CASE REPORT

DEAF CHILD

Presentator : dr. Feri Trihandoko

Moderator : dr. Ashadi Prasetyo, M.Sc, Sp.THT-KL

Bagian Ilmu Kesehatan Telinga Hidung Tenggorok Kepala Leher

Fakultas Kedokteran Universitas Gadjah Mada/ RS DR.SardjitoYogyakarta
2017
INTRODUCTION achievement and later employment

Hearing loss is extremely opportunities. There is a high

common and has a wide spectrum prevalence of psychosocial problems

ranging from a nearly undetectable among deaf children.2

degree of disability to a profound loss The prevalence of permanent

of ability to function in society. Nearly childhood hearing loss, which is mainly

10% of the adult population has some due to loss of cochlear function, is 1.2

hearing loss. Often, this impairment to 1.7 cases per 1000 live births.

presents early in life. One of every 1000 Between 20% and 30% of affected

babies born in the United States is children have profound hearing loss.

completely deaf, and more than 3 The prevalence increases up to 6 years

million children have hearing loss. of age as a result of meningitis, the

However, hearing loss can present at delayed onset of genetic hearing loss, or

any age. Between 30% and 35% of late diagnosis. In developing countries,

individuals over the age of 65 have a the prevalence is greater because of a

hearing loss sufficient to require a lack of immunization, greater exposure

hearing aid. Forty percent of people to ototoxic agents, and consanguinity;

over the age of 75 have hearing loss.1 about half the disabling cases of

In childhood, profound hearing hearing loss worldwide are preventable.

loss (a hearing level of >90 dB) has far- Approximately 30% of deaf children

reaching, lifelong consequences for have an additional disability most

children and their families. The most commonly, cognitive impairment.2

striking effect of profound hearing loss Nine out of 10 Deaf children are

is the lack of development of spoken born to hearing parents. Many of these

language, with its impact on daily deaf children are placed into

communication; this, in turn, restricts mainstream classrooms in part because

learning and literacy, substantially of a lack of knowledge on deaf

compromising educational education exists for these parents.

Parents of deaf children do not realize

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the different education opportunities nongenetic cause of SNHL.7,8
that are provided for them. There is not Mutations in this gene interrupt
enough support and education for potassium recycling, resulting in the
parents of deaf children and how to accumulation of potassium and,
cope with their emotions.3 ultimately, cell death.2
Deafness is a sensory Cytomegalovirus is the most
deficiency which prevents a person to prevalent cause of intrauterine viral
be able to hear. They are not able to infection, responsible for
grasp any sounds like hearing people approximately 40,000 congenitally
do. They cannot hear any sounds. The infected infants in the United States
actual hearing loss is measured by the annually.9,10 Direct transmission of
range of frequencies and the degree of CMV in humans can occur either
volume (loudness).3 Deafness is the vertically (i.e., from mother to child) or
pathological condition of profound horizontally (from person to person), as
hearing loss and that deaf people suffer well as through transplanted organs
from a sensory deficit.5 from seropositive donors. Primary
The etiology of hearing loss in infection, causing few symptoms in
children is almost equally attributed to immunocompetent hosts, can lead to
genetic and environmental factors. A months or years of viral shedding in
comprehensive diagnostic workup for saliva, urine, semen, and cervical or
the patient with sensorineural hearing vaginal fluids. Maternal viremia
loss (SNHL) is vital for guiding facilitates transplacental passage of
treatment and intervention options. CMV to the fetus. Congenital CMV
Mutations in the gap junction -2 infection rate is particularly high (20%
(GJB2) gene that encodes connexin 26 to 25%) for lower socioeconomic status
are the most common cause of (SES) mothers under 20 years of age,
hereditary nonsyndromic SNHL, most of whom are unmarried (80% in
whereas congenital cytomegalovirus one study).7
(CMV) infection is the most common

