Archive for February, 2010

Two New Important Studies Supporting Cochlear Implants for Young Children and the A-V Approach

Saturday, February 6th, 2010

Research is catching up to what parents and professionals working closely with our children already know from experience, that cochlear implants in combination with Auditory-Verbal therapy provide our children with the greatest chance of leading their lives successfully and happily in the mainstream alongside their peers born with normal hearing.

(Click here for link to article)

MEDIA RELEASE: New data shows deaf children can keep pace entering mainstream school.

Sunday 24th January 2010: As young hearing-impaired graduates from The Shepherd Centre make their final preparations to begin “big” school, new data released today shows they can expect to do just as well in their language and vocabulary development as their mainstream peers.

Preliminary data analysis from The Shepherd Centre shows that the vast majority of hearing impaired children who graduate from the Shepherd Centre to a mainstream school will score in the “normal” range; for vocabulary (79 % of children are in the normal range) and language (71 %of children are in the normal range) as they enter school.

Around 84% of the general population of children will be in the normal range for language and vocabulary skills.

The data is derived from a sample of 41 Shepherd Centre students who were diagnosed through the NSW SWISH newborn hearing screening program and who graduated from The Shepherd Centre’s Early Intervention Auditory-Verbal Therapy program into mainstream schools in 2007, 2008 & 2009.

The similarity between the scores for hearing impaired children and mainstream kids would have been unheard of ten years ago, where just integrating a hearing impaired child into a mainstream school was seen as a major achievement. In many cases children over a decade ago would have had very poor communication skills.

According to Acting Director of the Clinical Program at The Shepherd Centre, Aleisha Davis, the main developments underpinning these results are early diagnosis, improved technology and improved early intervention. Universal newborn screening in NSW was introduced in 2002, this has made a big difference.

“The average age of children with hearing problems joining the Shepherd Centre program is now eight weeks, whereas a decade ago the average age was over two years. Fitting the children early with the latest hearing devices allows them to participate in Auditory-Verbal therapy from a very early age. Early diagnosis, early fitting and early intervention are the keys to success,” said Ms Davis.

The Shepherd Centre was the first fully Auditory-Verbal Therapy Early Intervention Agency in the country starting 40 years ago. Today the centre has five internationally certified Auditory-Verbal therapists, which is 28% of those certified in NSW, 14% of those certified in Australia and approximately 1% of those in the world with this level of professional certification and ongoing mentoring.

“The results of this study show that these children, who have a range of hearing loss from profound to mild are achieving results that were unimaginable just ten years ago. This is a great outcome for parents of children with hearing loss, as well as the children themselves who can go on in life and lead fully normal integrated lives reaching their full potential,” said Ms Davis.

“Cochlear implants have also made a huge difference to the prospects and outcomes of children with severe and profound hearing losses. In many cases now, with early diagnosis and cochlear implants and early Auditory-Verbal therapy through early intervention programmes, their communication skills are excellent and many people would not know they were actually deaf.”

The clinical team at the Shepherd Centre will continue to test, analyse and report on the progress of their graduates. Data on speech production is currently being collected and will be analysed in the near future.

Click Here to download The Shepherd Centre Outcomes Document


(Click here for link to article)

Cochlear implantation enables quality of life equal to normal hearing peers

1. February 2010 01:11

New research gives voice to hearing-impaired children during kids E.N.T. health month

Children who have cochlear implants (CI) rank their quality of life (QOL) equal to their normally hearing (NH) peers, indicates new research in the February 2010 issue of Otolaryngology – Head and Neck Surgery.

A cochlear implant is an electronic device that restores partial hearing to the deaf. It is surgically implanted in the inner ear and activated by a device worn outside the ear. Unlike a hearing aid, it does not make sound louder or clearer. Instead, the device bypasses damaged parts of the auditory system and directly stimulates the hearing nerve, allowing deaf or severely hard of hearing individuals to receive sound. The National Institutes of Health estimate that as many as 59,000 people worldwide have received cochlear implants, with roughly half of those in the pediatric population.

Prior research has indicated that deaf children feel less socially accepted, experience more difficulty in making friends, and demonstrate greater adjustment problems than their hearing peers. The subsequent success of the multi-channel CI devices that improve speech perception and language development led researchers to look beyond speech and language performance to questions of psycho-social behaviors and adjustment.

