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A rare, genetically highly heterogeneous otorhinolaryngologic disease, resulting from inner and/or middle ear or hearing nerve anomalies, typically characterized by bilateral, severe to profound hearing loss (mean sensorineural hearing impairment of 60 dB or more for 500-, 1,000-, and 2,000-Hz frequency tones in the better ear) which occurs before the onset of speech development and is not associated with visible external ear abnormalities or any other medical problems. It is usually nonprogressive and impedes oral language acquisition.
No clinical trials have been registered for prelingual non-syndromic genetic hearing loss.
21 publications have been identified in PubMed for prelingual non-syndromic genetic hearing loss. Research spans Basic Science / Preclinical (33%), Case Report / Case Series (24%), and Epidemiology / Natural History (24%).
Research Type | Count | % of Total |
|---|---|---|
Laboratory research | 7 | 33% |
Data assembled from 3 of 12 sources · Last updated Sep 19, 2026, 12:33 AM UTC
Patient Advocacy Groups (PAGs) provide support, resources, and community for patients and caregivers.
European rare disease database
Patient case studies
5 |
24% |
Disease patterns and progression | 5 | 24% |
Research summaries | 2 | 10% |
Clinical study results | 1 | 5% |
New treatment approaches | 1 | 5% |
Balogoun R (2026). [PMID: 42195045](https://pubmed.ncbi.nlm.nih.gov/42195045/). *Genes (Basel)*. [Epidemiology / Natural History]
Owrang D (2026). [PMID: 41514136](https://pubmed.ncbi.nlm.nih.gov/41514136/). *Molecular neurobiology*. [Case Report / Case Series]
Theunis M (2026). [PMID: 41126390](https://pubmed.ncbi.nlm.nih.gov/41126390/). *Ophthalmic genetics*. [Basic Science / Preclinical]
Liedtke D (2026). [PMID: 41959831](https://pubmed.ncbi.nlm.nih.gov/41959831/). *medRxiv*. [Basic Science / Preclinical]
Xie 谢飞 F (2026). [PMID: 41637835](https://pubmed.ncbi.nlm.nih.gov/41637835/). *Hearing research*. [Basic Science / Preclinical]
Nitta Y (2025). [PMID: 40695665](https://pubmed.ncbi.nlm.nih.gov/40695665/). *Auris, nasus, larynx*. [Epidemiology / Natural History]
Soleimani F (2025). [PMID: 41307816](https://pubmed.ncbi.nlm.nih.gov/41307816/). *Biochemical genetics*. [Case Report / Case Series]
Krishnamoorthy M (2025). [PMID: 40070250](https://pubmed.ncbi.nlm.nih.gov/40070250/). *Annals of human genetics*. [Epidemiology / Natural History]
Riahi Z (2025). [PMID: 41359850](https://pubmed.ncbi.nlm.nih.gov/41359850/). *Proceedings of the National Academy of Sciences of the United States of America*. [Epidemiology / Natural History]
Nayak G (2025). [PMID: 40827380](https://pubmed.ncbi.nlm.nih.gov/40827380/). *Disease models & mechanisms*. [Case Report / Case Series]