Hello and welcome to the Osteogenesis
Imperfecta Foundation’s Monthly Podcast. My name is Tracy Hart and I am the CEO
of the Osteogenesis Imperfecta Foundation. Each month, the OI Foundation
brings you information about the diagnosis and treatment of OI
presented by an expert in the field of osteogenesis imperfecta and rare bone
Disease. The podcasts are a part of the ongoing educational effort of the newly
formed Brittle Bone Disorders Consortium, part of the National Institutes of
Health’s Rare Diseases Clinical Research Network. The professional education
activities of the Brittle Bone Disorders Consortium are led by the OIF. Our
podcast today will focus on Hearing Loss in Patients with Osteogenesis
Imperfecta and we’re so excited today to have with us Dr. David Vernick. Dr.
Vernick is associated with Harvard Medical School in Boston he is a surgeon
in Otolaryngology at Massachusetts Eye and Ear Infirmary, Beth Israel Deaconess
Medical Center, Brigham and Women’s Hospital, and Children’s Hospital in
Boston, MA. Dr. Vernick is also a member of our
Medical Advisory Council. So Dr. Vernick thank you so much for being with us
today. It’s a pleasure, thank you for asking me.
Great. We’re going to go right into our first question; can you tell us how
common it is for people with OI to have hearing loss and why does this occur in
a disorder that’s commonly thought of as a brittle bone disease?
Well the incidence of hearing loss in OI increases with age. By 50
years of age pretty much 50% or so of everyone who has OI has a significant
hearing loss that is handicapping to them. The incidence increases with age
but it can start as early as birth so that children, teenagers, and young adults
need to be cognizant of the fact that they can have hearing loss too and they
May need seek intervention sooner than many of their peers would
need to seek intervention for help for hearing loss.
Now in your experience do you see hearing loss is more
prevalent in people with different types of OI? I think that most of the
work is done certainly on the most common type of OI, which is type 1 OI
and that the numbers that I’m giving you are for Type 1 OI but the other
types, the numbers are much smaller, but as the incidence is at least that great. The progression as well is at least that
specific. I missed part of your first question so let me go back a minute
before we move on and that was why does this disorder, commonly thought to be
brittle bone disease, cause hearing loss? The answer to that really is a basic
question about how our inner ear works. Our inner ear is an environment which is
surrounded and shielded by bone and inside are the hearing and the balance
organs and they are held in a very specific environment that nourishes them
and keeps them healthy. The problem with OI is that the
environment doesn’t always stay as healthy as it should. The bone turnover,
the changes in the bone, the changes in the bone texture, all affect the
environment that the inner ear is living in and with a less healthy environment
the organ doesn’t do as well and that’s thought to be the cause of most of the
hearing loss that progresses with OI That’s interesting. I know you touched on
this just a minute ago but in your experience when do people really start
noticing that they have hearing loss related to their OI and if they if
they start to experience that what should they do, who should they go
to see? Should they go to their primary care doctor and say this is what I’m
experiencing and then what should they do? Okay let me start at the very
Beginning. In most states now, and I’m not sure
that it’s all 50 states, but in almost all states now we do newborn
screening and so that’s very critical in picking up whether you have OI
whether you don’t have OI whether a child has significant hearing loss or
not and so I think that process for screening for hearing loss begins at
that point for everyone and children and adults who have OI and have all the
other causes of hearing loss as well in addition to those which are caused by OI
and so typically if they have hearing loss at a newborn stage that should be
picked up before they’ve had significant delays in speech and language
development the kind of the next step that usually happens is over the first
couple of years with speech and language development if it is delayed then those
children should be seen by an otolaryngologist
and an audiologist to have their ears checked to make sure they look fine and
they don’t have infections or fluid going on in their ears and they should
be screened with a hearing test to make sure that they still have hearing in the
normal range. It’s not as easy to do a hearing test on a one year old or a two
year old as it is on somebody who is a teenager or an adult but you can still
there are still methods of testing hearing even on newborns and one and
two-year-olds before their responses can be accurate there are ways using what
are called auditory evoke potentials and otoacoustic emissions which are things
that the ear can do with stimulation that we can record electrically and
don’t require the child to give a feedback so we can get a pretty good
assessment of what a child’s hearing is even before they can tell us that
they’re having troubles. As the children get older the recommendation has been to
