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!

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!!!!

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.

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.

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. :wave:

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. You mean Super Seals? Those are cool. Maybe later on he'll want to get colored molds or even glow in the dark or color changing molds! You really should see all the cool earmolds out there! There are also hearing aid stickers out there. Ask your audi about them. Phonic-Ear's hearing aid stickers are very cool and HIGHLY recommended! They are very realistic looking and even adults would love them! They come in: tigerprint, holstein cow, peacock, snakeskin, Monarch butterfly (gold and black), motherofpearl, purple background with "bubbles", orange flowers, and one I can't really describe. It's a purple background with black sploches.
There are also REALLY cute hearing aid covers out there called EarWear. They come in all types: ear of corn, batwing (perfect if he likes Batman!), banana, lightening bolt,cupid,quilted,fish, polka dots,zigzag,inlay, wolf from Little Red Riding Hood, saying " All the better to hear you with my dear", fake fur, dog, cat, teddy bear and a rocket one. 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. I don't think he will. Not unless technology improves drasticly. The ITE aids are only for losses up to severe,(and only in certain cases) and V'rie's son has a profound loss. Besides, even with less severe losses, the ITE aids don't always have enough power. I have been told by two respected audis at two prestigous audilogical depts (Mass. Eye and Ear and Clarke School for the Deaf) that the tiny aids are purely cosmetic and don't give enough power for more then a mild loss. Asrias, I bet you anything that you're mostly lipreading and not really "hearing" Try going back to BTEs....I bet you'd be amazed at how much more you can hear with them as compared to ITEs.....and trust me...BTEs are not THAT noticable. You can actually get mini-BTEs, if you're really concerned. Hardly anyone notices MY hearing aids, and I have the ones in the cool colors plus a colored earmold, plus some accessories on the tubing...and I have pretty short hair too.

When I was young I had to wear those dorky over-the-ear type too and I hated them
I did too. Especially seeing as when we were little the BTE aids looked like only old people wore them. The "coolest" thing you could say about them was that they came in twelve shades of beige! I think the reason why I hated them was b/c they made me feel different. I grew up completely and totally mainstreamed, and for a long time I thought I was the only one in the entire world who was hoh. V'rie....my best advice would be to find out about organizations for the deaf and hoh, that you can join so your son can meet others like him. A good non-partison organization is Hands and Voices (has a pretty active unilateral membership if I recall correctly) Also, The American Society For Deaf Children has a very active listserv and welcomes ALL parents of deaf and hoh kids, irregardless of commuication preference or degree of hearing loss. There might even be a listserv out there for parents of unilateral loss kids. Some people might reccomend SHHH but it's not a very interesting or very helpful organziation. Most members who belong to SHHH are the stereotypical old person with an aqquired loss, and the info is pretty dull. Their newsletters mostly drone on and on about new hearing aids and Assitive Listening Devices. AG Bell is also not too helpful, seeing as it's mostly for bilaterally deaf kids (it is also VERY militant in it's assertain that speech is the only way to go) Also try support groups around where you live. Ask your audi for more info and resources!

Well, I think that since the boy is only 8 years of age by the time he reaches adulthood the advances in hearing aids will probably have been significant.

Furthermore I have what I believe is a half-shell hearing aid...it isn't all the way in the ear like the little tiny ones but it is far less noticeable as the BTE and more comfortable.

You can never, ever convince me to go back to the BTEs....the only thing those are good for are children since they need them due to the dramatic changes in their ear sizes as they grow and the molds can be redone without changing aids.

But hey...whatever works for you.

it isn't all the way in the ear like the little tiny ones but it is far less noticeable as the BTE and more comfortable. Less noticable? Oh, man.....I just LOVE how some people make BTEs sound like those old body worn aids in terms of cosmetics. Besides they now have a mini-BTE that's a lot less noticable! Comfort? Do you mean the occlusion factor? Or itchy-ears? B/c I've worn both BTEs and ITEs, and BTEs win HANDS down for comfort. Having a soft earmold is much more comfortable then having a hard piece of plastic jammed in your ear. If you're talking about itchy ear, try hypoallergenic earmolds.

You can never, ever convince me to go back to the BTEs Next time you're at the audi's ask to try a BTE. You may be extremely surprised at the difference in volumne and sound quality! I was just like you when I was in early high school. I had hated my BTEs and gone to ITEs in jr high (purely for cosmetic reasons) Thank GAWD I went to Mass. Eye and Ear Infirmery where an audi put a BTE in my ear...WOW! I am now hardcore for BTEs! ....the only thing those are good for are children since they need them due to the dramatic changes in their ear sizes as they grow and the molds can be redone without changing aids.
That's your opinon...that is not fact. I wonder if you had been brought up to be proud of your disabilty, instead of hiding it, if you'd be yapping about the wonders and invisabilty of tiny aids. Probaly not. Look I understand 100% your view on aids and being self-conscious about them. However, I think it's a lot better to expose dhh kids to other dhh kids who wear aids/CIs/other assitive devices and to view hearing aids as just like glasses. Then that way, the dhh kid won't feel "different"(in a bad way) or self-conscienous about their aids. Many of the kids I grew up with see my being hoh as just a "difference", just a part of me, the same way my blonde hair and my love for books is a part of me (and I grew up in a very snobby "Stepford Wives" suburb, where all the girls were anorexic with the stereotypical high achiever superfical football/cheerleader culture active in the schools) Almost forgot...one HUGE advantage to BTE aids is that they offer an unspoken "clue" that the person may have trouble hearing, and they're not stupid or retarded. Yes, in some ways that can be a negative, like when you run into people who think you're retarded b/c of being d/hh....but I've noticed that most of those people are almost retarded themselves! Most people don't even think twice about the fact that I am hoh!

