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autosomal dominant nonsyndromic hearing loss 36 - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

autosomal dominant nonsyndromic hearing loss 36

MONDO:0011708

Also known as:DFNA36TMC1 autosomal dominant nonsyndromic deafnessautosomal dominant deafness 36

Overview

Any autosomal dominant nonsyndromic deafness in which the cause of the disease is a mutation in the TMC1 gene.

GeneticsAutosomal dominant · TMC1 (chr 9)

OnsetJUVENILE

ResearchLimited published research (7 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: Inner ear hearing loss (sensorineural hearing impairment) and Tinnitus.

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

Age of onset: adolescence.

Explore clinical features

Genetic Landscape

TMC1 function has not been fully characterized.

Autosomal dominant nonsyndromic hearing loss 36 is associated with mutations in the TMC1 gene on chromosome 9.

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 TMC1 is available. Testing is considered confirmatory for diagnosis.

Biomarker and diagnostic research for autosomal dominant nonsyndromic hearing loss 36 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 36.

7 publications have been identified in PubMed for autosomal dominant nonsyndromic hearing loss 36. Research spans Case Report / Case Series (29%), Epidemiology / Natural History (29%), and Diagnostic / Biomarker (14%).

Recent Publications

Hoff FW (2026). [PMID: 41742372](https://pubmed.ncbi.nlm.nih.gov/41742372/). *The Journal of clinical endocrinology and metabolism*. [Case Report / Case Series]
Hoang NC (2026). [PMID: 41955303](https://pubmed.ncbi.nlm.nih.gov/41955303/). *Human molecular genetics*. [Epidemiology / Natural History]
Liu M (2025). [PMID: 39994654](https://pubmed.ncbi.nlm.nih.gov/39994654/). *BMC medical genomics*. [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 = Journal of Central South University. Medical sciences*. [Gene Therapy / Novel Therapeutics]
Damrongchietanon T (2025). [PMID: 40998904](https://pubmed.ncbi.nlm.nih.gov/40998904/). *Scientific reports*. [Diagnostic / Biomarker]

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 1:48 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:606705

GARD

Genetic and Rare Diseases Info Center

GARD:18115

Questions for Your Care Team

→Should I pursue genetic testing for TMC1, 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 36

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