Author(s)
Hilary C. McCrary MDsup>1
Anna Buhle BS2, Eric Babajanian MDsup>1
Steve Gordon MDsup>1
Kathryn Johnson AuD, CCC-Asup>1
Neil Patel MD1
Affiliation(s)
1University of Utah Department of Surgery, Division of Otolaryngology - Head and Neck Surgery,2Virginia Tech Carilion School of Medicine;
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.
Study Design: Case report.
Setting: Tertiary care academic center.
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 ABR, DPOAE, and acoustic reflex testing. Imaging revealed no central cause of hearing
impairment.
Interventions: Right-sided cochlear implantation.
Main Outcome Measures: Pre- and postoperative audiometric data. Intraoperative electrocochleography (ECoG).
Results: Preoperative audiologic and cochlear implant assessment for the right and left ear, respectively: PTA 15 dB, 8dB;
Word Recognition Score (WRS) 36%, 56%; CNC Words: 8%, 28%; and AzBio Quiet: 0%, 36%. 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, increased confidence, and averages over 14 hours of device use daily. Further
follow up data will be available at the time of presentation.
Conclusions: To our knowledge this is the first reported case of CI in an ear with normal PTA. Intraoperative and
postoperative audiometric findings add to the understanding of ECoG and CI in ANSD.
*Professional Practice Gap & Educational Need: To discuss the decision-making and workup, operative technique and
intraoperative testing, and postoperative results with CI for ANSD.
*Learning
Objective: To describe the application of cochlear implantation in ANSD patients with normal PTA.
*Desired
Result: Improved knowledge about the applications of cochlear implantation in ANSD patients and the role of
ECoG.
*Level of Evidence – Level V
*Indicate IRB or IACUC: Exempt