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Any autosomal recessive nonsyndromic deafness in which the cause of the disease is a mutation in the TSPEAR gene.
Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 9:42 PM UTC
Online Mendelian Inheritance in Man
Genetic and Rare Diseases Info Center
Features include always present findings: Inner ear hearing loss (sensorineural hearing impairment).
Organ System | Phenotype Count | Example Features |
|---|---|---|
Ears | 1 | Inner ear hearing loss (sensorineural hearing impairment) |
Age of onset: at birth.
TSPEAR function has not been fully characterized.
Autosomal recessive nonsyndromic hearing loss 98 is associated with mutations in the TSPEAR gene on chromosome 21.
Genetic testing for TSPEAR is available. Testing is considered confirmatory for diagnosis.
Biomarker and diagnostic research for autosomal recessive nonsyndromic hearing loss 98 has been reported in the published literature.
Phenotype severity distribution: 1 always present feature.
No clinical trials have been registered for autosomal recessive nonsyndromic hearing loss 98.
4 publications have been identified in PubMed for autosomal recessive nonsyndromic hearing loss 98. Research spans Diagnostic / Biomarker (25%), Review / Meta-Analysis (25%), and Basic Science / Preclinical (25%).
Ahmadkhani A (2026). [PMID: 41486137](https://pubmed.ncbi.nlm.nih.gov/41486137/). *J Med Case Rep*. [Review / Meta-Analysis]
Fazeli-Jezei R (2025). [PMID: 40122168](https://pubmed.ncbi.nlm.nih.gov/40122168/). *Acta Otorrinolaringol Esp (Engl Ed)*. [Epidemiology / Natural History]
Xu Y (2025). [PMID: 39461497](https://pubmed.ncbi.nlm.nih.gov/39461497/). *Clin Chim Acta*. [Diagnostic / Biomarker]
Teryutin FM (2024). [PMID: 39436953](https://pubmed.ncbi.nlm.nih.gov/39436953/). *PLoS One*. [Basic Science / Preclinical]