Original Issue Date (Created): August 28, 2012 Most Recent Review Date (Revised): March 25, 2014 Effective Date: June 1, 2014 I. POLICY Unilateral use of an auditory brainstem implant (using surface electrodes on the cochlear nuclei) may be considered medically necessary in patients with neurofibromatosis type 2, who are 12 years of age or older, and who are rendered deaf due to bilateral resection of neurofibromas of the auditory nerve. An auditory brainstem implant is considered investigational for all other conditions including non-neurofibromatosis-type 2 indications. Bilateral use of an auditory brainstem implant is considered investigational. Penetrating electrode auditory brainstem implant (PABI) is considered investigational. There is insufficient evidence to support a conclusion supporting the health outcomes or benefits associated with the above procedures Cross-reference: MP-1.019 Implantable Bone-Conduction and Bone-Anchored Hearing Prosthetic Devices MP-1.023 Cochlear Implant MP-4.020 Evaluation and Treatment of Hearing Loss MP-1.130 Semi-Implantable and Fully Implantable Middle Ear Hearing Aid for Moderate to Severe Sensorineural Hearing Loss II. PRODUCT VARIATIONS [N] = No product variation, policy applies as stated [Y] = Standard product coverage varies from application of this policy, see below [N] Capital Cares 4 Kids [N] PPO [N] HMO [Y] SeniorBlue HMO* [Y] SeniorBlue PPO* [N] Indemnity [N] SpecialCare [N] POS [Y] FEP PPO** Page 1
*Auditory Brainstem Implants are eligible for coverage. These devices are indicated only when hearing aids are medically inappropriate or cannot be utilized due to congenital malformations, chronic disease, severe sensorineural hearing loss, or surgery (Centers for Medicare and Medicaid Services (CMS) Medicare Benefit Policy Manual. Publication 100-2. Chapter 16, Revision 39, Section 100. Hearing Aids and Auditory Implants). **Refer to FEP Medical Policy Manual MP-7.01.83 Auditory Brainstem Implant. The FEP Medical Policy manual can be found at: www.fepblue.org III. DESCRIPTION/BACKGROUND The auditory brain stem implant is a device used to restore some hearing in patients with neurofibromatosis type II who are rendered deaf by bilateral removal of neurofibromas that involve the auditory nerve. The device consists of an external speech processor that provides auditory information to an electrical signal that is transferred to a receiver/stimulator that is implanted in the temporal bone. The receiver/stimulator is attached to an electrode array that is implanted on the surface of the cochlear nerve in the brainstem. The electrode stimulates multiple sites on the cochlear nucleus, which are then processed normally by the brain. One device has received approval by the U.S. Food and Drug Administration (FDA) for auditory brainstem implantation: the Nucleus 24 Auditory Brainstem Implant System (Cochlear Corporation). The speech processor and receiver are similar to the devices used in cochlear implants; the electrode array placed on the brainstem is the novel component of the device. The device is indicated for individuals 12 years of age or older who have been diagnosed with neurofibromatosis type 2 (NF2). IV. RATIONALE Unilateral Auditory Brainstem Implant In Patients With Neurofibromatosis Type 2 FDA approval of the Nucleus 24 Auditory Brainstem Implant System was based on results in a case series of 90 patients with neurofibromatosis type 2 (NF2), ages 12 years and older. (1, 2) Of the 90 subjects evaluated, 28 complications occurred in 26 patients; 26 of these complications resolved without surgical or extensive medical intervention. Two patients had infections of the postoperative flap requiring explantation of the device. A total of 60 patients had a minimum experience of 3 to 6 months with the device, and thus effectiveness outcomes were also evaluated. Overall 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% patients (57 of 60) derived benefit from the device. While the use of an auditory brainstem implant (ABI) is associated with a very Page 2
