What does medical science know about the genetics of hearing loss?
There are more than 90 genes that can cause asymptomatic hearing loss when they mutate. Additionally, any single gene can have different kinds of mutations and cause different types of hearing loss.
The genetics of hearing loss are also closely linked with symptomatic congenital diseases such as Usher syndrome. In a case of Usher syndrome, a baby will be born with a vision and hearing impairments. Approximately between 6 percent and 12 percent of children with some form of hearing impairment are born with this genetic disorder.
So How Do Mutated Genes Cause Hearing Loss?
In many cases, gene mutations cause hearing issues by disrupting protein synthesis.
The process of hearing involves a number of proteins that build up the hair cells that respond to noise. But if a gene is mutated, it may not produce enough of the necessary proteins. Thus, the inner ear cannot work the way it should. One of the genes in question and with very widespread mutiation is the GJB2 gene. About 40 percent of newborns with hereditary hearing loss have a mutation of this gene.
Are there any other ways that genes can cause hearing issues?
In cases of symptomatic hearing diseases such as Stickler syndrome, problems can come from newborns having a distinctive facial structure, which causes middle and inner ear might to not have enough space to function as they should.
Why Is It Important to Study the Genetics of Hearing Loss?
It’s important to understand the way that children inherit hearing problems from their parents. This can let people with hearing loss know what to expect when they decide to have a child.
Finding a cure is another important possibility. By using gene therapy, it may be possible to improve protein production and make the inner ear more sensitive even in late childhood or adulthood.