June 15, 2023 – (Innsbruck, Austria): Hearing loss is a common problem affecting 1 in 5 people around the world. Millions of people already benefit from a range of treatments such as hearing aids, bone conduction devices or cochlear implants that help them to hear again. But for some, a rare disease stops sound from reaching the brain, meaning these devices are not an option.
Neurofibromatosis type 2 (NF-2) is a genetic disease that affects just 1 in 35,000 - 40,000 people, usually at a younger age. A benign tumour grows on the auditory (hearing) nerve, expands into the brain and therefore needs to be removed. Removal usually damages the hearing nerve, which is why a cochlear implant will not work. Deafness is inevitable. For these people an Auditory Brainstem Implant (ABI) is their only chance to hear.
The ABI system consists of an implant with a small paddle-shaped electrode which is placed onto the brainstem. An audio processor that is worn behind the ear picks up the soundwaves, converts them into electrical signals and sends them on to the implant, which then stimulates the neurons of the auditory brainstem so that the person can hear again.
Despite the number of affected people being low, MED-EL, a leading global hearing implant manufacturer from Austria, did not want to leave NF2 patients behind. In close collaboration with specialised ENT and neurosurgeons, MED-EL developed an implant that electronically stimulates the brainstem. 20 years ago, the MED-EL ABI was officially launched for adults. In 2017 it also received CE-approval for children from 12 months of age.
Because the surgery is complex and involves working on the brainstem, only specialist surgeons worldwide are able to perform auditory brainstem implantation. One of the world’s leading ABI surgeons, Dr Robert Behr, former Head of the Department of Neurosurgery at the University Clinic in Fulda, Germany, has helped hundreds of children and young adults to hear by providing them an ABI. “In its early days, researchers were convinced that the ABI would only help users with lip-reading, but they would not reach open-set speech understanding”, comments Dr Behr.
20 years and more than 1,000 ABI users worldwide later, the initial scepticism towards hearing success with the device is gone. Many people communicate without lip-reading and live an independent life in the hearing world.
Hannah, a 36-year-old NF2 patient from Australia became profoundly deaf after her tumours were removed 2 years ago. “I lived in total silence for 5 months. The ABI gave me my life back, the world that I thought would always be lost for me”, the teacher says. Only two years after she received her ABI she is travelling Europe on her own.
MED-EL CEO Dr Ingeborg Hochmair states: “ABI technology and surgery have continuously advanced in the past 20 years, allowing for an effective restoration of functional hearing. We have seen significant improvements in users’ hearing performance. The ABI is an important part in reaching our mission of overcoming hearing loss as a barrier for communication.”
The development in the field of auditory brainstem implantation is ongoing. Other indications that involve an absent or damaged hearing nerve or conditions that do not allow the use of a cochlear implant are continually being explored. This should help a larger number of people with rare diseases affecting hearing to benefit from auditory brainstem implants and give them the gift of hearing back.
MED-EL Medical Electronics, a leader in implantable hearing solutions, is driven by a mission to overcome hearing loss as a barrier to communication and quality of life. The Austrian-based, privately owned business was co-founded by industry pioneers Ingeborg and Erwin Hochmair, whose ground-breaking research led to the development of the world’s first micro-electronic multi-channel cochlear implant (CI), which was successfully implanted in 1977 and was the basis for what is known as the modern CI today. This laid the foundation for the successful growth of the company in 1990, when they hired their first employees. To date, MED-EL has more than 2,500 employees from around 80 nations and 30 locations worldwide.
The company offers the widest range of implantable and non-implantable solutions to treat all types of hearing loss, enabling people in 140 countries enjoy the gift of hearing with the help of a MED-EL device. MED-EL’s hearing solutions include cochlear and middle ear implant systems, a combined electric acoustic stimulation hearing implant system, auditory brainstem implants as well as surgical and non-surgical bone conduction devices. www.medel.com
Doz. DI Dr DDr med. h.c. Ingeborg Hochmair
PR & Corporate Communications
MED-EL Medical Electronics
T: +43 5 7788-1029