have the hearing tested every three years to make sure they’re not
developing any slowly progressive hearing loss that
might be interfering with speech and language development or with their
schoolwork or learning in their day-to-day settings. Many schools have a
program where they do screen kids for hearing it’s important to check with the
school where your child goes to make sure that that’s being done and that
your child is passing those tests there are also some simple things that
you can do at home to see if the child is turning up the TV too loud not
responding appropriately to questions where the response should be positive
and it isn’t those kind of things should alert a parent that their child
needs to be screened. And then as we get older just increasing troubles with
communication I think warrant a continued hearing
screening periodically and again every three to four years is probably
reasonable if you’re not having any troubles until such time as you either
are doing great and you never need it or you’re starting to develop some hearing
loss and then the screening of the hearing loss is present probably should
be once a year. Okay I know for those Listening, Dr. Vernick is a regular
participant at our conferences and meetings and I know Dr. Vernick you get a
lot of questions from adults that are experiencing hearing loss and are ready
for some sort of intervention. What do you tell them? What are
their choices or what can people look to help them with their
hearing loss? Okay the types of interventions that are available really
depend upon the type of hearing loss that the person has. We generally take
hearing loss and divide it into two main categories the first category is a
problem with the ear itself, the ear drum, the little bones of hearing and the
second category is the nerve of hearing the inner ear function and
the nerve of hearing back to the brain the first part with the little bones of
hearing where sound is conducted through the ear canal the eardrum the little
bones of hearing into the middle ear is called a conductive hearing loss and the
second kind which is involves the inner ear and the nerve of hearing is called a
sensory neural hearing loss there are things we can do to help both of those
kinds of hearing loss depending upon how severe they are the easiest thing to do
and the simplest thing to do which is non-interventional if the if the hearing
loss is mild from either kind is to simply be aware that that’s what’s going
on and to try and improve the listening environment so if someone is sitting up
front in a classroom that has a mild hearing loss they can usually do much
better than if they’re sitting in the Back, carrying on conversations in
quieter areas helps a lot, paying attention to people’s lips to pick up
additional clues helps. so those things will help with mild hearing loss of
either type if the loss gets to the point where that’s not adequate then the
next intervention to consider if it is a conductive hearing loss which is a
problem with the eardrum and the little bones of hearing there are really two
things that you can do one is considered an operation where you can go in and try
and fix whatever the problem is and the other is hearing aids. it used to be said
in the in the old days that hearing aids didn’t work for sensory neural hearing
loss but that’s just not the case in fact most people who wear hearing aids
now are being treated for sensory neural hearing loss not the conductive hearing
loss so that is certainly an option when kids are under age 10 or 12 they can
have troubles with fluid in their ears or recurrent infections and tubes can
help them sometimes to drain off the fluid or help stop the ear infections
and that may be all that’s needed for intervention if they have troubles with
the little bones of hearing then surgery on those is really not
a good idea until they get to be a teenager or young adult because the
results are just not as good when they’re little kids the area hasn’t
fully grown at that point and the success rate is significantly lower so
most children who have hearing loss whether it’s conductive or sensory
neural if it’s not something simple like fluid in their ear the best option is to
get them hearing aids to boost up their Hearing, make sure there’s their teachers
know that they’re having hearing problems and make sure they’re sitting
up front in the classroom so they don’t have all the competing background noise
around them when they’re trying to get their lessons. As people get older if
they have a conductive hearing loss the bones that you can repair in somebody
who has OI or not as good and not as sturdy building blocks as somebody
who doesn’t have OI but there are still operations to replace those little bones
where you can put in artificial prosthesis to try and replace the bones
the most common one is the littlest bone the stapes bone and it in
OI that bone can be either malformed or bony changes can be around
the base of it and prevent it from vibrating normally there’s also problems
at times with head injuries or even loud noises where the little bones can break
in the middle ear and surgery can many times fix those that’s not always
possible and those operations are not as successful as they are in people who