I understand what you are saying but I also feel that children are going to be children and tease this child. You can't shelter the boy from the negative aspects.

Making the hearing aids less noticable will help the child who wears them be less conscious about them which will help keep the childs self-esteem up.

Yes, I agree with you that he needs to exposed to other dhh children and that he needs to be reminded that his deafness is not something to be ashamed of. However, you have to remember to see things through the eyes of a child. He will be self-conscious about the wearing of the hearing aids and no amount of parental reassurances is going to stop that.

I think there is a reason why most people go to the ITE instead of the BTE...it isn't as noticeable and that does make a difference to many people.

I think we should agree to disagree here...you almost seemd to get angry with me in your above post.

Just a by the way-- the cosmetics is the reason i went with very bland blend in aids in the first place-- now he wants to show them. He has relatives with severe disabilities. In our former home he was one of few in school with aids-- he knew no one else- here there is a school for deaf and blind locally and I have seen many people with aids just shopping. I feel good about his adjustment that he wants colors on his aids and is kinda seeing them as accessories like his sisters wear jewelry and hair bows! He's got a personality where he really doesn't care--one child made fun of him at first and he told him he was a bully anyway and his opinion didn't count! LOL!

I understand what you are saying but I also feel that children are going to be children and tease this child. You can't shelter the boy from the negative aspects.

Making the hearing aids less noticable will help the child who wears them be less conscious about them which will help keep the childs self-esteem up.

Yes, I agree with you that he needs to exposed to other dhh children and that he needs to be reminded that his deafness is not something to be ashamed of. However, you have to remember to see things through the eyes of a child. He will be self-conscious about the wearing of the hearing aids and no amount of parental reassurances is going to stop that.

Asrias, There might be teasing....but I have noticed that kids tend to pick up on self-conscienoiusness which is already there. If a kid and a parent is positive about a particlular difference, then other kids will just not really think twice about it. I remember when I was little and VERY self-conscienous about my aids (I picked up on my parent's feelings about having a kid with an "obvious" difference) I got teased up the wazoo. Now that I'm older and have cooler looking aids, and am actually PROUD of being dhh, people don't even think twice about the fact that I have to wear them. Matter of fact, whenever I run into someone I went to school with, they always say they didn't think twice about the fact that I was hoh...and they always forgot that I wore aids in the first place. It seems like I was a victim of bullies who would pick on anyone for any perceived difference. Parental and child attitude can go a really really long way. You probaly got bullied b/c the bullies sensed that it was a weak spot with you. Acting like a hearing aid's just a part of you really builds a good defense against bullies. I have a lot more to say....but I'm being kicked off...so I'll finish this post a little later!

Making the hearing aids less noticable will help the child who wears them be less conscious about them which will help keep the childs self-esteem up.
Perhaps....but the thing is the BTE aids really aren't as noticable or cosmeticly jarring as some detractors would have you believe. Some of the adverstising makes BTE sound like a body worn aid or an ear horn!
Besides, JIMHO, the ITE aids are far more noticable (and ugly) then the BTEs.

hi, I can definatly tell you what's wrong. I have had problems with my ears my whole life. I failed every hearing test I have ever taken. When I was in kindergarten 1990. I had taken so many test that the doctors could not get a staple through my paper work. they never did find out what was wrong except for the fact that I could not communicate with that ear. I hear at 92 db in that ear and the same in the other one now. Because of other recent ear problems with my hearing ear,I have done tons on research and actually test myself. What the problem is, is during birth, somehow some nerves got tweeked, by that happeneing, I could never understand. Ask your son if he hears noise out of that ear aka just unrecongnize sounds... by the nerves being pinched, I causes the sounds to travel or process wrong, ending up with a disorted message. I can only clearly understand vowel sounds. I can not hear these unless, I have phone up to the ear or a whisper. I have to get back to class, but I hope I helped
Take good care of his ears because even with these conditions, it is going to be hard to live with society. Not hard, he will have to adapt. when you ear with only one ear, you can not tell the direction of where sounds are coming from. keep that in mind.

having a hearing situation, has made me a stronger,more independent, self-motivated person. Kids are mean, but just tell him, you are special, there is nothing wrong with him, if these people can not except him for who he is, then they do not deserve to be his friend. Good Luck the support is always here.

having a hearing situation, has made me a stronger,more independent, self-motivated person. Same with me. Besides it's something cool and unique... I am very glad I have it. Without it I'd probaly be a cookie cutter Stepford Suburban kid Kids are mean, True...kids are mean. On the other hand, I have heard amazing stories of acceptance. Attitude can really make a HUGE difference. I've heard of hearing kids being jealous of a hoh kid's "cool ears"!!! (b/c now they have cool colored aids rather then the old geezer flesh colored aids) Plus, kids are very accepting early on. I grew up in a cookie cutter Stepford Suburb and while I was teased and picked on, nowadays when I run into kids I grew up with they say they never really thought twice about the fact that I am hoh.