Latest Review Date: March 2024

Category:  Surgery

POLICY:

Unilateral auditory brain stem implants that are FDA approved may be considered medically necessary in individuals 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.

Bilateral use of an auditory brainstem implant is considered investigational.

Upgrades of existing components for next generation devices may be considered medically necessary when the individual’s existing components are inadequate to the point of interfering with the activities of daily living, or the components are no longer functional. 

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 considered not medically necessary.

An auditory brainstem implant is considered investigational for all other conditions, including non-neurofibromatosis type 2 indications.

DESCRIPTION OF PROCEDURE OR SERVICE:

An auditory brainstem implant (ABI) is designed to restore some hearing in people with neurofibromatosis type 2 who are rendered deaf by bilateral removal of neurofibromas involving the auditory nerve. ABIs have also been studied to restore hearing for other non-neurofibromatosis indications.

The auditory brainstem implant (ABI) is intended to restore some hearing in people with neurofibromatosis type 2 who are rendered deaf by bilateral removal of the characteristic neurofibromas involving the auditory nerve. The ABI consists of an externally worn speech processor that provides auditory information by electrical signal that is transferred to a receiver/stimulator implanted in the temporal bone. The receiver stimulator is, in turn, attached to an electrode array implanted on the surface of the cochlear nerve in the brainstem, thus bypassing the inner ear and auditory nerve. The electrode stimulates multiple sites on the cochlear nucleus, which is then processed normally by the brain. To place the electrode array on the surface of the cochlear nucleus, the surgeon must be able to visualize specific anatomic landmarks. Because large neurofibromas compress the brainstem and distort the underlying anatomy, it can 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.

ABIs are also being studied to determine whether they can restore hearing for other non-neurofibromatosis causes of hearing impairment in adults and children, including absence of or trauma to the cochlea or auditory nerve. It is estimated that 1.7 per 100,000 children are affected by bilateral cochlea or cochlear nerve aplasia and 2.6 per 100,000 children are affected by bilateral cochlea or cochlear nerve hypoplasia.

KEY POINTS:

The most recent literature update was performed through December18, 2023.

Summary of Evidence

For individuals who are deaf due to bilateral resection of neurofibromas of the auditory nerve who receive an auditory brainstem implant (ABI), the evidence includes a large, prospective case series and a technology assessment that included observational studies. Relevant outcomes are functional outcomes, quality of life, and treatment-related morbidity. The technology assessment found the highest quality evidence for improvement in hearing function, but evidence on other outcomes was lacking. The U.S. Food and Drug Administration approval of the Nucleus 24 device in 2000 was based on a prospective case series of 90 patients 12 years of age or older, of whom 60 had the implant for at least 3 months. From this group, 95% had a significant improvement in lip reading or improvement on sound-alone tests. While use of an ABI is associated with a very modest improvement in hearing, this level of improvement is considered significant for those patients who have no other treatment options. A systematic review of 16 studies found that ABI was associated with improved sound recognition and speech perception. Based on these results, ABIs are considered appropriate for the patient population age ≥12 years with neurofibromatosis type 2 and deafness following tumor removal. The evidence is sufficient to determine that the technology results in an improvement in the net health outcome.

For individuals who are deaf due to nontumor etiologies who receive an auditory brainstem implant (ABI), the evidence includes case series and systematic reviews of case series. Relevant outcomes are functional outcomes, quality of life, and treatment-related morbidity. In general, ABIs have not demonstrated hearing benefits over cochlear implants for many conditions not related to neurofibromatosis type 2, and some older (now obsolete) ABI models have been associated with high rates of device failure and adverse events in this population. In addition, ABI studies have shown inferior outcomes in children with other disabilities. However, ABIs hold promise for select patients when the cochlea or cochlear nerve is absent. Evaluation is currently ongoing with the recently available Nucleus ABI541 to determine its efficacy and durability in children. Thus, further study is needed to define populations that would benefit from these devices. The evidence is insufficient to determine that the technology results in an improvement in the net health outcome.

Practice Guidelines and Position Statements

National Institute for Health and Care Excellence

In 2005, National Institute Health and Care Excellence issued guidance on interventional procedures for auditory brainstem implants. The guidance stated: “…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.”

