Idiopathic Hearing loss in 8yo - Hearing Disorders Message Board

vrie · 04-16-2004, 04:31 PM

I've posted before, but really had nothing to go on. Now it's even worse. My 8 yo son failed a hearing screening at school recently, and subsequently shows a 90db + sensorineural hearing loss in his left ear with his right ear fine. A ct scan, 5 tubes of blood, uranalysis, and several appointments later, we still can't find any reason for the loss. According to my son, his hearing has always been bad in that ear, but he wasn't screened as a newborn. We don't know if it started this bad or was progressive.

We have one more thing to do. We need to consult a pediatric urologist. The only abnormality in ANY tests was a borderline positive of red blood cells in the urine. His ENT wants to rule out Alport Syndrome, even though he thinks this is highly unlikely.

I have a few questions. From what I've read, Alport is usually diagnosed because of kidneys or family history rather than hearing loss. Also, both ears are affected in Alport. Am I right in this? We don't have a big family history of hearing loss (noise exposure or fluid but nothing else), but our family history includes a billion things that cause kidney problems. No one with the kidney problems had the hearing problems and vice versa. Should I be realistically looking at Alport? It's my feeling that the ENT just wants it ruled out since it is the last possible syndrome that could cause hearing loss.

Basically, is there anyone out there with unknown causes of hearing loss or information on Alport that can answer some vague questions I have? Thanks alot!

Biblophile · 04-30-2004, 06:33 PM

Hi V'rie!
There are now hundreds of identified hearing loss syndromes, but from what I recall, most of the time doctors and experts don't know what the root pathology of the loss is, especially in kids.
I heard (from another board that I lurk at, but don't post at b/c I've been banned one too many times there) that you're planning to get your child hearing aids! AWESOME!!!! I know that traditional wisdom has been that unilateral kids don't need aids...however, they can't hurt and might even help!Bit of advice....get colored aids, so your son accepts them more. The flesh colored ones are just for old people. HTH!!!!

Asrias · 08-07-2004, 04:28 PM

I have Alport's Syndrome. I also have the sensorneural (sp?) hearing loss in the high frequencies..

I have worn hearing aids since about the age of 11 or 12.

I am not a doctor but I know that I have always had abnormal blodd cells in my urine.

From what I have heard Alport's can skip a generation so is there a chance that either of the boy's grandparents had a kidney problem?

It's really hard to say how my family history started. My mother was the first to get kidney disease but one has to remember that in previous generations medical diagnosises were often wrong and there may have been a grandparent down the family line that died from kidney failure who's death was attributed to something else.

I suggest you go to a pediatric nephrologist and have your son checked by him/her.

vrie · 08-08-2004, 01:52 PM

My son went through about a million tests- Alport's wasn't really ruled out but his kidneys are fine so it kinda is ruled out. He will get annual kidney and hearing checks. Oh and I'm using colored covers for his hearing aids themselves and got flesh colered molds-that way he can change colors at will-- sorta like his hair LOL.

Asrias · 08-08-2004, 05:47 PM

Just be sure to tell him one thing...when he grows up he will be able to get inner ear type hearing aids that aren't so visible.

When I was young I had to wear those dorky over-the-ear type too and I hated them but since a child's ear is always growing it is the only way you can keep up with the changes.

Tell him to keep his chin up.