This cross-sectional study of 88 families with CI children from 16 U.S. states used a generic QOL questionnaire. The group was then divided by age of the child when they filled out the questionnaire: an 8-11-year-old group and a 12-16-year-old group. Both parents and children were asked to fill out the QOL questionnaire, with the parents assessing their child. The study group was then compared to a control group of 1,501 NH children in fourth and eighth grades.

Results of the questionnaire revealed that overall QOL did not differ between CI and NH groups. However, examination of individual subscales revealed that 8-11-year-old CI children rate their QOL with family less positively than their NH peers. Younger CI recipients rated overall QOL more positively than the older 12-16-year-old CI group. However, the authors point out that this could be a reflection of standard adolescent behavior. Overall QOL showed a significant inverse association with age at implantation, and a significant positive correlation with duration of CI use in the 12-16-year-old group.

The authors point out that even though prior studies have assessed QOL in CI children, this study adds additional perspective to the literature, as it combines assessments by the actual CI recipients and parents, and it maps the results in context with NH children. In addition to findings about how CI children rank their QOL, the research reveals that parents proved to be reliable reporters for their children in areas where they could observe and participate.

The authors write, “For profoundly deaf children who regularly use a cochlear implant, feelings about life overall are no better or worse than their hearing peers. These findings indicate that cochlear implantation has a positive effect on certain psycho-social domains.”

Source: American Academy of Otolaryngology — Head and Neck Surgery

Obtaining a Correct Diagnosis and Becoming an Educated Parent

Monday, February 1st, 2010

Amy: Let’s discuss a topic which is tangentially related to Auditory Verbal Therapy– and that is, diagnosis.  Recently on CI circle, the topic of Usher Syndrome came up.  Many parents proclaimed that their children did not have Usher Syndrome, though they did not know exactly why they were deaf.  In the past, I have also heard parents discuss hearing loss, and found that often they had previously believed that their child could hear based on their own “tests” at home, or worse, “testing” that their family doctor performed.

There are two major problems here.  First, we want to disseminate factual information about Usher Syndrome.  Myths abound in the community of those with hearing loss.


-it’s rare

-you can tell if you have it, by early childhood

-typical gene testing done on deaf babies can usually diagnose early

-lots of babies walk late

First, it is not rare in deaf children.  More than 1 in ten are probably afflicted with Usher Syndrome.  Children usually don’t know until teens or adulthood.  There are only 3 ways to diagnose this, and it is not typically tested in the ordinary testing done by geneticists or family doctors who know a child is deaf.  Most kids have been tested for Connexin 26 and 30, had their kidneys tested and had an EKG. They may have had testing for CMV.  But only recently has the (very expensive) gene testing for most Usher Syndrome genes been available; not only that, but there are STILL unaccounted for genes for Usher Syndrome.  So, if you don’t know the cause of your child’s  deafness, you can be sure that Usher Syndrome is still one of the possible causes.

Once you have a child who is walking late, you have an even greater likelihood of falling into the genetic likelihood of your child having Usher Syndrome, if they are deaf.  Average babies walk at around 13 months, with the medical community suggesting concern or testing when a baby is not walking by 18 months of age.  And yet, we still hear of people pooh-poohing parents who are concerned about their 20 month old not walking.  Sure, there are kids whose range of normal is outside of that time window, but this is cause for concern, especially if they know that their child was not premature and has no other serious health problems which would inhibit walking.  There are also confirmed cases of Usher Syndrome in kids who walked within the normal time frames.

The only way to confirm Usher Syndrome is with an ERG test, which can be difficult for children.  Gene testing which is positive can also confirm, and lastly, the newer OCT (Optical Coherence Tomography) tests can confirm, if there are signs on the retina.  Dark adaptation testing can also confirm the need for further testing.

We also want to make sure that parents can identify children with hearing loss early.  Some babies missed their newborn screening, or were born with hearing but had a decline within months or years.  Don’t rely on the guesses of uninformed “professionals”– get a full ABR test to find out.  Don’t believe that it’s just “fluid in their ears” or that “boys talk later.”

Melissa, I believe you have some directly related stories?!?

