Evidence map›Paper›PMID 31453354›Full record

ReviewLaryngoscope investigative otolaryngology2019

Auditory synaptopathy, auditory neuropathy, and cochlear implantation.

Aiden Eliot Shearer, Marlan R Hansen

Registry-linked trialOpen access · goldAbstract readReview
PubMed Full text at PMC Publisher
In one paragraph

Review in Laryngoscope investigative otolaryngology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT07778641 (Genetic Study of OTOF- Related Auditory Neuropathy Spectrum Disorder), which is not on this map. Cited by 47 papers, 3 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
47citing papers in PubMed, 3 pooled it
5.6field-weighted citation impact, top 3% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT07778641 not yet recruitingnot on this mapstarted 2026, after this paper: background citation

Genetic Study of OTOF- Related Auditory Neuropathy Spectrum Disorder

Typeobservational_patient_registrySponsorSohag UniversityRan2026 to 2027Enrolled30ConditionsAuditory Neuropathy Spectrum Disorder, Auditory NeuropathyArmsWhole exome sequencing.
3 · Its place in the literature

Who cites it

47 citing papers in PubMed, 3 syntheses or guidelines pooled it, 141 citations in OpenAlex.

  1. Pooled it
  2. Guideline
  3. Guideline
  4. Effects of electrical stimulation rate in genetically confirmed pre- and post-Synaptic auditory neuropathy in children.European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery · 2026
    Trial
  5. Article
  6. Review
  7. Review
  8. Neural Hearing Loss: Mechanisms, Diagnosis and Treatment Horizons.Journal of the Association for Research in Otolaryngology : JARO · 2026
    Review
  9. Article
  10. Article
  11. Review
  12. Article
  13. UtilizingJournal of developmental biology · 2025
    Review
  14. Article
  15. Article
  16. Cochlear implantation in patients with Charcot-Marie-Tooth disease: two cases with a review of the literature.European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery · 2024
    Review
  17. Article
  18. Review
  19. Article
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

2 authors at 1 institution in 1 country.

Aiden Eliot ShearerDepartment of Otolaryngology-Head and Neck Surgery University of Iowa Carver College of Medicine Iowa City Iowa U.S.A.ORCID https://orcid.org/0000-0002-5324-4805
Marlan R HansenDepartment of Otolaryngology-Head and Neck Surgery University of Iowa Carver College of Medicine Iowa City Iowa U.S.A.ORCID https://orcid.org/0000-0002-6884-4897
University of Iowa · US

Funding

The University of Iowa Clinical and Translational Science AwardUL1TR002537 · NCATS · UNIVERSITY OF IOWA · PI HANSEN, MARLAN R, WINOKUR, PATRICIA · 2018 to 2022
$20.0M
Reduction of Intracochlear Trauma and Fibrosis Using Dual Network, Zwitterionic Hydrogel Thin Films on Cochlear Implant SurfacesR01DC012578 · NIDCD · UNIVERSITY OF IOWA · PI Allan Guymon, Marlan R Hansen · 2013 to 2026
$6.3M
NCATS NIH HHS UL1 TR002537NIDCD NIH HHS R01 DC012578
6 · The paper itself

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.

Indexed as

auditory neuropathy spectrum disorderCochlear implantsgenetics

Identifiers

PMID31453354
PMCPMC6703118
OpenAlexW2953862104

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
Read underepoch 390

Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.