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PLENARY TASK REPORT OF SECOND CASE
GROWTH AND DEVELOPMENT MODULE
Group 9:
Irma Nur Rizka Hanifah
Muhammad Fadhil A
Budi Hartono
Rahmad Ramadhani
Ledi Ratih Nurcahayani S
Anggita Serli Verdian
Agitya Goesvie Ajie
Makmur Sejati
I101114109
I101114138
I101114140
I101114158
I101114172
I101114174
I101114175
I101114178
STUDY PROGRAM OF MEDICAL EDUCATION
MEDICAL FACULTY
TANJUNGPURA UNIVERCITY
PONTIANAK
2015
1. What is the meaning of congenital rubella infection and congenital rubella
syndrome?
Congenital rubella infection (CRI) occurs when the rubella virus is
passed from an infected pregnant mother to her baby. Infants born with CRI
have laboratory confirmation of infection but no visible defects. The virus
may be shed in the infant’s urine or nasopharyngeal secretions for a year or
more. Infants infected at 20 weeks of pregnancy or beyond may still present
later in life (sometimes several years later) with deafness, chorioretinopathy,
developmental delay or other problems.1
Fetal infections with rubella, especially in the first 20 weeks of
pregnancy, may be associated with spontaneous abortion, intrauterine death
and a variety of other problems collectively known as congenital rubella
syndrome (CRS). This occurs as single or combined defects. Moderate and
severe cases of CRS are typically recognized at birth. In mild forms of the
disease, however, the anomalies may not be obvious at birth but become
apparent within the first year of life. The risk of infection producing damage
to the fetus evident at birth is as high as 90% if infection occurs in the first
trimester, falling to 10–20% by the 16th week, and becoming very low by the
20th week of pregnancy and beyond. Diabetes mellitus has been recognized as
a frequent late manifestation of CRS.1
Classical congenital rubella syndrome was originally described before
the virus could be cultured in the laboratory. The major features are congenital
heart disease (particulary patent ductus arteriosus and pulmonic stenosis) and
eye defects (particulary cataracts, glaucoma, and microphthalmia).
Microchephaly and deafness may also be present.2
2. What is the sign of congenital rubella infection?
Expanded congenital rubella syndrome was defined during he severe
epidemic in the US in 1964, when a number of other manifestations were
clearly recognized for the first time. These included intrauterine growth
retardation, jaundice with hepatosplenomegaly, thrombocytopenic purpura,
encephalitis, and myocarditis in various combination. The term “blueberry
muffin” baby was coined to describe the yellow baby with purple spots
because of jaundice and purpura or dermal erythropoiesis. Other possible
manifestation are a large anterior fontabel, transient longitudional bone
radiolucencies, failure to grow well, unusual dermatoglyphics, and dental
enamel defects. Retinitis manifested is neither progressive nor associated with
decreased vision.2
3. When do we call it congenital rubella syndrome?
Late-onset rubella syndrome is a form in which there are minimal
symptoms at birth but acute severe multisystem disease develops after 3-6
months, with interstitial pneumonia, skin rash, diarrhea,
hypogammaglobulinemia, and circulating immune complexes. Aseptic
meningitis, hepatosplenomegaly, thrombocytopenia, and Pneumocystis
pneumonia may also occur. Diabetes mellitus has been observed later in
childhood in follow-up studies of some infants with congenital rubella
infection. Hypotonia or convulsions or a chronic progressive panencephalitis
can also occur in later childhood.2
4. Find out about passive immunization.
Immunity is the ability of the human body to tolerate the presence
of material indigenous to the body (“self ”), and to eliminate foreign
(“nonself ”) material. This discriminatory ability provides protection from
infectious disease, since most microbes are identified as foreign by the
immune system. Immunity to a microbe is usually indicated by the presence
of antibody to that organism. Immunity is generally specific to a single
organism or group of closely related organisms. Passive immunity is
protection by products produced by an animal or human and transferred to
another human, usually by injection. Passive immunity often provides
effective protection, but this protection wanes (disappears) with time,
usually within a few weeks or months.