modest improvement in hearing, this level of improvement is considered significant in this group of patients who have no other treatment options. Among the 90 patients 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, patients with large, long-standing tumors may not benefit from the device. In 2012, Sanna and colleagues reported on 25 ABIs placed in 24 patients with NF2. (3) In this retrospective case study, patients were followed up for a range of 2 53 months. One patient died due to NF2 progression. Sound recognition was present in 19 patients, of whom 11 had some word recognition and 8 had good speech recognition (50% speech discrimination in 4 patients and 75-100% speech discrimination and telephone use in 4 patients). A multivariate analysis did not identify any factors that were statistically significant in predicting ABI performance outcomes. The authors also conducted a review of the literature on ABIs and found it difficult to compare outcomes as reporting methods and outcomes measured were inconsistent and imprecise. A retrospective review by Goffi- Gomez and colleagues report mixed results in audiologic outcomes in 5 adults (4 with NF2) and 4 children (all with non-tumor diseases) who received ABIs between 2005 and 2009 at a university center in Brazil. (4) Unilateral Auditory Brainstem Implant In Nontumor Patients In a 2004 study in Italy, V. Colletti and colleagues reported on the use of ABIs in patients with deafness unrelated to neurofibromatosis and who had a poor response to cochlear implants. (5) However, there are inadequate data from this study to permit scientific conclusions regarding this additional indication. V. Colletti and colleagues, in 2005, presented data from ABIs in 16 children and adults who had non-tumor diseases of the cochlear nerve or cochlea and 13 patients with NF2. (6) Ages ranged from 14 months to 70 years; the non-tumor group included patients with head trauma, complete cochlear ossification, 1 child with auditory neuropathy, and 5 children with bilateral cochlear nerve aplasia. Following implantation, the adult non-tumor group scored substantially higher than the patients with NF2 in open set speech perception tests. Some of the children showed dramatic improvements in word and sentence recognition over a 1-year follow-up. Short-term adverse effects included dizziness or tingling sensations in the leg, arm, and throat (20 of 29 patients). Page 3
Additional studies report improvement in hearing with ABIs in non-tumor patients; V. Colletti has reported results on 54 non-tumor patients, (7) and L. Colletti has reported results on 22 non-neurofibromatosis patients. (8) In a 2010 retrospective review, the authors previously cited, V. Colletti and colleagues, reported on the complications from ABI surgery in 83 adults and 31 children, 78 of whom had nontumor cochlear or cochlear nerve disorders. (9) The authors found complication rates were similar to cochlear implant surgery. Additionally, major and minor complications were significantly fewer in nontumor patients than in NF2 patients. These authors concluded ABIs can be used in a wider population of patients than only those with NF2. However, this review did not evaluate hearing outcomes. Sennaroglu et al., in 2009, reported on the use of ABIs in 11 prelingually deaf children ages 30-56 months. (10) Results showed mean performance on the Meaningful Auditory Integration Scale improved in all patients. However, the results of this small study are described as only preliminary. Bilateral Auditory Brainstem Implants Nucleus 24 Auditory Brainstem Implant System (Cochlear Corporation) labeling states (1) The efficacy of bilateral implantation with the ABI has not been studied. No evidence was identified to support bilateral auditory brainstem stimulation. The studies included to date only included patients with unilateral auditory brainstem implantation. Penetrating Electrode Auditory Brainstem Implant In 2008, Otto et al. (11) conducted a prospective clinical trial (n=10) with patients with NF2 who received a penetrating electrode auditory brainstem implant (PABI) after vestibular schwannoma removal. The PABI is an extension of the ABI technology that uses surface electrodes on cochlear nuclei. PABI uses 8 or 10 penetrating microelectrodes in conjunction with a separate array of 10 to 13 surface electrodes. The PABI met the goals of lower threshold, increased pitch range, and high selectivity, but these properties did not result in improved speech recognition. These data are inadequate to draw conclusions regarding the effectiveness of PABI as compared to conventional ABI. Three general reviews were identified on ABI. Sennaroglu and Ziyal review the history and development of ABI, indications, side selection criteria, surgical options and audiological outcomes after ABI. (12) Colletti and colleagues discuss new developments on ABI specifically for NF2. (13) Schwartz and colleagues (14) discuss in a 2008 review article the future directions in central implants for hearing, including PABI, the use of ABI in nontumor patients, and the auditory midbrain implant. Page 4
