This 3yr old child was brought to the clinic. His parents are worried about the future.
What is this condition? What are associated disabilities?
How will you proceed in managing this child?
What will you counsel the parents about?
No life threatening complications from UPPP
11 years ago
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10 comments:
Is it an atresia of the external auditory canal? Or more likely to be an anotia? Not very sure :)
Can audiological examinations such as BERA help? My rationale is that if the internal and middle ear is present, then the child may have the option of having surgery done to fashion an opening to allow him to hear.
Otherwise, the child and parents should be guided to learn other means of communicating with one another i.e. sign language.
I apologise if my answers sound crude.
congenital atresia. It may associated with shallow blind pit, no cavity, no pinna or middle ear and inner ear abnormality.
It depends whether it is unilateral or bilateral. If it is bilateral, the external meatus should be fashioned to allow the pathway of sound to the inner ear if the chochlear and nerve is normal. If the condition is unilateral and the unaffected ear does not have any hearing impairement, i think the child no need to go for surgery.
Microtia with atresia of the ear canal. The associated disabilties are:
1. down-slanting eyes
2. notched lower eyelids
3. underdevelopment or absence of cheekbones and the side wall and floor of the eye socket
4. small lower jaw and slanting
5. breathing problems and/or eating difficulties
6. hearing loss in each ear due to abnormalities of the outer and middle ear, which conduct sound to the nerve endings
•the eyes have a tendency to dry out, which can lead to infection
•cleft palate
Management:
-a conductive hearing aid- via BAHA
-reconstruction of the ears
-speech and language development
The parents will have to be counselled as if one parent is affected with Treacher collins, there is a 50% chance of passing it on to the offspring.
Good attempts all!
Yes, this is a CONGENITAL MICROTIA with CANAL ATRESIA of the left ear.
The associated disabilitIes of concern is SENSORINEURAL and CONDUCTIVE HEARING LOSS - secondary to congenital defects of the inner ear and middle ear respectively.
The priorities in management are:
1. Condition of other ear
2. Child's hearing level
3. Hearing rehabilitation - early intervention is crucial for speech
4. Reconstruction of left external ear canal if hearing on this ear is intact and imaging confirms intact inner ear structures (done at older age)
5. Reconstruction of left pinna for cosmetic purposes (also at older age)
Reconstruction of pinna generally does not give good enough cosmetic results and its is easier and better to fit a prosthetic ear. Bone anchored hearing aid has definitely transformed the life of these patients.
Don't you have to have his kidneys checked since this is a congenital ear malformation?
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