Hearing Loss:
A chat with Dr. David Vernick

Chief Otolaryngology
Beth Israel Deaconess Hospital


Q: I have OI and can hear very well; does everyone have hearing problems with OI?
A: Not everyone who has trouble hearing has OI. The incidence of hearing loss with OI is much higher than the general population - up to 50 percent.

Q: Does a person's age have anything to do with hearing loss in a person with OI? Should people with OI expect to start losing their hearing in their 20s?
A: The incidence of hearing loss in OI increases with age; fortunately not everyone gets hearing loss.

Q: I've heard hearing loss in people with OI begins in their 20s. Can it start earlier? How early?
A: It can start at any age.

Q: What type of hearing test is best?
A: Hearing tests should be done in a soundproof booth with headphones, that way you can measure hearing and determine if hearing loss is conductive (from the middle ear) or sensorineural. Hearing tests done in open rooms are inaccurate, as the background noise messes up the results.

Q: Are there any costs involved in testing?
A: You would have to ask your local ear, nose and throat doctor what the charges are. They vary a great deal and may be covered by insurance.

Q: Do you know what the cause of sensorineural hearing loss in OI is?
A: No one is sure why OI carries a greater risk of hearing loss. It may be that the bone turnover around the cochlea (the inner ear) releases enzymes into the inner ear that damage it. This is just speculation though.

Q: Do you usually see different types of hearing loss and ages for hearing loss within a family of OI Type I?
A: You can see both conductive and sensorineural hearing loss with all types of OI. They can vary in a family just as much as between unrelated people.

Q: Is there a difference between the ages of onset for either type of hearing loss?
A: Sensorineural hearing loss tends to be more frequent as one ages. The conductive loss usually occurs in the 20s and 30s, but these are generalizations that have many exceptions.

Q: Our 24-year-old son, whose hearing is rapidly deteriorating, has an appointment with an audiologist. Are there any special tests that he should ask for because he has OI?
A: The only tests are routine hearing tests with air and bone conduction and the speech reception threshold.

Q: Hearing tests for our son have been inconclusive. He had hydrocephalus in infancy and had to be shunted; the hydrocephalus was arrested long ago. His ears are positioned more in line with his mouth than his nose. He repeats words heard wrong and constantly asks to have things repeated. Are there any tests that you would advise?
A: There is a special type of hearing test called evoke response audiometry, which can determine the level of hearing, even when the person cannot cooperate. Many times the person has to be sedated to get an accurate test.

Q: Do you know of particular hearing complications related to hydrocephalus?
A: Not that I know of.

Q: Is there any connection with the receptive part of hearing when there is a tracheostomy involved?
A: Not that I know of.

Q: My son had a bone marrow transplant two years ago and is doing well. Is he still at risk for hearing loss?
A: I don't think that anyone has any long-term data on that. I would have him periodically tested just to make sure all is well.

Q: My husband faintly heard our small dog bark the other day, otherwise he is deaf, is there some sort of hope of hearing for him?
A: He should have a hearing test done to determine if he has any hearing; if he heard a dog bark, he may have some hearing that a hearing aid would help.

Q: I'm almost 27 years old, and my hearing gets muffled and then comes back, and at times I have ringing in my ears. Should I be concerned?
A: I would get a hearing test just to check out the hearing level.

Q: A friend of mine, who has OI, is using hearing aids in both ears. When she turns up the hearing aid in one of the ears to hear better, that ear then rings. What is the cause of that? If she doesn't turn the hearing aid up, then she really can't hear out of the ear. What can she do?
A: When hearing aids are turned up too loud, they give feedback, or the high-pitched squeal that you hear. To get around this, she may need a different kind of hearing aid or a better fitting mold. She should see her hearing aid dealer to find out what options she has.

Q: An audiologist told my friend, who has OI, that a hearing aid would not help her, but she does have some hearing. He did not give a reason. Can you speculate why that might be?
A: The main reasons for a hearing aid not working are: poor understanding of sounds in the ear, very poor hearing overall, poor fit of the hearing aid. Your friend should get a second opinion before giving up.

Q: Would you, as a doctor, advise a hearing aid over stapedectomy or cochlear implants to someone with some hearing?
A: If you can use a hearing aid, that is always preferable to try first, as it carries little risk.

