Down Syndrome : Hearing Impairment and early preparation and intervention.

1 in 800 children in the world has down syndrome.

80% of children with Down Syndrome suffers from hearing impairment. It can be caused by middle ear (conductive loss) or inner ear issues (sensorineural hearing loss).  A lot of precautions needed to be aware about when it comes to preparing a child with Down Syndrome as early intervention is the key.

As a pregnant woman i am right now, i too am very anxious when it comes to what to expect of the little one that i am carrying. But being an audiologist, i have been exposed to the importance of early intervention hence i have the courage to visit the sonographer and since i am an eager fan of prenatal screening. Nowadays, preparation for mothers to be are amazing and you can know as early as 10 weeks of pregnancy if you are carrying a child with down syndrome. It is imperative that us parents to check our little ones if they need early intervention or extra early care in advance.

As statistics and studies has further agreed that children with down syndrome also will go through a degree of mental retardation from mild to severe which further enhances the fact that these children needs early intervention. 

When they are born, precautions on how they are fed is important to avoid middle ear issues such as their middle ear tube ie: eustachian tube can be dysfuntional easily as they have a flatter tubing compared to us adults due to exposure to adult smoking or feeding milk while they are laid flat etc.

Our test battery to assists parents in understanding their childs status of hearing would be :

1- Hearing screening

- Otoscopic Examination

- Tympanometry

- Automated Auditory Brainstem Response (AABR) with inserts (to avoid collapsed ear canal)

- Otoacoustic Emissions (OAE)

Then, if found to have hearing impairment, further diagnostic assessment will be done.

2- Diagnostic Hearing Assessment

- Otoscopic Examination

- Tympanometry

- Diagnostic Auditory Brainstem Response (ABR) with inserts  (to avoid collapsed ear canal)

- oacoustic Emissions (OAE)

At birth, you can see their beautiful features :

• flat facial features
• small head and ears
• short neck
• bulging tongue
• eyes that slant upward  
• atypically shaped ears   
• poor muscle tone

Having said that, so many successful real life stories of these individuals i have encountered. My own daughter had an assistant kindergarten teacher with down syndrome, cousins and friends who are attending school because they had an amazing exposure and early intervention when they had the window of opportunity (0-5yo) for early intervention.

In Malaysia, we have Kiwanis and Persatuan Sindrom Down to name a few to support parents and children with Down  Syndrome. The support available is just incredible and as i have said before, children with down syndrome can have a bright bright future with early intervention.

Before i end this piece, here is an amazing story about this "Johns Crazy Socks". John’s Crazy Socks is a father-son venture inspired by John Lee Cronin, a young man with Down syndrome, and his love of colorful and fun socks, or what he calls his “crazy socks.” Crazy or not, to me its amazing and i wish for more successful stories from our community in Malaysia. 

The world is our oyster, if we know our strength..

As a parent, " their world is their oyster, as long as we know in advance their strength".

To parents out there, family, friends with down syndrom children, may the force be with you and do share with us your success stories so more parents in our community are empowered!

Love,

Dr Shasa Aziz

CEO and Founder of Eartistic Sdn Bhd

References:

http://www.puravidasanantonio.com/kids-chiropractor/childhood-ear-infections/

http://www.bbc.com/news/business-42353259 

https://www.healthline.com/health/down-syndrome#symptoms

Catch our promotion this month.
Free Hearing Assessment for anyone Down Syndrome  from the 26th -31st of March 2016.
Call us to book your appointment within this week or book an appt on our website.

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Unnoticed Hearing Loss in Children

Children learn many thing s via interaction with their environment. They use their senses to gain input from the environment mostly while  playing. One of the senses, which are crucial for a child’s development is hearing.

Nowadays , it is common procedure for hospital and hearing care centre to do Newborn Hearing Screening, thus, giving an early idea of a newborn  hearing status .

Parents  and caretakers are advised to  to monitor a child development according to the guidelines available,such as  Paediatric  Protocols  for Malaysian Hospitals, 3rd Edition. Below are the excerpts of the guideline ;-

 

 

Despite undivided attention to the needs and care of children, there  might be some  situations where hearing loss in children might go unnoticed  by the parents:

1.     Unilateral hearing loss:

Only one ear is having hearing problem and the other ear is normal hearing. A child will have   have  normal speech and language skills  however there might trouble  with sound localization. 

2.     Mild hearing loss :

Mild hearing loss is in between 25 to 40 dBHL either in one or both ears. The hearing might be adequate for communication but there are  difficulties with soft sounds or  understanding speech when in noisy environment .

3.     Progressive or gradual hearing loss :

 Although a child passed hearing screening during infancy (Newborn Hearing Screening) , there are cases of hearing loss that starts and gets progressively worse after infantile stage.

4.     Partial  hearing  loss: 

Usually  this concerns high frequency loss where speech clarity is diminished while the loudness of speech is still  normal. “ I can hear people talking, i just cant understand them .”

