Coverage Policy Manual
Policy #: 1998070
Category: Surgery
Initiated: February 1998
Last Review: September 2018
  Cochlear Implant

Description:
A Cochlear implant is a device for individuals with severe-to-profound hearing loss who only receive limited benefit from amplification with hearing aids. A cochlear implant 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 cochlear implant include both external and internal components. The external components include a microphone, an external sound processor, and an external transmitter. 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.
 
Sounds that are picked up by the microphone are carried to the external sound processor, which transforms sound into coded signals that are then transmitted transcutaneously to the implanted internal receiver. The receiver converts the incoming signals to electrical impulses that are then conveyed to the electrode array, ultimately resulting in stimulation of the auditory nerve.
  
Hearing loss is rated on a scale based on the threshold of hearing. Severe hearing loss is defined as a hearing threshold of 70-90 decibels (dB) and profound hearing loss is defined as a hearing threshold of 90 dB and above.
 
Regulatory Status
Several cochlear implants are commercially available in the U.S. and are manufactured by Cochlear Corporation, Advanced Bionics, and the Med El Corporation. Over the years, subsequent generations of the various components of the devices have been approved by the U.S. Food and Drug Administration (FDA), focusing on improved electrode design and speech-processing capabilities. Furthermore, smaller devices and the accumulating experience in children have resulted in broadening of the selection criteria to include children as young as 12 months. The labeled indications from the FDA for currently marketed implant devices are summarized below.
 
The following is a summary of the labeled indications from the FDA for currently marketed implanted devices.
 
Cochlear Corporation Devices
 
Currently marketed cochlear implant:  Cochlear® Nucleus 5
 
Predecessor cochlear implants:  Nucleus 22, Nucleus 24 and Freedom with Contour
 
*Note: Cochlear, Ltd. voluntarily recalled the Nucleus CI500 range in September 2011 for device malfunction in the CI512 implant. The external Nucleus 5 sound processor is not a part of the recall.
 
FDA labeled indications:    
Adults:
At least 18 years old
Pre- or post-lingual onset of moderate-to-profound bilateral sensorineural hearing loss
At least 50% sentence recognition in the ear to be implanted
At least 60% sentence recognition in the opposite ear or binaurally
 
Children 25 months to 17 years 11 months:
  • Severe to profound bilateral sensorineural hearing loss
  • Multi-syllabic Lexical Neighborhood Test (MLNT) scores of less than or equal to 30% in best-aided condition in children 25 months to 4 years 11 months
  • Lexical Neighborhood Test (LNT) scores of less than or equal to 30% in best-aided condition in children 5 years to 17 years and 11 months
  • Lack of progress in the development of auditory skills
 
Children 12 months to 24 months:
  • Profound sensorineural hearing loss bilaterally
  • Limited benefit from appropriate binaural hearing aids
  • Lack of progress in the development of auditory skills
 
Advanced Bionics Devices
 
Currently marketed cochlear implant:  HiResolution Bionic Ear System (HiRes 90K)
 
Predecessor cochlear implants:  CII Bionic Ear
 
*Note: Advanced Bionics HiRes90K was voluntarily recalled in November 2010 and given FDA-approval for re-entry to market the device in September 2011.
 
FDA labeled indications:
 
Adults:
  • At least 18 years of age
  • Post-lingual onset of severe-to-profound bilateral sensorineural hearing loss (greater than or equal to 70 decibels (dB))
  • Limited benefit from appropriately fitted hearing aids, defined as scoring less than or equal to 50% on a test of open-set Hearing in Noise Test (HINT) sentence recognition
 
Children:
  • 12 months to 17 years of age
  • Profound bilateral sensorineural deafness (>90dB)
  • Use of appropriately fitted hearing aids for at least 6 months in children 2 to 17 years of age or at least 3 months in children 12 to 23 months of age
  • Lack of benefit in children <4 years of age is defined as a failure to reach developmentally appropriate auditory milestones (e.g., spontaneous response to name in quiet or to environmental sounds) measured using the Infant-Toddler Meaningful Auditory Integration Scale or Meaningful Auditory Integration Scale or <20% correct on a simple open-set word recognition test (Multisyllabic Lexical Neighborhood Test) administered using monitored live voice (70 dB SPL [sound pressure level])
  • Lack of hearing aid benefit in children >4 years of age is defined as scoring <12% on a difficult open-set word recognition test (Phonetically Balanced-Kindergarten Test) or <30% on an open-set sentence test (HINT for Children) administered using recorded materials in the soundfield (70 dB SPL)  
 
MED-EL Corporation Devices
 
Currently marketed cochlear implant:  Med El® Maestro (Sonata or Pulsar)
 
Predecessor cochlear implants:  Combi 40+
 
FDA labeled indications:
 
Adults:
  • At least 18 years old
  • Severe to profound bilateral sensorineural hearing loss (greater than or equal to 70dB)
  • Less than or equal to 40% correct Hearing in Noise test (HINT) sentences with best-sided listening condition
 
Children:
  • 12 months to 18 years with profound sensorineural hearing loss (greater than or equal to 90dB)
  • In younger children, little or no benefit is defined by lack of progress in the development of simple auditory skills with hearing aids over a 3-6 month period
  • In older children, lack of aided benefit is defined as <20% correct on the MLNT or LNT, depending upon the child’s cognitive ability and linguistic skills
  • A 3-6 month trial with hearing aids is required if not previously experienced
 
In July, 2003, the FDA began notifying healthcare providers about a recent study showing that children with cochlear implants are at greater risk of developing bacterial meningitis caused by Streptococcus pneumoniae than children in the general population.  Recommendations for vaccination with Pneumococcal Conjugate Vaccine (PCV7) are found in the coverage section of this policy.
 
While cochlear implants have typically been used unilaterally, in recent years, interest in bilateral cochlear implantation has arisen. The proposed benefits of bilateral cochlear implants are to improve understanding of speech in noise and localization of sounds. Improvements in speech intelligibility may occur with bilateral cochlear implants through binaural summation; i.e., signal processing of sound input from 2 sides may provide a better representation of sound and allow one to separate out noise from speech. Speech intelligibility and localization of sound or spatial hearing may also be improved with head shadow and squelch effects, i.e., the ear that is closest to the noise will be received at a different frequency and with different intensity, allowing one to sort out noise and identify the direction of sound. Bilateral cochlear implantation may be performed independently with separate implants and speech processors in each ear or with a single processor. However, no single processor for bilateral cochlear implantation has been approved by the FDA for use in the U.S. In addition, single processors do not provide binaural benefit and may impair sound localization and increase the signal to noise ratio received by the cochlear implant.
 
 
 
 

Policy/
Coverage:
Effective September 2017
 
Meets Primary Coverage Criteria Or Is Covered For Contracts Without Primary Coverage Criteria
 
A FDA-approved cochlear implant and associated aural rehabilitation meets primary coverage criteria for effectiveness and is covered when the device is implanted for FDA approved indications subject to all member contract limitations.
 
Pneumococcal vaccination prior to cochlear implantation should be given based on recommendations from the Advisory Committee on Immunization Practices:
    • Children less than 24 months of age with cochlear implants should receive PCV7 , as is universally recommended;
    • Children aged 24-59 months of age with cochlear implants who have not received PCV7 should be vaccinated according to the high-risk schedule.  Children who have completed the PCV7 series should receive PPV 23 two months or more after vaccination with PCV7;
    • Persons aged 5-64 years with cochlear implants should receive PPV 23 according to the schedule used for persons with chronic illness; a single dose is indicated;
    • Persons planning to receive a cochlear implant should be up-to-date on age-appropriate pneumococcal vaccination at least two weeks prior to surgery, if possible.
 
