high frequency hearing loss

[Guest guest]

Anne,

Sorry to hear you are dealing with more issues for Sammy. I was interested in discovering the answer regarding your questions so I went to the old nursing books. From what I understand sensorineural hearing loss occurs with ototoxic drugs. Sensorineural hearing loss involves damage to the inner ear structure and/or auditory nerve. There is a loss in the perception of auditory stimuli, initially of high frequency sounds. This type of loss results in distortion of sound and problems with discrimination. My pediatric nursing book says that although the child hears some of everything going on around him or her, the sounds are distorted, severely affecting discrimination and comprehension. With high frequency loss, speech may be poorly understood when spoken quickly or when background noise is present.

Treatment of sensorineural hearing loss with hearing aids are of less value than with other hearing loss. Using cochlear implants are said to provide some hope for affected children. HTH, Loriann

[Guest guest]

We started Sam on chelation therapy Thursday night. His ferritin is up to 5000 (normal is 10-175 at our lab). Because of the risk of hearing loss from the desferol we did a baseline hearing test. Sam's hearing has last been tested 18 months ago - normal at that time. This time the test shows a high frequency hearing loss and questionable low frequency loss. He will go back to the audiologist Tuesday to look into this further. Needless to say I was shocked. His articulation has been getting worse but his hearing seemed fine to us. I know many of you deal with hearing loss issues. Is high frequency loss correctable? Is this typically how hearing loss presents in mito disease - as a high frequency loss first? I know very little about hearing loss with mito but I'm sure I'll be learning. Anne

[Guest guest]

Dear Anne;

Poor Sammy!!!!!! What a fighter he is .... and he comes by it honestly!

Quite often, with drugs which have ototoxic side effects, the high frequencies

of the sound spectrum are what are most vulnerable. High frequency losses

tend to affect the hearing of the consonant sounds, notably "S" and "Z",

"SH", "F" and "V" and "TH" first. This means that the words heard probably

are missing these sounds, which leads to both a distortion of the words heard

and the consequent effect on speech production.

Lower frequency loss removes the vowel sounds from words ... but vowels usually

are heard for longer during the course of a hearing loss.

Hearing loss is measured in decibels (Db) and NOT in %age. The Decibel scale

is a logarithmic scale so the greater the number of Db loss, the more involved

the loss. You don't mention the loss that the audiologist found. Generally,

a loss of under 30 Db is considered within the range of normal, 30 to 50Db

is a mild loss, 50 to 60/65 Db is a moderate loss, 65 to 80/85 Db is a severe

loss and above 85Db is considered to be severe to profound ... the higher

the number, the greater the loss. A loss of about 120Db would mean that

a person would not hear loud traffic or airplane engine noise .... but would

likely be very aware of the vibration sensation!!!!!

As I recall, Sammy used to use (or still does) sign language for communication.

He will have developed a very visual demeanour, which would have helped

him to appear to be hearing more than what he may otherwise have heard.

Anne ...... IF indeed this is a high frequency loss, this means that it would

be sensory-neural in nature, especially if it has been caused by ototoxic

drugs. Sensori-neural losses are there for keeps, I am afraid ... but Sam

has had the advantage of hearing for a number of years so language patterns

will have been developed normally.

I cannot comment on whether high frequency losses occur first in Mito .....

but that would be my bet as it takes more energy to create a high frequency

sound than it does a low frequency ....... think of your high school physics

and the thicker and finer strings and the longer and shorter strands .....

and the sounds that they produced.

Aminoglycosides are ototoxic for *some forms of Mitochondrial Cytopathies

- a specific genetic variation (Mitochondrial 12S and 16S ribosomal RNA -

mutation of A to G in the 12S rRNA at position 1555)*{Hutchins, Cortopassi

1994}. Aminoglycosides are *some* of the antibiotics which end in "_______mycin".

Ototoxic means that these drugs can cause hearing loss or deafness. ASK YOUR

PHARMACIST AND PRESCRIBING DOCTOR.

I am not aware if this chelation drug, desferol, is an aminoglycoside

...... or conversely, if it is ototoxic specifically to Mito or SOME forms

of Mito as the aminoglycosides are.

Generally, high frequency hearing losses of significant degree are helped

with a hearing aid, which has been adjusted so that the greatest "gain" (increase

of decibel strength or sound energy) is placed in the frequency range where

the loss is greatest. They have done some quite wonderful things with "tailor

making" hearing aids using computerized digital technology.

