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autosomal dominant nonsyndromic hearing loss 11 - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

autosomal dominant nonsyndromic hearing loss 11

MONDO:0011032

Also known as:DFNA11MYO7A autosomal dominant nonsyndromic deafnessautosomal dominant deafness 11

Overview

Any autosomal dominant nonsyndromic deafness in which the cause of the disease is a mutation in the MYO7A gene.

GeneticsAutosomal dominant · MYO7A (chr 11)

Clinical Presentation

Features include: Bilateral sensorineural hearing impairment, Abnormal vestibular function, and Vertigo.

Organ System	Phenotype Count	Example Features
Ears	3	Bilateral sensorineural hearing impairment, Abnormal vestibular function, Vertigo

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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.

MYO7A encodes myosin VIIA (2,215 aa). Myosins are actin-based motor molecules with ATPase activity. Unconventional myosins serve in intracellular movements. Highest expression in Testis (43.8 TPM) and Adrenal Gland (40.7 TPM).

Autosomal dominant nonsyndromic hearing loss 11 is associated with mutations in the MYO7A gene on chromosome 11.

The MYO7A protein participates in RPE65 isomero-hydrolyses atREs to 11cROL, Mechanoelectrical transduction (MET) channel transports cations into the cytosol of stereocilia of cochlear outer hair cell, and Mechanoelectrical transduction (MET) channel transports cations from the extracellular region into the cytosol of stereocilia of inner hair cell pathways.

MYO7A is classified as a druggable target with score 0.0.

Diagnosis

Drawn from GeneReviews diagnostic guidelines, newborn screening panel status (RUSP), and ClinGen gene-disease validity classifications. Reflects current clinical evidence and consensus where available.

Genetic testing for MYO7A is available. Testing is considered confirmatory for diagnosis.

Biomarker and diagnostic research for autosomal dominant nonsyndromic hearing loss 11 has been reported in the published literature.

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 11.

18 publications have been identified in PubMed for autosomal dominant nonsyndromic hearing loss 11. Research spans Basic Science / Preclinical (41%), Review / Meta-Analysis (24%), and Diagnostic / Biomarker (12%).

Research Type	Count	% of Total
Laboratory research	7	41%

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 8:03 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:601317

GARD

Genetic and Rare Diseases Info Center

GARD:18104

Questions for Your Care Team

→Should I pursue genetic testing for MYO7A, 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 11

Overview

Clinical Presentation

Explore clinical features

Genetic Landscape

Diagnosis

Research Landscape

Medical References

External Resources

OMIM

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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