U.S. Preventive Services Task Force Recommendations

Not applicable.

KEY WORDS:

Auditory brain stem implant (ABI), neurofibromatosis II, neurofibromas, auditory nerve, penetrating electrode brainstem implant, PABI, Nucleus 24® Auditory Brainstem Implant System, Nucleus ABI541

APPROVED BY GOVERNING BODIES:

In 2000, the Nucleus® 24 Auditory Brainstem Implant System (Cochlear Corp.) was approved by the U.S. Food and Drug Administration (FDA) through the premarket approval process. 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. Nucleus® 24 Auditory Brainstem Implant System (Cochlear Corp.) labeling states: “The efficacy of bilateral implantation with the ABI has not been studied.” The Nucleus® 24 is currently obsolete.

In June 2016, the Nucleus ABI541 Auditory Brainstem Implant (Cochlear Corp) was approved by the FDA through a supplement to the premarket approval for the Nucleus® 24. The implant is indicated for individuals 12 years of age or older who have been diagnosed with neurofibromatosis type 2.

BENEFIT APPLICATION:

Coverage is subject to member’s specific benefits. Group-specific policy will supersede this policy when applicable.

ITS: Home Policy provisions apply

FEP contracts: Special benefit consideration may apply. Refer to member’s benefit plan.  

CURRENT CODING:

CPT codes:

HCPCS:

REFERENCES:

1. Colletti L, Shannon R, Colletti V. Auditory brainstem implants for neurofibromatosis type 2. Curr Opin Otolaryngol Head Neck Surg. Oct 2012; 20(5):353-357.
2. Colletti L, Wilkinson EP, Colletti V. Auditory brainstem implantation after unsuccessful cochlear implantation of children with clinical diagnosis of cochlear nerve deficiency. Ann Otol Rhinol Laryngol. Oct 2013; 122(10):605-612.
3. Colletti L. Beneficial auditory and cognitive effects of auditory brainstem implantation in children. Acta Otolaryngol, September 2007; 127(9): 943-946.
4. Colletti V, Carner M, Miorelli V, et al. Auditory brainstem implant (ABI): New frontiers in adults and children. Otolaryngol Head Neck Surg, July 2005; 133(1): 126-138.
5. Colletti V, Fiorino FG, Carner M, et al. Auditory brainstem implant as a salvage treatment after unsuccessful cochlear implantation. Otol Neurotol. Jul 2004; 25(4):485-496; discussion 496.
6. 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.
7. Colletti V. Auditory outcomes in tumor vs. non-tumor patients fitted with auditory brainstem implants. Adv Otorhinolaryngol 2006; 64: 167-185.
8. Ebinger K, Otto S, Arcaroli J, et al.  Multichannel auditory brainstem implant:  U.S. clinical trial results. Journal of Laryngology Otology Supplement, 2000; 27: 50-3.
9. Food and Drug Administration. Nucleus 24 Auditory Brainstem Implant System. FDA Summary of Safety and Effectiveness. 2000; www.accessdata.fda.gov/cdrh_docs/pdf/P000015B.pdf. Accessed January 25, 2021.
10. Food and Drug Administration. Premarket Approval (PMA). Nucleus ABI541 Auditory Brainstem Implant. 2016. www.accessdata.fda.gov/scripts/cdrh/cfdocs/cfpma/pma.cfm?id=P000015S012. Accessed January 25, 2021.
11. 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. Feb 2012; 76(2):257-264.
12. IOM (Institute of Medicine). 2011. Clinical Practice Guidelines We Can Trust. Washington, DC: The National Academies Press.
13. Jackson KB, Mark G, Helms J, et al. An auditory brainstem implant system. American Journal of Audiology, 2002; 11(2): 128-33. (Abstract)
14. Kaplan AB, Kozin ED, Puram SV, et al. Auditory brainstem implant candidacy in the United States in children 0-17 years old. Int J Pediatr Otorhinolaryngol. Mar 2015; 79(3):310-315.
15. Lenarz M, Matthes C, et al. Auditory brainstem implant, part II: Subjective assessment of functional outcome, 2002; 23(5): 694-7.  (Abstract)
16. Lenarz T, Moshrefi M, Matthies C, et al. Auditory brainstem implant, part i: auditory performance and its evolution over time. Otology and Neurotology. 2001; 22(6): 823-33.  (Abstract)
17. Matthies C, Brill S, Kaga K, et al. Auditory brainstem implantation improves speech recognition in neurofibromatosis type II patients. ORL J Otorhinolaryngol Relat Spec. 2013; 75(5):282-295.
18. Medina M, Di Lella F, Di Trapani G, et al. Cochlear implantation versus auditory brainstem implantation in bilateral total deafness after head trauma: personal experience and review of the literature. Otol Neurotol. Feb 2014; 35(2):260-270.
19. Merkus P, Di Lella F, Di Trapani G, et al. Indications and contraindications of auditory brainstem implants: systematic review and illustrative cases. Eur Arch Otorhinolaryngol. Jan 2014; 271(1):3-13.
20. National Institute for Clinical Excellence (NICE). Interventional Procedure Guidance 108. Auditory brain stem implants. 2005; //guidance.nice.org.uk/IPG108.
21. Nevison B, Laszig R, Sollmann WP, et al. Results from a European clinical investigation of the nucleus multichannel auditory brainstem implant. Ear and Hearing, 2002; 23(3): 170-83. (Abstract) Accessed January 25, 2021.
22. Noij KS, Kozin ED, Sethi R, et al. Systematic review of nontumor pediatric auditory brainstem implant outcomes. Otolaryngol Head Neck Surg. Nov 2015; 153(5):739-750.
23. Nucleus ® 24 Auditory Brainstem Implant System. FDA Summary of Safety and Effectiveness. www.accessdata.fda.gov/scripts/cdrh/cfdocs/cftopic/pma/pma.cfm?num=p000015.
24. Ontario Health (Quality). Auditory brainstem implantation for adults with neurofibromatosis 2 or severe inner ear abnormalities:a health technology assessment. Ont Health Technol Assess Ser [Internet]. 2020 Mar;20(4): 185. www.hqontario.ca/evidence-to-improve-care/health-technology- assessment/reviews-and-recommendations/auditory-brainstem-implantation-for-adults-with- neurofibromatosis-2-or-severe-inner-ear-abnormalities. Accessed January 14, 2021.
25. Otto S, Brachmann D, Hitselberger W, et al. Brainstem electronic implants for bilateral anacusis following surgical removal of cerebello pontine angle lesions. Otolaryngologic Clinics of North America, 2001; 34(2).
26. Otto S, Brachmann D, Hitselberger W, et al. Multichannel auditory brainstem implant: Update on performance in 61 patients. Journal of Neurosurgery, 2002; 96(6): 1063-71. (Abstract)
27. Otto SR, Shannon RV, Wilkinson EP, et al. Audiologic outcomes with the penetrating electrode auditory brainstem implant. Otol Neurotol, December 2008; 29(8): 1147-1154.
28. Puram SV, Barber SR, Kozin ED, et al. Outcomes following Pediatric Auditory Brainstem Implant Surgery: Early Experiences in a North American Center. Otolaryngol Head Neck Surg. Jul 2016; 155(1):133-138.
29. 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.
30. Schwartz MS, Otto SR, Shannon RV, et al. Auditory brainstem implants. Neurotherapeutics, January 2008; 5(1): 128-136.
31. Sennaroglu L, Sennaroglu G, Yucel E, et al. Long-term Results of ABI in Children With Severe Inner Ear Malformations. Otol Neurotol. Aug 2016; 37(7):865-872.
32. 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. Sep 2009; 30(6):708-715.
33. Toh E, Luxford W. Cochlear and brainstem implantation. Otolaryngologic Clinics of North America, 2002; 35(2).

POLICY HISTORY:

Medical Policy Group, November 2003 (1)

Medical Policy Administration Committee, December 2003

Available for comment December 16, 2003-January 29, 2004

Medical Policy Group, November 2005 (1)

Medical Policy Group, August 2006 (2)

Medical Policy Administration Committee, August 2006

Available for comment August 30-October 13, 2006

Medical Policy Group, August 2008 (1)

Medical Policy Group, August 2010 (1): Description, Key Points updated, Key words added, new policy non-coverage statement added

Medical Policy Administration Committee, July 2010

Available for comment July 23-September 6, 2010

Medical Policy Group, September 2012 (1): Update to Key Points, and References related to MPP update; no change to policy statement

Medical Policy Panel March 2013

Medical Policy Group, December 2013 (2): No new references identified that would change policy statement. 