don’t have OI but it’s certainly something to look into and consider if
that’s not a consideration or if the loss is sensory neural then the answer
is to get properly fitted hearing aids as we get older it’s usually sensory
neural hearing loss that becomes a major issue and for those people hearing aids
help a great deal and for some people as they get older and the hearing loss
becomes much more severe so that hearing aids are not an effective means of
treating their hearing loss there is a device called a cochlear implant
that’s a surgical procedure where you implant a little fine electrode array or
a little fine tubing with wire in it into the inner ear directly it is placed
underneath the skin behind the ear and then there is an external processor like
a hearing aid that is attached to it with a magnet that stimulates the nerve
directly and bypasses the whole ear, ear Canal, ear drum, and inner ear issues so
it directly stimulates the nerve of hearing to give you hearing back. That
has been successful in kids and adults it has been done in patients who have OI
and is very successful in them as well the only consideration that is a little
bit different with OI is that the bone is not as dense so that these implants
work by triggering little electrical impulses and normally the bone around
the ear will shield those impulses and act as a barrier so they don’t spread
too far and since the OI bone isn’t as dense or as favorable to shielding those
sounds you sometimes have to turn the current down a little bit lower than
you might for someone else but you can still do a very good stimulation of
the inner ear to give back hearing in those patients. Is the cochlear
implant probably the most aggressive type of correction for hearing loss in
your opinion? Or what’s on the horizon for new interventions?
Well cochlear implants I think are probably the
fastest improving technology that we have for hearing right now. I don’t know
that I would call them aggressive because as long as you follow the
criteria which are if you can’t get anything effective with a hearing aid
then you either deaf or you have a cochlear implant to try and get some
hearing back and although they aren’t as good as normal hearing and not as good
as you can get with a hearing aid they are still far better than being deaf so
the criteria for fitting them I think is justified by the results that we get
from them so that somebody who can benefit from a hearing aid isn’t offered
a cochlear implant they’re not a candidate for that they’re only a
candidate for a cochlear implant if they fail hearing aid use or in kids
if they’re born deaf. Okay and how long is that surgery? What is that surgery
like for a cochlear implant? Is somebody put under anesthesia?
How long does that take? The surgery is actually pretty
straightforward even though it sounds like a fairly complex procedure. It’s a
general anesthesia where you are put to sleep there is an incision made behind
the ear there is some bone taken away out of the bone behind the ear called
the mastoid there is an opening made into the inner ear and the electrode
inserted into the inner ear and secured in place so the surgery takes somewhere
around two to three hours it can be done either as a day surgery so that people
come in and have the surgery and go home the same day or overnight stay so that
some people come in and have it done and stay overnight and go home the next
morning the area is then allowed to heal up so the person has some stitches
behind their ear that come out in a week A dressing over their ear which comes
off in a week and then they can pretty much do their
normal activities for another three to four weeks and usually at about a month
they will meet with the audiologist, have the external device fitted and adjusted
and start the process of learning how to use a cochlear implant. There are
some complications with the surgery that can occur people can have some dizziness
afterwards which clears up. People can have rarely but can have an injury to
their nerve that supplies motion to their face called the facial nerve and
people very often will lose any residual hearing that they have in their ear at
this point so that’s why you don’t do it in somebody who has good residual
hearing or can use a hearing aid they are working on some cochlear implants
that are called hybrid implants where they are done in such a way to try and
preserve any leftover hearing that’s in the ear but preserving
that hearing is not a guaranteed thing so they’re usually not done they’re done
with the hopes of preserving the hearing but they’re not done in patients who
that’s critical for them to be able to do that at least not yet. So one of the
goals of our medical education initiative here for professional
education is to provide consultation with experts in the field
so what would your recommendation be Dr. Vernick if you you know person with OI
went into their primary care provider and said I’m having hearing loss
this just isn’t right I’m 43 years old I have OI what should that
primary care provider do at that point in your opinion? How should they advise
Them? Well I think the first thing is to take a look and make sure that their
ears look okay that there’s not wax that’s blocking up their ears it’s not fluid
