Auditory Brainstem Implant Form

500 EXCHANGE STREET, PROVIDENCE, RI 02903-2699 MEDICAL COVERAGE POLICY | 1 (401) 274-4848 WWW.BCBSRI.COM EFFECTIVE DATE: 03|02|2010 POLICY LAST REVIEWED: 03|19|2025 OVERVIEW This policy documents the coverage determination for Auditory Brain Stem Implant. An auditory brainstem implant (ABI) is designed to restore some hearing in people with neurofibromatosis type 2 (NF2) 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. MEDICAL CRITERIA Not applicable PRIOR AUTHORIZATION Not applicable POLICY STATEMENT Medicare Advantage Plans Unilateral use of an auditory brainstem implant (using surface electrodes on the cochlear nuclei) is covered in individuals with neurofibromatosis type 2 (ICD-10 diagnosis code Q85.02), who are 12 years of age or older, and who are rendered deaf due to bilateral resection of neurofibromas of the auditory nerve.
The following are not covered as the evidence is insufficient to determine the effects of the technology on health outcomes:  An auditory brainstem implant for all other conditions including non-neurofibromatosis type 2 indications  Bilateral use of an auditory brainstem implant  Penetrating electrode auditory brainstem implant (PABI) Commercial Products Unilateral use of an auditory brainstem implant (using surface electrodes on the cochlear nuclei) is considered medically necessary in patients with neurofibromatosis type 2 (ICD-10 CM diagnosis code Q85.02), who are 12 years of age or older, and who are rendered deaf due to bilateral resection of neurofibromas of the auditory nerve.
The following are not medically necessary as the evidence is insufficient to determine the effects of the technology on health outcomes:  An auditory brainstem implant for all other conditions including non-neurofibromatosis type 2 indications  Bilateral use of an auditory brainstem implant  Penetrating electrode auditory brainstem implant (PABI) COVERAGE Benefits may vary between groups/contracts. Please refer to the appropriate Benefit Booklet, Evidence of Coverage, or Subscriber Agreement for applicable not medically necessary/not covered benefits/coverage. BACKGROUND Medical Coverage Policy | Auditory Brainstem Implant

500 EXCHANGE STREET, PROVIDENCE, RI 02903-2699 MEDICAL COVERAGE POLICY | 2 (401) 274-4848 WWW.BCBSRI.COM

The auditory brainstem implant (ABI) is a device designed 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 nonneurofibromatosis 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.

REGULATORY STATUS
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. The Nucleus® 24 Auditory Brainstem Implant System labeling states: “The efficacy of bilateral implantation with the ABI [auditory brainstem implant] has not been studied.” The Nucleus® 24 is now obsolete.

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

For individuals who are deaf due to bilateral resection of neurofibromas of the auditory nerve who receive an 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 (FDA) 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 NF2 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 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 also 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.

500 EXCHANGE STREET, PROVIDENCE, RI 02903-2699 MEDICAL COVERAGE POLICY | 3 (401) 274-4848 WWW.BCBSRI.COM

CODING Medicare Advantage Plans and Commercial Products The following code(s) are covered for patients 12 years of age older with a diagnosis of Neurofibromatosis type 2*:
92640 Diagnosis analysis with programming of auditory brainstem implant, per hour
S2235 Implantation of auditory brainstem implant

*ICD-10 Diagnosis Code: Q85.02 Neurofibromatosis, type 2

RELATED POLICIES None

PUBLISHED Provider Update, May 2025
Provider Update, May 2024 Provider Update, May 2023 Provider Update, June 2022 Provider Update, April 2021

REFERENCES:

1. Food and Drug Administration. Nucleus 24 Auditory Brainstem Implant System. FDA Summary of Safety and Effectiveness. 2000; https://www.accessdata.fda.gov/cdrh_docs/pdf/P000015B.pdf; Accessed December 31, 2024.
2. 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. PMID 25577282
3. Food and Drug Administration. Premarket Approval (PMA). Nucleus ABI541 Auditory Brainstem Implant.
4. https://www.accessdata.fda.gov/scripts/cdrh/cfdocs/cfpma/pma.cfm?id=P000015S012. Accessed December 31, 2024.
5. Garcia A, Haleem A, Poe S, et al. Auditory Brainstem Implant Outcomes in Tumor and Nontumor Patients: A Systematic Review. Otolaryngol Head Neck Surg. Jun 2024; 170(6): 1648-1658. PMID 38329219
6. Wang B, Yan M, Liu C, et al. Auditory brainstem implants for hearing rehabilitation in NF2- schwannomatosis: A systematic review and single-arm meta-analysis. NeuroRehabilitation. 2024; 54(2): 213-225. PMID 38427506
7. 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. https://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 December 31, 2024.
8. Ebinger K, Otto S, Arcaroli J, et al. Multichannel auditory brainstem implant: US clinical trial results. J Laryngol Otol Suppl. 2000; (27): 50-3. PMID 11211440
9. Otto SR, Shannon RV, Wilkinson EP, et al. Audiologic outcomes with the penetrating electrode auditory brainstem implant. Otol Neurotol. Dec 2008; 29(8): 1147-54. PMID 18931643
10. Daoudi H, Torres R, Mosnier I, et al. Long-term analysis of ABI auditory performance in patients with neurofibromatosis type 2-related schwannomatosis. Acta Neurochir (Wien). Oct 02 2024; 166(1): 390. PMID 39356313
11. 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. PMID 23404468

500 EXCHANGE STREET, PROVIDENCE, RI 02903-2699 MEDICAL COVERAGE POLICY | 4 (401) 274-4848 WWW.BCBSRI.COM

11. 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): 260-70. PMID 24448286
12. Sennaroglu L, Lenarz T, Roland JT, et al. Current status of pediatric auditory brainstem implantation in inner ear malformations; consensus statement of the Third International Pediatric ABI Meeting. Cochlear Implants Int. Jul 2024; 25(4): 316-333. PMID 39607757
13. 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): 739-50. PMID 26227469
14. 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): 605-12. PMID 24294682
15. Sennaroğlu L, Sennaroğlu G, Yücel E, et al. Long-term Results of ABI in Children With Severe Inner Ear Malformations. Otol Neurotol. Aug 2016; 37(7): 865-72. PMID 27273392
16. 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): 708-15. PMID 19704357
17. Baş B, Gökay NY, Aydoğan Z, et al. Do auditory brainstem implants favor the development of sensory integration and cognitive functions?. Brain Behav. Aug 2024; 14(8): e3637. PMID 39099332
18. Colletti V, Carner M, Miorelli V, et al. Auditory brainstem implant (ABI): new frontiers in adults and children. Otolaryngol Head Neck Surg. Jul 2005; 133(1): 126-38. PMID 16025066
19. Colletti V. Auditory outcomes in tumor vs. nontumor patients fitted with auditory brainstem implants. Adv Otorhinolaryngol. 2006; 64: 167-185. PMID 16891842
20. Colletti L. Beneficial auditory and cognitive effects of auditory brainstem implantation in children. Acta Otolaryngol. Sep 2007; 127(9): 943-6. PMID 17712673
21. Colletti V, Shannon RV, Carner M, et al. Complications in auditory brainstem implant surgery in adults and children. Otol Neurotol. Jun 2010; 31(4): 558-64. PMID 20393378

22. National Institute Health and Care Excellence (NICE). Auditory brain stem implants [IPG108]. 2005 https://www.nice.org.uk/guidance/ipg108. Accessed December 31, 2024.

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