Hybrid Cochlear Implant
Cochlear implantation with a hybrid cochlear implant/hearing aid device that includes the hearing aid integrated into the external sound processor of the cochlear implant (eg, the Nucleus® Hybrid™ L24 Cochlear Implant System) meets member benefit certificate primary coverage criteria for patients ages 18 years and older who meet all of the following criteria:
 
    • Bilateral severe-to-profound high-frequency sensorineural hearing loss with residual low-frequency hearing sensitivity; AND
 
    • Receive limited benefit from appropriately fit bilateral hearing aids; AND
 
    • Have the following hearing thresholds:
 
      • Low-frequency hearing thresholds no poorer than 60 dB hearing level up to and including 500 Hz (averaged over 125, 250, and 500 Hz) in the ear selected for implantation; AND
      • Severe-to-profound mid- to high-frequency hearing loss (threshold average of 2000, 3000, and 4000 Hz ≥75 dB hearing level) in the ear to be implanted; AND
      • Moderately severe to profound mid- to high-frequency hearing loss (threshold average of 2000, 3000, and 4000 Hz ≥60 dB hearing level) in the contralateral ear; AND
      • Aided consonant-nucleus-consonant word recognition score from 10% to 60% 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.
 
Replacement of Components
Replacement of internal and/or external components meets member benefit certificate primary coverage criteria and is covered only in a small subset of members who have inadequate response to existing component(s) to the point of interfering with the individual’s activities of daily living, or the component(s) is/are no longer functional and cannot be repaired. Copies of original medical records must be submitted either hard copy or electronically to support medical necessity.
 
*Note: See below section for statement addressing replacement of internal and/or external components solely for the purpose of upgrading and for aesthetic improvement
 
Does Not Meet Primary Coverage Criteria Or Is Investigational For Contracts Without Primary Coverage Criteria
 
Cochlear implantation does not meet member benefit certificate primary coverage criteria and is not covered for:
    • Deafness due to lesions of the acoustic nerve or central auditory pathway;
    • Otitis media or other active, unresolved ear problems;
    • Radiographic evidence of absent cochlear development;
    • Unilateral hearing loss with or without tinnitus
 
For contracts without primary coverage criteria, cochlear implantation is considered investigational for:
    • Deafness due to lesions of the acoustic nerve or central auditory pathway;
    • Otitis media or other active, unresolved ear problems;
    • Radiographic evidence of absent cochlear development;
    • Unilateral hearing loss with or without tinnitus
 
Investigational services are specific contract exclusions in most member benefit certificates of coverage
 
Upgrades and Replacement of Components
Upgrades of an existing, functioning external system to achieve aesthetic improvement, such as smaller profile components or a switch from a body-worn, external sound processor to a behind-the-ear model do not meet member benefit certificates of coverage.
 
For members with contracts without primary coverage criteria, upgrades of an existing, functioning external system to achieve aesthetic improvement, such as smaller profile components or a switch from a body-worn, external sound processor to a behind-the-ear model, are considered not medically necessary.  Services that are considered not medically necessary are specific contract exclusions in most member benefit certificates of coverage.
 
Replacement of internal and/or external components solely for the purpose of upgrading to a system with advanced technology or to a next-generation device does not meet member benefit certificates of coverage.
 
For members with contracts without primary coverage criteria, replacement of internal and/or external components solely for the purpose of upgrading to a system with advanced technology or to a next-generation device is considered not medically necessary. Services that are considered not medically necessary are specific contract exclusions in most member benefit certificates of coverage.
 
Effective August 2010- August 2017
A FDA-approved cochlear implant and associated aural rehabilitation meets primary coverage criteria for effectiveness and is covered when the device is implanted for FDA approved indications subject to all member contract limitations.
 
Pneumococcal vaccination prior to cochlear implantation should be given based on recommendations from the Advisory Committee on Immunization Practices:
    • Children less than 24 months of age with cochlear implants should receive PCV7 , as is universally recommended;
    • Children aged 24-59 months of age with cochlear implants who have not received PCV7 should be vaccinated according to the high-risk schedule.  Children who have completed the PCV7 series should receive PPV 23 two months or more after vaccination with PCV7;
    • Persons aged 5-64 years with cochlear implants should receive PPV 23 according to the schedule used for persons with chronic illness; a single dose is indicated;
    • Persons planning to receive a cochlear implant should be up-to-date on age-appropriate pneumococcal vaccination at least two weeks prior to surgery, if possible.
 
Cochlear implantation for:
    • Deafness due to lesions of the acoustic nerve or central auditory pathway;
    • Otitis media or other active, unresolved ear problems;
    • Radiographic evidence of absent cochlear development.
is not covered based on benefit certificate primary coverage criteria that there be scientific evidence of effectiveness.
 
For contracts without primary coverage criteria, cochlear implantation for:
    • Deafness due to lesions of the acoustic nerve or central auditory pathway;
    • Otitis media or other active, unresolved ear problems;
    • Radiographic evidence of absent cochlear development.
is considered investigational.  Investigational services are an exclusion in the member certificate of coverage.
 
 
Effective, October 2004 to July 2010
A FDA-approved cochlear implant and associated aural rehabilitation meets primary coverage criteria for effectiveness and is covered when the device is implanted for FDA approved indications subject to all member contract limitations including maximum dollar allowance.
 
Pneumococcal vaccination prior to cochlear implantation should be given based on recommendations from the Advisory Committee on Immunization Practices:
    • Children less than 24 months of age with cochlear implants should receive PCV7 , as is universally recommended;
    • Children aged 24-59 months of age with cochlear implants who have not received PCV7 should be vaccinated according to the high-risk schedule.  Children who have completed the PCV7 series should receive PPV 23 two months or more after vaccination with PCV7;
    • Persons aged 5-64 years with cochlear implants should receive PPV 23 according to the schedule used for persons with chronic illness; a single dose is indicated;
    • Persons planning to receive a cochlear implant should be up-to-date on age-appropriate pneumococcal vaccination at least two weeks prior to surgery, if possible.
 
Cochlear implantation for:
    • Deafness due to lesions of the acoustic nerve or central auditory pathway;
    • Otitis media or other active, unresolved ear problems;
    • Radiographic evidence of absent cochlear development.
is not covered based on benefit certificate primary coverage criteria that there be scientific evidence of effectiveness.
 
For contracts without primary coverage criteria, cochlear implantation for:
    • Deafness due to lesions of the acoustic nerve or central auditory pathway;
    • Otitis media or other active, unresolved ear problems;
    • Radiographic evidence of absent cochlear development.
is considered investigational.  Investigational services are an exclusion in the member certificate of coverage.
 
Next generation devices have typically offered a marginal improvement over previous devices, such that replacement of the internally implanted components is not routinely performed and would not be covered.  Upgrades of an existing, functioning external system to achieve aesthetic improvement, such as smaller profile components, or a switch from a body-worn, external sound processor to a behind-the-ear (BTE) model are not covered.  This denial is based on the "least costly alternative" language in the member benefit contract.
 
Effective, December 2001 to September 2004
An FDA-approved cochlear implant and associated aural rehabilitation is covered when the device is implanted for FDA approved indications.
 
Cochlear implantation is not covered for:  (1) Deafness due to lesions of the acoustic nerve or central auditory pathway; (2) Otitis media or other active, unresolved ear problems; (3) Radiographic evidence of absent cochlear development.
 
Next generation devices have typically offered a marginal improvement over previous devices, such that replacement of the internally implanted components is not routinely performed and would not be covered.  Upgrades of an existing, functioning external system to achieve aesthetic improvement, such as smaller profile components, or a switch from a body-worn, external sound processor to a  behind-the-ear (BTE) model are not covered.
 
