surface of the cochlear nucleus, the surgeon must be able to visualize specific anatomical landmarks. Because large neurofibromas compress the brainstem and distort the underlying anatomy, it may be difficult or impossible for the surgeon to correctly place the electrode array. For this reason, individuals with large, longstanding tumors may not benefit from the device.

Colletti and colleagues (2005a) presented data from ABI in 16 children and adults who had non-tumor diseases of the cochlear nerve or cochlea and 13 individuals with neurofibromatosis type 2 (NF-2). Ages of individuals ranged from 14 months to 70 years, the non-tumor group included individuals with head trauma, complete ossification, one child with auditory neuropathy, and five children with bilateral cochlear nerve aplasia. Following implantation, the adult non-tumor group scored substantially higher than the NF-2 group in open set speech perception tests, some of the children showed dramatic improvements in word and sentence recognition over a one year follow-up. Short-term side effects included dizziness or tingling sensations in the leg, arm, and throat (20 of 29 individuals). The investigators suggested that ABIs hold promise for individuals with cochlear and cochlear nerve abnormalities when cochlear implants are not indicated. Studies from Europe report improvement in hearing with these devices in "non-tumor" individuals. L. Colletti (2007) reported results on 22 non-neurofibromatosis individuals and V. Colletti (2006) reported results on 14 post-surgically removed NF-2 individuals and 25 non-tumor individuals who were fitted with an ABI. In the latter study, the non-tumor individuals had cochlear or cochlear nerve injuries or malfunctions including bilateral labyrinthine fractures, bilateral temporal bone fracture, incomplete cochlear partition (Type II or Mondini malformations) and alterations in cochlear patency (e.g. complete bilateral cochlear ossification, cochlear derangement due to meningitis, otosclerosis, and autoimmune diseases). Colletti (2006) reported better outcomes in the non-tumor group compared to NF-2 group when evaluated with a series of "psychophysical" tests. This study, however, is limited in drawing conclusions as to the benefit of an ABI in non-tumor individuals due to the small sample size and heterogeneous composition of the study population. In addition, the Nucleus^® 24 Auditory Brain Stem Implant has received FDA approval only for individuals with NF-2 following tumor removal.

Cochlear Implants

A CI provides direct electrical stimulation to the auditory nerve, bypassing the usual transducer cells that are absent or nonfunctional in deaf cochlea. The basic components of a CI include both external and internal components. The external components include a microphone headset, an external sound processor, and an external transmitter/audio input selector. The internal components are implanted surgically and include an internal receiver implanted within the temporal bone and an electrode array that extends from the receiver into the cochlea through a surgically created opening in the round window of the middle ear. The microphone picks up sounds that are carried to the external sound processor and transformed into coded signals that are then transmitted transcutaneously to the implanted internal receiver. The receiver converts the incoming signals to electrical impulses, which are conveyed to the electrode array, ultimately resulting in stimulation of the auditory nerve.

Once an individual is referred to a cochlear implant center, the implant team will perform additional testing to determine whether or not the individual is a suitable candidate for CI. This testing includes audiologic testing, psychological testing, medical examination, and additional tests performed by the surgeon. An otolaryngologist examines the ear canal and middle ear to ensure that no active infection or other abnormality precludes the implant surgery; a physical examination identifies any potential problems with the use of general anesthesia needed for the implant procedure. The process often involves evaluation using radiographic tests such as computerized tomography (CT scan) or magnetic resonance imaging (MRI) to evaluate the inner ear anatomy.