Cochlear Implants are done to deaf people whose loss is profound ... so I

would doubt that Sammy would be a candidate for a CI at this time. A CI

creates a mechanical form of hearing of its own and is a wonder for people

who have lost all hearing.

Anne .... If I can help any more, just ask.

Jean

Anne K Juhlmann wrote:

We started Sam on chelation therapy Thursday

night. His ferritin is up to 5000 (normal is 10-175 at our lab).

Because of the risk of hearing loss from

the desferol we did a baseline hearing test. Sam's hearing has last been

tested 18 months ago - normal at that time. This time the test shows a

high frequency hearing loss and questionable low frequency loss. He will

go back to the audiologist Tuesday to look into this further.

Needless to say I was shocked. His articulation

has been getting worse but his hearing seemed fine to us.

I know many of you deal with hearing

loss issues. Is high frequency loss correctable? Is this typically how

hearing loss presents in mito disease - as a high frequency loss first?

I know very little about hearing loss with mito but I'm sure I'll be learning.

Anne

Please contact mito-owner with any problems or questions.

[Guest guest]

Treatment of sensorineural hearing loss with hearing aids are of less value than with other hearing loss

While this may be the case, we are currently using hearing aids to treat my daughters sensorineural hearing loss due to her mito. It has helped her especially with localization of sound in the classroom and in areas where there is a lot of noise and many people talking such as a store or church, school, etc. There is a huge difference when she wears them and when she does not. Just FYI.

Holly

[Guest guest]

Hi. I'm sorry you are going through this. My son is completely deaf. He was implanted with a cochlear implant at 7 months of age. The implants are amazing and if done soon after a hearing loss is detected almost no speech quality is lost. I now work at a deaf pre-school where there are several deaf children with implants. We are finding that the children who were implanted earliest (or close to the time they lost their hearing) by age 6 are in regular classes with very little assistance. If you have any questions about deafness, hearing loss or implants please e-mail off list and I would be happy to give you my number.

Sue Ann Bube

President, Indiana Mitochondrial Support Group

Re: high frequency hearing loss

Anne,

Sorry to hear you are dealing with more issues for Sammy. I was interested in discovering the answer regarding your questions so I went to the old nursing books. From what I understand sensorineural hearing loss occurs with ototoxic drugs. Sensorineural hearing loss involves damage to the inner ear structure and/or auditory nerve. There is a loss in the perception of auditory stimuli, initially of high frequency sounds. This type of loss results in distortion of sound and problems with discrimination. My pediatric nursing book says that although the child hears some of everything going on around him or her, the sounds are distorted, severely affecting discrimination and comprehension. With high frequency loss, speech may be poorly understood when spoken quickly or when background noise is present.

Treatment of sensorineural hearing loss with hearing aids are of less value than with other hearing loss. Using cochlear implants are said to provide some hope for affected children. HTH, Loriann Please contact mito-owner with any problems or questions.

[Guest guest]

I am an adult with cochlear implants. Anne knows me. I can also

attest to their ability to keep a child in the hearing world.

Cochlear implantation can be done for a child with severe to

profound hearing loss. Sammy would need undergo extensive CI

testing to see if his needs are this extensive. Certainly, hearing

aids can help and I do think that Sammy might be a candidate for

considering them. Cochlear Implants provide quality of life when

the hearing loss becomes more than hearing aids can help.

For anyone who wishes to learn more about cochlear implants, you can

go to the following sites:

http://www.bionicear.com

http://www.cochlear.com

http://www.medel.com

Also.. I am listowner of a listserv called CI Hear Sue - you should

get on this one. I'm glad (but not surprised) to read that your son

is doing so well with his CI. To join the list, anyone interested

can go to

http://www.groups.yahoo.com/group/cihear

and click on join.

My best wishes go to anyone in dealing with this issue. I'm always

available to provide support if needed.

Alice

justagram@...

> Hi. I'm sorry you are going through this. My son is completely

deaf. He was implanted with a cochlear implant at 7 months of age.

The implants are amazing and if done soon after a hearing loss is

detected almost no speech quality is lost. I now work at a deaf pre-

school where there are several deaf children with implants. We are

finding that the children who were implanted earliest (or close to

the time they lost their hearing) by age 6 are in regular classes

with very little assistance. If you have any questions about

deafness, hearing loss or implants please e-mail off list and I

would be happy to give you my number.

