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hearing loss, autosomal dominant 89 - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

hearing loss, autosomal dominant 89

MONDO:0859528

Overview

GeneticsAutosomal dominant · ATOH1 (chr 4)

ResearchLimited published research (5 articles)

Clinical Presentation

Features include: Hearing loss (hearing impairment).

Organ System	Phenotype Count	Example Features
Ears	1	Hearing loss (hearing impairment)

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.

ATOH1 encodes atonal bHLH transcription factor 1 (354 aa). Transcriptional regulator. Activates E box-dependent transcription in collaboration with TCF3/E47, but the activity is completely antagonized by the negative regulator of neurogenesis HES1. Highest expression in Colon Transverse (17.1 TPM) and Small Intestine Terminal Ileum (16.9 TPM).

Hearing loss, autosomal dominant 89 is associated with mutations in the ATOH1 gene on chromosome 4.

ATOH1 is classified as a druggable target (Transcription Factor category) with score 0.0.

Diagnosis

Genetic testing for ATOH1 is available. Testing is considered confirmatory for diagnosis.

Biomarker and diagnostic research for hearing loss, autosomal dominant 89 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 hearing loss, autosomal dominant 89.

5 publications have been identified in PubMed for hearing loss, autosomal dominant 89. Research spans Basic Science / Preclinical (40%), Epidemiology / Natural History (40%), and Diagnostic / Biomarker (20%).

Recent Publications

González-Aguado R (2025). [PMID: 40178561](https://pubmed.ncbi.nlm.nih.gov/40178561/). *Eur Arch Otorhinolaryngol*. [Epidemiology / Natural History]
Wang W (2025). [PMID: 40389765](https://pubmed.ncbi.nlm.nih.gov/40389765/). *J Assist Reprod Genet*. [Diagnostic / Biomarker]
Curtis SW (2025). [PMID: 41172132](https://pubmed.ncbi.nlm.nih.gov/41172132/). *Hum Mol Genet*. [Basic Science / Preclinical]
Wu KL (2025). [PMID: 40413265](https://pubmed.ncbi.nlm.nih.gov/40413265/). *Sci Rep*. [Epidemiology / Natural History]
Zhang H (2024). [PMID: 38627775](https://pubmed.ncbi.nlm.nih.gov/38627775/). *BMC Med Genomics*. [Basic Science / Preclinical]

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 3:32 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:620284

Questions for Your Care Team

→Should I pursue genetic testing for ATOH1, 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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hearing loss, autosomal dominant 89

Overview

Clinical Presentation

Explore clinical features

Genetic Landscape

Diagnosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

Related Diseases

Questions for Your Care Team

Questions for Your Care Team