that’s blocking up their ear and giving them the hearing loss because people
with OI can have all the regular hearing loss problems that everybody
else does if the ear looks perfectly healthy and
normal or both ears look perfectly healthy and normal since we usually have
two then they need to get a hearing test and I think referring them to an
audiologist makes a lot of sense. Some of the the primary care offices
use these little screening devices where they play some tones in the ear to see
if the person can hear them or not to try and get a generalized level of
hearing and that’s okay but I don’t think that’s appropriate for people that
have OI because they can have fairly significant hearing loss in some
frequencies and not all frequencies so they may not that may not be a really
good screen for them I think anybody with the history of OI oughta go
directly from a normal exam to getting a hearing test. Okay okay great
you mentioned hearing aids you were talking a little bit about hearing aids
are there who fits hearing aids for people? Would be the audiologist or who
if somebody needs hearing aids are there different types? How do they find the
right one for them? Well I think the key to getting the right hearing aids
has to do with first getting a good hearing test so you know exactly what
kind of hearing loss you have and how severe it is there are a lot of
different companies which make hearing aids and there are lots of people that
dispense hearing aids there are audiologists who are trained
to do that who have skill in dispensing and fitting hearing aids and that’s who
I would recommend that people go to there are stores that sell hearing aids
there are internet sites that sell hearing aids there are sales people who
go around selling hearing aids to people and I would caution against that because
you’re getting somebody who’s been trained or not trained very well to sell
hearing aids not to fit them properly and I think it’s really important for a
person who has OI everyone in general that
they’re fitting the proper and be adjusted because you can take some to
people who have the exact same hearing level and they may need totally
different settings for their hearing aids depending upon what their listening
environment is it’s not like glasses where you can go in do your eye exam no
matter where it is by your pair of glasses stick it on and walk out the
door and you’re fine. To fit somebody properly with hearing aids and get them
to get the most out of them usually it requires two or three sometimes more
visits back into the audiologist to get the hearing aids adjusted once they’ve
been set at a reasonable level given the hearing loss and those those kind of
things depend a lot on again what the listening environment is and what the
requirements are of that person there are lots of bells and whistles
that can go on with hearing aids including directional microphones, the
ability to filter out background noise, Bluetooth connection to TVs and phones
so there are a lot of different possibilities that you can use to
improve your ability to use a hearing aid that the general salesman is not
going to be able to help you with and so I recommend that everyone go in to see a
trained audiologist for proper fitting. Excellent thank you well we have a few
minutes left and would love for you to share anything we haven’t talked
about you think it’s really important for people to to know or some last
comments that you have? Right well I think a few things – I think
it’s important that people be more assertive I shouldn’t say aggressive but
more assertive about checking hearing in people that have OI and in the
general population especially in the children growing up we tend to think a
lot of times our kids don’t pay attention to us and they may not but
it’s important to give them the benefit Of the doubt and make sure that it’s not because
they don’t hear us or they’re not doing as well in school as they should because
they can’t hear the teacher I think that’s really a good take-home message
and I think it’s important as well to look at some very recent data
that has come out that shows that not hearing well as we age gives you a
greater chance of developing earlier dementia that it’s important for us to
continue to interact socially with other people and that requires hearing that
it’s important to do everything we can to maintain that connection
and that whether it includes hearing aids or surgery it’s just important
people that don’t hear tend to isolate themselves from the general population
and that leads to cognitive decline and so I think one of the other take-home
messages is if it’s a problem that you can fix and many times hearing loss is a
problem that you can fix or at least help that you need to do everything you
can to do that and keep people Interactive. Excellent excellent very
good advice well Dr. Vernick thank you so much for for joining us you can find
out more information about the medical education initiative that we have going
on at the OIF by visiting our website at www.oif.org Dr. Vernick is a member of our Medical
Advisory Council and so his information is on our website as well so Dr.
Vernick’s thank you so much for this really valuable information. It was a
pleasure thank you for having me
Very informative. Answered some questions that I had.