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autosomal dominant nonsyndromic hearing loss 50 - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

autosomal dominant nonsyndromic hearing loss 50

MONDO:0013114

Also known as:DFNA50autosomal dominant deafness 50autosomal dominant nonsyndromic deafness 50

Overview

An autosomal dominant nonsyndromic deafness that is characterized postlingual onset in the second decade of life with flat progressive hearing loss and has material basis in mutation in the MIRN96 gene on chromosome 7q32.

GeneticsAutosomal dominant · MIR96 (chr 7)

ResearchLimited published research (8 articles)

Clinical Presentation

Features include sometimes findings: Tinnitus. 4 total HPO annotations.

Organ System	Phenotype Count	Example Features
Ears	4	Progressive sensorineural hearing impairment, Inner ear hearing loss (sensorineural hearing impairment), Tinnitus

Explore clinical features

Genetic Landscape

Assembled from HGNC gene records, ClinGen gene-disease validity evidence, and ClinVar pathogenic variant data. Validity classifications reflect the strength of published evidence linking each gene to this disease.

MIR96 encodes microRNA 96. Highest expression in Adipose Subcutaneous (0.0 TPM) and Adipose Visceral Omentum (0.0 TPM).

Autosomal dominant nonsyndromic hearing loss 50 is associated with mutations in the MIR96 gene on chromosome 7.

MIR96 is classified as a druggable target with score 0.0.

Diagnosis

Genetic testing for MIR96 is available. Testing is considered confirmatory for diagnosis.

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 autosomal dominant nonsyndromic hearing loss 50.

12 publications have been identified in PubMed for autosomal dominant nonsyndromic hearing loss 50. Kisho has analyzed 8 by research type. Research spans Basic Science / Preclinical (63%), Case Report / Case Series (25%), and Review / Meta-Analysis (13%).

Recent Publications

Ranganathan R (2025). [PMID: 40213817](https://pubmed.ncbi.nlm.nih.gov/40213817/). *Development*. [Basic Science / Preclinical]
Soleimani F (2025). [PMID: 41307816](https://pubmed.ncbi.nlm.nih.gov/41307816/). *Biochem Genet*. [Case Report / Case Series]
Zhang Y (2025). [PMID: 41015553](https://pubmed.ncbi.nlm.nih.gov/41015553/). *Zhong Nan Da Xue Xue Bao Yi Xue Ban*. [Basic Science / Preclinical]
Sekiguchi R (2024). [PMID: 39388000](https://pubmed.ncbi.nlm.nih.gov/39388000/). *JA Clin Rep*. [Case Report / Case Series]
Zhang L (2024). [PMID: 39556694](https://pubmed.ncbi.nlm.nih.gov/39556694/). *Adv Sci (Weinh)*. [Review / Meta-Analysis]

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 12:32 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:613074

GARD

Genetic and Rare Diseases Info Center

GARD:18132

Questions for Your Care Team

→Should I pursue genetic testing for MIR96, and what would the results mean for my family?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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autosomal dominant nonsyndromic hearing loss 50

Overview

Clinical Presentation

Explore clinical features

Genetic Landscape

Diagnosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team