A speech-language pathologist and audiologist are involved in the CI evaluation process, performing extensive hearing tests that include pure-tone and speech audiometric tests to determine how much the individual can hear with or without a hearing aid. An audiologist may perform objective measures of auditory function in infants and the youngest children as an alternative to standard testing methods. Electrophysiological and objective measures have a valuable role in the management of individuals receiving CI; and in particular, young children, complex cases, and difficult-to-test persons. A challenge of performing these studies in children less than age one year is the lack of available, effective tools for measuring speech perception abilities, including concern regarding the reliability of audiometric results for this age group. Behavioral audiometric testing, the standard for measuring hearing sensitivity, is performed in infants using visual reinforcement audiometry but is not appropriate for infants less than age 5.5 months because they do not respond to sound with directed head turns (Holt and Svirsky, 2008). Therefore, audiologists use objective measures of auditory function as an alternative, including evoked otoacoustic emissions (OAE) testing, auditory brainstem response testing (ABR), and auditory steady-state response testing (ASSR) to assess various elements of the auditory system. Electrically evoked auditory brainstem responses (EABR), middle latency responses (MLR), or acoustic reflexes (EART) may be used intraoperatively with stimuli delivered to the CI prior to leaving the operating room or postoperatively on an outpatient basis to facilitate the CI fitting process. These objective measures are also useful in a small subset of children who are unable to respond consistently to the electrical stimuli used to program the speech processor after CI. The promontory/round window EABR (prom-EABR) stimulation test (or PST) may be indicated in a small subset of infants and children with conditions such as congenital malformations, cochlear nerve dysplasia or selected aplasia, or narrow internal auditory canal, to confirm the integrity of the auditory nerve (Nikolopoulos, 2000). For cochlear implantation performed in centers in the United States, the PST is no longer considered to be a prognostic factor, as the majority of the test results are recorded as "positive;" however, some centers continue to use the PST results during the CI evaluation process as an exclusion for candidates with complete absence of preoperative ability to stimulate the auditory nerve (Kuo, 2002).

One of the most commonly used speech recognition tests performed in the evaluation of adolescent and adult CI candidates is the Hearing In Noise Test (HINT), which measures an individual's ability to hear speech in quiet and in noise in the context of sentences. Psychological counseling of candidates is also performed. These tests are completed to ensure that the candidate will benefit from a CI and will have the motivation to participate in the process. It is important that the candidate understands what the implant will and will not do, and also understands the commitment required for care and follow-up services (AAO-HNS, 2011; NIDCD, 2009).

Several cochlear implants are commercially available in the United States: the Nucleus^® series of devices, including the Nucleaus^® 22 and 24 Channel Systems (Cochlear Americas, Englewood, CO); the Clarion^® Implants (Advanced Bionics Corporation, Sylmar, CA); and the MED-EL COMBI 40+ Cochlear Implant System^® (MED-EL Corporation, Durham, NC). Subsequent generations of the various components of these devices have been approved by the FDA, focusing on improved electrode design and speech processing capabilities. Furthermore, smaller devices and the accumulating experience with use of CI in children have resulted in broadening of the candidate selection criteria to include children as young as 12 months of age. Specific criteria vary with the type of device. The FDA-labeled indications for each device are currently available on the FDA Premarket Approval (PMA) web site.

While the benefits of single CI are well accepted, the implantation of a single device does not provide normal (binaural) hearing to an individual with severe bilateral hearing loss. Binaural hearing provides certain auditory effects that assist in localizing sound and understanding speech in a noise environment. The auditory benefits enabled by binaural hearing include addressing "head shadow," "binaural summation," and "binaural squelch" (Gantz, 2002). Head shadow is the barrier the head creates between sounds emitted from one direction and the contralateral ear. Head shadow dampens noise reaching the contralateral ear and delivers an intelligible signal to noise (SNR) or "speech-to-noise" ratio. Head shadow is believed to permit a bilateral CI user the flexibility of attenuating to the ear with the better SNR. This shadowing or attenuating effect works best for high frequency sounds.

With binaural hearing, each ear receives both unique and redundant information (acoustical representation) that is processed in the brain. The processing of this redundant information, "binaural summation," improves hearing threshold and increases sensitivity to small differences in sound frequency and intensity. Binaural summation can lead to improved speech perception in both quiet and noise. The third effect of binaural hearing is "binaural squelch." With two ears, the brain uses 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 (Tyler, 2003).

CDC and FDA Notifications

On June 4, 2007, the Centers for Disease Control and Prevention (CDC) posted fact sheets for the general public and healthcare professionals titled, Vaccines and Preventable Diseases: Use of Meningitis Vaccine in Persons with Cochlear Implant. Each posting recommends the pneumococcal vaccination for individuals with CI. Specific vaccination schedules are referenced and linked to a July 31, 2003 Early Release of Morbidity and Mortality Weekly Report (MMWR). Following the CDC notification, the FDA issued two separate Medical Device Safety notices concerning the increased, life-threatening risk of bacterial meningitis in CI recipients and the importance of being fully vaccinated (FDA, 2007). Theses warning were based on the reported deaths of two individuals with CI from bacterial meningitis caused by Streptococcus pneumoniae. Neither of the individuals was fully vaccinated; one of these two individuals likely died because of the lack of vaccination.

CI recipients should be up-to-date on age-appropriate pneumococcal vaccination in accordance with current CDC and the Advisory Committee on Immunization Practices (ACIP) recommendations. 

