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STRC gene
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Gene

STRC gene

The STRC gene provides instructions for making the protein stereocilin, which is essential for the function of hair cells in the inner ear.

Normal Function

The STRC gene provides instructions for making a protein called stereocilin. This protein is found in the inner ear and is involved in hearing. Stereocilin is associated with hairlike structures called stereocilia, which project from specialized cells called hair cells in the inner ear. Stereocilin links the tips of neighboring stereocilia to one another. Stereocilia must be physically connected to carry out certain auditory functions, such as making quiet sounds louder and detecting sound frequency.

Health Conditions Related to Genetic Changes

Nonsyndromic hearing loss

Pathogenic variants in the STRC gene have been found to cause nonsyndromic hearing loss, which is a partial or total loss of hearing that is not associated with other signs and symptoms. Specifically, these variants cause a form of hearing loss called DFNB16. This condition is inherited in an autosomal recessive pattern. The loss of functional stereocilin likely prevents stereocilia from connecting to one another, impairing auditory functions.

Deafness-infertility syndrome

Deafness-infertility syndrome is caused by the deletion of genetic material on the long (q) arm of chromosome 15. This region contains multiple genes, including the STRC gene and the CATSPER2 gene. The lack of stereocilin (due to the loss of STRC) impairs auditory functions, while the loss of CATSPER2 impairs the movement of sperm, leading to infertility.

Other Names for This Gene

  • DFNB16

Genomic Location

The STRC gene is found on chromosome 15.

Frequently Asked Questions

STRC-related hearing loss, specifically DFNB16, follows an autosomal recessive inheritance pattern. This means a person must inherit two non-working copies of the STRC gene, one from each parent, to develop the condition. Parents who carry one copy of the variant are typically unaffected carriers with normal hearing. If both parents are carriers, there is a 25 percent chance with each pregnancy that the child will have hearing loss, a 50 percent chance the child will be a carrier, and a 25 percent chance the child will inherit neither copy. Genetic counseling and carrier testing are recommended for couples with a family history of unexplained hearing loss.
Hearing loss associated with STRC variants is typically nonsyndromic, meaning it is not accompanied by other physical abnormalities or systemic health issues. The severity can range from mild to profound and is often present from birth or develops in early childhood. Because the condition affects the hair cells in the inner ear, it is usually sensorineural. Early identification through newborn hearing screenings is critical, as timely intervention with hearing aids or cochlear implants can significantly improve speech development and communication skills in affected children.
Yes, in specific cases involving a larger deletion of genetic material on the long arm of chromosome 15, STRC mutations can contribute to deafness-infertility syndrome. This condition occurs when the deletion removes both the STRC gene and the neighboring CATSPER2 gene. While the loss of STRC leads to hearing impairment, the loss of CATSPER2 impairs the movement of sperm, resulting in male infertility. This syndrome is distinct from the more common isolated hearing loss caused by smaller mutations within the STRC gene alone.
Currently, there is no cure for STRC-related hearing loss, but effective management strategies exist. The primary treatment involves amplification devices such as hearing aids for mild to moderate loss or cochlear implants for severe to profound loss. Regular audiological monitoring is essential to adjust devices as the individual grows or as hearing thresholds change. Research into gene therapy for specific genetic causes of hearing loss is ongoing, but it is not yet available as a standard clinical treatment for STRC-related conditions.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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