Science 3 min read

Scientists Use Genetic Editing to Cure Congenital Hearing Loss

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In over half of cases, congenital hearing loss in humans is due to genetic causes.

There are dozens of known genes associated with hereditary deafness.

In Autosomal Recessive Deafness 9 (DFNB9), which accounts for 80 percent of cases, the culprit gene is called OTOF.

Mutations in the OTOF gene, which regulate the otoferlin protein responsible for the transmission of sound data in the inner ear, are one of the most common causes of hearing loss.

For the time being, cochlear implants constitute the sole available option for people affected by DFNB9 to restore their hearing to a certain degree.

Now, it may be possible to cure this hearing loss without the need for an electronic device.

In a new study, a team of American and French researchers has shown the potential of gene-based therapies in reversing hearing loss.

Replacing Defective Hearing Genes

Blindness, cancer, and muscular dystrophy are examples of health conditions where gene editing has made progress with promising perspectives.

In 2015, scientists used gene therapy to repair hair cells in the inner ear to help deaf individuals to recover hearing. But the technique is “aimed at patients who have lost their hearing due to years of loud noise or music, or the toxic effects of certain drugs.”

Now, thanks to the work of a Franco-American research team, we can add hereditary hearing loss to the gene therapy list of theoretically curable conditions.

Researchers managed to restore hearing in mice affected by congenital deafness by replacing defective genes.

The team used harmless viruses, called Adeno-Associated Viruses (AAVs), as vectors to transfer healthy genes that replace defect ones “to reconstruct the otoferlin coding region” in DFNB9 mice with otoferlin protein deficiency.

The injection of AAVs stocked with otoferlin-coding genes led to:

“long-term restoration of otoferlin expression in the inner hair cells, and then restored hearing… The scientists have therefore obtained initial proof of the concept of viral transfer of fragmented cDNA in the cochlea using two vectors, showing that this approach can be used to produce otoferlin and durably correct the profound deafness phenotype in mice.”

The results of the research “suggest that the therapeutic window for local gene transfer in patients with DFNB9 congenital deafness could be wider than thought”

This is a world-first breakthrough for congenital hearing loss.

Although AAV-based gene therapy will likely take some time to go through human clinical trials, patients who were born deaf can hope to one day be able to hear without implants.

Read More: Stargazing for the Deaf: The Future of Accessible Astronomy

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Zayan Guedim

Trilingual poet, investigative journalist, and novelist. Zed loves tackling the big existential questions and all-things quantum.

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