Hearing Restoration in Cochlear Nerve Deficiency: the Choice Between Cochlear Implant or Auditory Brainstem Implant, a Meta-analysis

Objective: To answer the dilemma clinician's face when deciding between cochlear implant (CI) and auditory brainstem implant (ABI) treatment options in patients with cochlear nerve deficiency (CND). Study Design: Case study supplemented with literature review and meta-analysis. Setting: Tertiary referral center. Patient(s): Child with CHARGE syndrome and congenital deafness. Intervention(s): ABI as there was no benefit after bilateral cochlear implantation. Main Outcome Measures: Speech and language development, quality of life. Results: In one ear the cochleovestibular nerve was present on magnetic resonance imaging (MRI) without preoperative ABR responses. In the contra lateral ear the nerve could not be identified, despite present ABR responses. Nevertheless, there was no positive outcome with CI. The patient had improved speech and language and quality of life with ABI. Of the 108 patients with CND and CI identified in the literature review, 25% attained open-set speech perception, 34% attained closed-set speech perception, and 41% detected sounds or less. The appearance of the cochlear nerve on MRI was a useful predictor of success, with cochlear nerve aplasia on MRI associated with a smaller chance of a positive outcome post cochlear implantation compared with patients with cochlear nerve hypoplasia. Conclusion: Although patients with (apparent) cochlear nerve aplasia are less likely to benefit from CI, CI before ABI is supported as some patients attain closed or open-set levels of speech perception after cochlear implantation. Address correspondence and reprint requests to Annemarie Vesseur, M.D., Radboud Universitair Medisch Centrum, P.O. Box 9101 (377), 6500 HB Nijmegen, The Netherlands; E-mail: Annemarie.vesseur@radboudumc.nl Funding received for this work from: Cochlear Benelux BV. The authors disclose no conflicts of interest. Supplemental digital content is available in the text. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (http://ift.tt/2i6WMKr). Copyright © 2018 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company

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