Archive for category Speech Therapy

Down Syndrome and Ear Problems

Down Syndrome and Ear Problems
Contributed by Dr. Marcella Bothwell, MD

Children born with Down Syndrome may have significant problems in the region of the ear, nose, and throat.  This article will focus on major ear problems and interventions for children with ear issues.  The 3 major issues to be concerned about are the following and will be explained in more detail.

  • External Ear Canal Stenosis or narrowing
  • OME (Otitis Media with Effusion) or fluid in the middle ear
  • Two types of hearing loss, Conductive Hearing Loss (CHL) and Sensorinerural (SNHL)

1) External Ear Canal Stenosis: affects up to 50% of newborns with Down Syndrome and can make it difficult to see your child’s ear drum (tympanic membrane).  Visualization is required for the diagnosis of ear infections or chronic ear fluid.  An ENT or Otolaryngologist / Head and neck surgeon may recommend cleaning with a microscopic exam up to every 3 months for ideal visualization.  Ideally by 2-3 years, the ear canals may have grown enough not to need such frequent cleaning.  Please do not use any Q-tips to clean ears because it actually makes the wax impaction (clogging of ear canal) worse.

2) Otitis Media with Effusion (OME): fluid in the middle ear may be chronic and not be acutely infected. Sometimes however it can also be a more typical ear infection with fever and pain.  Reasons for having more ear problems are:

  • Reduction of the immune system (both T and B cell function) which may increase upper respiratory infections or colds
  • Eustachian tube (ET) dysfunction or abnormal function of the tube between the ear and nose.  Causes are usually anatomical or structural such as:  mid face hypoplasia or flattening; the shape of the Eustachian may have a smaller width;  and / or the cartilage density may make it weaker causing collapse.  Hypotonia or general muscle weakness may not just be in the core muscles but also in the muscle that opens the ear or ET i.e. the tensor veli palatini muscle dysfunction which opens and closes ET.  Because of the above reasons, Eustachian Tube Dysfunction (ETD) persists much longer compared to other children without DS.  However there are no studies that show the expected length of time for persistent OME.

3.) Hearing loss can either be conductive (CHL) or sensorineural (SNHL):

In a prospective study of children with Down Syndrome it was reported by Shott that 98% had normal hearing at one year enrollment but 83% of children required ear tubes due to chronic ear infections or OME. Balkany in 1979 reported 83% of hearing loss is conductive with 60% of the conductive loss due to chronic fluid or perforations.  40% was ossicular (hearing bones of the ear) either congenital or due to secondary erosion. However, Harado and Sandi in 1981 reported, 25% of patients with DS were evaluated at surgery where no obvious anatomic abnormalities were found. 4-20% SNHL or mixed hearing loss is also reported.

Hearing testing is done at birth by BAER (Brainstem Audio Evoked Potential)/ OAE (Otoacoustic Emission) testing and every 6 months following until ear specific information can be attained with Behavioral or pure tone audiogram (hearing testing)

At age 3, 12% of children were able to get ear specific information and by age 4, 41% were successful at behavioral hearing testing.

Remedies to hearing loss include:  Ossicular reconstruction and Amplification (hearing aides).  Remedy is important due to delayed expressive language skills compared to cognitive abilities.  By helping children with Down syndrome express their needs, their disabilities may improve.

Down Syndrome and Ear Problems

Children born with Down Syndrome may have significant problems in the region of the ear, nose, and throat. This article will focus on major ear problems and interventions for children with ear issues. The 3 major issues to be concerned about are the following and will be explained in more detail.

  • External Ear Canal Stenosis or narrowing
  • OME (Otitis Media with Effusion) or fluid in the middle ear
  • Two types of hearing loss, Conductive Hearing Loss (CHL) and Sensorinerural (SNHL)

1) External Ear Canal Stenosis: affects up to 50% of newborns with Down Syndrome and can make it difficult to see your child’s ear drum (tympanic membrane). Visualization is required for the diagnosis of ear infections or chronic ear fluid. An ENT or Otolaryngologist / Head and neck surgeon may recommend cleaning with a microscopic exam up to every 3 months for ideal visualization. Ideally by 2-3 years, the ear canals may have grown enough not to need such frequent cleaning. Please do not use any Q-tips to clean ears because it actually makes the wax impaction (clogging of ear canal) worse.