Passive immunity is the transfer of antibody produced by one human
or other animal to another. Passive immunity provides protection against
some infections, but this protection is temporary. The antibodies will
degrade during a period of weeks to months, and the recipient will no
longer be protected. The most common form of passive immunity is that
which an infant receives from its mother. Antibodies are transported across
the placenta during the last 1–2 months of pregnancy. As a result, a full-
term infant will have the same antibodies as its mother. These antibodies
will protect the infant from certain diseases for up to a year. Protection is
better against some diseases (e.g., measles, rubella, tetanus) than others
(e.g., polio, pertussis).3
5. How is the methods and way of working in examining ear with OAE
methods? During normal hearing, OAEs originate from the hair cells in
the cochlea and are detected by sensitive amplifying processes. They travel
from the cochlea through the middle ear to the external auditory canal, where
they can be detected using miniature microphones. Transient evoked OAEs
(TEOAEs) may be used to check the integrity of the cochlea. In the neonatal
period, detection of OAEs can be accomplished during natural sleep, and
TEOAEs can be used as screening tests in infants and children for hearing at
the 30 dB level of hearing loss. They are less time consuming and elaborate
than ABRs and are more sensitive than behavioral tests in young children.
TEOAEs are reduced or absent owing to various dysfunctions in the middle
and inner ears. They are absent in patients with >30 dB of hearing loss and are
not used to determine the hearing threshold; rather, they provide a screen for
whether hearing is present at >30-40 dB. Diseases such as OM or congenitally
abnormal middle-ear structures reduce the transfer of TEOAEs and may
incorrectly indicate a cochlear hearing disorder. If a hearing loss is suspected
based on the absence of OAEs, the ears should be examined for evidence of
pathology, and then ABR testing should be used for confirmation and
identification of the type, degree, and laterality of hearing loss.4
6. How is the methods and way of working in examining ear with ABR
methods?
Auditory Brainstem Response, most commonly abbreviated as ABR,
is a test that evaluates how well the sound travels from the ear to the level of
the brainstem. ABR responses are recorded by placing 3-4 stickers called
electrodes on the child’s head and behind the ears, stimulating the ear with
brief auditory signals via earphones. By placing electrodes on the child’s
head, the ABR is testing neural synchronysynchronous electrical activity of
many neurons firing at the same time from areas within the cochlea, 8th nerve,
and the brainstem in response to an auditory stimulus.5 This synchronization
allows clinicians to mark neural responses on the waves recorded in response
to stimulation presented at different intensity levels across low and high
frequencies. The lowest level at which neural responses are obtained is
considered the threshold of hearing for that particular frequency.6
Automated Auditory Brainstem Response (AABR) is used as a
screening test in newborn babies. Newborn babies are screened for hearing
loss prior to hospital discharge. Screening tests are performed using one
loudness level and the baby either passes or fails the screen. Newborns or
infants who do not pass the initial screen are re-screened either prior to
discharge or as a separate visit. Infants not passing in one or both ears after
two screening attempts are referred for a diagnostic ABR evaluation to further
evaluate their auditory function at different loudness levels across frequencies.
On whom can this test be performed? ABR can be recorded in newborns,
infants, children, and adults. ABR test is performed while the patient is asleep
or resting quietly as noise and movement can affect test results. If the child is
younger than 4-5 months, ABR can most likely be done while the child is
asleep. ABR testing under sedation is recommended for patients who are
unable to sleep naturally or rest quietly for the duration of the test. Sedated
ABR testing is also recommended on older children for whom there is a
concern or suspicion of hearing loss that could not be confirmed through
standard hearing test procedure.6
7. What is the indication that tells us to do OAE examination?
Automated ABR, OAE, or OAE followed by ABR testing is considered
medically necessary to screen for hearing disorders for any of the following
indications:7
As initial screening for newborns and infants; or
Infants (age less than 1 year) admitted to an ICU for 2 or more days; or
Neonates during the 1st month of life when exposed to potential causes of
hearing loss (for example, chemotherapy, hyperbilirubinemia that requires
exchange transfusions, meningitis, or culture-positive sepsis); or
To screen for hearing disorders in infants and children when behavioral
audiometry is not reliable related to, but not limited to, inability to
cooperate with other methods of hearing testing; or
To screen for hearing disorders in children less than 36 months of age who
passed the neonatal hearing screening test but are at risk of having
sensorineural hearing loss; or
To monitor for ototoxicity in individuals undergoing treatment with an
ototoxic agent (for example, aminoglycosides, chemotherapy agents, or
heavy metals).
