Journal article
Auditory synaptopathy, auditory neuropathy, and cochlear implantation
Laryngoscope investigative otolaryngology, Vol.4(4), pp.429-440
08/01/2019
DOI: 10.1002/lio2.288
PMCID: PMC6703118
PMID: 31453354
Abstract
Cochlear implantation has become the standard-of-care for adults and children with severe to profound hearing loss. There is growing evidence that qualitative as well as quantitative deficits in the auditory nerve may affect cochlear implant (CI) outcomes. Auditory neuropathy spectrum disorder (ANSD) is characterized by dysfunctional transmission of sound from the cochlea to the brain due to defective synaptic function or neural conduction. In this review, we examine the precise mechanisms of genetic lesions causing ANSD and the effect of these lesions on CI outcomes. Reviewed data show that individuals with lesions that primarily affect the cochlear sensory system and the synapse, which are bypassed by the CI, have optimal CI outcomes. Individuals with lesions that affect the auditory nerve show poor performance with CIs, likely because neural transmission of the electrical signal from the CI is affected. We put forth a nuanced molecular classification of ANSD that has implications for preoperative counseling for patients with this disorder prior to cochlear implantation. We propose that description of ANSD patients should be based on the molecular site of lesion typically derived from genetic evaluation (synaptopathy vs. neuropathy) as this has implications for expected CI outcomes. Improvements in our understanding of genetic site of lesions and their effects on CI function should lead to better CI outcomes, not just for individuals with auditory neuropathy, but all individuals with hearing loss.
Details
- Title: Subtitle
- Auditory synaptopathy, auditory neuropathy, and cochlear implantation
- Creators
- Aiden Eliot Shearer - Roy J. and Lucille A. Carver College of MedicineMarlan R. Hansen - Roy J. and Lucille A. Carver College of Medicine
- Resource Type
- Journal article
- Publication Details
- Laryngoscope investigative otolaryngology, Vol.4(4), pp.429-440
- Publisher
- Wiley
- DOI
- 10.1002/lio2.288
- PMID
- 31453354
- PMCID
- PMC6703118
- ISSN
- 2378-8038
- eISSN
- 2378-8038
- Number of pages
- 12
- Grant note
- R01 DC012578; UL1TR002537 / National Institutes of Health; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA
- Language
- English
- Date published
- 08/01/2019
- Academic Unit
- Molecular Physiology and Biophysics; Neurosurgery; Otolaryngology
- Record Identifier
- 9984297605802771
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