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Expanding Understanding of Electrocochleography in Cochlear Implantation: Auditory Neuropathy Spectrum Disorder With Normal Pure Tone Average.

Authors :
Buhle AC
McCrary HC
Gordon SA
Johnson KM
Babajanian EE
Patel NS
Source :
Otology & neurotology open [Otol Neurotol Open] 2023 Jun 15; Vol. 3 (2), pp. e035. Date of Electronic Publication: 2023 Jun 15 (Print Publication: 2023).
Publication Year :
2023

Abstract

Objective: Describe the preoperative decision-making, intraoperative electrocochleographic (ECoG) findings, and outcome of cochlear implantation (CI) in a patient with auditory neuropathy spectrum disorder (ANSD) and normal pure-tone thresholds.<br />Patients: A 19-year-old with a history of hypoxic ischemic encephalopathy and seizures was referred for hearing rehabilitation in the setting of typical hearing by pure tone audiometry but poor speech understanding. A diagnosis of ANSD was made based on acoustic brainstem response (ABR), distortion product otoacoustic emission, and acoustic reflex testing. Imaging revealed no central cause of hearing impairment.<br />Interventions: Right-sided CI.<br />Main Outcome Measures: Preoperative and postoperative audiometric data. Intraoperative ECoG.<br />Results: Preoperatively the patient underwent comprehensive audiologic testing with behavioral audiometry, ABR testing, and CI candidacy evaluation. In the right ear, the pure tone average (PTA) was 15 dB and word recognition score was 36%. ABR confirmed ANSD. Preoperative CNC and AzBio in quiet were 8% and 0%, respectively. Intraoperative ECoG amplitudes and audiometry showed responses in the 100 uV range and estimated PTA of 42 dB HL. Postoperative testing at 1-month post-initial activation revealed PTA of 45 dB HL and unchanged word and sentence scores. However, the patient cites an improved ability to communicate and increased confidence and averages over 14 hours of device use daily.<br />Conclusions: To our knowledge, this is the first reported case of CI in an ear with normal PTA. Given that nearly all presently available ECoG data comes from patients with greater degrees of hearing loss, this unique case adds to our understanding of hearing preservation in CI.<br />Competing Interests: None declared.<br /> (Copyright © 2023 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

Details

Language :
English
ISSN :
2766-3604
Volume :
3
Issue :
2
Database :
MEDLINE
Journal :
Otology & neurotology open
Publication Type :
Academic Journal
Accession number :
38516124
Full Text :
https://doi.org/10.1097/ONO.0000000000000035