Auditory Brainstem Implants

The ABI is a device designed to restore some hearing in individuals with neurofibromatosis type 2 rendered deaf by bilateral surgical removal of neurofibromas involving the auditory nerve. The device consists of an externally worn speech processor that provides auditory information to an electrical signal that is transferred to a receiver/stimulator implanted in the temporal bone. The receiver stimulator is, in turn, attached to an electrode array implanted on the surface of the cochlear nerve in the brainstem, thus bypassing the inner ear and auditory nerve. The electrode stimulates multiple sites on the cochlear nucleus, which is then processed normally by the brain. One device has received FDA approval for auditory brainstem implantation, the Nucleus^® 24 Auditory Brain Stem Implant System. The speech processor and receiver are similar to the devices used in CI; the electrode array placed on the brainstem is the novel component of the device.

Auditory brainstem response (ABR): A neurologic test of auditory brainstem function in response to auditory (click) stimuli; the most common application of auditory evoked responses.

Auditory evoked potential: Evaluates the nerve pathways from the ear to the brain; consists of a very small electrical voltage originating from the brain recorded from the scalp in response to an auditory stimulus (e.g. different tones, speech sounds).

Cochlea: Part of the inner ear that processes sound.

Decibel (dB): Unit that measures the intensity or loudness of sound.

Electrically evoked auditory brainstem response (EABR): A measurement of auditory brainstem (ABR) integrity using an electrical stimulus with the purpose of determining if the auditory nerve responds as expected to electrical stimulation.  The EABR test may be used presurgically in selected individuals to determine if a CI should be attempted and postsurgically to determine if the CI is working properly.

Evoked otoacoustic emissions (OAE): Sounds measured in the external ear canal that are a reflection of the working of the cochlea. OAE is used in the screening as well as the diagnosis of hearing impairments in neonates and young children. While the test is considered part of the standard battery of tests in infants, it is considered a specialized test in children and adults.

Meningitis: Inflammation of the meninges, the membranes that surround the brain and the spinal cord; may result in hearing loss or deafness.

Neural plasticity: Ability of the brain, certain parts of the nervous system, or both to adapt to new conditions, such as an injury.

Neurofibromatosis Type 2 (NF-2): Group of inherited disorders in which noncancerous tumors grow on several nerves that usually include the nerve involved with hearing.

Otitis media: Inflammation of the middle ear caused by infection.

Postlingual deafness: Hearing loss that occurs after acquiring language or speech.

Prelingual deafness: Hearing loss that occurs before the development of language or speech.

Promontory/round window stimulation test (PST): The application of controlled electrical current to the promontory or round window niche of the middle ear. The current is delivered via an electrode that is inserted either through the tympanic membrane by myringotomy or puncture by the otolaryngologist. The test has been used to predict the electrical response of surviving spiral ganglion nerve fibers and felt to verify a functioning cochlear nerve.

Scala tympani: The lower tube of the cochlear canal extending from the opening in the medial wall of the middle ear leading into the cochlea.

Sensorineural hearing loss: Hearing loss caused by damage to the sensory cells and/or nerve fibers of the inner ear

Severity of hearing loss: Severity of hearing loss is defined in terms of decibels; graded as Mild (26-40 dB), Moderate (41-55 dB), Moderately Severe (56-70 dB), Severe (71-90 dB), Profound (90 dB).

Speech recognition test: A test frequently used to determine cochlear implant candidacy; currently used tests include the following:

• Central Institute for the Deaf (CID) Test
• City of New York (CUNY) Sentence Test
• Hearing in Noise Test (HINT)
• Lexical Neighborhood Test (LNT)
• Multi-syllabic Lexical Neighborhood Test (MLNT)
• Phonetically Balanced-Kindergarten (PBK) Test

The following codes for treatments and procedures applicable to this document are included below for informational purposes.  Inclusion or exclusion of a procedure, diagnosis or device code(s) does not constitute or imply member coverage or provider reimbursement policy.  Please refer to the member's contract benefits in effect at the time of service to determine coverage or non-coverage of these services as it applies to an individual member.

Cochlear Implants
When services may be Medically Necessary when criteria are met:

When services are Investigational and Not Medically Necessary:
For the procedure and diagnosis codes listed above when criteria are not met, for all other diagnoses not listed; or when the code describes a procedure indicated in the Position Statement section as investigational and not medically necessary.

