First-ever gene therapy trial to cure form of deafness begins

Good news!

"The trial aims to show whether gene therapy can provide hearing for children born with hearing loss due to auditory neuropathy, a condition caused by the disruption of nerve impulses travelling from the inner ear to the brain.

Up to 18 children under the age of 18 years from three participating countries – the UK, Spain and the USA – will be included in the trial and followed up for five years to see the extent to which their hearing improves.

Auditory neuropathy can be due to a variation in a single gene – known as the OTOF gene – which produces a protein called otoferlin. This protein typically allows the inner hair cells in the ear to communicate with the hearing nerve. Mutations in the OTOF gene can be identified by standard NHS genetic testing.

About 20,000 people across the US and EU5 (UK, Germany, France, Spain and Italy) are thought to have auditory neuropathy due to OTOF mutations. ...
Gene therapy aims to deliver a working copy of the faulty OTOF gene using a modified, non-pathogenic virus. It will be delivered via an injection into the cochlea during surgery under general anesthesia. ...

The trial will consist of three parts, which must be done in order, with children receiving:

1. A starting dose of gene therapy (DB-OTO) in one ear only.
2. A higher dose of gene therapy in one ear only, following proven safety of the starting dose.
3. Gene therapy in both ears with the optimal dose selected after ensuring the safety and effectiveness of DB-OTO in parts 1 and 2. ..."

"... The gold standard treatment for the condition is currently cochlear implants, but they have limitations. They do not filter out background noise in complex hearing environments, such as when multiple people are speaking, often making it difficult to participate in group conversations or to enjoy music. ..."

First-ever gene therapy trial to cure form of deafness begins | Ars Technica Researchers hope to transform treatment of auditory neuropathy.

World-first trial to provide hearing for children with rare type of genetic hearing loss launches in Cambridge Addenbrooke’s Hospital in Cambridge is participating in a world-first trial to see whether gene therapy can provide hearing for children with severe to profound hearing loss due to a rare genetic condition.

Credits: Human Progress Weekly Progress Roundup

Johns Hopkins study links hearing loss with dementia in older adults

This study only confirms age old common wisdom that hearing or eyesight loss can promote dementia or other forms of cognitive degeneration.

However, remember Beethoven who was still able to compose incredible music pieces despite deafness. How one handles progressive deafness is very important too.

"... The findings, from a nationally representative sample of more than 2,400 older adults, are consistent with prior studies showing that hearing loss might be a contributing factor to dementia risk over time, and that treating hearing loss may lower dementia risk. ..."

Johns Hopkins study links hearing loss with dementia in older adults | Hub But the likelihood of dementia was lower among patients with hearing aids, researchers say

Restoring Hearing With Beams of Light

Good news! Amazing stuff! However, this is still in a very early stage of development. 

A new generation of cochlear implants is coming (however not before the next decade)! 

"... Some 466 million people worldwide suffer from disabling hearing loss that requires intervention ...
a new kind of cochlear implant that uses light emitters and genetically altered cells that respond to light. By using precise beams of light instead of electrical current to stimulate the cochlear nerve, we expect our optical cochlear implants to better replicate the full spectral nature of sounds and better mimic natural hearing. We aim to start clinical trials in 2026 and, if all goes well, we could get regulatory approval for our device at the beginning of the next decade. ...
Instead of getting the semiconductor emitter itself into the cochlea, the alternative approach puts the light source, such as a laser diode, farther away in a hermetically sealed titanium housing. Optical fibers then bring the light into the cochlea and to the light-sensitive neurons. ... We’re still thinking about how to get the viral vector to deliver the necessary genes to the correct neurons in the cochlea. The viral vector we’ve used in experiments thus far, an adeno-associated virus, is a harmless virus that has already been approved for use in several gene therapies, and we’re using some genetic tricks and local administration to target cochlear neurons specifically."

The article is actually written by one of the developers of this new device.

Restoring Hearing With Beams of Light - IEEE Spectrum Gene therapy and optoelectronics could radically upgrade hearing for millions of people