Effective, February 1998 to November 2001
A single or multi-channel cochlear implantation is considered medically necessary in patients of at least two years of age with severe or profound sensorineural deafness (hearing threshold of 70 decibels or greater) who cannot benefit from a hearing aid, including those with hearing loss due to meningitis.
 

Rationale:
Cochlear implants are recognized effective treatment of sensorineural deafness, as noted in a 1995 National Institutes of Health Consensus Development conference, which offered the following conclusions:
    • Cochlear implantation has a profound impact on hearing and speech reception in post lingually deafened adults with positive impacts on psychological and social functioning.
    • The results are more variable in children. Benefits are not realized immediately but rather are manifested over time, with some children continuing to show improvement over several years.
    • Prelingually deafened adults may also benefit, although to a lessor extent than post lingually deafened adults. These individuals achieve minimal improvement in speech recognition skills.  However, other basic benefits, such as improved sound awareness, may meet safety needs.
    • Training and educational intervention are fundamental for optimal post implant benefit.
    • Cochlear implants in children under 2 years old are complicated by the inability to perform detailed assessment of hearing and functional communication. However, a younger age of implantation may limit the negative consequences of auditory deprivation and may allow more efficient acquisition of speech and language. Some children with post meningitis hearing loss have been implanted under the age of 2 years due to the risk of new bone formation associated with meningitis, which may preclude a cochlear implant at a later date.
 
2010 Update
While use of a monolateral cochlear implant in patients with severe to profound hearing loss has become standard clinical practice, bilateral cochlear implantation was less common.  A search of the MEDLINE database was conducted. A summary of the available relevant literature follows.
 
A number of studies have reported results with bilateral cochlear implants. Litovsky reported that 9 of 13 (70%) children with bilateral cochlear implants discriminated source separations of ≥20 degrees and 7 of 9 performed better when using bilateral (vs. unilateral) devices (Litovsky, 2006).  Schoen and colleagues reported that bilateral cochlear implants were able to restore spatial hearing in 11 cochlear implant patients (Schoen, 2005). Litovsky and colleagues reported on a multicenter prospective study of 37 adults with post-linguistic bilateral hearing loss (Litovsky, 2006).  Bilateral benefit (speech understanding in quiet and noise) was seen in 32 of the 34 subjects. The authors indicate that the 3dB 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 (subjects used only the “best” unilateral device for 3 weeks) also indicated that bilateral users perceived their performance to be better than when using a single device. Ricketts and colleagues reported on 16 similar adults with postlinguistic bilateral hearing loss (Ricketts, 2006). 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 patients followed up to 17 months, a consistent bilateral advantage was noted.
 
Ramsden and colleagues reported on 30 adults in England who had bilateral cochlear implants and received their second implant a mean of 3 years after the first (Ramsden, 2005).  At 9 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 when 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. Kuhn-Inacker reported on a group of 39 European children who had bilateral cochlear implants (Kuhn-Inacker, 2004).  From qualitative and quantitative data, they concluded that bilateral implants improve the children’s communicative behavior, especially in complex listening situations.
 
Bond et al. authored a technology assessment in the UK to investigate the clinical and cost-effectiveness of unilateral cochlear implants (using or not using hearing aids), and bilateral cochlear implants with a single cochlear implant (unilateral or unilateral plus hearing aid) for severely to profoundly deaf children and adults (Bond, 2009). 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 cochlear implant 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 cochlear implants 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.”
 
In January 2009, the National Institute for Health and Clinical Excellence (NICE) released technology appraisal guidance 166, Cochlear Implants for children and adults with severe to profound deafness.  The guidance includes the following recommendations:
 
1. “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.
2. 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
· adults who are blind or who have other disabilities that increase their reliance on auditory stimuli as a primary sensory mechanism for spatial awareness.
3. Sequential bilateral cochlear implantation is not recommended as an option for people with severe to profound deafness.
4. For purposes of this guidance, severe to profound deafness is defined as hearing only sounds that are louder that 90 dB HL at frequencies of 2 and 4 k hertz (Hz) 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.
5. 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).”
 
A review article by Firszt et al. evaluates the advantages of binaural hearing and the disadvantages of hearing with only one ear or hearing with two ears with significantly different sound thresholds (Firszt, 2008).  A case study is presented that demonstrates the benefit of bimodal hearing (i.e., a cochlear implant [CI] in one ear and a contralateral hearing aid [HA]) in a nontraditional CI candidate with asymmetrical hearing thresholds. Selected studies in adult recipients who use a CI and contralateral HA or who use two CIs are summarized. The data overall demonstrates that bilateral CI recipients experience substantial binaural hearing advantages, including improved speech recognition in noise, localization, and functional everyday communication. These results suggest that bilateral stimulation of the auditory system through a CI and contralateral HA or two CIs is beneficial.
 
Olson et al. conducted a systematic review (Olson, 2008) to answer the clinical question: "Does amplification in the ear opposite of a cochlear implant provide improved communication function for adult users?" Several trends about bimodal stimulation were observed, which include: (1) significantly better speech understanding in the bimodal condition for many participants; (2) in noise, the largest bimodal benefits in speech recognition; (3) variable findings on localization tasks; and (4) overall significant improvement in functional ability based on self-assessments. The preponderance of evidence received grades of B or C. The evidence available indicates "moderate" (II) strength in support of bimodal stimulation for adult implant users. The authors noted that “additional research is needed about optimal time frame for introducing bimodal fittings as well as establishing a clinical profile of patients who may benefit most from this intervention compared to bilateral implantation.”
 
In May 2008, the British Cochlear Implant Group (BCIG) released a position paper on Bilateral Cochlear Implants.  The position paper includes the following regarding indications for use of bilateral implantation:
 
· “For all profoundly deaf children in order to stimulate both auditory pathways and optimize speech, language and auditory development and maximize potential academic achievement.
· For all profoundly deaf adults, unable to benefit from bimodal hearing;
· For patients following meningitis or other risk of ossification, where failure to implant may result in obliteration or the cochlea, preventing future stimulation;
· For patients with additional sensory handicap, where there is greater reliance on binaural hearing;
· For patients who experience a loss of performance in the first implanted ear or loss of device function in the first ear but re-implantation in the same ear is contra-indicated;
· For patients who agree to participate in research studies into bilateral implantation.”
 
Johr et al. highlight the surgical and anesthesiological considerations when performing cochlear implant surgery in very young infants (younger than 1 year of age) (Johr, 2008). This is an observational and literature review by pediatricians at a tertiary children’s hospital in Switzerland. Patients younger than 1 year of age undergoing cochlear implant surgeries were analyzed concerning surgical techniques, and anesthesiological aspects of elective surgeries in small infants. The results demonstrated that the age of the patient and the pediatric experience of the anesthesiologist, but not the duration of the surgery, are relevant risk factors. The authors concluded, “Further research is needed to provide more conclusive evidence that the performance outcome for children implanted before 12 months of age does not converge with the results of children implanted between 12 and 18 months.” Currently, there is no conclusive published evidence to support performing cochlear implant surgery on an infant younger than 12 months of age.
 
A number of small studies from outside the U.S. have reported results on cochlear implantation in infants younger than 12 months, which would be an off-label indication. For example, in a study from Australia, Ching and colleagues (Ching, 2009) published an interim report on early language outcomes of children with cochlear implants. This study evaluated 16 children who had implants before 12 months of age compared to 23 who had implants after 12 months (specific time of implantation was not provided). The preliminary results demonstrated that children who received an implant before 12 months of age developed normal language skills at a rate comparable to normal-hearing children, while those with later implants performed at two standard deviations below normal. The authors noted that these results are preliminary as there is a need to examine the effect of multiple factors on language outcomes and the rate of language development. Similarly, in a study from Italy, Colletti (40) reported on findings from 13 infants who had implants placed before 12 months. The procedures were performed between 1998 and 2004. In this small study, the rate of receptive language growth for these early implant infants overlapped scores of normal-hearing children. This overlap was not detected for those implanted at 12–23 or 24–36 months. Data from these small studies are viewed as preliminary and not conclusive.
 