When services may also be Medically Necessary when criteria are met:

When services are Not Medically Necessary:
For the replacement procedure code listed above, when criteria are not met; or when the code describes a procedure indicated in the Position Statement section as not medically necessary

Auditory Brainstem Implants
When services may be Medically Necessary when criteria are met: 

When services are Investigational and Not Medically Necessary:
For the procedure and diagnosis codes listed above when criteria are not met; for all other diagnoses, or when the code describes a procedure indicated in the Position Statement section as investigational and not medically necessary.

When services may also be Medically Necessary when criteria are met:

When services are Not Medically Necessary:
For replacement components, when criteria are not met; or when the code describes a procedure indicated in the Position Statement section as not medically necessary

Future ICD-10 coding (effective 10/01/2013)
A draft of ICD-10 Coding related to this document, as it might look today, is available for reference and comments at: Appendix 1: Future ICD-10 coding

Peer Reviewed Publications

1. Biernath KR, Reefhuis J, Whitney CG, et al. Bacterial meningitis among children with cochlear implants beyond 24 months after implantation. Pediatrics. 2006; 117(2):284-289.
2. Ching TY, Incerti P, Hill M. Biaural benefits for adults who use hearing aids and cochlear implants in opposite ears. Ear Hear. 2004; 25(1):9-21.
3. Ching TY, Incerti P, Hill M, et al. An overview of binaural advantages for children and adults who use binaural/bimodal hearing devices. Audiol Neurotol. 2006; 11(suppl 1):6-11.
4. Colletti L. Beneficial auditory and cognitive effects of auditory brainstem implantation in children. Acta Otolaryngol 2007; 127(9):943-946.
5. Colletti L. Long-term follow-up of infants (4-11 months) fitted with cochlear implants. Acta Otolaryngol. 2009; 129(4):361-366.
6. Colletti V. Auditory outcomes in tumor vs. non-tumor patients fitted with auditory brainstem implants. Adv Otorhinolaryngol 2006; 64:167-185.
7. Colletti V, Carner M, Miorelli V, et al. Auditory brainstem implant (ABI): new frontiers in adults and children. Otolaryngol Head Neck Surg. 2005a; 133(1):126-138.
8. Colletti V, Carner M, Miorelli V, et al. Cochlear implantation at under 12 months: report on 10 patients. Laryngoscope. 2005b; 115(3):445-449.
9. Copeland BJ, Pillsbury HC III. Cochlear implantation for the treatment of deafness. Annual Rev Med. 2004; 55:157-167.
10. Dettman SJ, Pinder D, Briggs RJ, et al. Communication development in children who receive the cochlear implant younger than 12 months: risks versus benefits. Ear Hear. 2007; 28(2 Suppl):11S-18S.
11. Donaldson GS, Peters MD, Ellis MR, et al. Effects of the Clarion Electrode Positioning System on auditory thresholds and comfortable loudness levels in pediatric patients with cochlear implants. Arch Otolaryngol Head Neck Surg. 2001; 127(8):956-960.
12. El-Kashlan H, Ashbaugh C, Zwolan T, Telian S. Cochlear implantation in prelingually deaf children with ossified cochleae. Otol Neurotol. 2003; 24(4):596-600.
13. Gantz BJ, Tyler RS, Rubinstein JT, et al. Biaural cochlear implants placed during the same operation. Otol Neurotol. 2002; 23(2):169-180.
14. Harrison RV, Gordon KA, Mount RJ. Is there a critical period for cochlear implantation in congenitally deaf children? Analyses of hearing and speech perception performance after implantation. Dev Psychobiol. 2005; 46(3):252-261.
15. Hehar SS, Nikolopoulos TP, Gibbin KP, O'Donoghue GM. Surgery and functional outcomes in deaf children receiving cochlear implants before age 2 years. Arch Otolaryngol Head Neck Surg. 2002; 128(1):11-14.
16. Holt RF, Kirk KL, Eisenberg LS, et al. Spoken word recognition development in children with residual hearing using cochlear implants and hearing aids in opposite ears. Ear Hear. 2005; 26(4 suppl):82S-91S.
17. Holt RF, Svirsky MA. An exploratory look at pediatric cochlear implantation: is earliest always best? Ear Hear. 2008; 29(4):492-511.
18. Johnson MH, Hasenstab MS, Seicshnaydre MA, Williams GH. CT of postmeningitic deafness: observations and predictive value for cochlear implants in children. Am J Neuroradiol. 1995; 16(1):103-109.
19. 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. 2008; 29(3):310-313.
20. Kuhn-Inacker H, Shehata-Dieler W, Muller J, Helms J. Bilateral cochlear implants: a way to optimize auditory perception abilities in deaf children? Int Jl Pediatr Otorhinolaryngol. 2004; 68(10):1257-1266.
21. Kuo S, Gibson W. The role of the promontory stimulation test in cochlear implantation. Cochlear Implants Int. 2002; 3(1):19-28.
22. 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):958-968.
23. Litovsky R, Johnstone, PM, Godar S, et al. Bilateral cochlear implants in children: localization acuity measured with minimum audible angle. Ear Hear. 2006; 27(1):43-59.