ABR with or without OAE testing is considered medically
necessary to diagnose hearing disorders for any of the following
indications:
To make a confirmatory diagnosis of a hearing disorder in infants and
children (birth to 36 months) who did not pass the initial hearing
screening; or
To evaluate infants and children suspected of having a hearing
disorder when behavioral audiometry are not reliable, or ear-specific
thresholds cannot be obtained, or when results are inconclusive
regarding the type, degree, or configuration of hearing levels; or
To assess suspected hearing disorders in individuals who are unable to
cooperate in other methods of hearing testing (e.g. behavioral
audiometry); or
To evaluate in the diagnosis and monitoring of acoustic neuroma.
Not Medically Necessary:
ABR or OAE for hearing disorders is considered not medically necessary
when the above criteria are not met, or for the evaluation of suspected
presbycusis, or for the evaluation of suspected otosclerosis, or for individuals
able to undergo standard audiometry.7
8. What is the indication that tells us to do ABR examination?
Brainstem auditory evoked responses (ABR), also known as auditory
brainstem response (ABR), test both the ear and the brain. They measure the
timing of electrical waves from the brainstem in response to clicks or tone
bursts in the ear. Computer averaging over time to filters background noise to
generate an averaged response of the auditory pathway to an auditory stimulus
three waves (1, 3 and 5) are plotted for each ear. The waveform represents
specific anatomical points along the auditory neural pathway: the cochlear
nerve and nuclei (waves I and II), superior olivary nucleus (wave III), lateral
lemniscus (wave IV), and inferior colliculi (wave V). Delays of one side
relative to the other suggest a lesion in the 8th cranial nerve between the ear
and brainstem or the brainstem itself.8
The main indication for ABR is when an acoustic neuroma is
suspected. This generally comes about when there is an asymmetrical
sensorineural hearing loss. ABR testing is more cost effective for this purpose
than MRI but MRI provides additional information. The most reliable
indicator for acoustic neuromas from the ABR is the interaural latency
differences in wave V: The latency in the abnormal ear is prolonged.
According to a meta-analysis of ABR, they are 93.4% sensitive to acoustic
neuroma. ABR testing may also be useful in situations where an auditory
neuropathy is suspected. In this case, it may be combined with otoacoustic
emission testing.8
ABR testing may show some subtle abnormalities in persons with
tinnitus. ABR's are commonly abnormal in brainstem disorders such as
multiple sclerosis, brainstem stroke, or brainstem degenerative disorders.
These are much less common than inner ear disorders, but also are
intrinsically much more dangerous.7 ABR testing requires reasonable high-
frequency hearing. This means that it is often not worth doing in persons over
the age of 70. We recommend that either an audiogram or at least a screening
test for high frequency hearing be done prior to ABR testing.8
9. What is the function of OAE and ABR examination, what's it that we want to
see from this examination?