Guidelines and Position Statements
 
In 2006, the American Academy of Otolaryngology-Head and Neck Surgery Foundation (Blakely, 2006) released criteria for cochlear implants for adults and pediatric patients.
 
Adult Criteria
1. Be 18 years or older, with bilateral, severe to profound sensorineural hearing loss, i.e., 70dB or greater PTA (pure-tone air-conduction average) at 500, 1000, and 2000 Hz;
2. Have tried but have limited benefit from adequately fitted binaural hearing aid; or
3. Have sentence recognition score of 50 percent or less in the ear to be implanted and 60 percent or less in the contralateral ear in best aided conditions using Hearing in Noise Test (HINT) or City University of New York (CUNY) tests.
 
Pediatric Criteria
1. Be 12 months to 17 years of age.
2. Infants age 12-24 months should have bilateral, profound hearing loss with thresholds of 90dB or greater at 100 Hz.
3. Children 24 months to 17 years should have bilateral severe to profound (greater than 70dB) hearing loss.
4. Infants and older children should demonstrate lack of progress in simple auditory skills in conjunction with appropriate auditory amplification and participation in intensive aural habilitation for three to six months. Less than 0.14520 percent correct on the Multi-syllabic Lexical Neighborhood Test (MLNT) or Lexical Neighborhood Test (LNT), depending on the child’s cognitive and linguistic abilities.
5. A three-to six-month trial of appropriate hearing aids is required. If meningitis is the cause of hearing loss or if there is radiologic evidence of cochlear ossification a shorter hearing aid trial and earlier implantation may be reasonable.”
 
In May 2008, The British Cochlear Implant Group (BCIG) released a position paper on “Bilateral Cochlear Implants.”  The position paper includes indications for use of bilateral implantation as noted above.
 
In January 2009, the National Institute for Health and Clinical Excellence (NICE) released technology appraisal guidance 166, “Cochlear Implants for children and adults with severe to profound deafness,” which includes recommendations for use of unilateral and bilateral cochlear implants in children and adults as noted above.
 
Summary
In summary, these studies show consistent improvement in speech reception (especially in noise) and in sound localization with bilateral devices. These are important attributes. Studies also suggest that earlier implantation may be preferred.  Based on the available evidence, the use of unilateral and bilateral cochlear implant devices is sufficient to improve net health outcomes.
 
2012 Update
 
Cochlear Implantation in Adults: Unilateral Stimulation
In April 2011, a technology assessment was completed by the Tufts Evidence-based Practice Center for the Agency for Health Care Research and Quality on the effectiveness of cochlear implants in adults (Raman, 2011). This assessment examined 22 studies with 30 or more patients and concluded that while the studies reviewed were rated as poor to fair quality, unilateral cochlear implants are effective in adults with sensorineural hearing loss. Pre- and post-cochlear implant scores on multi-syllable tests and open-set sentence tests demonstrated significant gains in speech perception regardless of whether a contralateral hearing aid was used along with the cochlear implant. Additionally, the assessment found generic and disease-specific health-related quality of life improved with unilateral cochlear implants. However, the available evidence was insufficient to draw conclusions on improvements in open-set sentence test scores (i.e., >40% and ≤50% or >50% and ≤60%), and any relationship between pre-implantation patient characteristics and outcomes (e.g., age, duration of hearing impairment, Hearing in Noise Test [HINT] scores and pre- or post-linguistic deafness). A 2012 systematic review of 11 studies by Bittencourt et al. also concluded cochlear implants improved hearing outcomes over conventional hearing aids in patients with severe to profound postlingual deafness (Bittencourt, 2012).
 
In October 2011, Berrettini and colleagues published results of a systematic review of cochlear implant effectiveness in adults (Berrettini, 2011). Included in the review, were 8 articles on unilateral cochlear implants in advanced age patients. All of the studies reported benefits with cochlear implantation despite advanced age at time of implant (age 70 years or older). In 6 studies, results were not significantly different between younger and older patients. However, 2 studies reported statistically significant inferior perceptive results (e.g., hearing in noise test and consonant nucleus consonant test) in older patients. This systematic review also examined 3 studies totaling 56 adults with pre-lingual deafness who received unilateral cochlear implants. The authors concluded unilateral cochlear implants provided hearing and quality-of-life benefits in prelingually deaf patients, but results were variable.
 
Cochlear Implantation in Adults: Bilateral Stimulation
The April 2011 technology assessment, noted above, completed by the Tufts Evidence-based Practice Center on the effectiveness of cochlear implants in adults examined 16 studies on bilateral cochlear implantation of fair to moderate quality published since 2004 (Raman, 2011). The assessment concluded bilateral cochlear implants provide greater benefits in speech perception test scores, especially in noise, when compared to unilateral cochlear implants (with or without contralateral hearing aids). Significant binaural head shadow benefits were noted along with some benefit in binaural summation, binaural squelch effects, and sound localization with bilateral cochlear implants. However, it was unclear if these benefits were experienced under quiet conditions, although benefits increased with longer bilateral cochlear implant usage indicating a need for longer term studies. Hearing-specific quality of life could not be assessed because only one study evaluated this outcome. Additionally, the evidence available on simultaneous bilateral implantation was found to be insufficient, although gains were experienced in speech perception using open-set sentences or multi-syllable tests compared to unilateral cochlear implants or unilateral listening conditions. The assessment noted longer term studies are needed to further understand the benefits with bilateral cochlear implantation and identify candidacy criteria given the risks of a second surgery and the destruction of the cochlea preventing future medical intervention.
 
In the 2011 Berrettini et al. review of cochlear implant effectiveness in adults (noted above), 13 articles on bilateral cochlear implants were reviewed (Berrettini, 2011). Sound localization improved with bilateral cochlear implants compared to monaural hearing in 6 studies. Significant improvements in hearing in noise and in quiet environments with bilateral implants compared to unilateral implants were reported in 10 studies and 7 studies, respectively. Five of the studies reviewed addressed simultaneous implantation, 5 studies reviewed sequential implantation, and 3 studies included a mix of simultaneous and sequential implantation. However, no studies compared simultaneous to sequential bilateral implantation results, and no conclusions could be made on the timing of bilateral cochlear implantation. Smulders et al. also examined the timing of cochlear implantation in a systematic review of 11 studies; 5 studies addressed postlingually deafened adults and 7 studies addressed prelingually deafened children (discussed below) (Smulders, 2011). One study on adults showed a delay in the timing of the second implantation resulted in poorer outcomes in quiet environments. Nevertheless, all studies reported benefits with bilateral implants, but all studies were considered to be of poor quality and with a high risk of bias.
 
Cochlear Implantation in Pediatrics
In 2011, Forli and colleagues conducted a systematic review of 49 studies on cochlear implant effectiveness in children (Forli, 2011). Heterogeneity of studies precluded performance of a meta-analysis. Early implantation was examined in 22 studies, but few studies compared outcomes of implantations performed prior to one year of age to implantations performed after one year of age. Studies suggest improvements in hearing and communicative outcomes in children receiving implants prior to one year of age, although, it is not certain whether these improvements are related to duration of cochlear implant usage rather than age of implantation. However, the reviewers noted hearing outcomes have been shown to be significantly inferior in patients implanted after 24-36 months. Bilateral cochlear implants improved verbal perception in noise and sound localization compared to unilateral implants in 19 of 20 studies reviewed, but none of the studies compared learning development and language in bilateral versus unilateral cochlear implant recipients. Simultaneous versus sequential bilateral cochlear implantation results were not examined in any of the studies reviewed. Finally, 7 studies were reviewed that examined cochlear implant outcomes in children with associated disabilities. In this population, cochlear implant outcomes were inferior and occurred more slowly but were considered to be beneficial.
 