Seek a hearing professional if you are suspicious about a childs hearing status. Parents’  instinct and suspicion should NEVER be taken lightly as a reason for an assessment as they are more aware of their child ‘s progress and development. Hearing impairment will  lead to issues in a child’s social development and may lead to further psychosocial problems  as they grow up.

Early assesment leads to early intervention and early intervention may lead to  better outcome.

Written by,

Faiz Salleh

Eartistic Hearing and Balance Centre, Audiologist at Kota Bharu

References:
Lang-Roth R (2014) ,  Hearing impairment and language delay in infants: Diagnostics and genetics , GMS Curr Top Otorhinolaryngol Head Neck Surg.

J H hedges et al  (2013) , Play, attention, and learning: How do play and timing shape the development of attention and influence classroom learning?   Ann N Y Acad Sci.

S C P M theunissen et al (2015), Symptoms of Psychopathology in Hearing-Impaired Children , Ear Hear.

A M Korver et al ( 2010) , Newborn hearing screening vs later hearing screening and developmental outcomes in children with permanent childhood hearing impairment., JAMA.

Paediatric Protocols for Malaysian Hospitals , 3rd Edition.

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“CHICKEN RUN IN A MARATHON”

Cluck! Cluck! Cluck! Cluck! Cluck!

Listen, I heard that sound. It’s a clucking sound.

Cluck! Cluck! Cluck! Cluck! Cluck!

Uh-oh listen, that sound is getting louder and closer towards us. 

Cluck! Cluck! Cluck! Cluck! Cluck!

Look, it’s a chicken who makes the clucking sound. That chicken ran so fast until I could barely see its little legs. I wonder why the chicken is running. Is the chicken doing a fun run event or someone is trying to catch that chicken. I think someone is trying to catch that chicken and that chicken is scared. That is why the chicken is running.

……..YEEeeEEeeEEeeEEeeEEeettttt!.........

 

Wait! What is happening here? Chicken?? Running??

This chicken might get inspiration from Chicken Run the Movie in the year of 2000. But why is this chicken related to hearing?

It is just a tactic to attract you to read more and in a fun way. Let’s practice things like this with our kids. For a child who is wearing a hearing aid, spending time by making a lot of sounds motivates them to wear hearing aids more often. It can be animal sounds, like baa, baa, baa for a sheep, moo, moo for a cow and many more. Here, I will share with you several sounds that you could play around with your child.

·       “aaaa” - aeroplane sound or riding a rollercoaster

·       “moo” - a cow

·       “broooom” - a moving car

·       “chip chip” - a bird

·       “meow” - a cat

·       “bummm” - something crashing

·       “ting” - when the oven is ready

Be a child and activate your imagination. You can make any kind of sound to represent anything. Make it in a fun way, I believe children would love it and so will you.

Did you know some writers use ONOMATOPOEIA to give sound effect in their writing?

                  ONOMATOPOEIA

The act of creating or using words that include sounds that are similar to the noises the words refer to. “pop” “boom” “squelch” are onomatopoeic words (Cambridge Dictionary).

Try search onomatopoeia dictionary and you will find millions of sounds.

Let us get back to the little chicken story. Participating in a running/marathon event requires us to be prepared. In a nutshell, you need to prepare your body, plan your day, and mainly you need to prepare your mind to join the event. We need a lot of preparation for that, don't we?

The same thing should go with our kids especially those with hearing aids. Wearing hearing aids is a really NEW EXPERIENCE for you, your family and your child. Accepting hearing aid has been a big challenge for a parent, kids and even for ourselves. We do prepare ourselves accepting hearing aids but how about our child? Even when the child goes to school we need to prepare our child’s mentally and physically so that they would not cry due to separation or meeting new strangers.

THE MAIN THING IS TO GET YOUR CHILD READY TO WEAR HEARING AID/S.

Fitting hearing aids to children requires parent MOTIVATION, COMMITMENT and TIME. Not only a parent but all family members, siblings, friends, teachers, neighbours and community support. Small steps start from our home where the children meets most of the time. Rehabilitation starts with wearing hearing aid all the time.

GOAL 1            :  TOWARDS OPTIMAL HEARING ACCESS

TARGET           : PARENT AND CHILD

STRATEGIES   :

Ø  Attitude towards hearing aids

o   Not only your child but all family member

o   Prepare yourself before preparing others

o   Research about the benefit and limitations of hearing aids

o   Get review from parent with hearing impaired kids and join support group

o   Build a relationship with successful young hearing aid wearers

o   Maintain a good relationship with professional including Audiologist, Speech Therapist, ENT Doctors

o   Share with family member, neighbour, school teachers and community about hearing aids

Ø  Get your child’s mental ready towards hearing aid

o   Story to your child about hearing and introduce to hearing aids.

o   Role play with your child meeting with professional.

o   Let your child play with hearing aid wearer kids.

o   Picture your child’s mind with professional by showing their picture.

