Ear Infections in Children with CdLS

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Ear Infections in Children with CdLS
Plus . . . An Opportunity to Help With Current Research
may help to solve that problem, as
way—including a technique developed
r. Robert Thayer Sataloff is Professor
discussed further on.
by our physicians of custom-making
of Otolaryngology at Thomas
tubes from polyethylene tubing
Jefferson University in Philadelphia,
What We Have Learned
available at hobby stores.
Pennsylvania. He is a member of the
In rare instances, it may be
Foundation's CdLS LINK program, and
worthwhile to consider surgery to
has served as a consultant to our families
In 1987, 45 patients ranging in age
widen the ear canal. This will make it
on medical issues relating to the ears,
from 5 months to 20 years were
easier to examine and clean the ears,
nose and throat. In the following article,
examined at an annual CdLS confer-
and allow for the placement of tubes.
Dr. Sataloff offers the latest information
ence. At the time, 22 had serous otitis
However, deciding which patients can
on ear infections in people with CdLS,
media and 7 had myringotomy tubes
be helped is tricky, and the final
plus two opportunities for our readers to
in place, primarily because of
decision to proceed with surgery
help with current research.
previously diagnosed serous otitis
media. Combined, this is an incidence
should be determined by a skilled
of 64%. Thirteen of the patients had
otologist experienced in managing
The Cornelia de Lange Syndrome extremely stenotic (very small) ear
other genetic conditions of the ear.
Foundation receives many inquiries canals, and all of these children had
Other conditions that may contribute
about ear infections from parents of serous otitis media.
to the accumulation of ear fluid should
children with CdLS. Since most
We were able to perform hearing
also be considered and treated,
otolaryngologists—ear, nose and throat tests on 38 children. Twenty had
including allergies. Although parents
doctors—have no knowledge of the profound hearing loss, 9 had moderate have questioned the association of
special otologic problems of children hearing loss, and 9 had mild hearing
gastroesophageal acid reflux and ear
with CdLS and the difficulties in their loss. It is important to realize that fluid infection, there is no evidence to link
management and care, it is important in the middle ear can cause
the two conditions at this time, other
for families to have as much substantial hearing loss even in
than they both tend to appear in
information as possible. This brief someone with healthy inner ear nerve children with CdLS. In most children
article will summarize much of what function. If such a mechanical hearing with CdLS, however, fluid is caused by
we know—and don't know—about ear loss is superimposed upon someone
developmental defects in the skull base
problems in children with CdLS.
who already has a moderate
and Eustachian rubes, and medical,
Three ear problems that may occur
sensorineural (nerve) hearing loss, the
non-surgical, management usually
in people with CdLS are acute otitis
combined effect may be a severe or
fails. Surgical placement of ventilating
media, chronic otitis media, and
profound hearing loss. This is
ear tubes should not be delayed longer
serous otitis media. Acute otitis media
extremely important, since there
than necessary. Even in children with
means an acute ear infection, usually
appears to be a direct correlation
normal sensorineural hearing levels and
associated with fever, pain, pus and
between hearing loss and language
normal learning capacity, prolonged
some danger of more serious
mild to moderate hearing loss from
In our study, 8 of the 9 children with
fluid has been associated with learning
Chronic otitis media means a long-
mild hearing loss had vocabularies
deficits. Children with CdLS should
term infection, usually characterized
ranging from 50 words to greater than
have their hearing optimized as soon as
by a perforated eardrum and
1,000 words. One child was younger
possible in order to avoid potential
intermittent ear drainage. Serous otitis than one year of age. All 9 of the
consequences for learning and
media refers to the common problem
children with moderate hearing loss
language development.
of fluid in the middle ear, which causes had fewer than 30 words in their
Acute ear infections can be difficult
a conductive or mechanical hearing
vocabulary. Of the 20 children with
to diagnose in children with stenotic ear
loss— often correctable. Hearing loss
severe hearing loss, none had a
canals. In most cases, if the child can be
is often the only symptom of serous
vocabulary of more than three words.
held still for examination, there is
otitis media. However, middle ear
It is essential to optimize hearing by enough of an ear canal opening, after it
fluid may predispose some patients to
treating any easily correctable
is cleaned, to permit visualization of a
more frequent episodes of acute otitis
condition, such as middle ear fluid.
portion of the eardrum through an
media, in addition to the middle car
This is usually done by the placement
operating microscope. Most ear, nose
of ear tubes. Placing ear tubes in
and throat specialists have such a micro-
The true incidence of acute otitis
children with CdLS can be challenging,
scope in their offices. However, the
media and chronic otitis media is
and initially may even seem impossible.
examination can be challenging if the
unknown, but readers of Reaching Out
However, there is almost always a
child is not cooperative or sedated, and