2) Otitis Media with Effusion (OME): fluid in the middle ear may be chronic and not be acutely infected. Sometimes however it can also be a more typical ear infection with fever and pain. Reasons for having more ear problems are:

  • Reduction of the immune system (both T and B cell function) which may increase upper respiratory infections or colds
  • Eustachian tube (ET) dysfunction or abnormal function of the tube between the ear and nose. Causes are usually anatomical or structural such as: mid face hypoplasia or flattening; the shape of the Eustachian may have a smaller width; and / or the cartilage density may make it weaker causing collapse. Hypotonia or general muscle weakness may not just be in the core muscles but also in the muscle that opens the ear or ET i.e. the tensor veli palatini muscle dysfunction which opens and closes ET. Because of the above reasons, Eustachian Tube Dysfunction (ETD) persists much longer compared to other children without DS. However there are no studies that show the expected length of time for persistent OME.

2) Hearing loss can either be conductive (CHL) or sensorineural (SNHL):

In a prospective study of children with Down Syndrome it was reported by Shott that 98% had normal hearing at one year enrollment but 83% of children required ear tubes due to chronic ear infections or OME. Balkany in 1979 reported 83% of hearing loss is conductive with 60% of the conductive loss due to chronic fluid or perforations. 40% was ossicular (hearing bones of the ear) either congenital or due to secondary erosion.

However, Harado and Sandi in 1981 reported, 25% of patients with DS were evaluated at surgery where no obvious anatomic abnormalities were found. 4-20% SNHL or mixed hearing loss is also reported.

Hearing testing is done at birth by BAER (Brainstem Audio Evoked Potential)/ OAE (Otoacoustic Emission) testing and every 6 months following until ear specific information can be attained with Behavioral or pure tone audiogram (hearing testing)

At age 3, 12% of children were able to get ear specific information and by age 4, 41% were successful at behavioral hearing testing.

Remedies to hearing loss include: Ossicular reconstruction and Amplification (hearing aides). Remedy is important due to delayed expressive language skills compared to cognitive abilities. By helping children with Down syndrome express their needs, their disabilities may improve.

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OT – Playing With Shaving Cream (soapy foam)

If your child is not putting everything in their mouth for sensory input, or can tell the difference between something they can eat or not eat, then shaving cream or soapy foam can be used to help your baby with tactile stimulation, learning to open their index finger (pointer finger) and practice drawing. The whole body is engaged in this kind of play and opportunities for language development abound, too.

A typical baby’s index finger begins to “unfurl” and become useful to them around the age of  8 to 9 months. At this time, they can pass an object from hand to hand and use their index finger and thumb to grasp objects (pincher’s grasp) such as small bites of food. In a baby with low muscle tone, or with cognitive delay, this interaction between brain and finger comes later. It is important that it is encouraged early on in their development since it is the basis for the vast majority of finger and hand operations regarding self-care. Just think about how you use your pointer finger every hour! For a child, they can begin to push buttons, point out what interests them, poke holes in playdough, touch things for sensory input, feed themselves and a long list of other things.

In the video here, 22 month old Arabella is trying an activity that will encourage her to draw with shaving cream if she opens her fingers and (hopefully) uses her index finger to draw in the shaving cream. She is not using her index finger fully, and we are trying to get her brain’s awareness of that finger and its control heightened.

Watch how Tad Bruneau, OTR/L, introduces Arabella to playing with shaving cream. She has played with it before, when it was safely encased in a Ziplock bag, but this time she has the full sensory experience of smell, touch, taste and visual reaction to the foam on her play table. Read the rest of this entry »

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Speech Therapy – Free Class from the San Diego Unified School District for Parents of Special Needs Infants and Toddlers

Alcott Elementary, in the San Diego Unified School District, offers a speech therapy program through their Infant and Toddler Program to help parents learn the basics of how a baby begins to learn speech.  The class is ideal if your baby with special needs is at or near the age of beginning to talk. It offers the opportunity to learn some significant techniques to use to encourage “child lead learning” and enhance the interactions between parent and child. You really ought to talk with your public school system and see if they offer any classes for parents.

I signed up for a 12 week class and I have a lot to learn. Our instructor Ms. Dale Bushnell-Revel SLP, a veteran speech therapist, impressed me from the start.

[Note: as of 1/8/10 the company that Alcott uses in their free speech therapy program, while they have not threatened legal action,  would prefer that I do not blog about what I learned in the class.]

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