In a 2011 systematic review of 38 studies, Black and colleagues sought to identify prognostic factors for cochlear implantation in pediatric patients (Black, 2011). A quantitative meta-analysis was not able to be performed due to study heterogeneity. However, 4 prognostic factors: age at implantation, inner ear malformations, meningitis, and Connexin 26 (a genetic cause of hearing loss), consistently influenced hearing outcomes.
 
Pakdaman et al. conducted a systematic review of cochlear implants in children with cochleovestibular anomalies in 2011 (Pakdaman, 2011). Anomalies included inner ear dysplasia such as large vestibular aqueduct and anomalous facial nerve anatomy. Twenty-two studies were reviewed totaling 311 patients. The authors found implantation surgery was more difficult and speech perception was lower in patients with severe inner ear dysplasia. However, heterogeneity in the studies limited interpretation of these findings.
 
In another 2011 systematic review, Roush and colleagues examined the audiologic management of children with auditory neuropathy spectrum disorder (Roush, 2011). The review included 15 studies that addressed cochlear implantation in these patients. All of the studies reported auditory benefit with cochlear implantation in children with auditory neuropathy spectrum disorder. However, the studies were noted to be limited methodologically and further research is needed in this population.
 
Ongoing Clinical Trials
A May 26, 2012 search of clinical trials at online site: ClinicalTrials.gov identified 2 randomized trials on cochlear implant outcomes. In a prospective, randomized, controlled, multicenter study in Finland, auditory performance skills will be assessed in 40 children with bilateral or unilateral cochlear implants for up to 5 years of hearing age (NCT00960102). In this study, speech perception ability, language acquisition, and speech production and speech recognition will be evaluated in addition to quality of life and parent assessment of functional communication performance. In another prospective, randomized trial in the United States, auditory, linguistic and cognitive development, along with quality-of-life outcomes will be evaluated in 252 children up to age 5 years with developmental delay and deafness (NCT01256229). Children with cochlear implants or hearing aids and developmental delays will be compared to children with cochlear implants and without developmental delay.
 
Summary
The available evidence for use of unilateral and bilateral cochlear implant devices is sufficient to improve net health outcome. Therefore, the policy statement for their use in accordance with FDA approval is unchanged.
 
2013 Update
A literature search conducted using the MEDLINE database through August 2013 did not reveal any new information that would prompt a change in the coverage statement. The key identified literature is summarized below.
 
Bilateral Hearing Loss
In 2013, Gaylor and colleagues published an update to the AHRQ technology assessment (discussed previously) (Gaylor, 2013). Sixteen (of 42) studies published through May 2012 were of unilateral cochlear implants. 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 of 4 studies revealed a significant improvement in cochlear-implant relevant QOL after unilateral implantation (standard mean difference: 1.71; 95% confidence interval [CI]: 1.15-2.27). However, these studies varied in design and there was considerable heterogeneity observed across studies (Gaylor, 2013).
 
Crathorne and colleagues published an update of the NICE systematic review (discussed previously) in 2012 (Crathorne, 2012). The objective was to conduct a systematic review of the clinical and cost-effectiveness of bilateral multichannel cochlear implants compared with unilateral cochlear implantation alone or in conjunction with an acoustic hearing aid, in adults with severe-to-profound hearing loss. A literature search was updated in July 2011 and January 2012. Nineteen studies were included in this update; 6 of these studies were included in the original NICE review. Two studies were RCTs with waiting list controls, 10 were prospective pre-⁄post repeated measure or cohort designs, 6 were cross-sectional in design, and one was an economic evaluation. The studies were conducted in the U.S. and Europe; all compared bilateral with unilateral implantation, and 2 compared bilateral implants with a unilateral implant plus acoustic hearing aid (Crathorne, 2012).
 
The included studies in the Crathorne review were of moderate-to-poor quality, including 2 RCTs (Crathorne, 2012). Meta-analyses could not be performed due to heterogeneity between studies in outcome measures and study design. However, all studies reported that bilateral cochlear implants improved hearing and speech perception. One RCT found a significant binaural benefit over the first ear alone for speech and noise from the front (12.6 + 5.4%, p<0.001) and when noise was ipsilateral to the first ear (21 + 6%, p<0.001), and another RCT found a significant benefit for spatial hearing at 3 months post-implantation compared with preimplantation (mean difference [standard deviation] scores: 1.46 [0.83–2.09], p<0.01). QOL results varied, showing bilateral implantation may improve QOL in the absence of worsening tinnitus (Crathorne, 2012).
 
van Schoonhoven and colleagues independently published a systematic review in 2013 as an update to the original NICE review (van Schoonhoven, 2013). As with the Crathorne review, all studies (n=19, published through March 2011) showed a significant bilateral benefit in localization over unilateral cochlear implantation. Similarly, meta-analyses could not be performed due to the heterogeneity of the studies and the level of evidence of the included studies, which was of moderate-to-poor quality (van Schoonhoven, 2013).  
 
Unilateral Hearing Loss
A number of small observational studies from outside the U.S. have reported results on cochlear implantation primarily for adult patients with unilateral deafness, which would be an off-label indication (Arndt, 2011; Van de Heyning, 2008). At least 3 narrative literature reviews on this indication have also been published (Arts, 2012; Kamal, 2012; Sampaio, 2011).
 
Arndt and colleagues, for example, published a German pilot study in 2010 of 11 adult patients with unilateral hearing loss of various causes (Arndt, 2011). The aim was to evaluate the use of unilateral electrical stimulation with normal hearing on the contralateral side and after a period of 6 months compared with the preoperative unaided situation, conventional contralateral routing of signal or bone-anchored hearing aid hearing aids. Ten (of 11) patients also suffered from tinnitus. Two tests were used to assess speech comprehension, localization was assessed using an array of multiple speakers, and QOL was evaluated using 3 questionnaires. The study results were presented as p values without adjustment for multiple testing. The authors reported that cochlear implantation improved hearing abilities in these study patients and was superior to the above alternative treatment options. The use of the cochlear implant did not interfere with speech understanding in the normal-hearing ear.
 
The application of cochlear implants for tinnitus relief in patients with unilateral deafness has also been described in previous studies. van de Heyning and colleagues, for example, published a study in 2008 of 21 patients with unilateral hearing loss accompanied by severe tinnitus for at least 2 years who underwent cochlear implants at a university center in Belgium (Van de Heyning, 2008). Three (of 21) patients showed complete tinnitus relief, whereas the majority demonstrated a significant reduction in tinnitus loudness based on a visual analogue scale (2 years after implantation, 2.5 +/- 1.9; before implantation, 8.5 +/- 1.3). Based on the above data and narrative reviews, the evidence-base to date on unilateral hearing loss is based on a few observational studies with a small number of patients (n ≤30), with a tendency toward reporting bias across these studies (Arts, 2012).
 
2016 Update
A literature search conducted through January 2016 did not reveal any new information that would prompt a change in the coverage statement. The key identified literature is summarized below.
 
Studies published since the above systematic reviews suggest that cochlear implant removal and reimplantation (due to device malfunction or medical/surgical complications) in children is not associated with worsened hearing outcomes (Sterkers, 2015).
 