Ongoing Clinical Trials A search of online site ClinicalTrials.gov identified one ongoing study on auditory brainstem implant. Study of Nucleus 24 Auditory Brainstem Implant (ABI) in Adult Non-Neurofibromatosis Type 2 Subjects (NCT01736267) The purpose of this single-arm study is to analyze whether ABI surgery improves the hearing and quality of life of non-nf2 patients based on subjective and objective measures of their hearing before and after ABI surgery. This study has an estimated enrollment of 10 adult patients (with bilateral deafness who are not candidates for cochlear implants) at the Massachusetts Eye and Ear Infirmary in the U.S. The primary outcome measures are perioperative and postoperative complications, audiologic performance, and ABI electophysiologic parameters at 5 years from date of surgery. This study is currently recruiting participants with the estimated completion date of November 2022. Summary The Nucleus 24 Auditory Brainstem (ABI) Implant received FDA approval only for patients with neurofibromatosis type 2 (NF2) following tumor removal. The available evidence for unilateral use of ABI devices in patients with NF2 is sufficient to demonstrate improvements in net health outcomes. Therefore, the policy statement indicates an auditory brainstem implant may be considered medically necessary in this condition. ABIs hold promise for patients with cochlear and cochlear nerve abnormalities when cochlear implants are not indicated. However, studies on ABIs for non-nf2 conditions are limited, with small numbers of patients and insufficient data to make scientific conclusions. Given the lack of both high-quality evidence and FDA approval, ABI for non-nf2 conditions and bilateral ABI are considered investigational. Penetrating electrode auditory brainstem implant is also considered investigational, since the very limited evidence available is insufficient to draw conclusions on health outcomes. Practice Guidelines and Position Statements In January 2005, National Institute for Clinical Excellence (NICE) issued Interventional Procedure Guidance 108, Auditory Brain Stem Implants. (15) The guidance states the following: evidence on safety and efficacy of auditory brain stem implants appears adequate to support the use of this procedure by surgical teams experienced in this technique. Page 5
V. DEFINITIONS NEUROFIBROMA is a tumor of the connective tissue of the nerve. NEUROFIBROMATOSIS is a group of genetic disorders that affects the cell growth of neural tissues. NEUROFIBROMATOSIS TYPE II (NF-2) is an autosomal dominant disease affecting 1 in 50,000 persons and which causes intracranial and spinal tumors. VI. BENEFIT VARIATIONS The existence of this medical policy does not mean that this service is a covered benefit under the member's contract. Benefit determinations should be based in all cases on the applicable contract language. Medical policies do not constitute a description of benefits. A member s individual or group customer benefits govern which services are covered, which are excluded, and which are subject to benefit limits and which require preauthorization. Members and providers should consult the member s benefit information or contact Capital for benefit information. VII. DISCLAIMER Capital s medical policies are developed to assist in administering a member s benefits, do not constitute medical advice and are subject to change. Treating providers are solely responsible for medical advice and treatment of members. Members should discuss any medical policy related to their coverage or condition with their provider and consult their benefit information to determine if the service is covered. If there is a discrepancy between this medical policy and a member s benefit information, the benefit information will govern. Capital considers the information contained in this medical policy to be proprietary and it may only be disseminated as permitted by law. VIII. REFERENCES 1. Nucleus 24 Auditory Brainstem Implant System. FDA Summary of Safety and Effectiveness. Available online at: http://www.accessdata.fda.gov/scripts/cdrh/cfdocs/cftopic/pma/pma.cfm?num=p000015. Last accessed March 6, 2012. 2. Ebinger K, Otto S, Arcaroli J et al. Multichannel auditory brainstem implant: US clinical trial results. J Laryngol Otol Suppl 2000; (27):50-3. 3. Sanna M, Di Lella F, Guida M et al. Auditory brainstem implants in NF2 patients: results and review of the literature. Otol Neurotol 2012; 33(2):154-64. 4. Goffi-Gomez MV, Magalhaes AT, Brito Neto R et al. Auditory brainstem implant outcomes and MAP parameters: report of experiences in adults and children. Int J Pediatr Otorhinolaryngol 2012; 76(2):257-64. Page 6
