Goldenhar Syndrome is a congenital condition that usually affects just one side of the sufferer’s body. Common characteristics include facial and ear abnormalities, breathing problems, deafness, heart, lung, kidney and eye health issues, feeding problems and spinal and rib abnormalities. Each person with Goldenhar Syndrome is affected differently, so will have a different mix of the symptoms. The condition does not affect the intelligence or lifespan of the sufferer and 70% of suffers are male. The cause is not currently known. There are various surgical procedures available, depending on the symptoms of the sufferer.
Oliver is affected mainly by his profound deafness but also suffers from a fused section of vertebrae (between T1 and T6) on one side of his body which is causing a marked scoliosis of his spine. He also has two missing ribs. His face, and in particular his head, are not symmetrical and he has also had large skin tags removed from the front of both ears.
What is an ABI?
The MED-EL Auditory Brainstem Implant (ABI) is a solution for individuals with hearing loss due to a non-functioning auditory nerve. Bypassing both the inner ear and the auditory nerve, the MED-EL ABI stimulates the cochlear nucleus (CN) and provides users with a variety of hearing sensations to assist with sound awareness and communication.An ABI consists of two components, the external audio processor and the implant. The audio processor is either an OPUS 2 or OPUS 1. The implant consists of an electronics housing and an implantable soft silicone matrix with a 12 contact electrode array. The 5.5 × 3.0 mm matrix is the active interface between the stimulation electronics and the neural tissue. An additional reference electrode is used for advanced telemetry measurements providing added functional reliability and control.
The soft silicone matrix with a 12 contact electrode array is surgically inserted directly onto the brainstem. The implant stimulates the cochlear nucleus, which enables the recipient to distinguish a variety of sounds. Following surgery and the post-operative healing period of 14 days, the user is required to undergo an intensive training period with a qualified audiologist to learn how to interpret sounds and understand speech. Generally, it is important to realise that speech understanding with an ABI is not on the same level as speech understanding for cochlear implant recipients. Extensive training is all the more crucial for this reason, allowing the recipient to gain the best benefit from an ABI and to make the most of this advanced technology.