24. Litovsky RY, Parkinson A, Arcaroli J, et al. Bilateral cochlear implants in adults and children. Arch Otolaryngol Head Neck Surg. 2004; 130(5):648-655.
25. Litovsky RY, Parkinson A, Arcaroli J, et al. Simultaneous bilateral cochlear implantation in adults: a multicenter clinical study. Ear Hear. 2006; 27(6):714-731.
26. 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 An. 2003; 114(3):1565-1574.
27. Loundon N, Blanchard M, Roger G, et al. Medical and surgical complications in pediatric cochlear implantation. Arch Otolaryngol Head Neck Surg. 2010; 136(1):12-15.
28. Morera C, Manrique M, Ramos A, et al. Advantages of binaural hearing provided through bimodal stimulation via a cochlear implant and a conventional hearing aid: a 6-month comparative study. Acta Otolaryngol. 2005; 125(6):596-606.
29. 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):198-206.
30. Nicholas JG, Geers A. Effects of early auditory experience on the spoken language of deaf children at 3 years of age. Ear Hear. 2006; 27(3):286-298.
31. Nikolopoulos TP, Mason SM, Gibbin KP, O'Donoghue GM. The prognostic value of promontory electric auditory brain stem response in pediatric cochlear implantation. Ear Hear. 2000; 21(3):236-241.
32. Peters BR, Litovsky R, Parkinson A, Lake J. Importance of age and postimplantation experience on speech perception measures in children with sequential bilateral cochlear implants. Otol Neurotol. 2007; 28(5):649-657.
33. Peters BR, Wyss J, Manrique M. Worldwide trends in bilateral cochlear implantation. Laryngoscope. 2010; 120 (Suppl 2):S17-S44.
34. Philippon D, Bergeron F, Ferron P, Bussières R. Cochlear implantation in postmeningitic deafness. Otol Neurotol. 2010; 31(1):83-87.
35. Ramsden R, Greenham P, O'Driscoll M, et al. Evaluation of bilaterally implanted adult subjects with the Nucleus 24 cochlear implant system. Otol Neurotol. 2005; 26(5):988-998.
36. Reefhuis J, Honein MA, Whitney CH, et al. Risk of bacterial meningitis in children with cochlear implants. NEJM. 2003; 349(5):435-445.
37. Ricketts TA, Grantham DW, Ashmead DH, et al. Speech recognition for unilateral and bilateral cochlear implant modes in the presence of uncorrelated noise sources. Ear Hear. 2006; 27(6):763-773.
38. Roland JT Jr, Cosetti M, Wang KH, et al. Cochlear implantation in the very young child: long-term safety and efficacy. Laryngoscope. 2009; 119(11):2205-2210.
39. Schoen F, Mueller J, Helms J, Nopp P. Sound localization and sensitivity to interaural cues in bilateral users of the Med-El Combi 40/40+ cochlear implant system. Otol Neurotol. 2005; 26(3):429-437.
40. Sharma A, Dorman MF. Central auditory development in children with cochlear implants: clinical implications. Adv Otorhinolaryngol. 2006; 64:66-88.
41. Sharma A, Dorman MF, Kral A. The influence of a sensitive period on central auditory development in children with unilateral and bilateral cochlear implants. Hear Res. 2005; 203(1-2):134-143.
42. Tait M, De Raeve L, Nikolopoulos TP. Deaf children with cochlear implants before the age of 1 year: comparison of preverbal communication with normally hearing children. Int J Pediatr Otorhinolaryngol. 2007; 71(10):1605-1611.
43. Tomblin JB, Barker BA, Spencer LJ, et al. The effect of age at cochlear implant initial stimulation on expressive language growth in infants and toddlers. J Speech Lang Hear Res. 2005; 48(4):853-867.
44. Tyler R, Dunn CC, Witt SA, Preece JP. Update on bilateral cochlear implantation. Curr Opin Otolaryngol Head Neck Surg. 2003; 11(5):388-393.
45. Tyler RS, Gantz BJ, Rubinstein JT, et al. Three-month results with bilateral cochlear implants. Ear Hear. 2002; 23(1 Suppl):80S-89S.
46. Valencia DM, Rimell FL, Friedman BJ, et al. Cochlear implantation in infants less than 12 months of age. Int J Pediatr Otorhinolaryngol. 2008; 72(6):767-773.
47. van Hoesel RJ, 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):137-149.
48. van Hoesel RJ, Typer RS. Speech perception, localization, and lateralization with bilateral cochlear implants. J Acoust Soc Am. 2003; 113(3):1617-1630.
49. Verschuur, CA, Lutman, ME, Ramsden R, et al. Auditory localization abilities in bilateral cochlear implant recipients. Otol Neurotol. 2005; 26(5):965-971.
50. Vlastarakos PV, Candiloros D, Papacharalampous G, et al. Diagnostic challenges and safety considerations in cochlear implantation under the age of 12 months. Int J Pediatr Otorhinolaryngol. 2010a; 74(2):127-132.
51. Vlastarakos PV, Proikas K, Papacharalampous G, et al. Cochlear implantation under the first year of age-the outcomes. A critical systematic review and meta-analysis. Int J Pediatr Otorhinolaryngol. 2010b; 74(2):119-126.
52. Waltzman SB, Roland JT Jr. Cochlear implantation in children younger than 12 months. Pediatrics. 2005; 116(4):e487-493.
53. Young NM, Hughes CA, Byrd SE, Darling C. Postmeningitic ossification in pediatric cochlear implantation. Otolaryngol Head Neck Surg. 2000; 122(2):183-188.

