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progressive sensorineural hearing loss-hypertrophic cardiomyopathy syndrome - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

progressive sensorineural hearing loss-hypertrophic cardiomyopathy syndrome

MONDO:0016424

Also known as:progressive neurosensory deafness-hypertrophic cardiomyopathy syndromeprogressive neurosensory hearing loss-hypertrophic cardiomyopathy syndromeprogressive sensorineural deafness-hypertrophic cardiomyopathy syndrome

Overview

Progressive sensorineural hearing loss - hypertrophic cardiomyopathy is an extremely rare disorder described in one family to date that is characterized by progressive, late onset, autosomal dominant sensorineural hearing loss, QT interval prolongation, and mild cardiac hypertrophy.

ResearchLimited published research (6 articles)

Prognosis

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for progressive sensorineural hearing loss-hypertrophic cardiomyopathy syndrome.

6 publications have been identified in PubMed for progressive sensorineural hearing loss-hypertrophic cardiomyopathy syndrome. Research spans Case Report / Case Series (67%) and Review / Meta-Analysis (33%).

Recent Publications

Dodulík J (2026). [PMID: 41682788](https://pubmed.ncbi.nlm.nih.gov/41682788/). *J Clin Med*. [Case Report / Case Series]
Goda T (2026). [PMID: 41956113](https://pubmed.ncbi.nlm.nih.gov/41956113/). *J Pediatr Endocrinol Metab*. [Case Report / Case Series]
Shahab S (2025). [PMID: 41589183](https://pubmed.ncbi.nlm.nih.gov/41589183/). *Cureus*. [Case Report / Case Series]
Yen B (2025). [PMID: 38569205](https://pubmed.ncbi.nlm.nih.gov/38569205/). *Retin Cases Brief Rep*. [Case Report / Case Series]
Antolínez-Fernández Á (2024). [PMID: 38855161](https://pubmed.ncbi.nlm.nih.gov/38855161/). *Front Cell Dev Biol*. [Review / Meta-Analysis]

Data assembled from 3 of 12 sources · Last updated Sep 20, 2026, 2:17 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:228012

GARD

Genetic and Rare Diseases Info Center

GARD:17145

Questions for Your Care Team

→Are there specialists or centers of excellence for this condition in my area?
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progressive sensorineural hearing loss-hypertrophic cardiomyopathy syndrome

Overview

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team