The Deaf Child

Anak-anak dengan kehilangan pendengaran mendalam (>90 dB) atau tuli total gagal
mengembangkan kemampuan berbicara dan sering disebut tuli-bisu atau tuli dan bisu. Namun,
anak-anak ini tidak memiliki cacat dalam kemampuan bicara mereka. Cacat utama adalah tuli.
Mereka belum pernah mendengar ucapan dan karena itu mereka tidak bisa mengembangkan
kemampuan bicaranya. Dalam derajat gangguan pendengaran yang lebih rendah, bicara memang
berkembang tetapi rusak.

Periode dari lahir hingga 5 tahun kehidupan sangat penting untuk perkembangan bicara dan bahasa,
oleh karena itu, perlu dilakukan identifikasi dan penilaian dini gangguan pendengaran dan
rehabilitasi dini pada bayi dan anak-anak. Anak-anak yang gangguan pendengarannya diamati dan
dikelola sebelum usia 6 bulan memiliki skor kosakata yang lebih tinggi, kemampuan bahasa
ekspresif dan komprehensif yang lebih baik daripada mereka yang didiagnosis dan dikelola setelah
usia 6 bulan yang menekankan pentingnya identifikasi dan pengobatan dini.

AETIOLOGY

Hearing loss in a child may develop from causes before birth (prenatal), during birth (perinatal) or
thereafter (postnatal).

A. PRENATAL CAUSES

1. INFANT FACTORS. Seorang bayi dapat lahir dengan anomali telinga bagian dalam karena
penyebab genetik atau nongenetik. Anomali dapat mempengaruhi telinga bagian dalam saja
(nonsyndromic) atau dapat membentuk bagian dari sindrom (syndromic). Anomali yang
mempengaruhi telinga bagian dalam mungkin hanya melibatkan labirin membran atau kedua labirin
membran dan tulang. Mereka termasuk:

(a) Scheibe dysplasia. Ini adalah anomali telinga bagian dalam yang paling umum. Labirin tulang
normal. Bagian superior dari labirin membran (saluran utrikel dan semicircular) juga normal.
Displasia terlihat di koklea dan saccule; Oleh karena itu juga disebut cochleosaccular dysplasia. It is
inherited as an autosomal recessive nonsyndromic trait.

(b) Alexander dysplasia. Ini hanya mempengaruhi belokan basal koklea membranosa. Dengan
demikian hanya frekuensi tinggi yang terpengaruh. Sisa pendengaran hadir dalam frekuensi rendah
dan dapat dimanfaatkan dengan amplifikasi dengan alat bantu dengar.

(c) Bing-Siebenmann dysplasia. Tidak ada labirin membran sama sekali.

(d) Michel aplasia. Tidak ada labirin bertulang dan membran sama sekali. Bahkan puncak petrous
tidak ada, tetapi telinga eksternal dan tengah mungkin sama sekali tidak terpengaruh. Tidak ada alat
bantu dengar atau implantasi koklea yang dapat digunakan.
(e) Mondini dysplasia. Only basal coil is present or cochlea is 1.5 turns. Ada partisi yang tidak
lengkap antara scalae karena tidak adanya lamina spiral osseous. Kondisinya unilateral atau bilateral.
Deformitas ini dapat dilihat pada sindrom Pendred, Waardenburg, branchio-oto-renal, Treacher-
Collins dan Wildervanck.

(f) Enlarged vestibular aqueduct. Vestibular aqueduct membesar (>2 mm), endolymphatic sac juga
membesar dan dapat dilihat pada MRI T2. Ini menyebabkan gangguan pendengaran sensorineural
onset dini yang progresif. Vertigo mungkin ada. Fistula perilymphatic dapat terjadi.

(g) Semicircular canal malformations. Baik superior dan lateral atau hanya lateral dari semicircular
canal malformations dapat dilihat. Mereka dapat diidentifikasi pada teknik pencitraan.

2.MATERNAL FACTORS

(a) Infections during pregnancy.