Government Agency, Medical Society, and Other Authoritative Publications:

1. American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS). Available at: http://www.entnet.org/. Accessed on March 31, 2011.
   • Cochlear implants. Reaffirmed December 27, 2007.
   • Minimal test battery for cochlear implants. Reaffirmed March 1, 1998.
2. Bond M, Mealing S, Anderson R, et al. The effectiveness and cost-effectiveness of cochlear implants for severe to profound deafness in children and adults: a systematic review and economic model. Health Technol Assess. 2009; 13(44):1-330.
3. Centers for Disease Control and Prevention (CDC). Vaccines and preventable diseases: cochlear implants & meningitis vaccination. September 14, 2009. Available at: http://www.cdc.gov/Vaccines/vpd-vac/mening/cochlear/dis-cochlear-faq-hcp.htm. Accessed on March 31, 2011.
4. Centers for Medicare and Medicaid Services (CMS). National Coverage Determination: Cochlear Implantation. NCD #50.3. Effective April 4, 2005. Available at: http://www.cms.hhs.gov/mcd/index_list.asp?list_type=ncd. Accessed on March 31, 2011.
5. U.S. Food and Drug Administration (FDA). Center for Devices and Radiological Health (CDRH). FDA Public Health Notifications. Importance of vaccination in cochlear implant recipients.  Rockville, MD: FDA. October 10, 2007. Available at: http://www.fda.gov/cdrh/safety/101007-cochlear.html. Accessed on March 31, 2011.
6. U.S. Food and Drug Administration (FDA). Center for Devices and Radiological Health (CDRH). Medical Device Safety. Advice for patients with cochlear implants: new information on meningitis risk (1^st advisory). Rockville, MD: FDA. February 6, 2006. Available at: http://www.fda.gov/MedicalDevices/Safety/AlertsandNotices/PatientAlerts/ucm064737.htm. Accessed on March 31, 2011.
7. U.S. Food and Drug Administration (FDA). Medical Devices Databases. Cochlear implants. Rockville, MD: FDA. Available at: http://www.accessdata.fda.gov/scripts/cdrh/devicesatfda/index.cfm. Accessed on March 31, 2011.

1. National Institute on Deafness and other Communication Disorders (NIDCD). Cochlear implants. March 2011. Available at: http://www.nidcd.nih.gov/health/hearing/coch.asp. Accessed on March 31, 2011.
2. National Institute on Deafness and other Communication Disorders (NIDCD). Vestibular schwannoma (acoustic neuroma) and neurofibromatosis. February 2004. Available at: http://www.nidcd.nih.gov/health/hearing/acoustic_neuroma.asp. Accessed on March 31, 2011.

Auditory Brainstem Implant
Cochlear Implant

The use of specific product names is illustrative only. It is not intended to be a recommendation of one product over another, and is not intended to represent a complete listing of all products available.