This seemed to be confirmed at our following two audiology appointments at least according to the official paperwork. The new audiologist was reporting that Robyn had almost completely normal hearing levels with a combination of both ears. However increasingly this is not what I was seeing at home, I also had some concerns about how the appointments were being conducted having moved away from an audiologist who was very familiar with Robyn and had been working with us since she was very young so had experienced first hand how loud Robyn's breathing used to be for example.
After raising my concerns again we were re referred to the consultant audiologist's clinic with our original audiologist doing the testing alongside our consultant. Mixed emotions about the results. Obviously we are not hoping for a hearing loss but also we were seriously concerned that Robyn was missing out on a lot.
The test returned results showing a 90dbl hearing loss. This places her hearing loss directly on the line between a severe hearing loss and a profound hear loss. For those unfamiliar with the scale hearing loss is measured on a scale from mild - moderate - severe - profound. So as you can see a very long way from the normal hearing levels previously recorded.
In a way I was glad that my instincts were correct and that I became 'that mum' pushing for things others didn't think she needed. But on the other hand we have now confirmed our child has a severe hearing loss which is really reducing her contact with the rest of the world. Having recently watched a hearing loss simulation video I was quite shocked by how little it shows she can actually hear. It also goes a long way to explain why Robyn's speech is very delayed for her age.
The good news of course is now we have a definitive result we can put in place a plan to help with it. Robyn has been issued with a BaHa (Bone anchored hearing aid). This works by bi-passing Robyn's ears altogether. The processor transmits sound directly into the skull and thus using vibration directly stimulates the cochlea and allows Robyn to hear sound. We know Robyn does have permanent hearing loss but at least part of the los is caused by glue ear which has built up again since her grommets have come out. A normal behind the ear hearing aid works by amplifying sound and if we used these on days when Robyn's glue ear has fluctuated and isn't so bad the hearing aids would be too loud and cause her discomfort. The Baha by passes this issue and so we don't have to worry about the noise being too loud on any given day. It is usually worn behind the ear to do this most effectively but works on any part of the skull which is handy as Robyn still spends a lot of time lying down due to her hypotonia and her proprioception sensory issues. This causes constant feedback noise if the BaHa is in the 'correct' position so we will make do with it being on the front of her head for now.
Robyn modelling her BaHa
Robyn will wear the Baha on a softband to begin with until we see how her hearing loss develops as she grows, it is quite common for hearing in CHARGE to deteriorate with age but it is also common for children to grow out of having glue ear so her hearing could also improve with time. We are reluctant to insert more grommets at this time as each time you do this it weakens the ear drum. It ws helpful to carry out the first occasion as it allowed us to determine that Robyn had glue ear as well as a permanent hearing loss but if the glue ear is likely to re occur after grommets as it has it is probably best to wait a while before we try it again.
If it becomes apparent that Robyn will need a BaHa permanently at some stage we would move to the more permanent version of attachment. Previously this involved an operation to attach what was essentially a screw into the skull with the end of this protruding from the skull just behind the ear. The processor would then clip onto this to work. Once in it was a permanent feature so even if the BaHa was no longer needed in future the patient would be left with the protruding attachment piece. More recently it has been changed. The connector is no longer protruding from the skull. The processor attaches with a magnet. One is placed under the skill on the skull behind the ear. When the processor is not attached the magnet is virtually invisible once the operation site has healed completely.
Obviously as it involves an operation it is not something that is rushed into so its probably a long way off if we ever need that but I thought people might be interested.
We have only had the BaHa since Friday so not even a full week yet but already we have noticed HUGE changes. Robyn responded to me calling her name for the first time that I ever really remember. And in those few days we are now regularly hearing the 'm' sound which we didn't have before and today for the first time we heard a 'g' sound. Amazing progress in 5 days. The processor does come unclipped from the band quite easily due to Robyn moving around quite carelessly but already she notices such a difference without it on that she immediately picks up the processor and crawls to me to have it reattached. She clearly loves beign able to hear what is going on round about her finally.