>

> Sue Ann Bube

> President, Indiana Mitochondrial Support Group

Service.

[Guest guest]

Holly;

I agree with you!

Actually, I believe the nursing text book may have this treatment turned

around somewhat. Use of hearing aids, which have been customized to the

loss as seen on an audiogram, is the STANDARD treatment for

mild, moderate and severe losses which are SENSORI-NEURAL in nature. Hearing

aids are also useful for SOME patients with profound sensori-neural losses,

although many otolaryngologists are opting for Cochlear Implants now in cases

where little cochlear function is happening.

Traditional in-the-ear type hearing aids are not as widely used to rectify

CONDUCTIVE losses (losses happening in either the outer ear or the middle

ear). Generally, although not always, conductive losses are treated with

surgeries such as insertion of tubes in the eustachian tube (myringotomy)

or ear drum repair, because conductive losses are generally accessible without

causing subsequent neurological damage. Generally if a hearing aid is used

for a conductive loss, the aid stimulates BONE CONDUCTION with the use of

a kind of earphone like device with a pad which sits on the bone behind the

ear.

Surgery which enters the area of the inner ear is generally done when

the cochlea and/or the the auditory nerve has irreparable damage since Cochlear

Implants REPLACES the normal cochlear function!

Cochlear Implantation has provided the miracle of "hearing" again to many

people with profound loss. Cochlear Implantation uses mechanical stimulation

to create an "electronic sound". People using CIs have to relearn how to

use this electronic sound and interpret it. I agree with Sue Ann Bube when

she says that children fitted with CI do well and learn to interpret the

sound well. Many adults do well with their CI also.

Some years ago, I had a discussion with an adult, deafened by Mito, who was

facing a CI implantation. I told her that I felt that the results would

likely be very beneficial if the damage was in the cochlea ...... BUT that

I would have a concern that the Mito may instead have done its damage in

the "Hearing Area" of the brain and, if that was the case, the CI may eventually

overdrive the Mito energy production in the cells of that area of the brain

and the CI would cease to be beneficial. Unfortunately, this likely was

the case ... and the adult now has a CI implanted in her other ear.

In my humble opinion, Cochlear Implants are a gift to the world of hearing

loss BUT its use with Mito patients carries with it the possible potential

of further harm because it would be difficult to determine where the Mito

cellular damage is located. My opinion is purely a para-medical one which

comes from a HEARING adult with Mito who has had lifelong experience with

deafness.

Jean Shepherd, adult with Complexes I, III and IV

Teacher of the Deaf

Child of a profoundly deaf and likely Mito mom, who uses a hearing aid

Sibling of a profoundly deaf and likely Mito brother, who uses a hearing

aid.

Hollyorn@... wrote:

In a message dated 10/25/2003 10:59:23 PM US Eastern Standard Time,

LMelby1000@... writes:

Treatment

of sensorineural hearing loss with hearing aids are of less value than with

other hearing loss

While this may be the case, we are currently using hearing aids to treat

my daughters sensorineural hearing loss due to her mito. It has helped her

especially with localization of sound in the classroom and in areas where

there is a lot of noise and many people talking such as a store or church,

school, etc. There is a huge difference when she wears them and when she

does not. Just FYI.

Holly

Please contact mito-owner with any problems or questions.

[Guest guest]

