More speech, and an informative phone call...

Finally, our final speech assessment! Lots more listening, more words, more sentences, some copying, which she hates...but I felt confident Jonica's responses were pretty indicative of her true abilities. She's pretty co-operative and is reliable with her responses, so she's pretty easy to assess.

The speechie had scored her first round of tests, and she was mostly in the average range! This is amazing considering how much hearing she doesn't have, but she has always outperformed her audiogram and her language development has always been excellent so in ways I wasn't surprised. Comprehension was in the average range, Word Structures was mildly delayed (mostly due to missing S's off the ends of words) and Expressive Vocab was in the average range. Quite pleased :-)

Earlier this same week, on Tues 1st July, I received a phone call from our implant surgeon with results from Jonica's CT scan. As it turns out, Jonica has what's called Enlarged Vestibular Aqueduct on both sides, and also the Mondini defect on both sides. EVA I had fully expected, but the Mondini was quite a surprise. A brief explanation...

EVA... There's a small bony canal & lymphatic duct in your head that runs from the inner ear towards the brain cavity, and there's a lymphatic sac between the skull and the lining of the brain. When the little canal is slightly larger than normal, the fluid in the sac can flow back towards the inner ear which it's not supposed to do, and a hearing loss occurs. One of main characteristics of EVA is a deteriorating hearing loss, usually in sudden, large changes and sometimes with recovery but sometimes not. Head trauma can be a catalyst, even a mild bump. This is exactly how Jonica's hearing loss has progressed, so I fully expected EVA to show up on the scan.

Mondini defect...the cochlea is a tiny, snail-shell-shaped organ in the inner ear which contains all the small hair cells which detect and transmit sound to the brain via the relevant cranial nerve (cranial nerve VIII, for those who are interested). A normal cochlea has 2.5 turns, but one with the Mondini defect only has 1.5 full turns, leading to hearing loss. This condition is not necessarily inherited, it's usually due to an interruption to development of the inner ear in about the 6th week of pregnancy. It seems that Mondini and EVA tend to travel together, so at this stage it's a bit hard to tell if one caused the other or if they just happen to both be present in this case.

So, we have a clear reason for her hearing loss. There still may be a genetic component, but we haven't had genetic testing done yet so we won't know until we have that done. We also have some ideas about how to preserve the hearing she has left, and thankfully she's not a very bump-my-head kind of kid. Now we just have to wait for an appointment with the surgeon to do paperwork and arrange a surgery date...currently scheduled for 28th July, although we're on there cancellation list in case something comes up sooner!

Next appt: Mon 28th July, Implant Surgeon