5. Colletti V, Fiorino FG, Carner M et al. Auditory brainstem implant as a salvage treatment after unsuccessful cochlear implantation. Otol Neurotol 2004; 25(4):485-96; discussion 96. 6. Colletti V, Carner M, Miorelli V et al. Auditory brainstem implant (ABI): new frontiers in adults and children. Otolaryngol Head Neck Surg 2005; 133(1):126-38. 7. Colletti V. Auditory outcomes in tumor vs. nontumor patients fitted with auditory brainstem implants. Adv Otorhinolaryngol 2006; 64:167-85. 8. Colletti L. Beneficial auditory and cognitive effects of auditory brainstem implantation in children. Acta Otolaryngol 2007; 127(9):943-6. 9. Colletti V, Shannon RV, Carner M et al. Complications in auditory brainstem implant surgery in adults and children. Otol Neurotol 2010; 31(4):558-64. 10. Sennaroglu L, Ziyal I, Atas A et al. Preliminary results of auditory brainstem implantation in prelingually deaf children with inner ear malformations including severe stenosis of the cochlear aperture and aplasia of the cochlear nerve. Otol Neurotol 2009; 30(6):708-15. 11. Otto SR, Shannon RV, Wilkinson EP et al. Audiologic outcomes with the penetrating electrode auditory brainstem implant. Otol Neurotol 2008; 29(8):1147-54. 12. Sennaroglu L, Ziyal I. Auditory brainstem implantation. Auris Nasus Larynx 2012; 39(5):439-50. 13. Colletti L, Shannon R, Colletti V. Auditory brainstem implants for neurofibromatosis type 2. Curr Opin Otolaryngol Head Neck Surg 2012; 20(5):353-7. 14. Schwartz MS, Otto SR, Shannon RV et al. Auditory brainstem implants. Neurotherapeutics 2008; 5(1):128-36. 15. National Institute for Clinical Excellence (NICE). Interventional Procedure Guidance 108. Auditory brain stem implants. 2005. Available online at: http://guidance.nice.org.uk/ipg108. Last accessed February 2013. 16. Medicare Policy Benefit Manual. Chapter 16 - General Exclusions from Coverage. Available online at: http://www.cms.gov/manuals/downloads/bp102c16.pdf. Last accessed February 2013 Other: Taber s Cyclopedic Medical Dictionary, 19 th edition. IX. CODING INFORMATION Note: This list of codes may not be all-inclusive, and codes are subject to change at any time. The identification of a code in this section does not denote coverage as coverage is determined by the terms of member benefit information. In addition, not all covered services are eligible for separate reimbursement. Page 7
Covered when medically necessary: CPT Codes 61875 92640 Current Procedural Terminology (CPT) copyrighted by American Medical Association. All Rights Reserved. HCPCS Code L8699 S2235 Description Cochlear device, includes internal and external components Implantation of auditory brain stem implant ICD-9-CM Diagnosis Description Code* 237.72 Neurofibromatosis Type 2 [acoustic neurofibromatosis ] *If applicable, please see Medicare LCD or NCD for additional covered diagnoses. The following ICD-10 diagnosis codes will be effective October 1, 2015: ICD-10-CM Diagnosis Description Code* Q85.02 Neurofibromatosis, type 2 *If applicable, please see Medicare LCD or NCD for additional covered diagnoses. X. POLICY HISTORY MP 1.085 CAC 8/28/12 Information regarding auditory brain stem implants extracted from MP 1.023 and this new policy created. Added investigational indications for all other conditions including non-neurofibromatosis-type. Coded 8/14/12 klr 04/22/2013- Admin code review. CAC 7/30/13 Consensus review. References updated, but no changes to the policy statements CAC 3/25/14 Consensus review. References updated, no changes to the policy statements. Rationale added. Policy coded. Health care benefit programs issued or administered by Capital BlueCross and/or its subsidiaries, Capital Advantage Insurance Company, Capital Advantage Assurance Company and Keystone Health Plan Central. Independent licensees of the BlueCross BlueShield Association. Communications issued by Capital BlueCross in its capacity as administrator of programs and provider relations for all companies. Page 8