Cochlear implantation (CI) has been used as a treatment option for children with auditory neuropathy spectrum disorder (ANSD) who do not benefit from conventional hearing aids. Up to date, there is no robust indicator for predicting CI outcomes in these patients. In addition, programming settings selected for individual ANSD patients might not be optimal since many ANSD children are not able to provide reliable behavioral responses due to their young ages and/or comorbid conditions. These conditions make the audiologic management of implanted ANSD children extremely challenging and at many times uncertain. The long-term goal of this program of research is to develop objective tools that can be used in clinical settings for accurately predicting post-implant speech perception performance and optimizing programming settings for individual ANSD children. As the first step towards fulfilling this long-term goal, this project aims to evaluate how well electrical stimulation is transmitted by the auditory nerve by evaluating temporal response properties of the nerve in implanted ANSD children. In addition, this project also aims to explore the extent to which neural responses recorded at a peripheral and more central level are correlated with each other and whether they are correlated with speech perception performance in ANSD patients.
Specific Aim 1 characterizes temporal response properties of the auditory nerve in pediatric CI users by measuring the electrically evoked auditory compound action potential (ECAP) at different stimulation rates. Both children with ANSD and children with sensorineural hearing loss will be tested. Results from these experiments will provide a better understanding of sources of across-patient variations in post-implant speech perception performance among ANSD children.
Specific Aim 2 evaluates the association among temporal response properties of the auditory nerve, gap detection threshold and speech perception performance in implanted ANSD children. Results of these studies should expand understandings of how auditory processing in the central auditory cortex is affected by the input from the auditory nerve. Overall, results of this project could potentially lead to the development of an objective tool for identifying a subgroup of ANSD patients who are unlikely to receive substantial benefit from their devices at an early post-implant stage. In addition, these results could also potentially lead to the development of specific signal processing modifications that might result in improved performance for individual implanted ANSD patients. Therefore, this project has tremendous translational significance.

Public Health Relevance

The proposed study aims to characterize temporal response properties of the auditory nerve in pediatric cochlear implant (CI) users, including both children with auditory neuropathy spectrum disorder (ANSD) and children with sensorineural hearing loss (SNHL). In addition, this study also aims to investigate the possible association among auditory-nerve temporal response properties, gap detection thresholds, and post-implant speech perception performance in implanted ANSD children. The long-term goal of this research program is to develop objective tools for accurately predicting CI outcomes and optimizing programming settings for individual ANSD patients. It is anticipated that the electrophysiological measures investigated in this program of research will be adopted as clinical tools by clinicians who work with implanted ANSD patients, which will have an important impact on clinical practice.

Agency
National Institute of Health (NIH)
Institute
National Institute on Deafness and Other Communication Disorders (NIDCD)
Type
Small Research Grants (R03)
Project #
7R03DC013153-04
Application #
9135579
Study Section
Communication Disorders Review Committee (CDRC)
Program Officer
Donahue, Amy
Project Start
2013-05-20
Project End
2016-04-30
Budget Start
2015-08-01
Budget End
2016-04-30
Support Year
4
Fiscal Year
2015
Total Cost
$150,000
Indirect Cost
$50,000
Name
Father Flanagan's Boys' Home
Department
Type
DUNS #
073136806
City
Boys Town
State
NE
Country
United States
Zip Code
68010
He, Shuman; Teagle, Holly F B; McFayden, Tyler C et al. (2018) Longitudinal Changes in Electrically Evoked Auditory Event-Related Potentials in Children With Auditory Brainstem Implants: Preliminary Results Recorded Over 3 Years. Ear Hear 39:318-325
He, Shuman; Shahsavarani, Bahar S; McFayden, Tyler C et al. (2018) Responsiveness of the Electrically Stimulated Cochlear Nerve in Children With Cochlear Nerve Deficiency. Ear Hear 39:238-250
He, Shuman; McFayden, Tyler C; Shahsavarani, Bahar S et al. (2018) The Electrically Evoked Auditory Change Complex Evoked by Temporal Gaps Using Cochlear Implants or Auditory Brainstem Implants in Children With Cochlear Nerve Deficiency. Ear Hear 39:482-494
Teagle, Holly F B; Henderson, Lillian; He, Shuman et al. (2018) Pediatric Auditory Brainstem Implantation: Surgical, Electrophysiologic, and Behavioral Outcomes. Ear Hear 39:326-336
He, Shuman; Teagle, Holly F B; Buchman, Craig A (2017) The Electrically Evoked Compound Action Potential: From Laboratory to Clinic. Front Neurosci 11:339
He, Shuman; McFayden, Tyler C; Teagle, Holly F B et al. (2016) Electrically Evoked Auditory Event-Related Responses in Patients with Auditory Brainstem Implants: Morphological Characteristics, Test-Retest Reliability, Effects of Stimulation Level, and Association with Auditory Detection. Ear Hear 37:634-649
He, Shuman; Abbas, Paul J; Doyle, Danielle V et al. (2016) Temporal Response Properties of the Auditory Nerve in Implanted Children with Auditory Neuropathy Spectrum Disorder and Implanted Children with Sensorineural Hearing Loss. Ear Hear 37:397-411