Assorted stuff...

I found this link, http://www.sens.com/helps/helps_intro.htm , which has some demos of what it is like for a person with hearing loss. Demo 1 is a severity of hearing loss simulator. Lily's hearing in her "good" ear is the one labeled "HL5". No wonder Lily doesn't seem to care about music very much. Demo 5 has a cochlear implant simultation.

I discovered that there is a doctor in Jacksonville that does cochlear implants at a children's hospital and is not affiliated with UF. This doctor implants the brand "Advanced Bionics" (AB) implants. AB has focused on making music more enjoyable for implantees and has something called a 120 Virtual Channel external processor coming out in the spring. There are currently 16 to 24 electrodes that are implanted internally, depending on the cochlear implant company, but the AB technology works with the outer processor to create more realistic sound somehow. I think. I don't know much about the differences between the three companies, but I guess I will have to do some research. I did find this chart comparing the three implants, but it is slightly outdated and does not include the new AB Harmony. I am not sure if the doctor in Jax would even be an option for us though, considering Lily's abnormal inner ears. Dr. Antonelli is the one that doctors around the state refer their toughest cases to. This wait to hear if they will implant Lily is hard and we don't know the date of their team meeting. I just hope to hear by the end of the month.

I have joined a Yahoo group for LVAS and have learned quite a bit about Lily's condition there. A medical doctor with LVAS himself moderates the group and quickly answers questions and clears up misinformation. There was a recent discussion about Pendred's Syndrome and genetics and progressive hearing loss after these links were posted by someone from the National Institute of Health:

Enlarged Vestibular Aqueducts and Childhood Hearing Loss

http://www.nidcd.nih.gov/health/hearing/eva.asp



More About Childhood Hearing Loss and EVA

http://www.nidcd.nih.gov/health/hearing/eva-intro.htm



Genetics of Enlarged Vestibular Aqueducts (EVA)

http://www.nidcd.nih.gov/health/hearing/genetics.asp



Pendred Syndrome

http://www.nidcd.nih.gov/health/hearing/pendred.asp



NIDCD Research on EVA and Hearing Loss

http://www.nidcd.nih.gov/health/hearing/research.asp

A pretty interesting discussion followed. One person pointed out that the first article says that Pendred's Syndrome likely causes the progressive loss, but made no mention of enlarged vestibular aqueducts/non-Pendred's causing progressive hearing losses. Several people replied saying that their children do not have Pendred's but have had progressive losses so the article must be inaccurate. One mother replied with this, which has made a lot of sense to me out of some overwhelming information:

I've excerpted some passages below from the following genetic encyclopedia. One point this article makes is that there are many, many different possible mutations in the PDS gene most likely causing a RANGE of symptoms that include LVAS. We're all dying to know whether our kids fall into the genetic definition of Pendred Syndrome when the definition of Pendred Syndrome is really a symptomatic one. There were a set of symptoms, someone gave them a name, and medicine looked at the genetics later as the science advanced. The genetics showed that this range of disorders was a little more complicated than that particular set of symptoms.

http://health.enotes.com/genetic-disorders-encyclopedia/pendred-syndrome

"As of March 2001, genetic researchers identified at least 47 different types of alterations in the PDS gene among different families. However, four of these are more common than the others, and it is estimated that approximately 75% of affected people have these common changes.

" ... it is believed that changes in the pendrin gene actually cause a number of overlapping conditions. These conditions range from Pendred syndrome (i.e. sensorineural hearing loss [SNHL] and thyroid changes) to SNHL with EVA.

"Changes in the PDS gene have been found in people who have SNHL and EVA, even though they do not have thyroid changes that would have helped make a clear diagnosis of Pendred syndrome in the past. Thus, future genetic studies on large groups of individuals with SNHL will help researchers understand how common Pendred syndrome truly is, as well as the range of symptoms that are caused by changes in the PDS gene."

So that is it for the assortment of thoughts for now. I plan to get on here this weekend and talk about Lily's progress.