An otoacoustic emission (OAE) is a low-level sound emitted by the
cochlea either spontaneously or evoked by an auditory stimulus. Specifically,
OAEs provide information related to the function of the outer hair cells
(OHC). Over the past 20 years, their use in routine audiological assessments
has increased significantly. Today, OAEs are used commonly in the
audiological assessment of difficult to test patients, such as persons who
cannot or will not volunteer reliable behavioral responses. OAEs are routinely
used in the pediatric population to verify behavioral responses and obtain
additional frequency-specific information. In addition, they are routinely used
in newborn hearing screening programs across the world. OAEs have many
benefits: they are easy to obtain, non-invasive, and provide reliable
information regarding cochlear status in a relatively short time.9
Present OAEs in an ear indicate many things about the auditory
system. First, a present OAE tells us that the conductive mechanism of the ear
is functioning properly. This includes proper forward and reverse
transmission, no blockage of the external auditory canal, normal tympanic
membrane movement, and a functioning impedance matching system. Present
OAEs also indicate that OHC function is normal, which, in most cases,
correlates with normal hearing sensitivity. OAE testing does have some
limitations. OAE testing does not evaluate the inner hair cells (IHC), nVIII,
ascending central auditory pathway, or auditory processing function.9
The use of OAEs to assist in the diagnosis of retrocochlear pathologies
has become standard in clinical practice. OAEs arise from the peripheral
auditory system; therefore, a logical conclusion is that they will be present in
cases of retrocochlear pathology. In most cases this is true; however,
neoplasms in the internal auditory canal and/or posterior fossa may impinge
on the internal auditory artery and compromise blood flow to the cochlea.
This will affect the presence of OAEs. Among various studies, the proportion
of patients with retrocochlear pathology showing normal OAEs is about 20%.
Probably the most common use of OAEs in the diagnosis of retrocochlear
pathologies is in the diagnosis of auditory neuropathy (also called auditory
dysynchrony or auditory neuropathy spectrum disorder (ANSD)). ANSD is
characterized by absent or severely abnormal auditory brainstem responses,
poor word recognition, variable audiogram findings, possibly present OAEs,
absent middle ear muscle reflexes, and a "mirror image" cochlear microphonic
with a change in stimulus polarity. The latter is the gold standard for ANSD,
as relying upon an abnormal or absent ABR and present OAEs to diagnose
ANSD is not reliable; OAEs may be initially present but disappear over time
in individuals with ANSD.9
Brainstem auditory evoked response (BAER) and auditory brainstem
response (ABR) are two names for the same test. It is a way for the
audiologist to measure how the ear is receiving sound and sending it to the
brain through the auditory nerve. Unlike standard hearing tests, it does not
involve a voluntary response from the patient. Once your child is sleeping, the
test will be started. It does not hurt. Sounds are sent through a small, soft
earphone placed in each ear. The responses are picked up through small
electrodes (metal disks), taped in place on the forehead and behind each ear.
The audiologist uses a computer to read and record the brain’s response to
sounds.10 The test is done to:11
Help diagnose nervous system problems and hearing loss (especially in
newborns and children)
Find out how well the nervous system works
Check hearing ability in people who cannot do other hearing tests.
This test may also be performed during surgery to decrease the risk of
injury to the hearing nerve and brain.
10. What is ASSR?
An auditory steady-state response (ASSR) is an electrophysiological
response that follows the envelope of a periodically repeated narrow band
stimulus. The stimuli may consist of pure tones modulated in amplitude
and/or frequency, repeated filtered clicks or repeated band-limited chirp
signals. The portion of the basilar membrane being stimulated is restricted to
the stimulus frequency range, thus a frequency specific assessment of hearing
is possible.ASSR can be recorded over a wide range of stimulus rates.
Different stimulus rates result in stimulation at different levels of the auditory
pathway. At fast stimulus rates (> 70 Hz, so called 80-Hz-response) the
response is dominated by early evoked activity from the brain stem and is
therefore not affected by subject state (sleep, sedation, attention, level of
arousal). So, the 80-Hz-response is mainly utilized in sedated or lightly
anesthetized children/babies. At stimulus rates around 40 Hz, named as 40-
Hz-response, components of the Middle Latency Response (MLR) contribute
to the ASSR. Therefore the response includes activity from the higher
auditory pathways in the Thalamus and the Cortex. They are mainly used in
awake adults. The narrow band stimuli allow either single frequency or
multiple frequency stimulation to one or both ears simultaneously. When
multiple frequencies are tested, different stimulus rates are used so that the
ASSR to each frequency can be detected separately . Recordings of ASSR are
obtained differentially from electrodes placed on the scalp at locations
typically used for the recording of other auditory evoked potentials (vertex,
mastoid). The ASSR consists of neural activity that follows the rate of the
narrow band stimulus. So the frequency of interest in the brainwaves is that
corresponding to the stimulus rate.12
Averaging is commonly used to extract the ASSR from other electrical
activity (e.g. EEG) to increase the signal-to-noise ratio and with that enabling
response detection. Unlike ABR, ASSR analysis occurs in the frequency
domain rather than the time domain. Objective response detection algorithms
use amplitude and/or phase of the response and its variability to determine
whether a response is present or not.12
Clinical Use: The ASSR is mainly used in the audiological diagnosis.
