TECTA

TECTA is a gene that encodes the protein alpha-tectorin, which is a major component of the tectorial membrane in the inner ear. Mutations in this gene can lead to autosomal dominant non-syndromic sensorineural types of hearing impairment, such as DFNA8 and DFNA12, or autosomal recessive type of hearing impairment, DFNB21.

Function

The TECTA gene provides instructions for making a protein called alpha-tectorin. This protein is a crucial part of the tectorial membrane, a structure in the inner ear that plays a critical role in hearing. The tectorial membrane responds to sound waves by moving back and forth, which stimulates sensory cells to send signals to the brain that are interpreted as sound.

Clinical significance

Mutations in the TECTA gene can cause autosomal dominant non-syndromic sensorineural hearing loss, which is a type of hearing loss that occurs when the inner ear or nerve pathways from the inner ear to the brain are damaged. This type of hearing loss is typically not associated with other signs and symptoms.

There are three types of hearing loss associated with TECTA gene mutations: DFNA8, DFNA12, and DFNB21. DFNA8 and DFNA12 are inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. DFNB21 is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations.

See also

• DFNA8
• DFNA12
• DFNB21
• Tectorial membrane
• Sensorineural hearing loss

External links

• NCBI Gene
• Genetics Home Reference