Elizabeth had her routine hearing check at Sick Kids last week and as it is becoming quite monotonous. Elizabeth has to listen for the sounds and place pegs when she hears the sound, this is the same activity she has been doing for the past 3 years (see above old picture). I despise this activity for many reasons; the pegs I am sure have never been cleaned, she of course will still put them in her mouth, the abundance of pegs are tempting for Elizabeth and her sensory needs (she loves run her hands through the bucket), I have to attempt to distract her from running her hands through them and keeping Elizabeth still for any length of time is a challenge. Elizabeth does pretty good despite how boring it really is but after 10-15 minutes we have both run out of patience. We have had the same Audiologist since Elizabeth's Auditory Neuropathy diagnosis and she doesn't mind all Elizabeth demands for stickers and for her to play the animal that make sounds. We also saw her ENT and he is still amazed by her progress as she is one of the few children to not need a cochlear implant. Only 15% of babies with AN regain their hearing and she is one of them which is such a blessing.
Normally her test results show a mild hearing loss in her left ear and her right ear tests fine. Last weeks results showed her left ear with a mild hearing loss and now a loss to her right ear as well which is not typical and is not related to congestion. AN can be such a frustrating diagnosis as it is does fluctuate day to day and we do not know how much distortion Elizabeth deals with. A great example of how AN distorts speech is here.
We will go again in 4 months to be retested and hopefully her right ear will be better. It is a good reminder for me to increase how much we use her FM unit at home and when we are out but the times when she really needs she cannot wear it (the pool and gymnastics). I must remind myself to bring a different activity for Elizabeth, any ideas would be much appreciated.

We will take option 'No'

What I despise the most in the summer is going for all Elizabeth's medical appointments. I absolutely hate spending a summer day sitting in dirty hospital waiting rooms trying to keep her occupied (as above we were playing playdough that I had stashed in my purse) and fighting hours of traffic to get in and out of Toronto. Elizabeth continues to puzzle and baffle every Doctor we meet with her varying symptoms. This summer we have seen some of her regular Doctors and also some new ones each one mystified by this 'very interesting and bright child'. As her Movement Disorder Specialist is away we met with the Doctor who trained her. I thought Dr. L in in his endless years of working may give us a glimpse as to what her diagnosis is. Dr. L. was interested in how she moves and specifically how she gets up to standing (think beginning walker on both feet and then pushing up with her hands). He feels she has both Dystonia and Ataxia but is not sure why, he does not feel this is related to prematurity. He was honest in stating that there was nothing than can be done for Ataxia presently and at least he was not too favorable of deep brain stimulation. The only options left for diagnosing were muscle biopsy and cerebral spinal fluid testing, we opted 'no thanks'. We also have seen the Geneticist who once again found Elizabeth's symptoms puzzling and does not feel her balance issues relate to prematurity. Her options; more blood for DNA testing and repeat Friedrich's Ataxia testing (this will be her 3rd time being tested) to rule out a rarer form. She also questioned whether Elizabeth has Perrault syndrome but this cannot be tested presently so we would need to wait until she hits (or doesn't due to the syndrome) puberty. We once again opted for 'no thanks'. Both Dr's think that her hearing impairment (Auditory Neuropathy) may be a key to her diagnosis as it is recently being linked with many disorders.
We feel all this invasive testing at the present is not fair to Elizabeth. A diagnosis of a rare syndrome will not change who she is or what we are doing for her and the search to find out why she is the way she is would just add extra stress on her and us. Her Neurologist was not too happy with our decision and said it could impact her children but Elizabeth has lots of time to decide what she wants done with her body until then we will stick up for her.
We of course tomorrow need to get up at the crack of dawn to see her Audiologist and the ENT, I hope it is rainy so we won't be cooped up on one our few summer days we have left.

AVT Graduate

Elizabeth has officially been discharged from Auditory Verbal Therapy (AVT). Elizabeth began AVT when she diagnosed with a hearing impairment (Auditory Neuropathy) at 6months old. AVT teaches children how to listen, process verbal language and how to speak. We have always credited AVT for Elizabeth's amazing language and conversation skills. The best part of AVT is that Elizabeth learned through play and exploration. Making volcanoes (as above) was a way to teach Elizabeth to follow a sequence of steps and reiterate what would happen next and now it is also another way Elizabeth can get muck with her beloved dinosaurs. Elizabeth loved AVT, her therapist's were amazing dedicated professionals whose creativity allowed Elizabeth to learn while she was having fun, it was not work for her.
We knew that her being discharged was inevitable as her listening skills are amazing and as per her last assessment she scored 75% in her receptive language and 98% in her expressive vocabulary. The only saddening part of her latest assessment was her articulation as it scored 6%. This 6% saddens me as Elizabeth has so much to say but many people cannot understand her. Elizabeth is now being followed by a Speech Pathologist for a 9 week block to work on her articulation which is great. Of course the downside is due to her age she will be discharged from the preschool service and will probably receive 10 session a year which is pitiful considering her articulation difficulties. We have a great health insurance plan that covers a lot of her needs but it will only cover 2 1/2 sessions of speech a year (not very helpful).
After great discussions with her ENT and Audiologist we have decided to try an FM Unit. With Elizabeth's hearing loss being unique we do not know how it impairs her ability to hear in all situations. We do notice that in noisy environments she does not hear and we have to touch her to get her attention and speak to her face to face. An FM unit can help her to hear a primary speaker in noisy environments (school, gymnastics, family gatherings, car etc). My fear is that her articulation is not only related to her poor oral motor skills and that some of her articulation difficulties are due to the fact that she cannot hear everything. We will continue to have her hearing checked every 4 months to monitor her closely.
We will definitely miss AVT but it is great that she has done so well and has graduated.