Fernandes and colleagues evaluated 18 published studies and 2 dissertations that reported hearing performance outcomes for children with ANSD and cochlear implants (Fernandes, 2015).  Studies included 4 nonrandomized controlled studies considered high quality, 5 RCTs considered low quality, and 10 clinical outcome studies. Most studies (n=14) compared the speech perception in children with ANSD and cochlear implants with the speech perception in children with sensorineural hearing loss and cochlear implants. Most of these studies concluded that children with ANSD and cochlear implants developed hearing skills similar to those with sensorineural hearing loss and cochlear implants; however, these types of studies do not allow comparisons of outcomes between ANSD patients treated with cochlear implants and those treated with usual care.
 
In a subsequent small retrospective case series not included in the Humphriss, Fernandes, or Roush systematic reviews, Kontorinis and colleagues described pre- and post-cochlear implantation hearing outcomes (Kontorinis, 2014).among 27 children with ANSD.24 Candidacy for cochlear implants in patients with ANSD was considered if they had severe or even moderate hearing loss when there were deficits in auditory skills and the perception and development of language, and it is not clear how many children would have qualified for a cochlear implant without ANSD.
 
In 2015, van Zon et al published another systematic review of studies evaluating cochlear implantation for single-sided deafness or asymmetric hearing loss (van Zon, 2015). The authors reviewed 15 studies, 9 of which (n=112 patients) were considered high enough quality to be included in data review. The authors identified no high-quality studies of cochlear implantation in this population. Data were not able to be pooled for meta-analysis due to high between-study heterogeneity, but the authors conclude that studies generally report improvements in sound localization, QOL scores, and tinnitus after cochlear implantation, with varying results for speech perception in noise.
  
In 2014, Blasco and Redleaf published a systematic review and meta-analysis of studies evaluating cochlear implantation for unilateral sudden deafness (Blasco, Redleaf, 2014).  The review included 9 studies with a total of 36 patients. In pooled analysis, subjective improvement in tinnitus occurred in 96% of patients (of 27 assessed), subjective improvement in speech understanding occurred in 100% of patients (of 16 assessed), and subjective improvement in sound localization occurred in 87% of patients (of 16 assessed). However, the small number of patients in which each outcome was assessed limits conclusions that may be drawn.
 
Santa Maria and colleagues conducted a systematic review and meta-analysis of hearing outcomes after various types of hearing-preservation cochlear implantation, including implantation hybrid devices, cochlear implantation with surgical techniques designed to preserve hearing, and the use of post-operative systemic steroids (Santa, 2014).  The study included 24 studies, but only 2 focused specifically on a hybrid cochlear implant system, and no specific benefit from a hybrid system was reported.
 
A small number of studies in a small number of patients suggest that a hybrid cochlear implant system is associated with improvements in hearing of speech in quiet and noise. However, there are currently not available studies that compare the use of a standard hearing aid with a hybrid cochlear implant, which would be an appropriate comparison to determine if a hybrid device improves outcomes for patients who currently have hearing loss but might not be candidate for a cochlear implant. In addition, there is only limited data to suggest that the preservation of residual hearing associated with a hybrid device is associated with improved outcomes compared with a standard cochlear implant.
 
The available evidence suggests that a hybrid cochlear implant system is associated with improvements in hearing of speech in quiet and noise. However, the evidence is not sufficient to demonstrate that hybrid cochlear implant/hearing aid systems to improve outcomes compared with either hearing aid or cochlear implant alone; therefore, they are considered to be investigational.
 
Cochlear Implantation in Infants Younger Than 12 Months
A recent (2015) prospective study of 28 children with profound sensorineural hearing loss who were implanted early with cochlear implants (mean age at device activation: 13.3 months) reported that children had social and conversational skills in the range of normal peers (Guerzoni, 2015).
 
Cochlear Implantation in Children: Bilateral Stimulation
Studies, ranging in size from 91 to 961 patients, generally report improved speech outcomes with bilateral implantation, compared with unilateral implantation. In another retrospective case series of 73 children and adolescents who underwent sequential bilateral cochlear implantation with a long (>5 year) interval between implants, performance on the second implanted side was worse than the primary implanted side, with outcomes significantly associated with the inter-implant interval (Illg, 2013).
 
Cochlear Implantation for Unilateral Hearing Loss
Several individual studies have reported on outcomes for cochlear implantation for single-sided deafness since the publication of the systematic reviews described above. The longest follow-up was reported by Mertens and colleagues, in a case series with structured interviews, including 23 individuals who received cochlear implants for single-sided deafness with tinnitus (Mertens, 2016). Eligible patients had either single-sided deafness or asymmetric hearing loss and ipsilateral tinnitus. Subjects had a mean 8 years of experience with their cochlear implant (range, 3-10 years). Tinnitus symptoms were assessed by structured interview, visual analog scale (VAS), and the Tinnitus Questionnaire. Patients demonstrated improvements in VAS from baseline (mean score, 8) to 1 month (mean score: 4; p<0.01 vs baseline) and 3 months (mean score: 3; p<0.01 vs baseline) after the first fitting. Tinnitus scores improved from baseline to 3 months post fitting (55 vs 31, p<0.05) and were stable for the remainder of follow-up.
 
In 2015, Arndt and colleagues reported outcomes for 20 children who underwent cochlear implantation for single-sided deafness, which represented a portion of their center’s cohort of 32 pediatric patients with single-sided deafness who qualified for cochlear implants (Arndt, 2015). Repeated-measure analyses of hearing data sets were available for 13 implanted children, excluding 5 who had undergone surgery too recently to be evaluated and 2 children who were too young to be evaluated for binaural hearing benefit. There was variability in the change in localization ability across the tested children. Self- (or child-) reported hearing benefit was measured with the Speech, Spatial and Qualities of Hearing Scale (SSQ). Significant improvements were reported on the child and parent evaluations for the scale’s 3 subcategories: speech hearing, spatial hearing, hearing quality, and total hearing.
 
Earlier, smaller studies, such as those reported by Arndt and colleagues (2010) with 11 adult patients, and by Hanset  and colleagues (2013) with 29 patients, reported improvements in hearing ability compared with baseline.
 
Cochlear Implant for Tinnitus Relief in Patients With Unilateral Deafness
The application of cochlear implants for tinnitus relief in patients with unilateral deafness has also been described. Studies of cochlear implants in patients with bilateral hearing loss, which is often associated with tinnitus, have reported improvements in tinnitus. For example, Von Zon and colleagues reported on a prospective study focusing on tinnitus perception conducted as a part of a multicenter RCT comparing unilateral with bilateral cochlear implantation in patients with severe bilateral sensorineural hearing loss (van Zon, 2015). This analysis included 38 adults enrolled from 2010-2012 and randomized to simultaneous bilateral or unilateral cochlear implants. At 1 year post-implantation, both unilaterally and bilaterally implanted patients had significant decreases in score on the Tinnitus Handicap Inventory (THI; a validated scale): change in score of 8 to 2 (p=0.03) and from 22 to 12 (p=0.04) for unilaterally and bilaterally implanted patients, respectively. Bilaterally implanted patients had a significant decrease in Tinnitus Questionnaire (TQ; a validated scale): change in score of 20 to 9 (p=0.04).
 
Several individual studies have reported on outcomes for cochlear implantation for single-sided deafness since the publication of the systematic reviews described above.
 
The longest follow-up was reported by Mertens and colleagues, in a case series with structured interviews, including 23 individuals who received cochlear implants for single-sided deafness with tinnitus (Mertens, 2016). Eligible patients had either single-sided deafness or asymmetric hearing loss and ipsilateral tinnitus. Subjects had a mean 8 years of experience with their cochlear implant (range, 3-10 years). Tinnitus symptoms were assessed by structured interview, visual analog scale (VAS), and the Tinnitus Questionnaire. Patients demonstrated improvements in VAS from baseline (mean score, 8) to 1 month (mean score: 4; p<0.01 vs baseline) and 3 months (mean score: 3; p<0.01 vs baseline) after the first fitting. Tinnitus scores improved from baseline to 3 months post fitting (55 vs 31, p<0.05) and were stable for the remainder of follow-up.
 
