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autosomal dominant nonsyndromic hearing loss 56 - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

autosomal dominant nonsyndromic hearing loss 56

MONDO:0014283

Also known as:DFNA56TNC autosomal dominant nonsyndromic deafnessautosomal dominant deafness 56

Overview

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

GeneticsAutosomal dominant · TNC (chr 9)

ResearchLimited published research (3 articles)

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include always present findings: Inner ear hearing loss (sensorineural hearing impairment). 2 total HPO annotations.

Organ System	Phenotype Count	Example Features
Ears	2	Abnormal vestibular function, Inner ear hearing loss (sensorineural hearing impairment)

Explore clinical features

Genetic Landscape

TNC function has not been fully characterized.

Autosomal dominant nonsyndromic hearing loss 56 is associated with mutations in the TNC gene on chromosome 9.

Diagnosis

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

Prognosis

Phenotype severity distribution: 1 always present feature.

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 56.

3 publications have been identified in PubMed for autosomal dominant nonsyndromic hearing loss 56. Research spans Case Report / Case Series (67%) and Basic Science / Preclinical (33%).

Recent Publications

Chen R (2026). [PMID: 41078281](https://pubmed.ncbi.nlm.nih.gov/41078281/). *Am J Med Genet A*. [Case Report / Case Series]
Guan J (2025). [PMID: 40068948](https://pubmed.ncbi.nlm.nih.gov/40068948/). *Yi Chuan*. [Basic Science / Preclinical]
Vroegindewey L (2024). [PMID: 39700338](https://pubmed.ncbi.nlm.nih.gov/39700338/). *Endocrinol Diabetes Metab Case Rep*. [Case Report / Case Series]

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 2:12 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:615629

GARD

Genetic and Rare Diseases Info Center

GARD:18137

Questions for Your Care Team

→Should I pursue genetic testing for TNC, 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 56

Overview

Clinical Presentation

Explore clinical features

Genetic Landscape

Diagnosis

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team