Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2015 Mar;79(3):310-315.
doi: 10.1016/j.ijporl.2014.11.023. Epub 2014 Dec 15.

Auditory brainstem implant candidacy in the United States in children 0-17 years old

Alyson B Kaplan #  1 , Elliott D Kozin #  1 , Sidharth V Puram  1 , Maryanna S Owoc  1 , Parth V Shah  1 , A E Hight  1 , Rosh K V Sethi  1 , Aaron K Remenschneider  1 , Daniel J Lee  1
Affiliations

Auditory brainstem implant candidacy in the United States in children 0-17 years old

Alyson B Kaplan et al. Int J Pediatr Otorhinolaryngol. 2015 Mar.

Abstract

Objectives: The auditory brainstem implant (ABI) is an option for hearing rehabilitation in profoundly deaf patients ineligible for cochlear implantation. Over the past decade, surgeons have begun implanting ABIs in pediatric patients who are unable to receive cochlear implants due to congenital or acquired malformations of the inner ear. No study has examined the potential population-level demand for ABIs in the United States (US). Herein, we aim to quantify the potential need for pediatric ABIs.

Methods: A systematic literature review was conducted to identify studies detailing the rates of congenital cochlear and/or cochlear nerve (CN) anomalies. Absolute indications for ABI include bilateral cochlea or CN aplasia (Group A), and relative indications for ABI include bilateral cochlea or CN hypoplasia (Group B). Data was subsequently correlated to the US Census Bureau, the National Health Interview Survey, and the Gallaudet Research Institute to provide an estimation of pediatric ABI candidates.

Results: Eleven studies documented rates of bilateral findings. Bilateral cochlea aplasia was identified in 0-8.7% of patients and bilateral CN aplasia in 0-4.8% of patients (Group A). Bilateral cochlea hypoplasia was identified in 0-8.7% of patients and bilateral CN hypoplasia in 0-5.4% of patients (Group B). Using population-level sensorineural hearing loss data, we roughly estimate 2.1% of potential implant candidates meet absolute indications for an ABI in the United States.

Conclusion: Congenital cochlear and cochlear nerve anomalies are exceedingly rare. This study provides the first preliminary estimate of cochlea and CN aplasia/hypoplasia at the population level albeit with limitations based on available data. These data suggest the need for dedicated ABI centers to focus expertise and management.

Keywords: Auditory brainstem implant; Cochlear abnormalities; Cochlear implant; Cochlear nerve abnormalities; Neuroprosthesis; Pediatric.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Flowchart of methodology used to determine absolute and relative indications for ABI.
See this image and copyright information in PMC

References

    1. Harrison RV, Gordon KA, Mount RJ. Is there a critical period for cochlear implantation in congenitally deaf children? Analyses of hearing and speech perception performance after implantation. Dev Psychobiol. 2005;46:252–261. - PubMed
    1. NIDCD [8/18/2014];Cochlear Implants. Available at: http://wwwnidcdnihgov/health/hearing/pages/cochaspx.
    1. Williams C. Hearing restoration: Graeme Clark, Ingeborg Hochmair, and Blake Wilson receive the 2013 Lasker~DeBakey Clinical Medical Research Award. The Journal of clinical investigation. 2013;123:4102–4106. - PMC - PubMed
    1. Ebinger K, Otto S, Arcaroli J, Staller S, Arndt P. Multichannel auditory brainstem implant: US clinical trial results. J Laryngol Otol Suppl. 2000;27:50–53. - PubMed
    1. Otto SR, Shannon RV, Brackmann DE, Hitselberger WE, Staller S, Menapace C. The multichannel auditory brain stem implant: performance in twenty patients. Otolaryngol Head Neck Surg. 1998;118:291–303. - PubMed

Publication types