(b) Drugs during pregnancy.

(c) Radiation to mother in the first trimester.

(d) Other factors.

(a) Infections during pregnancy. Infeksi yang dapat mempengaruhi janin yang sedang berkembang
adalah toxoplasmosis, rubella, cytomegaloviruses, herpes type 1 and 2 and syphilis. Remember
mnemonic, TORCHES.

(b) Drugs during pregnancy. Streptomycin, gentamicin, tobramycin, amikacin, quinine or

chloroquine, ketika diberikan kepada ibu hamil, bisa menembus barrier plasenta dan merusak
cochlea. Thalidomide Tidak hanya mempengaruhi telinga tetapi juga menyebabkan kelainan anggota
badan, jantung, wajah, bibir dan langit-langit mulut.

(c) Radiation to mother in the first trimester.

(d) Other factors. Kekurangan gizi, diabetes, toksemia dan kekurangan tiroid. Alkoholisme pada ibu
juga teratogenik terhadap sistem pendengaran yang sedang berkembang.

B. PERINATAL CAUSES

They relate to causes during birth or in early neonatal period. They are as follows.

1. ANOXIA. It damages the cochlear nuclei and causes haemorrhage into the ear. Placenta praevia,
prolonged labour, cord round the neck and prolapsed cord can all cause fetal anoxia.

2. PREMATURITY AND Low BIRTH WEIGHT. Born before term or with birth weight less than 1500 g
(3.3 lb).

3. BIRTH INJURIES. e.g. forceps delivery. They may cause intracranial haemorrhage with
extravasation of blood into the inner ear.

4. NEONATAL JAUNDICE. Bilirubin level greater than 20 mg% damages the cochlear nuclei.

5. NEONATAL MENINGITIS
6. SEPSIS

7. TIME SPENT IN NEONATAL ICU

8. OTOTOXIC DRUGS. used for neonatal meningitis or septicaemia.

C. POSTNATAL CAUSES

1. GENETIC. Though deafness is genetic, it manifests later in childhood or adult life. Deafness may
occur alone as familial progressive sensorineural deafness or in association with certain syndromes,
e.g. Alport, Klippel-Feil, Hurler, etc.

2. NONGENETIC. They are essentially same as in adults

and include:

(a) Viral infections (measles, mumps, varicella, influenza),

meningitis and encephalitis.

(b) Secretory otitis media.

(c) Ototoxic drugs.

(d) Trauma, e.g. fractures of temporal bone, middle ear

surgery or perilymph leak.

(e) Noise-induced deafness.

EVALUATION OF A DEAF CHILD

FINDING THE CAUSE

This may require a detailed history of prenatal, perinatal or

postnatal causes, family history, physical examination and

certain investigations depending on the cause suspected.

1. Suspicion of hearing loss. Hearing loss is suspected if

(i) the child sleeps through loud noises unperturbed or

fails to startle to loud sounds, (ii) fails to develop speech

at 1–2 years. A partially hearing child may have a defective

speech and perform poorly in school and be labelled

mentally retarded. It is essential that all children

at risk for hearing loss should be screened and followed.

2. Risk factors for hearing loss in children (Recommendations

of Joint Committee on Infant Hearing—

updated to 1994).

(a) Family history of hearing loss.

(b) Prenatal infections (TORCHES).

(c) Craniofacial anomalies including those of pinna

and ear canal.

(d) Birth weight less than 1500 g (3.3 lbs).

(e) Hyperbilirubinaemia requiring exchange transfusion.

(f) Ototoxic medications included but not limited

to aminoglycosides used in multiple courses or in

combination with loop diuretics.

(g) Bacterial meningitis.

(h) 1Apgar score of 0–4 at 1 min or 0–6 at 5 min.

(i) Mechanical ventilation for 5 days or longer.

(j) Stigmata or other findings associated with a

syndrome known to include sensorineural and/or

conductive hearing loss.