Q: My dad has OI and hearing loss. Can an operation help?
A: It depends upon what type of hearing loss he has and how severe it is. He should see an ear, nose and throat doctor and have his hearing checked.

Q: I've been using hearing aids since I was about nine years old, and they are getting expensive, because I no longer have insurance, so I'm exploring my options as far as surgery. Do you have any advice?
A: It depends upon what type of hearing loss you have, whether conductive or sensorineural, whether surgery is even an option. You need a current hearing test to determine that.

Q: I traveled from Cincinnati to Memphis to find a doctor with experience in OI and stapedectomy. I have had both ears successfully operated on. The last surgery, in September 2000, left me dizzy at times, and I have had at least one ear infection since then. Is this normal?
A: The dizziness can occur because the surgery opens up the inner ear, which usually clears with time. I cannot explain the ear infection; you should be no more susceptible to one now than before the surgery.

Q: What are the risks of the stapedectomy?
A: The major risks of stapedectomy are hearing loss, dizziness and change in taste to part of the tongue. The hearing loss can be total loss, the dizziness usually goes away, and the taste problem usually clears in a few months.

Q: I've seen an ear, nose and throat doctor who said the difference between a conductive loss and a sensorineural loss should be at 40 db before a stapedectomy would be able to make a significant improvement. I had a 34 db. Can you explain this?
A: Some doctors will not operate on hearing loss that is conductive until it is great enough to justify the risks of surgery. Your hearing loss is certainly close to that. With newer techniques and prosthesis for reconstructing the hearing bones, that rule is not as strict as it used to be.

Q: Why does a stapedectomy work in some people and not in others?
A: There are many reasons for failure of a stapedectomy operation. Just to list a few: there may be more problems than just with the stapes, the attachment of the prosthesis to the incus may be faulty, the incus may be too thin to hold the prosthesis long term, and damage can occur to the inner ear leading to nerve loss. Success of the surgery is highly dependent on the surgeon and on the person's ear anatomy (both can be highly variable).

Q: My sister had a stapedectomy and could hear for a while. Then one day she woke up and couldn't hear out of that ear. What happened?
A: See previous answer.

Q: I had a stapedectomy about 35 years ago in both ears. The surgery was successful, but now my hearing is declining. Is there anything I can do to prevent further loss? Can I have a second stapedectomy?
A: You need your hearing checked to see if the loss you have is nerve loss or conductive loss. You can have a second stapedectomy if your loss is mainly conductive. Surgery wouldn't help if it were nerve loss.

Q: What is the bone implant, and is it a good idea for persons with OI?
A: There are two types of bone implants. One is a cochlear implant, which is implanted in someone who is deaf. The other is a bone-anchored hearing aid, which is implanted in someone who has a conductive (middle ear) hearing loss. The cochlear implant has been performed in at least two people with OI. The only trouble with the implant, so far, is that because the bone is not as solid or dense as the other bone, the electrical signals seem to travel a little easier through the bone. This means that the facial nerve, which runs near the inner ear, sometimes gets stimulated from the implant. To date, there have been no bone-anchored hearing aids implanted in OI patients that I know of.

Q: What kind of sound would someone gain from the cochlear implant?
A: Hearing with a cochlear implant is variable. It is not like normal speech, but many adapt to it and can even talk on the telephone.

Q: Does a cochlear implant allow an otherwise deaf person to understand the spoken language?
A: Cochlear implants allow some people to understand speech. Not everybody that gets an implant can understand speech though.

Q: How many people with OI had cochlear implants and were they successful?
A: Two people, whom I know of, have had cochlear implants, and both are using the implants. Some of the electrodes had to be turned off, because they stimulated the facial nerve as well. Otherwise, they did fine.

Q: Did turning off some of the electrodes decrease the hearing capacity of the implant?
A: It limited the coding strategies that could be used.

Q: Is the implant major surgery or an outpatient procedure?
A: Cochlear implants are significant ear surgery. However, most of the patients are able to go home that evening or the next morning.

Q: Does a cochlear implant pose more risks for a person who has OI than for the general public? Do the risks increase with age?
A: There are only a couple of implanted people with OI. I'm not sure there is an answer to your question yet.

Q: What are the risks of a cochlear implant for a "senior" person with OI?
A: The risks of a cochlear implant are dizziness and facial nerve injury. The facial nerve is monitored during the surgery, so that should not occur too often. The dizziness can occur because of the surgery on the inner ear, but it usually clears.