It is a powerful method to provide frequency specific estimation of the
behavioural pure-tone thresholds in the entire audiometric frequency and
level range.12
11. Download a journal about modified hearing ability testing instrument and a
questionnaire regarding to hearing ability test with its interpretation!
(Attached in folder)13
REFERENCES
1. Alberta Health and Wellness Public Health Notifiable Disease Management
Guidelines. 2011. Congenital Rubella Infection/Syndrome. Government of
Alberta
2. Randall G. Fisher, Thomas G. Boyce; Hugh L. Moffet (2005). Moffet’s
Pediatric Infectious Disease: A Problem-oriented Approach. Lippincott
Williams & Wilkins pp. 637
3. Patankar, AuD, CCC-A, Avni. 2014. Auditory Brainstem Response Explained
Pediatric Ear, Nose & Throat of Atlanta, P.C.Atlanta,Georgia ; Winter, from
www.childrensent.com.
4. Kliegman, Robert M, Richard E. Behrman, Hal B. Jenson, Bonita F. Stanton.
2015. Nelson Textbook Of Pediatrics. 20th Edition.Philadelphia
5. Crumley, W. (2011). Good practices in auditory brainstem response, part 1.
Retrieved August 28, 2013. from www.audiologyonline.com.
6. Patankar, AuD, CCC-A, Avni. 2014. Auditory Brainstem Response Explained
Pediatric Ear, Nose & Throat of Atlanta, P.C.Atlanta,Georgia ; Winter, from
www.childrensent.com.
7. American Academy of Audiology Childhood Hearing Screening Guidelines
(2011). Available at: http://www.cdc.gov/ncbddd/hearingloss/documents/
AAA_Childhood%20Hearing%20Guidelines_2011.pdf. Accessed on June
24, 2014.
8. Koors PD, Thacker LR, Coelho DH. ABR in the diagnosis of vestibular
schwannomas: A meta-analysis. Am J Otolaryngol. 2013 Jan 14. pii: S0196-
0709(12)00260-8. doi: 10.1016/j.amjoto.2012.11.011. [Epub ahead of print]
9. Cunningham Rebekah F. 2011. Otoacoustic Emissions: Beyond Newborn
Hearing Screening. USA: Department of Audiology at A. T. Still University,
the Arizona School Of Health Sciences.
10. Childrens Hospitals and Clinics of Minnesota. 2013. Brainstem Auditory
Evoked Response or Auditory Brainstem Response : for Hearing Assesmen.
Chicago. Retrieved from https://www.childrensmn.org/Manuals/PFS/ Rehab
Public/018782.pdf September, 26th 2015 01.33 pm
11. Emerson RG, Pedley TA. 2014. Clinical neurophysiology:
Electroencephalography and evoked potentials. In: Bradley WG, Daroff RB,
Fenichel GM, Jankovic J, eds.Neurology in Clinical Practice.
12. Al-Noury K. Distortion product otoacoustic emission for the screening of
cochlear damage in children treated with cisplatin. Laryngoscope. 2011 May;
121(5):1081-4. doi: 10.1002/lary.21740.
13. Andriani, Rini, dkk. 2010. Peran Instrumen Modifikasi Tes Daya Dengar
sebagai Alat Skrining Gangguan Pendengaran pada Bayi Risiko Tinggi Usia
0-6 Bulan. Jakarta: Sari Pediatri 2010;12(3):174-83.