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 Infection of toxoplasma gondii, subthalamic nuclei; sectors H2 to H3 of
treponema pallidum, herpes simplex the hippocampus; substantial nigra;
virus, rubella virus, mumps virus, and reticular formation of the pons, inferior
some bacterial which can make olivary nuclei, and cranial nerve nuclei
meningitis like Haemophilus oculomotor, facial, vestibular, and
influenzae type B, Neisseria ventrocochlear nucleus, which can
meningitides, and Streptococcus result in neurologic sequelae, including
pneumonia, during pregnancy can also SNHL (retrocochlear). Manifestations
make a pediatric hearing loss or deaf of chronic postkernicteric bilirubin
child. Beside infection, deaf child can encephalopathy include athetosis,
also be caused by ototoxic drugs and intellectual deficits, gaze disturbance
chemical agent, like aminoglycosides, including limitation of upward gaze,
loop diuretic, cisplatin, quinine and and SNHL. Kernicterus can occur in
chloroquoine.7 term infants with hyperbilirubinemia
Significant perinatal anoxia or and in premature infants without
hypoxia is statistically correlated with hyperbilirubinemia.7
subsequent SNHL in affected infants. A case history should include
Had been reported there is association detailed prenatal, birth, and postnatal
of severe neonatal respiratory distress history for the child and a complete
among term and near-term survivors family history for hearing loss, speech
and delayed-onset, possibly or language disorders, ear, nose, and
progressive, SNHL. At 2 years of age throat disorders, and craniofacial
35% (30 of 86) had SNHL and by age deformities, as well as such syndromic
4, 53% exhibited SNHL.7 features as kidney disorders, sudden
Kernicterus results when death of a family member at a young
bilirubin, crossing the blood brain age, thyroid disease, intracranial
barrier, is deposited in a neonate's basal tumors, and progressive blindness.
ganglia, most often globus pallidus and Marital consanguinity in the pedigree
should be carefully documented. The

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physical examination must focus on profound, and anacusic); the
subtle components of hearing loss audiometric configuration and
syndromes such as auricular symmetry of the impairment; and
displacement or malformation, finally, with serial assessment, the
preauricular or branchial pits, white stability or progression of the loss.
forelock, heterochromia irides, blue Behavioral, electrophysiologic, and
sclerae, dystopia canthorum, and facial middle ear measures should be used, as
asymmetry. Ophthalmologic appropriate. Computerized rotational
evaluation with vision testing should be testing facilitates pediatric vestibular
accomplished. Other measures that system evaluation. Progressive and
should be obtained, as appropriate, fluctuating SNHL, with or without
could include an electrocardiogram, vertigo, in children raises the specter of
basic hematology and chemistry tests, other potentially treatable disorders and
thyroid studies, tests for congenital more extensive workup is indicated in
infection [e.g., toxoplasmosis, syphilis, these cases. Prompt management of
cytomegalovirus (CMV)], renal middle ear infections is mandatory to
ultrasound, and temporal bone imaging ensure accurate threshold results. A
studies. Although congenital CMV diligent search for the cause of
infection accounts for a significant childhood SNHL may prove
percentage of childhood deafness, inconclusive in 30% to 40% of cases.
confirmation must be based on positive Clinicians must be prepared to address
laboratory findings (e.g., viral families' concerns regarding the lack of
isolation) within the first 2 or 3 weeks a definitive diagnosis, because of its
of life.6,7 impact on the risk of hearing loss in
The pediatric audiologic future offspring and the possibility of
evaluation must determine the type of progression of their child's hearing
hearing loss (i.e., conductive, loss.7
sensorineural, or mixed); the degree of
loss (i.e., mild, moderate, severe,

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 Deafness may be considered a CASE REPORT
model system for understanding
A one year old female children,
neurosensory restoration. For example,
come to ENT departement with her
cochlear implants can bypass the
parents. The parent was complained
sensory end organ, stimulate the
about their child, whose has limited
neurobiologic and neurocognitive
respons to sound stimulations since his
substrates for speech and language
birth. She has been diagnosed with
processing, and consequently promote
cerebral CMV and under treatment at
cognitive development. The children
pediatric departement of Sardjito
with the best results from cochlear
hospital and then she is reffered to ENT
implantation are among those who have
departement.
received implants before 2 years of age.
She had normal delivery, aterm
These children will enter first grade
but with low birth weight (1900 gr). In
with expressive and receptive spoken
pregnant phase, mother was denied
language skills that are close to those of
about history of spesific illness like
children with normal hearing.2
Jaundice, Measles/ Rubella and
Cochlear implantation was
Varicella. Mother did not notice the
significantly associated with
presence of fever, cough, and another
improvement of quality of life,
symptom.
especially in communication,
Form general status, she is in
happiness and relations with their
compos mentis state. With child weight
friends and family members.11 Bone-
at 5, 4 kg. Form vital sign we found
anchored hearing aids are a durable
pulse 130 beats per minute, respiration
treatment option that can achieve
rate 28 rate perminute, and basal
noticeable improvements in hearing in
temperature rate is 36oC.
10
noise and in listening difficulties.
From otolaryngology
examination, at otoscopy membrane
tympani was intak and positive cone of

