3. Zoom on the cochlear implant

Faced with Connexin 26 there is no treatment, however there are solutions.

For mild, moderate and severe deafness there are conventional devices with the contours of the ears, they will amplify the sound vibrations and stress even more the functioning cells.

For severe and deep deafness there is the cochlear implant. The first cochlear implant was placed in 1957 by André Djourno. This implant artificially reproduces the functioning of the cochlea via electrodes that stimulate the auditory nerve. They thus reproduce the sounds heard by a healthy ear.

Extrait de l'interview du Professeur Sandrine Marlin

Coordinateur Centre de référence « Surdités Génétiques »
réalisée par Gabriel, Ruben et Julien le 17/12/2019

The cochlear implant is however very different from a device for simple hearing impaired since it affects all frequencies.

The cochlear implant is made up of three parts:

-a processor

-an antenna

-the implant

source : https://www.journee-audition.org/pdf/guide-MEDEL-implants.pdf

source : https://images.app.goo.gl/UwjTcRSiHvL3EbN79

The sound is received by the microphone, it is then encoded into digital signals which are then sent to the transmitting antenna. The transmitting antenna sends signals through the skin to the implant where they are converted into electrical signals. The signals are sent to the electrode bundle to stimulate the fibers of the auditory nerve in the cochlea. The signals are transmitted to the brain where they are identified as sounds.

1 Sounds are picked up by the microphone of the audio processor
2 The audio processor analyzes and codes sounds in specific sequences of electrical pulses.
3 These pulses are sent to the coil and transmitted through the skin to the implant.
4 The implant sends the pulses to the electrodes in the cochlea.
5 The auditory nerve transmits electrical signals to the auditory centers of the brain which interprets them as sounds

source : https://www.journee-audition.org/pdf/guide-MEDEL-implants.pdf

The cochlear implant is a good solution but it needs to be placed as soon as possible: before the child is 3 years old and even better before 1 year old so that the adaptation period is as short as possible.

Indeed we do not hear with the ear but with the brain and it must not stay too long without being stimulated otherwise the brain will not be able to decipher the messages received. The perception of noise allows the development of the brain.