Thank you everyone for your helpful knowledge and advice about hearing loss. It all is very new to me. Jean - I think that the high frequency was 60 and the low more ambiguous at 35? He was due to be re-tested on Tuesday but unsure if he will be now because his ear is bleeding. His ears had been examined pre hearing test and looked fine and tympanogram was normal so we don't think that interfered with his test but who knows. He has a severe bleeding disorder and bleeds everywhere spontaneously - the last few days it has been ears, GI tract, and kidneys so my guess it the bleeding is not related to the hearing but we'll see. Sam has actually never had any aminoglycosides or any other ototoxic drugs. Our doctor has been very careful about never putting Sam on aminoglycosides. The desferol is (in some cases) ototoxic but Sam had not yet received a dose at the time his hearing was tested on Friday. This was a "baseline" exam before we started the therapy. Unfortunately - I can not choose to not give Sam the desferol. He is very iron overloaded and without the desferol he has only a 12-25% chance of living much longer. His heart is already in failure and the iron is exacerbating that. Because he is transfusion dependent , he will continue to accumulate iron which will cause death from heart failure if we can not get some of it out of his body. The nurse that is in charge of the chelation program at the Children's Hospital told me that in 20 years they have not had one case of hearing loss from the desferol now that the medical community understands what doses to use. The reports of hearing loss are all from 20+ yrs ago when physicians prescribed larger doses. So that makes me feel a bit better. Thank you again for all of your experience sharing. It helps to know that others are dealing with this successfully. Sammy did sign when he was a toddler but learned tot alk when he was 3 and has not signed since then. He seems to hear us ok but does have progressive issues with poor articulation (exactly the sounds you mentioned Jean or Lorianne) and he also has slower response time when we do talk to him. I will let you all know if we are able to go on Tuesday and what they say. ThanksAnne

[Guest guest]

Anne;

A thought with regard to the ototoxicity. .... Is it possible that the iron

overload has caused the hearing loss? It is toxic to so many organs ....

could it, in itself, be mito-toxic?

Under normal conditions, bleeding from the ears is indicative of rupture

of the tympanic membrane (eardrum) ... so it sounds as if, with a normal

tympanogram, the audiogram would be reliable, assuming that Sammy understood

the testing and was cooperative (I doubt that that was the issue!!!! <grin>)

You might ask the audiologist if there could be a possibility of the actual

testing causing the rupture, since Sammy's body is so very fragile.

It is not uncommon for a mild (35Db) loss at the low end frequencies to be

ambiguous, because, in a sensitive individual, there might be vibration pick

up. A 60 Db loss in the high frequencies falls into the moderate loss range

...... so I wouldn't be thinking of using Cochlear Implantation for this degree

of loss. Apart from the fact that his loss isn't "enough", CI are done using

general anaesthesia ..... which I doubt you are going to be interested in

when a hearing aid would be helpful!! I would be asking about the possibility

that the sound energy force enough to remedy a 60 Db loss coming down the

ear canal may cause bleeding .... and they may look at you as if you are

crazy!!! Heheheh ..... aren't all Mito parents a little crazy, EH??????

As for the use of the drug for chelation ....... sometimes we have to do

what we have to do ..... and Sam's life is important to all of us. Ototoxicity

is the lesser of the two evils.

Anne, I think of you often ..... and I remember when Sam was born! Can it

really be so long ago? You inspire many moms on this list .... and your

answerts are always so wise. My thoughts here are based upon my gut hunches

..... and I know you would rather hear them than have me say nothing about

them.

On Tuesday, my heart will be with you, my friend

Jean

Anne K Juhlmann wrote:

Thank you everyone for your helpful knowledge

and advice about hearing loss. It all is very new to me.

Jean - I think that the high frequency

was 60 and the low more ambiguous at 35? He was due to be re-tested on

Tuesday but unsure if he will be now because his ear is bleeding. His

ears had been examined pre hearing test and looked fine and tympanogram

was normal so we don't think that interfered with his test but who knows.

He has a severe bleeding disorder and bleeds everywhere spontaneously

- the last few days it has been ears, GI tract, and kidneys so my guess

it the bleeding is not related to the hearing but we'll see.

Sam has actually never had any aminoglycosides

or any other ototoxic drugs. Our doctor has been very careful about never

putting Sam on aminoglycosides.

The desferol is (in some cases) ototoxic

but Sam had not yet received a dose at the time his hearing was tested

on Friday. This was a "baseline" exam before we started the therapy.

Unfortunately - I can not choose to

not give Sam the desferol. He is very iron overloaded and without the

desferol he has only a 12-25% chance of living much longer. His heart

is already in failure and the iron is exacerbating that. Because he is

transfusion dependent , he will continue to accumulate iron which will

cause death from heart failure if we can not get some of it out of his

body. The nurse that is in charge of the chelation program at the Children's

Hospital told me that in 20 years they have not had one case of hearing

loss from the desferol now that the medical community understands what

doses to use. The reports of hearing loss are all from 20+ yrs ago when

physicians prescribed larger doses. So that makes me feel a bit better.

Thank you again for all of your experience

sharing. It helps to know that others are dealing with this successfully.