other examination methods may be
healthy circumstances. I have not had
in all children cannot be over-
an opportunity to measure ear temper-
emphasized. Prompt and accurate
Ultrasound is used to identify the
atures in children with stenotic ear
attention is especially important.
presence of fluid within the middle ear canals, nor have we established how
Through our combined efforts facilitated
and mastoid areas, but will not
much temperature difference is
by the CdLS Foundation, oto-
discriminate between serous otitis
laryngologic care of CdLS patients has
media (clear fluid) and acute otitis
I invite families reading this to
already improved. Through our
media (pus in the middle ear). X-rays
purchase such a thermometer, if there is continued collaboration, it should
and CT scans have the same
not already one in the house. Measure
improve even more.
shortcoming most of the time. In
the temperatures in both of your child's
general, if a child has a fever, is
ears every day for two weeks, and
Robert Thayer Sataloff, M.D., D.M.
"fussy" and pulling at one or both
record the temperature in each ear,

ears, an ear infection should be
when your child is healthy. This should

assumed and treated with antibiotics.
provide good baseline information on
If infections are frequent, the
normal temperatures in each ear, and
placement of tubes may be considered. differences between the two ears, if any.
Then, when you suspect an ear
You Can Help Us Learn More
infection, repeat the measurements and
see your doctor. If the temperature is
Described below are two oppor-
elevated one-half degree or one degree,
tunities for caregivers to participate in
for example, in the afflicted ear, and
research I am conducting on ear
your doctor is able to confirm that that
infections and people with CdLS. To
ear is infected, we should be able to
help you easily record your
determine whether this is a useful
information and return it to me, you
measure for helping to diagnose acute
may use the data collection sheet on
otitis media in children with CdLS.
page 11.
Please send me your data through the
CdLS Foundation, and I will let you
1. Identifying Ear Infections by
know the results through Reaching Out.
Measuring Ear Temperature

2. "How Common Are Ear
Since receiving an inquiry about
Infections in Children With
identifying ear infections in infants
with stenotic ear canals, I have been
investigating a new technique which I
While we are working together on
will share for the first time in Reaching this collaborative research, I invite you
Out. Readers should recognize that this to participate with me in another project
technique is very preliminary and its
to determine the incidence of ear
long-term value undetermined. I offer
infections in children with CdLS.
it here with the request that caregivers
For 12 months, beginning today, keep
run their own experiment and let me
a written record of the number of times
know the results through the CdLS
your child experiences acute otitis
media. For the data to be meaningful,
Thermometers that measure body
we will need the following information:
temperature electronically through a
your child's name, age, date of ear
device that is easily inserted in the ear
infection, infected ear (right, left, both),
(it looks like an otoscope) are available
whether the diagnosis was confirmed by
at most pharmacies. After simply
a physician (and if so, was it a
inserting the thermometer in the ear
pediatrician, family practitioner, or
and pushing a button, the temperature
otolaryngologist) and the treatment
is read on a screen.
given. If enough of you respond, by
For the last few months, I have used
next year I will let you know, through
this device in patients, mostly adults,
the newsletter, the answer to your
who have an ear infection in one ear
often-asked question, "How common
and no problem in the other. It appears
are ear infections in children with
as if the temperature is elevated in the
infected ear—the temperature of the
The importance of good hearing and
two ears being virtually the same under aggressive management of otitis media