Evidence map›Paper›PMID 41430449›Full record

SynthesisEuropean 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 Surgery2026

Impact of enzyme replacement therapy on hearing sensitivity in Fabry disease: A systematic review.

Sreeraj Konadath, Praveen Prakash, Achu Abraham, Adithya Sreedharan Sanitha, Yerraguntla Krishna, Abdulaziz Almudhi, Irfana Madathodiyil, Reesha Oovattil Hussain

Abstract readSystematic Review
PubMed Publisher
In one paragraph

Synthesis in 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. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
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

0 citing papers in PubMed.

No citing paper in PubMed yet.

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.

Sreeraj KonadathDepartment of Medical Rehabilitation Sciences, College of Applied Medical Sciences, King Khalid University, Abha, 61481, Saudi Arabia.
Praveen PrakashJSS Institute of Speech and Hearing, Dharwad, 580007, India. praveenprakashp1849@gmail.com.ORCID http://orcid.org/0000-0001-6636-9343
Achu AbrahamJSS Institute of Speech and Hearing, Dharwad, 580007, India.
Adithya Sreedharan SanithaDepartment of Audiology and Speech Language Pathology, Kasturba Medical College Mangalore, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India.
Yerraguntla KrishnaDepartment of Medical Rehabilitation Sciences, College of Applied Medical Sciences, King Khalid University, Abha, 61481, Saudi Arabia.
Abdulaziz AlmudhiDepartment of Medical Rehabilitation Sciences, College of Applied Medical Sciences, King Khalid University, Abha, 61481, Saudi Arabia.
Irfana MadathodiyilDepartment of Medical Rehabilitation Sciences, College of Applied Medical Sciences, King Khalid University, Abha, 61481, Saudi Arabia.
Reesha Oovattil HussainDepartment of Medical Rehabilitation Sciences, College of Applied Medical Sciences, King Khalid University, Abha, 61481, Saudi Arabia.

Funding

Deanship of Research and Graduate Studies at King Khalid University through the Large Research Project RGP2/548/46
6 · The paper itself

Abstract

aimThis review systematically evaluated the impact of enzyme replacement therapy (ERT) on audiological outcomes in Fabry disease, focusing on the nature of audiological findings, therapeutic effects on hearing function, and factors influencing treatment response.

methodThe systematic review was conducted following PRISMA 2020 guidelines. Literature searches were performed across PubMed, PubMed Central, ScienceDirect, J-Gate, ProQuest, Web of Science, EBSCO, and Google Scholar up to 2025. Reference lists of eligible studies were also manually screened to identify additional relevant publications on audiological outcomes in Fabry disease patients undergoing ERT.

resultsTen studies comprising 404 patients were included. Across studies, ERT generally stabilized hearing thresholds, with modest improvements reported in some cases, particularly when therapy was initiated early. Factors such as disease stage and timing of therapy influenced the extent of auditory benefit.

conclusionERT appears effective in preventing further auditory deterioration in Fabry disease, with potential for modest improvement when initiated early. Evidence is limited by variability in study designs, small sample sizes, and challenges inherent to rare conditions, including difficulties in longitudinally tracking patients across treatment timelines.

Indexed as

alpha-GalactosidaseEnzyme Replacement TherapyFabry DiseaseHearing LossHumansTreatment Outcomealpha-GalactosidaseAlpha galactosidase aAnderson–Fabry diseaseAudiological outcomesEnzyme replacement therapyFabry diseaseHearing lossSystematic reviewTinnitus

Identifiers

PMID41430449

What OpenQuestion holds

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Registered trials

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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.