Q: Our son has been receiving pamidronate for two years now; is there a bone density minimum to qualify for an implant?
A: There is no bone density measurement that is done to determine if someone is a candidate for cochlear implants.

Q: Have you heard if pamidronate might aggravate hearing problems by thickening the small bones in the ear?
A: There is work at NIH to see if hearing is affected for better or worse by pamidronate.

Q: Is there a way we could get information on the NIH study about pamidronate's effect on hearing?
A: Not until they have analyzed the data.

Q: Can hearing loss be cured or somewhat corrected?
A: Hearing loss can be treated with hearing aids and surgery depending on the type and severity of the loss. There is no preventative to take yet.

Q: My nine-year-old grandson, who has OI, complains that the T.V., radio or someone yelling hurts his ears. Will loud noises "break" the bones in our ears?
A: Only extremely loud noises, such as jet airplanes and bombs, would have enough energy to damage the ossicles in someone with OI. I know only of a few reported incidences like that. Most of the time, the reasons to avoid loud noises are the same for everyone - loud noises cause inner ear damage and can lead to hearing loss.

Q: Can a hearing test determine if my loss is due to OI or loud noises, such as music and gunfire?
A: You cannot tell what the cause of the hearing loss is from a hearing test. Some patterns are more suggestive of noise exposure, but many times multiple causes add together to give the final loss.

Q: Does hearing loss just happen on its own or does noise, head injuries, etc., influence the loss? Can we prevent more loss?
A: All of those things can cause hearing loss. They are additive so that hearing loss can be from many causes in a person. Avoiding head injuries and loud noise can help reduce your chances of hearing loss.

Q: My hearing is much worse in my left ear than in my right. What could be causing this?
A: You need to get a hearing test and an exam of your ears to determine what kind of loss you have and what the cause is.

Q: Our OI Type II, eight-year-old son's doctor has always said his ear canals are too convoluted to detect infection or wax build-up. Would a routine flushing of the ear canals be justified or possibly dangerous?
A: The only time I have seen a problem with actually seeing the eardrum was in congenitally abnormal ear canals, which is not a feature of OI. If the ear canal is very narrow or abnormally shaped, washing out an ear may make matters worse as the water will push the wax in and make it wet.

Q: A pediatrician told us that my 10-month-old grandson has flattened ear canals and a lot of wax. Will this pose any long or short-term problems?
A: It's hard to know if the ear canals are going to be a problem in a 10-month-old. Their canals are tiny anyway and a little wax goes a long way.

Q: I have had doctors tell me the inside of my ears look blue like a Smurf's. What does this mean?
A: I do not know, sorry. Ask them the next time you see them.

Q: The doctor used a utensil to remove some of the wax in my grandson's ear, because he suspected an earache. The utensil seemed particularly painful. Is that common?
A: Cleaning out a little tike's ears can be a challenge in anyone's hands.

Q: When is wax production too much or too dark?
A: Wax has many colors and textures; they all can be normal in different people.

Q: What causes wax to produce?
A: It is a natural product of the ear canal. There are wax glands in the ear canal that make the wax. Wax is there to lubricate the skin and to prevent infections. It usually falls out on its own without the help of q-tips.

Q: At what age should a child with OI start having his hearing checked? How often? Is once a year enough? Can this be done by the pediatrician or by an audiologist?
A: In Massachusetts, all newborns are screened for hearing loss. Most kids are screened every few years for school as well. Other hearing tests should be done if there is any suspicion of hearing loss. An audiologist is the one to give an accurate hearing test. Pediatricians can screen kids, but don't have the equipment to do the accurate testing.

Q: My daughter has OI Type III and has borderline hearing. We have the audiologist test her every year. What else do I need to do?
A: It sounds as if you are doing everything you need to do to check the hearing.

Q: Do we need to seek out specialists for our hearing, or is a good ear, nose and throat doctor satisfactory?
A: A good ear, nose and throat doctor is fine. If you need surgery though, you need someone who is experienced with ear surgery.


This information is brought to you by the
Osteogenesis Imperfecta Foundation.

For more information, contact:

The Osteogenesis Imperfecta Foundation, Inc.
804 West Diamond Ave., Suite 210
Gaithersburg, MD  20878

Phone: (301) 947-0083 or (800) 981-2663
Fax: (301) 947-0456
E-mail: bonelink@oif.org
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