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.