Medical Policy Panel, March 2015

Medical Policy Group, March 2015 (6):  Updates to Key Points, Key Words and References; no change to policy statement.

Medical Policy Panel, September 2016

Medical Policy Group, September 2016 (6): Updates to Key Points, Key Words, Regulatory Status, Summary and References. No change to policy intent.

Medical Policy Panel, February 2017

Medical Policy Group, February 2017 (6): Updates to policy statement to include “Bilateral use of an auditory brainstem implant is considered investigational and not medically necessary.” added “unilateral” to policy statement , and “An auditory brainstem implant is considered investigational and not medically necessary for all other conditions, including non-neurofibromatosis type 2 indications.”, Description and Key Points.

Medical Policy Administration Committee, March 2017

Available for comment March 1 through April 14, 2017

Medical Policy Panel, February 2018

Medical Policy Group, February 2018 (6): Updates to Description and Key Points.

Medical Policy Panel, February 2019

Medical Policy Group, February 2019 (6): Updates to Description.

Medical Policy Panel, February 2020

Medical Policy Group, February 2020 (6): Updates to Key Points and References.

Medical Policy Panel, February 2021

Medical Policy Group, February 2021 (6): Updates to Description, Key Points and References.

Medical Policy Panel, February 2022

Medical Policy Group, February 2022(6): Updates to Description and Key Points.

Medical Policy Panel, February 2023

Medical Policy Group, February 2023 (6) Updates to Key Points, Benefit Application and References.

Medical Policy Panel, February 2024

Medical Policy Group, March 2024 (9): Updates to Key Points. Minor update to Benefit Application. No change to Policy Statement.  

This medical policy is not an authorization, certification, explanation of benefits, or a contract. Eligibility and benefits are determined on a case-by-case basis according to the terms of the member’s plan in effect as of the date services are rendered. All medical policies are based on (i) research of current medical literature and (ii) review of common medical practices in the treatment and diagnosis of disease as of the date hereof. Physicians and other providers are solely responsible for all aspects of medical care and treatment, including the type, quality, and levels of care and treatment.

This policy is intended to be used for adjudication of claims (including pre-admission certification, pre-determinations, and pre-procedure review) in Blue Cross and Blue Shield’s administration of plan contracts.

The plan does not approve or deny procedures, services, testing, or equipment for our members. Our decisions concern coverage only. The decision of whether or not to have a certain test, treatment or procedure is one made between the physician and his/her patient. The plan administers benefits based on the member’s contract and corporate medical policies. Physicians should always exercise their best medical judgment in providing the care they feel is most appropriate for their patients. Needed care should not be delayed or refused because of a coverage determination.

As a general rule, benefits are payable under health plans only in cases of medical necessity and only if services or supplies are not investigational, provided the customer group contracts have such coverage.

The following Association Technology Evaluation Criteria must be met for a service/supply to be considered for coverage:

1. The technology must have final approval from the appropriate government regulatory bodies;

2. The scientific evidence must permit conclusions concerning the effect of the technology on health outcomes;

3. The technology must improve the net health outcome;

4. The technology must be as beneficial as any established alternatives;

5. The improvement must be attainable outside the investigational setting.

Medical Necessity means that health care services (e.g., procedures, treatments, supplies, devices, equipment, facilities or drugs) that a physician, exercising prudent clinical judgment, would provide to a patient for the purpose of preventing, evaluating, diagnosing or treating an illness, injury or disease or its symptoms, and that are:

1. In accordance with generally accepted standards of medical practice; and

2. Clinically appropriate in terms of type, frequency, extent, site and duration and considered effective for the patient’s illness, injury or disease; and

3. Not primarily for the convenience of the patient, physician or other health care provider; and

4. Not more costly than an alternative service or sequence of services at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of that patient’s illness, injury or disease.