Melissa: I first became concerned that Rachel could not hear when she was all of a week old when I noticed that she did not startle.  When I began informal testing on my own, I could not get her to respond.  At her first visit with the pediatrician when she was three weeks old, I voiced my concerns.  His response was, “You are an overly anxious new mother who does not understand how newborns react to sound.”  At her two-month visit, I voiced my concerns more forcefully.  He told me that he would show me she hears and banged something behind her head while I was holding her.  He tried a few times, thought he saw her eyes blink once, but agreed to refer her for an ABR.  A few months later, I showed him an article on the importance of early detection and early intervention for babies with hearing loss.  He responded, “I think we caught it in time.”  Awhile later, we left him.  When our son was born, he was the pediatrician on call, but he sent his partner instead.  We knew why, but his partner didn’t.  We mentioned concern about hearing, and he clapped right in front of Adam’s face, and then Adam blinked.  He then said he hears, and I told him he needed to clap behind Adam’s head far enough away that Adam wouldn’t feel air from his clapping.

When Jessica came along, we found out that she was deaf when she was less than 24 hours old.  When our pediatrician in Atlanta saw Jessica at an early infant visit, she noted her arching her back and her overall floppiness.   She proclaimed to me, “She has the most amazing collagen vascular system I’ve seen.”  I looked at her thinking but not saying that that was one of the most off-the-wall diagnoses I’d ever heard and explained to her about vestibular issues.

These stories point out that we, as parents, need to trust our instincts.  No one, including professional experts, knows our children as well as we do, and no one spends as much time with them as we do.  In addition, while medical professionals have to learn about multiple problems and issues, we get to subspecialize in just our children’s issues, and so, if we take the time to educate ourselves, we may actually learn things that the professionals do not know.

Looking at the flip side, we can also use these opportunities to educate the professionals.  My husband’s cousin, who is a pediatrician, told me that, after hearing the story of our path to having Rachel’s deafness confirmed, he always takes parents’ concerns about their children’s hearing seriously and will also observe for hearing during their visits.  When Rachel was a baby and toddler, we had both medical and educational professionals insisting that she would not be able to master spoken English, that she needed to learn sign, and, later, that cochlear implants don’t work.  Several years later, when Rachel was a hearing, speaking teen, I contacted three of these professionals, all of whom remembered us, and told them about Rachel and emailed them links for videos of Rachel so that they could learn how wrong they were and, I hope, counsel future parents differently.

Amy: I think that, in a nutshell, we both think that parents’ suspicions should not be discounted, and if they have a feeling that something isn’t quite right, they should try to get an answer.  It is never wise to simply rely on the advice of one or two professionals.  Read, become educated, insist on appropriate testing.

Being concerned is not just alleviating the mystery.  Late diagnosis of deafness might result in inability to speak and delay in education for years.  Late diagnosis of Usher Syndrome has the potential to result in inability to take advantage of the upcoming therapies which are on the horizon.  Make it your business to ask questions, research, and find answers.

Definitions and Resources:

Hearing Tests:

You need either an ABR or ASSR to determine if your child or newborn can hear. An ABR should be done as soon as possible because older babies and children may need to be sedated. ABR requires quiet stillness in order to be performed.  Click here for additional information.

An ASSR test can distinguish the amount of hearing in the significant loss areas (severe to profoundly deaf) with great accuracy. For me (Amy), my younger son’s ASSR showed no response to 120 db. This not only encouraged my surgeon toward our preference of early implantation, but gave me a “break” regarding the hearing aid trial, which had been going along with little or no results (but a lot of aggravation). I knew that he had no residual hearing.  Click here for more information.

OAE tests are often used in screening, and should be followed up with an ABR test. Anecdotally, I (Amy) scheduled an ABR for my 4th son because my 3rd was deaf. When I arrived, they had accidentally scheduled an OAE test, which he “sort of passed” (I will leave the part out about how my husband stormed around and threatened when they insisted that they didn’t have the appropriate people that could administer the ABR on that day). We waited another 48 hours only to see him completely fail the ABR. We weren’t terribly surprised, but we were somewhat surprised, as we have two hearing sons as well. The point I am making is that the OAE was not sufficient for a child in a family who already had hearing loss. We were trying to bypass the screening and go straight to the findings… but as we have often found, the “professionals” were confused and didn’t realize that we knew what we were doing when we scheduled the ABR test. Suffice it to say, the person who said he has passed the OAE was chagrined and learned a lesson. Whether it was her incompetence or the OAE itself, I will never know. OAEs record the sound produced by the cochlear hair cells, a “byproduct” of the hearing process. It is a cheaper screening tool, but a full ABR is required eventually. ABR tests record evoked electrical responses to sound at the brainstem level.

It’s a complex science, but with either the OAE or the ABR, one will eventually have to have an ABR.

Otochip testing for hearing loss and Usher Syndrome is available here.

Consent and ordering information regarding testing is available here.

Specific Usher Syndrome information is available here.