In 2015, Arndt and colleagues reported outcomes for 20 children who underwent cochlear implantation for single-sided deafness, which represented a portion of their center’s cohort of 32 pediatric patients with single-sided deafness who qualified for cochlear implants (Arndt, 2015). Repeated-measure analyses of hearing data sets were available for 13 implanted children, excluding 5 who had undergone surgery too recently to be evaluated and 2 children who were too young to be evaluated for binaural hearing benefit. There was variability in the change in localization ability across the tested children. Self- (or child-) reported hearing benefit was measured with the Speech, Spatial and Qualities of Hearing Scale (SSQ). Significant improvements were reported on the child and parent evaluations for the scale’s 3 subcategories: speech hearing, spatial hearing, hearing quality, and total hearing.
 
Earlier, smaller studies, such as those reported by Arndt and colleagues (2010) with 11 adult patients, and by Hanset and colleagues (2013) with 29 patients, reported improvements in hearing ability compared with baseline.
 
Based on observations about tinnitus improvement with cochlear implants, several studies have reported on improvements in tinnitus after cochlear implantation in individuals with unilateral hearing loss. In the meta-analysis by Vlastarakos and colleagues (2013) described above, for example, unilateral tinnitus improved in most patients (95%).
 
Ramos Macias et al (2015) reported results of a prospective multicenter study with repeated measures related to tinnitus, hearing, and quality of life, among 16 individuals with unilateral hearing loss and severe tinnitus who underwent cochlear implantation.47 All patients had a severe tinnitus handicap (THI score ≥ 58%). Eight (62%) of the 13 patients who completed the 6-month follow-up visit reported a lower tinnitus handicap on the THI score. Perceived loudness/annoyingness of the tinnitus was evaluated with a 10-point VAS. When the CI was on, tinnitus loudness decreased from 8.4 preoperatively to 2.6 at the 6-month follow-up; 11 of 13 patients reported a change in score of 3 or more.
 
 
Hybrid Cochlear Implantation
Roland and colleagues reported FDA documentation and in per-reviewed form on pivotal trial was a prospective, multicenter, 1-arm, nonrandomized, non-blinded, repeated-measures clinical study among 50 subjects at 10 U.S. sites.
 
Performance was compared pre- with post-implant within each subject; outcomes were measured at 3, 6, and 12 months postoperatively. The study tested 2 co-primary efficacy hypotheses: (1) that outcomes on consonant-nucleus-consonant (CNC; a measure of word recognition); and (2) that AzBio sentences in noise presented through the hybrid implant system would be better at 6 months postimplantation than preoperative performance using a hearing aid.
 
Hearing Benefit With Shorter Cochlear Array
Causon and colleagues evaluated factors associated with cochlear implant outcomes in a meta-analysis of articles published from 2003 to 2013 which reported on PTA measurements pre- and post-cochlear implantation (Causon, .015). Twelve studies with available audiometric data (total N=200 patients) were included. The authors applied a formula to attempt to standardize degree of hearing preservation after cochlear implant, described as the HEARRING consensus statement formula, which calculates a percentage of hearing preservation at a specific frequency band, which is scaled to the preoperative audiogram by dividing the change in hearing by the difference between the maximum measurable threshold and the preoperative hearing threshold. The association of a variety of patient- and surgery-related factors, including insertion depth, and improvement in low-frequency hearing were evaluated. In this analysis, insertion depth was not significantly associated with low-frequency residual hearing.
 
In a retrospective review that included 10 subjects implanted with a cochlear implant with either a standard electrode (n=12) or the Nucleus Hybrid L24 electrode (n=10), loss of acoustic hearing to the severe-profound level occurred in a higher proportion of patients who received a standard electrode (58% vs 30%).
 
Prospective and retrospective studies using a single-arm, within-subjects comparison pre- and post-intervention suggest that a hybrid cochlear implant system is associated with improvements in hearing of speech in quiet and noise. For patients who would typically meet criteria for a cochlear implant, the evidence would suggest that outcomes with a hybrid device are at least as good as those with a standard cochlear implant. There are currently not available studies that compare the use of a standard hearing aid with a hybrid cochlear implant, which would be an appropriate comparison to determine if a hybrid device improves outcomes for patients who currently have hearing loss but are not necessarily current candidates for a cochlear implant.
 
Ongoing and Unpublished Clinical Trials
Some currently unpublished trials that might influence this policy are listed below:
 
(NCT02204618) Cochlear Implantation in Single Sided Deafness and Asymmetrical Hearing Loss: a Cost/Utility Study; planned enrollment 150; completion date August 2017.
 
(NCT02203305) Cochlear Implantation in Cases of Single-Sided Deafness; planned enrollment 30; completion date December 2018
 
The evidence for cochlear implants in individuals who have bilateral sensorineural hearing loss includes randomized controlled trials (RCTs) and multiple systematic reviews and technology assessments. Relevant outcomes include symptoms, functional outcomes, and treatment-related morbidity and mortality. The available studies report improvements in speech reception and quality of life measures. Although the available RCTs and other studies measure heterogeneous outcomes and have varying patient populations, the findings are consistent across multiple studies and settings. In addition, studies show consistent improvement in speech reception (especially in noise) and in sound localization with bilateral devices. Studies also suggest that earlier implantation may be preferred. The evidence is sufficient to determine qualitatively that the technology results in a meaningful improvement in the net health outcome.
 
The evidence for cochlear implants in individuals who have unilateral sensorineural hearing loss includes prospective and retrospective studies reporting within-subjects comparisons and systematic reviews of these studies. Relevant outcomes include symptoms, functional outcomes, and treatment-related morbidity and mortality. Given the natural history of hearing loss, pre- and postimplantation comparisons may be appropriate for objectively measured outcomes. However, the available evidence for the use of cochlear implants in improving outcomes for patients with unilateral hearing loss, with or without tinnitus, is limited by small sample sizes, short follow-up times, and heterogeneity in evaluation protocols and outcome measurements. The evidence is insufficient to determine the effects of the technology on health outcomes.
 
2017 Update
 
A literature search conducted using the Medline database through August 2017. The key identified literature is summarized below. There were no new randomized controlled trials identified.
 
Several case series assessing cochlear implantation for unilateral sensorineural hearing loss were identified.
 
In 2017, Sladen et al retrospectively reviewed prospectively collected data of short-term (6-month) follow-up for 23 adults and children with single-sided deafness from a variety of mechanisms who received a cochlear implant (Sladen, 2017). In the implanted ear, CNC word recognition improved significantly from preimplantation to 3 months postactivation (p=0.001). However, for AzBio sentence understanding in noise (+5 dB signal-to-noise), there was no ignificant improvement from preimplantation to 6 months postactivation.
 
In a prospective repeated-measures cohort study that included 20 subjects with single-sided deafness implanted with cochlear implants (15 of whom had reached 6-month follow-up), Sladen et al (2016) reported on speech recognition and QOL (Sladen, 2017). Pure-tone audiometry improved with air conduction in the implanted ear. CNC scores in quiet improved from 4.8% in the preoperative period to 42.3% at the 6-month postactivation check in patients who reached that follow-up.
 
