Evidence map›Paper›PMID 21669267›Full record

ReviewHearing research2011

Hearing loss associated with enlargement of the vestibular aqueduct: mechanistic insights from clinical phenotypes, genotypes, and mouse models.

Andrew J Griffith, Philine Wangemann

Registry-linked trialOpen access · greenAbstract readReview
In one paragraph

Review in Hearing research, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02798783 (Enlarged Vestibular Aqueduct Patient Registry at University Hospitals Rainbow Babies and Children's Hospital), which is not on this map. Cited by 47 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
47citing papers in PubMed, 1 pooled it
3.2field-weighted citation impact, top 9% 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.

NCT02798783 completednot on this mapstarted 2021, after this paper: background citation

Enlarged Vestibular Aqueduct Patient Registry at University Hospitals Rainbow Babies and Children's Hospital

Typeobservational_patient_registrySponsorUniversity Hospitals Cleveland Medical CenterRan2021 to 2023Enrolled165ConditionsEnlarged Vestibular Aqueduct, Sensorineural Hearing Loss
3 · Its place in the literature

Who cites it

47 citing papers in PubMed, 1 synthesis or guideline pooled it, 91 citations in OpenAlex.

  1. Guideline
  2. Review
  3. Review
  4. Assessing the Functional Significance of Novel and Rare Variants of theInternational journal of molecular sciences · 2025
    Article
  5. Article
  6. Article
  7. Article
  8. Review
  9. Review
  10. Article
  11. Article
  12. Article
  13. Article
  14. The role ofFrontiers in molecular neuroscience · 2024
    Review
  15. Article
  16. Cochlear Health and Cochlear-implant Function.Journal of the Association for Research in Otolaryngology : JARO · 2023
    Review
  17. Article
  18. SOX9 and SOX10 control fluid homeostasis in the inner ear for hearing through independent and cooperative mechanisms.Proceedings of the National Academy of Sciences of the United States of America · 2022
    Article
  19. Molecular Features of SLC26A4 Common Variant p.L117F.Journal of clinical medicine · 2022
    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 2 institutions in 1 country.

Andrew J GriffithOtolaryngology Branch, National Institute on Deafness and Other Communication Disorders, 5 Research Court, Rockville, Maryland 20850-3320, USA. griffita@nidcd.nih.gov
Philine Wangemann
Kansas State University · USNational Institute on Deafness and Other Communication Disorders · US

Funding

Molecular Analysis Of Human Hereditary DeafnessZIADC000060 · NIDCD · NATIONAL INSTITUTE ON DEAFNESS AND OTHER COMMUNICATION DISORDERS · PI ROUX, ISABELLE · 2009 to 2022
$18.4M
Molecular Analysis Of Human Hereditary DeafnessZ01DC000060 · NIDCD · NATIONAL INSTITUTE ON DEAFNESS AND OTHER COMMUNICATION DISORDERS · PI GRIFFITH, ANDREW J · 2001 to 2008
$3.0M
CYTOSOLIC REGULATION OF INNER EAR ION TRANSPORTR01DC001098 · NIDCD · KANSAS STATE UNIVERSITY · PI WANGEMANN, A. PHILINE · 1997 to 2007
$2.1M
Intramural NIH HHS Z01 DC000060Intramural NIH HHS ZIA DC000060NIDCD NIH HHS R01 DC001098
6 · The paper itself

Abstract

Enlargement of the vestibular aqueduct (EVA) is one of the most common inner ear malformations associated with sensorineural hearing loss in children. The delayed onset and progressive nature of this phenotype offer a window of opportunity to prevent or retard progression of hearing loss. EVA is not the direct cause of hearing loss in these patients, but rather is a radiologic marker for some underlying pathogenetic defect. Mutations of the SLC26A4 gene are a common cause of EVA. Studies of an Slc26a4 knockout mouse demonstrate that acidification and enlargement of the scala media are early events in the pathogenesis of deafness. The enlargement is driven by fluid secretion in the vestibular labyrinth and a failure of fluid absorption in the embryonic endolymphatic sac. Elucidating the mechanism of hearing loss may offer clues to potential therapeutic strategies.

Indexed as

MutationAnimalsAnion Transport ProteinsDisease Models, AnimalGenetic Predisposition to DiseaseHearing Loss, SensorineuralHumansMembrane Transport ProteinsMiceMice, KnockoutPhenotypeSulfate TransportersVestibular AqueductAnion Transport ProteinsMembrane Transport ProteinsSLC26A4 protein, humanSlc26a4 protein, mouseSulfate Transporters

Identifiers

PMID21669267
PMCPMC3183377
OpenAlexW1972814800

What OpenQuestion holds

Textmetadata
LicenceTDM
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.