o   Visit hearing aid centre regularly just to familiarize your kids with the environment, Audiologist and hearing device itself

Ø  Fit your child’s hearing aid every morning every day

Ø  Spend time with your child by introducing sounds (used Chicken Run in a Marathon Story Technique)

Ø  Be creative and decorate child’s hearing aid based on child’s preference

Ø  Reward your child wearing hearing aids with things that interest them

Ø  Wearing huggie or BTE clip

                                 

                                                        Figure 1 Huggie                                                             

Figure 2 BTE Clip

More strategies and explanation have been shared by my colleague, Miss Tasnim in her post- http://eartisticmalaysia.blogspot.my/2017/03/keeping-hearing-devices-on-your-little.html

Do follow and join us for more exciting activities and tips in Earichment Programme at Eartistic Hearing and Balance Centre.

Shared with love by,

Zahidah Zainal

Audiologist

Eartistic Hearing and Balance Centre, Shah Alam

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HEARING LOSS WITH AUTISM SPECTRUM DISORDER INDIVIDUAL

HEARING LOSS WITH OTHER DISABILITIES
 

Individual with hearing loss and additional disabilities represent by extensively varied and complex population. They vary in the type and degree of their hearing loss, the type and degree of their associated disability, and their general level of functioning.  About 30-40% of newborns who have hearing loss have additional neurodevelopmental disorder. Table 1 below shows the percentage of disabilities that occur in children with hearing loss by Gallaudet Research Institute (2005).

Additional disabilities	% of children with hearing loss
No additional disabilities	57.6
Low vision / legal blindness	4.6
Intellectual disabilities	10.0
Autism	1.0
Orthopedic disabilities (including cerebral palsy)	3.7
Learning disabilities	9.2
Attention deficit disorder/ attention deficit hyperactivity disorder	6.3
Emotional disabilities	1.9
Other	6.9

Table 1: The percentage of disabilities that occur in children with hearing loss by Gallaudet Research Institute (2005)

AUTISM SPECTRUM DISORDER (ASD)

What is Autism? Autism is a developmental disorder characterized by a triad of symptoms including qualitative impairments in social interaction, qualitative in communication, and restricted, repetitive, and stereotyped patterns of behaviours, activities, and interests (American Psychiatric Association, 1994). Autism spectrum disorder (APD) also includes pervasive developmental disorder (PDD), Asperger’s syndrome, Rett’s syndrome and childhood disintegrative disorder. 

            There is no solid evidence to suggest that individual with autism have a greater risk of hearing loss than the general population but the incidence of unusual sensory responses, including abnormal responses to sounds is considered as associated characteristic of autism.  For example, individual with autism normally to be too sensitive to sound, when loud or unexpected sounds arise, they covering theirs ears with their hands.  

HEARING TEST FOR ASD INDIVIDUAL 

            Children with autism who have hearing loss are diagnosed on average, almost 1 year later than those without hearing loss (Madell et al., 2005). Majority those with autism show cognitive deficits, behaviours abnormalities, and hypersensitivity to sensory stimulation, thus modification to traditional hearing testing should be considered. 

Audiologist will want to minimize physical contact with autistic children who have tactile sensitivities. For example audiologist may require initial testing in sound filed which is using the speaker instead of using the earphone. 

            Regardless of the chronological age of the individual, audiologist will use behavioural test procedures that are appropriate for their cognitive.  For example typical behavioural testing procedure used with infant or young children such as visual reinforcement audiometry (VRA) or play audiometry will be used with older children or even adults. 

            If an individual with autism not allow the placement of earphone or probes for individual ear testing, audiologist may have to re-establish to sedate procedures for certainly for those plans to fit with hearing aids. Individual with autism are hard to sedate with pediatric sedation agent and are at risk for seizure while under sedation (Mehta et al, 2004).

HEARING AID FOR INDIVIDUAL WITH ASD

            Individual with autism who has tactile sensitivities and hearing loss, we should expect some struggle and rejection to wear hearing aids.  

Technique to introduce the hearing aids to individual with AS3D:

1.     Start by having the parent or caregiver gently massage the patient’s ear several times a day until little or no resistance is offered.  This may require some time; days, weeks or months depending to the degree of tactile sensitivity.

2.     Introduce to one ear only, with a soft earmould first, without connected with the hearing aid until they willing to wear for longer period of time.

3.     Once the earmould is tolerated with little resistance, the hearing aid can be couple with the earmould and switch on. Then, introduce the hearing aids at another side.

4.     The hearing aid need to be secured to their clothing by using the special hearing aid clip so that even they pull the hearing aids from their ears, the hearing aid is secured to their clothing.    

REFERANCE

Katz et al. (2009). Handbook of Clinical Audiology 6^th.. Edition. Lippincott Williams & Wilkins.  

ATIQAH SOLEHAH BINTI JAMIL
AUDIOLOGIST EARTISTIC HEARING AND BALANCE CENTER, TT

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