JERUSALEM, Sept. 14 (Xinhua) -- A research collaboration between scientists from Israel, Palestine and the United States has identified a gene essential for normal hearing, offering new clues about how inherited hearing loss develops, Israel's Tel Aviv University (TAU) said in a statement on Monday.
The findings were published in the U.S. journal Proceedings of the National Academy of Sciences by researchers from TAU, Bethlehem University in the West Bank and the University of Washington.
The researchers found that mutations in a gene called FMN1 cause hearing loss and can also affect hair and skin pigmentation.
The investigation began with an extended Palestinian family in which several children were born with bilateral hearing loss and unusually light-colored hair. Genomic analysis revealed that the affected children carried two altered copies of the FMN1 gene.
In animal studies, the researchers observed hearing impairments among mice lacking functional FMN1. Microscopic analysis showed that the gene is crucial for maintaining the structural organization of supporting cells within the cochlea, a fluid-filled, spiral-shaped cavity in the inner ear that transforms sound waves into electrical signals for the brain.
The researchers explained that without a functional gene, these inner-ear supporting cells become disorganized, compromising the ear's mechanical integrity and hindering sound transmission from the cochlea to the brain.
The team also discovered that FMN1 plays a role in transporting pigment inside cells, explaining the light hair color observed in affected individuals.
The researchers said their discovery adds FMN1 to the list of genes essential for hearing, providing critical insight for the development of future genetic therapies for inherited deafness. ■