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light form both ears. Rhinoscopy
anterior, within normal limit without
oedem mucose, laceration,discharge
and concha oedem, hyper/athropy.
Oropharynx was within normal limit to,
T1- T1.
Patient has been got supporting
examination, namely ottoacoustic
emission/OAE and Brain Evoked
Response Auditory. The result form
OAE was refered or negative in both
ears. Form BERA the conclussion is
profound with distal neural hearing loss
in both ears.
Based on the result from
anamnesis, physical examinations and
supporting examinations, this patient
was diagnosed with deaf child or
pediatric hearing loss with CMV
infection.
The treatment that given to this
patient, namely hearing aid, speech
therapy, and education. Patient also was
treated for CMV infection by
pediatrician. Result for this therapy will
be evaluated 6 month again.
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DISCUSSION
Diagnosis pediatric hearing loss
or deaf child based on anamnesis,
physical examination and supporting
examination. From anamnesis we had
found that patient cannot speak and
when she had been called, was no
response. She did not response or
reaction when the presence of thunder.
From physical examination, we did not
found any anatomical disorder and
abnormality especially at her ear. We
had performed loud sound test for this
patient but no response. From
supporting examination, BERA, we
found that she had profound distal
neural hearing loss at both ear. From
anamnesis we also found that she had
CMV infection since she born and now
still under treatment from the
pediatrician in pediatric department
Sardjito hospital. We had conclusion
that patient had pediatric hearing loss
because of CMV infection which make
disturbance in maturation process or
genetic damage during pregnancy.
According to Misono, et al, 2011,
genetic factor which influence in
hearing process is GJB2/6 and
SLC26A4. In children with congenital during these early years. Respond to
CMV infection, it has been estimated their babbling and language efforts.
that 35% to 65% of symptomatic and Children vary in their language
7% to 15% of asymptomatic newborns development during these first years, so
will ultimately develop HL, with a parents should allow for some variation
median age at onset of 44 months.12 in children's abilities at different ages.
According to Brotherson, 2005, They should encourage language
The "prime time" for auditory development, be patient and seek
development, or a child's capacity for assistance from a qualified professional
learning to hear, is from birth to if concerns arise about a child's
between 4 and 5 years old. The progress in this area. So for this patient
development of these sensory speech, therapy was needed to be done.
capacities is very important for Hearing aids could be given in order to
allowing children, especially babies, to make more sound wave inserts to her
perceive and interact with the world ear and then make stimulus for the
around them. During the first few hearing nerve.13
months, especially, babies need Piracetam could be given
exposure to a variety of sounds so their because it has neurotropic effect which
brain can learn to process that can make increase of hearing nerve and
information and allow for brain function. According to Ashrafi et
responsiveness by hearing something. al, 2002, Zovadenko, et al, 2008,
The "prime time" for language Fusenko UA, 2009, piracetam dosage is
development and learning to talk is 30 - 50 mg/kg body weight/day.14,15,16
from birth to 10 years of age. Children Problem in this case was
are learning language during this entire Management. This patient was
period. However, the "prime time" for managing without follow PERHATI-
language learning is the first few years KL flow chart. In PERHATI-KL flow
of life. Children need to hear the chart, after founding neural
constantly talk, sing and read to them abnormality without conductive

7
abnormality in BERA or OAE, patient 3. Nguyen TL, Born Into A Hearing
must be performed visual Family: A Guide For Hearing
reinforcement audiometry, and Parents With Deaf Children, 2011
behavioral observation audiometry. 4. Barret EK, Barman MS, Boitano S,
After that patient must be performed Brooks LH, Ganongs Review of
communication and psychology Medical Physiology 23th ed,
assessment. In this case patient was McGraw-Hill Companies, 2010.
treated in order to cure CMV infection 5. Burke BT, Quest For A Deaf Child
first and was tried to improve hearing : Ethics And Genetics, The
quality. University Of New Mexico, 2011.
CONCLUSSION 6. Kimani WJ, Buchman AC, Booker
Had been reported patient KJ, Huang YB, Castillo M, Powell
female, 2 years old with diagnose MC, et al, Sensorineural Hearing
pediatric hearing loss or deaf child with Loss in a Pediatric Population
CMV infection. This patient had been Association of Congenital
treated by speech therapy, hearing aid, Cytomegalovirus Infection With
and education. Patient also was treated Intracranial Abnormalities, Arch
for CMV infection by pediatrician. Otolaryngol Head Neck Surg.
Result for this therapy will be evaluated 2010;136(10):999-1004
6 month again. 7. Bailey BJ, Johnson JT, et al, Head
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