ASSESSMENT OF HEARING IN INFANTS

AND CHILDREN

Assessment of auditory function in neonates, infants and

children demands special techniques. They are grouped

under the following heads (Table 19.2):

1. SCREENING PROCEDURES. They are employed to test

hearing in “high-risk” infants and are based on infant’s

behavioural response to the sound signal. It is now observed

that 95% of children with one or more risk factors

have normal hearing. On the contrary, 50% of children

with sensorineural hearing loss had no risk factor. This

leads to a programme of universal neonatal screening for

early detection.

Two important tests are to study otoacoustic emissions

(OAEs) and auditory brainstem responses (ABR).

(a) OAEs are generated at outer hair cells and can be

picked up from the external ear as the energy produced

by them travels in reverse direction from outer

hair cells ! ossicles ! tympanic membrane ! ear

canal where it is picked up. OAEs are absent if outer

hair cells in the cochlea are nonfunctional or there is

middle ear effusion or canal debris due to meconium

which may persist for 3–4 days. They are normal even

when VIIIth nerve is nonfunctional. Thus can be used

in the diagnosis of neuropathy of VIIIth nerve.

(b) ABRs are generated in response to sound stimulus presented

to the ear and picked up from the scalp. With

a response of 30–35 dB nHL, the infant who passes

the test and the hearing is considered normal. Infants

who fail these tests are followed up with repeat tests.

Arousal test. A high-frequency narrow band noise is

presented for 2 s to the infant when he is in light sleep. A

normal hearing infant can be aroused twice when three

such stimuli are presented to him.

Auditory response cradle is a screening device for newborns,

where baby is placed in a cradle and his behaviour

(trunk and limb movement, head jerk and respiration) in

response to auditory stimulation are monitored by transducers.

It can screen babies with moderate, severe or profound

hearing loss.

2. BEHAVIOUR OBSERVATION AUDIOMETRY. Auditory signal

presented to an infant produces a change in behaviour,

e.g. alerting, cessation of an activity, widening of

eyes or facial grimacing. Moro’s reflex is one of them and

consists of sudden movement of limbs and extension of

head in response to sound of 80–90 dB. In cochleopalpebral

reflex, the child responds by a blink to a loud sound. In

cessation reflex, an infant stops activity or starts crying in

response to a sound of 90 dB.

3. DISTRACTION TECHNIQUES. are used in children

6–7 months old. The child at this age turns his head to

locate the source of sound. In this test, the child is seated

in his mother’s lap, an assistant distracts the child’s attention

while the examiner produces a sound from behind or

from one side to see if the child tries to locate it. Sounds

used are high frequency rattle (8 kHz), low-frequency

hum, whispered sound as “S, S, S”, xylophone, warbled

tones or narrow band noise (500–4000 Hz).

4. CONDITIONING TECHNIQUES

(a) Visual reinforcement audiometry (VRA). It is a conditioning

technique in which child is trained to look for an

auditory stimulus by turning his head. This behaviour

is reinforced by a flashing light or an animated toy.

This test helps to determine the hearing threshold using

standard audiometric techniques. The auditory

stimulus is delivered by headphones or better still by

insert earphones which are accepted better and are

also light weight. Test is well-suited between the developmental

age of 6 months to 2 years.

(b) Play audiometry. The child is conditioned to perform

an act such as placing a marble in a box, putting a ring

on a post or putting a plastic block in a bucket each

time he hears a sound signal. Each correct performance

of the act is reinforced with praise, encouragement or

reward. Ear specific thresholds can be determined by

standard audiometric techniques. This test can be used

in children with developmental age of 2–4 or 5 years.

(c) Speech audiometry. The child is asked to repeat the

names of certain objects or to point them out on the

pictures. The voice can be gradually lowered. In this

way, hearing level and speech discrimination can be

tested. The test can also be used to examine the child’s

expressive ability when he is asked to name the toys

like horse, duck or objects like cup, plate, etc.

5. OBJECTIVE TESTS

(a) Evoked response audiometry.

