10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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9PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingsensory perception of soundextracellular regionnephritisNephropathyliver diseasebiliary tract disease
OTOS (otospiralin) is a small protein expressed by fibrocytes in the inner ear cochlea and vestibule 1. These non-sensory cochlear cells are critical for maintaining ionic and hydric homeostasis in the endolymph 2. OTOS appears essential for normal auditory function; Otos-deficient mice exhibit moderate deafness with degeneration of type II and IV fibrocytes, while hair cells and stria vascularis remain intact 2. This suggests OTOS dysfunction impairs fibrocyte-dependent cochlear physiology and may predispose to age-related hearing loss 2. OTOS has significant clinical relevance in cisplatin-induced ototoxicity. Genetic variants in OTOS associate with cisplatin-induced tinnitus in cancer survivors, with a genome-wide significant SNP near OTOS (rs7606353, P = 2 × 10⁻⁶) 3. G alleles of OTOS SNPs rs77124181 and rs2291767 are over-represented in ototoxicity-free patients, suggesting a protective role 4. OTOS overexpression in auditory cell lines confers resistance to cisplatin-induced cytotoxicity 3. These findings position OTOS as a potential otoprotective therapeutic target for chemotherapy-induced hearing loss, with pathway analysis implicating potassium ion transport mechanisms 3.
1
OTOS encodes a novel protein produced by non-sensory fibrocytes of the inner ear; OTOSP gene structure and chromosomal localization
PMID: 126874212
Otos-deficient mice show moderate deafness with fibrocyte degeneration; OTOS role in cochlear physiology and endolymph homeostasis
PMID: 156320833
OTOS variants associated with cisplatin-induced tinnitus (rs7606353); OTOS overexpression confers cisplatin resistance in auditory cells
PMID: 309526444
G alleles of OTOS SNPs rs77124181 and rs2291767 protective against cisplatin-induced ototoxicity
PMID: 25410892⚠Limited data available — This gene has 4 indexed publications. Summary and analysis may be incomplete.
liver diseaseOpen Targets
biliary tract diseaseOpen Targets
autosomal dominant nonsyndromic hearing lossOpen Targets
hearing loss, autosomal dominant 87Open Targets
hearing loss, autosomal dominant 85Open Targets
hearing loss, autosomal dominant 86Open Targets
hearing loss, autosomal recessiveOpen Targets
X-linked nonsyndromic hearing lossOpen Targets
hearing loss, autosomal dominant 75Open Targets
hearing loss, autosomal dominant 77Open Targets
hearing loss, autosomal recessive 118, with cochlear aplasiaOpen Targets
Non-syndromic genetic deafnessOpen Targets
hearing loss, autosomal dominant 80Open Targets
autosomal recessive nonsyndromic hearing loss 9Open Targets
X-linked mixed deafness with perilymphatic gusherOpen Targets
X-linked mixed hearing loss with perilymphatic gusherOpen Targets
Autosomal recessive cerebellar ataxia - blindness - deafnessOpen Targets
No pathogenic variants reported on ClinVar for this gene.