Sammy did sign when he was a toddler but learned tot alk when he was 3

and has not signed since then. He seems to hear us ok but does have progressive

issues with poor articulation (exactly the sounds you mentioned Jean or

Lorianne) and he also has slower response time when we do talk to him.

I will let you all know if we are able

to go on Tuesday and what they say.

Thanks

Anne

Please contact mito-owner with any problems or questions.

[Guest guest]

Jean, From what I've read about iron overload it is the liver, heart and endocrine glands that get affected....but that is not to say that the ears may not also. I just don't know. Sam was cooperative only when I bribed him with a present from the gift shop - after that he was perfect! Interesting question about the exam causing bleeding. The audiologist was very inexperienced and kept saying things like "I don't want to damage his ear but this test - he's not responding>". I am going to have one of the experienced audiologists do the next round of testing and I will ask the question. It does seem odd that he started bleeding from the left ear that night - though bleeding is certainly not new to him so who knows. He is getting old - 6 years old now! Thank you for your help. Will keep you all updated. Anne Re: high frequency hearing lossAnne;A thought with regard to the ototoxicity. .... Is it possible that the iron overload has caused the hearing loss? It is toxic to so many organs .... could it, in itself, be mito-toxic?assuming that Sammy understood the testing and was cooperative (I doubt that that was the issue!!!! <grin>)You might ask the audiologist if there could be a possibility of the actual testing causing the rupture, since Sammy's body is so very fragile.Anne, I think of you often ..... and I remember when Sam was born! Can it really be so long ago? Jean

[Guest guest]

Anne;

I will add my comments in red.

Jean

Anne K Juhlmann wrote:

Jean,

From what I've read about iron overload

it is the liver, heart and endocrine glands that get affected....but that

is not to say that the ears may not also. I just don't know.

Reading through

the list of areas that iron overload affects ... heart, liver and endocrine

glands ... these are all areas which are relatively high energy consumers.

Specifically ... does iron overload cause mitochondrial function changes?

Is it possible that, in reality, what is being damaged are the mitochondria

and no one has thought to explore that possibility. Certainly the metabolism,

or lack thereof, of iron could be considered to be a metabolic function!

Has Dr Cohen been consulted from afar by your doctors?

Sam was cooperative only when I bribed

him with a present from the gift shop - after that he was perfect!

Aha .....

works like a charm .... nothing like an intelligent bribery to achieve cooperation!

AND .... nothing like an intelligent child to achieve bribery!!!! Hahahah

Interesting question about the exam causing

bleeding. The audiologist was very inexperienced and kept saying things

like "I don't want to damage his ear but this test - he's not responding>". I

am going to have one of the experienced audiologists do the next round

of testing and I will ask the question. It does seem odd that he started

bleeding from the left ear that night - though bleeding is certainly not

new to him so who knows.

Audiologists

do tympanograms prior to testing for a reason! IF there is fluid build-up

in the middle ear, a tympanogram can be painful because the tympanic membrane

cannot move as it should. With fragile tissue such as Sammy's, I would think

it isn't entirely impossible for a tympanogram to actually cause rupture

and consequent bleeding, especially IF Sam had some pre-existing fluid in

the middle ear cavity. Has the doctor looked in his ears since the test?

I think that asking for a more experienced audiologist makes sense, Anne!

He is getting old - 6 years old now! Thank

you for your help. Will keep you all updated.

WOW!!!!!

Hard to believe it has been that long, my friend! Grandson, Cole just turned

4 .... and he has a baby brother, who will be 2 in March! Talking

of grandbabies (the pride and joy of all grannies) ... our oldest one just

graduated in June!!!! Can hardly believe it! Glad to be of help .... and

sometimes it can be helpful to ask the brainstorming questions out loud ...

not only for you, but for others on list.

Jean

Re: high frequency hearing loss

Anne;

A thought with regard to the ototoxicity. .... Is it possible that the

iron overload has caused the hearing loss? It is toxic to so many organs

..... could it, in itself, be mito-toxic?

assuming that Sammy understood the testing and was cooperative (I doubt

that that was the issue!!!! <grin>)

You might ask the audiologist if there could be a possibility of the

actual testing causing the rupture, since Sammy's body is so very fragile.

Anne, I think of you often ..... and I remember when Sam was born! Can

it really be so long ago?

Jean