Also in 2016, Rahne et al reported on a retrospective review of 4 children and 17 adults with single-sided deafness treated with cochlear implants and followed for 12 months (Rhane, 2016). Sound localization with aided hearing improved from preimplantation for all individuals. The speech recognition threshold in noise (signal-to-noise) ratio improved from -1.95 dB (CI off, SD=2.7 dB) to -4.0 dB after 3 months (SD=1.3 dB; p<0.05), with continued improvements through 6 months.
 
The available evidence for the use of cochlear implants in improving outcomes for patients with unilateral hearing loss, with or without tinnitus, is limited by small sample sizes, short follow-up times, and heterogeneity in evaluation protocols and outcome measurements. The coverage statement is changed to specify non-coverage for cochlear implants for the treatment of unilateral sensorineural hearing loss.   
 
2018 Update
 
Annual policy review completed with a literature search using the MEDLINE database through September 2018. No new literature was identified that would prompt a change in the coverage statement. The key identified literature is summarized below.
 
PRACTICE GUIDELINES AND POSITION STATEMENTS
 
American Academy of Otolaryngology - Head and Neck Surgery Foundation The American Academy of Otolaryngology - Head and Neck Surgery Foundation has a position statement on cochlear implants that was revised in 2014 (American Academy of Otolaryngology, 2014). The Foundation “...considers unilateral and bilateral cochlear implantation as 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 [rather] than one, bilateral cochlear implantation is accepted medical practice.”

CPT/HCPCS:
69930Cochlear device implantation, with or without mastoidectomy
92601Diagnostic analysis of cochlear implant, patient younger than 7 years of age; with programming
92602Diagnostic analysis of cochlear implant, patient younger than 7 years of age; subsequent reprogramming
92603Diagnostic analysis of cochlear implant, age 7 years or older; with programming
92604Diagnostic analysis of cochlear implant, age 7 years or older; subsequent reprogramming
L8614Cochlear device, includes all internal and external components
L8615Headset/headpiece for use with cochlear implant device, replacement
L8616Microphone for use with cochlear implant device, replacement
L8617Transmitting coil for use with cochlear implant device, replacement
L8618Transmitter cable for use with cochlear implant device, replacement
L8619Cochlear implant, external speech processor and controller, integrated system, replacement
L8621Zinc air battery for use with cochlear implant device and auditory osseointegrated sound processors, replacement, each
L8622Alkaline battery for use with cochlear implant device, any size, replacement, each
L8623Lithium ion battery for use with cochlear implant device speech processor, other than ear level, replacement, each
L8624Lithium ion battery for use with cochlear implant device speech processor, ear level, replacement, each
L8627Cochlear implant, external speech processor, component, replacement
L8628Cochlear implant, external controller component, replacement
L8629Transmitting coil and cable, integrated, for use with cochlear implant device, replacement

References: American Academy of Otolarygology -- Head and Neck Surgery.(2014) Position Statement: Cochlear Implants. http://www.entnet.org/Practice/policyCochlearImplants.cfm. Accessed January 25, 2018.

Arndt S, Aschendorff A, Laszig R et al.(2011) Comparison of pseudobinaural hearing to real binaural hearing rehabilitation after cochlear implantation in patients with unilateral deafness and tinnitus. Otol Neurotol 2011; 32(1):39-47.

Arndt S, Prosse S, Laszig R, et al.(2015) Cochlear implantation in children with single-sided deafness: does aetiology and duration of deafness matter? Audiol Neurootol. 2015;20 Suppl 1:21-30. PMID 25999052

Arts RA, George EL, Stokroos RJ et al.(2012) Review: cochlear implants as a treatment of tinnitus in single-sided deafness. Curr Opin Otolaryngol Head Neck Surg 2012; 20(5):398-403.

Berrettini S, Baggiani A, Bruschini L et al.(2011) Systematic review of the literature on the clinical effectiveness of the cochlear implant procedure in adult patients. Acta Otorhinolaryngol Ital 2011; 31(5):299-310.

Bittencourt AG, Ikari LS, Della Torre AA et al.(2012) Post-lingual deafness: benefits of cochlear implants vs. conventional hearing aids. Braz J Otorhinolaryngol 2012; 78(2):124-7.

Black J, Hickson L, Black B et al.(2011) Prognostic indicators in paediatric cochlear implant surgery: a systematic literature review. Cochlear Implants Int 2011; 12(2):67-93.

Blakely BW.(2006) Hearing: when surgery is appropriate for age-related hearing loss. In Geriatric Care Otolaryngology . American Academy of Otolaryngology-Head and Neck Surgery Foundation, Pages 11- 14. Available at: http://www.entnet.org/EducationAndResearch/upload/Chapter-1.pdf

Blamey PJ, Maat B, Baskent D, et al.(2015) A Retrospective Multicenter Study Comparing Speech Perception Outcomes for Bilateral Implantation and Bimodal Rehabilitation. Ear Hear. Jul-Aug 2015;36(4):408-416. PMID 25695925

Blasco MA, Redleaf MI.(2014) Cochlear implantation in unilateral sudden deafness improves tinnitus and speech comprehension: meta-analysis and systematic review. Otol Neurotol. Sep 2014;35(8):1426-1432. PMID 24786540

Bond M, Mealing S, Anderson R et al.(2009) 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.

British Cochlear Implant Group (BCIG).(2007) Position Statement - Bilateral Cochlear Implantation. Revised May 2008. Accessed September 2009. Available at: http://www.bcig.org.uk/downloads/pdfs/BCIG%20position%20statement%20-%20Bilateral%20Cochlear%20Implantation%20May%2007.pdf .

Causon A, Verschuur C, Newman TA.(2015) A retrospective analysis of the contribution of reported factors in cochlear implantation on hearing preservation outcomes. Otol Neurotol. Aug 2015;36(7):1137-1145. PMID 25853614

Centers for Medicare & Medicaid.(2013) Cochlear Implantation. https://www.cms.gov/Medicare/Coverage/Coverage-with-Evidence-Development/Cochlear-Implantation-.html. Accessed January 25, 2018.

Ching TY, Dillon H, Day J et al.(2009) Early language outcomes of children with cochlear implants: Interim findings of the NAL study on longitudinal outcomes of children with hearing impairment. Cochlear Implants Int 2009; 10(suppl 1):28-32.

Choi JS, Betz J, Li L, et al.(2016) Association of using hearing aids or cochlear implants with changes in depressive symptoms in older adults. JAMA Otolaryngol Head Neck Surg. Jul 01 2016;142(7):652-657. PMID 27258813

Colletti L.(2009) Long-term follow-up of infants (4-11 months) fitted with cochlear implants. Acta Otolaryngol 2009; 129(4):361-6.

Crathorne L, Bond M, Cooper C et al.(2012) A systematic review of the effectiveness and cost-effectiveness of bilateral multichannel cochlear implants in adults with severe-to-profound hearing loss. Clin Otolaryngol 2012; 37(5):342-54.

Fernandes NF, Morettin M, Yamaguti EH, et al.(2015) Performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implant: a systematic review Braz J Otorhinolaryngol. Jan-Feb 2015;81(1):85-96. PMID 25458263

Firszt JB, Reeder RM, Skinner MW.(2008) Restoring hearing symmetry with two cochlear implants or one cochlear implant and a contralateral hearing aid. J Rehabil Res Dev 2008; 45(5):749-67.

Forli F, Arslan E, Bellelli S et al.(2011) Systematic review of the literature on the clinical effectiveness of the cochlear implant procedure in paediatric patients. Acta Otorhinolaryngol Ital 2011; 31(5):281-98.

Friedmann DR, Peng R, Fang Y, et al.(2015) Effects of loss of residual hearing on speech performance with the CI422 and the Hybrid-L electrode. Cochlear Implants Int. Sep 2015;16(5):277-284. PMID 25912363

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