Evidence map›Paper›PMID 31516706›Full record

ArticleSAGE open medical case reports2019

Cochlear implantation in a patient with mucopolysaccharidosis IVA.

Kyoko Nagao, Cassidy Walter, William J Parkes, Michael Teixido, Mary C Theroux, Stacy Szymkowski, Thierry Morlet, Shunji Tomatsu

Open access · goldAbstract readCase Reports
In one paragraph

Article in SAGE open medical case reports, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed
0.5field-weighted citation impact, top 32% 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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

7 citing papers in PubMed, 7 citations in OpenAlex.

  1. Article
  2. Natural History of Morquio A Syndrome.Journal of inherited metabolic disease · 2026
    Review
  3. Review
  4. Article
  5. Article
  6. Article
  7. Review
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

8 authors at 4 institutions in 1 country.

Kyoko NagaoNemours Biomedical Research, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.ORCID https://orcid.org/0000-0003-0917-6603
Cassidy WalterDepartment of Linguistics and Cognitive Science, University of Delaware, Newark, DE, USA.
William J ParkesOtolaryngology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Michael TeixidoOtolaryngology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Mary C TherouxSidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
Stacy SzymkowskiDepartment of Audiology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Thierry MorletNemours Biomedical Research, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Shunji TomatsuNemours Biomedical Research, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Alfred I. duPont Hospital for Children · USChristiana Care Health System · USSalus University · USUniversity of Delaware · US

Funding

Center for Pediatric Research Pilot ProgramP30GM114736 · NIGMS · NEMOURS CHILDREN'S HOSPITAL, DELAWARE · PI SHAFFER, THOMAS H · 2015 to 2019
$5.1M
NIGMS NIH HHS P30 GM114736
6 · The paper itself

Abstract

Mucopolysaccharidosis IVA (OMIM 253000; also known as Morquio A syndrome) is associated with skeletal, airway, and hearing abnormalities. Cochlear implantation is an effective intervention for patients with severe-to-profound hearing loss. Patients can gain substantial improvement in auditory performance, speech perception, and their quality of life from cochlear implantation. Although severe progressive sensorineural hearing loss is a common feature of mucopolysaccharidosis IVA, no detailed description of cochlear implantation for mucopolysaccharidosis IVA has been reported. To review the effectiveness and special considerations associated with cochlear implantation in patients with mucopolysaccharidosis IVA, we here report the case of cochlear implantation in mucopolysaccharidosis IVA by a multidisciplinary team. A retrospective chart review was conducted on a 34-year-old female with mucopolysaccharidosis IVA, who received a cochlear implant. Audiometric thresholds, speech perception scores, and cochlear implant processor mapping information were reviewed during the first 12 months following cochlear implantation. The results of audiological tests indicate improved hearing thresholds as well as remarkable enhancement of speech perception skills over 12 months of cochlear implant use. Cochlear implantation improved auditory performance in a mucopolysaccharidosis IVA patient with postlingually severe-to-profound sensorineural hearing loss. The benefits of cochlear implantation could be meaningful for other Morquio patients with progressive hearing loss, although the risks of surgery and anesthesia should be carefully considered by a multidisciplinary team of experts during the cochlear implant candidacy process.

Indexed as

cochlear implantationhearing lossMucopolysaccharidosisspeech perception

Identifiers

PMID31516706
PMCPMC6719565
OpenAlexW2970460668

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
Read underepoch 390

Registered trials

None linked

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.