(i) Electrocochleography. It can measure auditory sensitivity

to within 20 dB. But it is an invasive procedure

requiring placement of electrodes through

the tympanic membrane.

(ii) Auditory brainstem response. It is not a direct test

of hearing but correlates highly with the puretone

thresholds. Identifiable waveforms in ABR

are generally present 10–20 dB above behavioural

threshold. ABR provides an ear-specific information

as sound stimulus can be presented to each

ear separately by headphones or ear inserts. It is an

objective test and can be done under sedation as

the latter has no effect on ABR. ABR is used both as

a screening test and as a definitive hearing assessment

test in children. In a screening test, a response

to a click stimulus of less than 40 dB nHL or less

is the criterion of passing the test. To find hearing

threshold in an infant, ABR tracing is obtained first

at higher sound stimulus and then gradually lowered

till wave V is just identifiable but repeatable.

(b) Otoacoustic emissions (see p. 29). Transient evoked

emissions (TEOAEs) are absent in ears where hearing

loss exceeds 30 dB. Distortion product emissions

(DPOAEs) are absent when hearing loss exceeds 50 dB.

(c) Impedance audiometry. Normally, stapedius muscle

contracts reflexly in response to a sound of 70–100 dB

HL and this reflex can be recorded. Absence of acoustic

reflex indicates middle ear disorder, retrocochlear

hearing loss or severe to profound SNHL. Used with

behaviour audiometry, acoustic reflexes are useful

component to cross-check. Absence of acoustic reflex,

but a normal tympanometry with parental concern

for hearing loss suggests possibility of SNHL of severe

to profound degree. Absence of acoustic reflex but an

abnormal tympanogram generally indicates conductive

loss. Since ABR and OAEs provide more information,

use of acoustic reflexes in assessment of paediatric

testing has declined.

OAEs and ABR have been used both in screening programmes

and in hearing evaluation in infants and children.

MANAGEMENT

It is essential to know the degree and type of hearing loss,

and other associated handicaps such as blindness or mental

retardation and whether hearing loss is prelingual (before

development of speech) or postlingual. Aetiology of

hearing loss remains obscure in about half the cases.

Aims of habilitation of any hearing-impaired child are

development of speech and language, adjustment in society

and useful employment in a vocation.

1. PARENTAL GUIDANCE. It is a great emotional shock

for parents to learn that their child is deaf. They should

be dealt with sympathetically, so as to accept the child.

They should be told of child’s disability and how to

care for it. Habilitation of the deaf demands a lot from

parents: care and periodic replacement of hearing aid,

change of ear-moulds as child grows, follow-up visits for

re-evaluation, education at home and the selection of

vocation.

2. HEARING AIDS. Most deaf children have a small but

useful portion of residual hearing which can be exploited

by amplification of sound. Hearing aids should be prescribed

as early as possible. If necessary, binaural aids, one

for each ear, can be used. Hearing aids help to develop

lip-reading also.

3. COCHLEAR IMPLANTS (SEE P. 138)

4. DEVELOPMENT OF SPEECH AND LANGUAGE. Communication

is a two way process, depending on the receptive

and expressive skills. Reception of information is through

visual, auditory or tactile faculties while expression is

through oral or written speech or the manual sign language.

In the hearing impaired, auditory faculty is poor or

totally absent (Figure 19.3). Thus, for proper communication,

there is need either to improve hearing through amplification

of the residual hearing or cochlear implants;

and in the absence of the feasibility of developing the auditory

faculty, one has to develop visual or tactile means

of communication.

(a) Auditory-oral communication. This is the method used

by a normal person and is the best way of communication.

In the deaf, it can be used in those with moderate

to severe hearing loss or those who are postlingually deaf. Hearing aids are provided to augment
auditory

reception. At the same time, training is also imparted

in speech reading, i.e. to read movements of lips, face,

and natural gestures of hand and body. Expressive

skill is encouraged through oral speech.

(b) Manual communication. It makes use of the sign language

or finger-spelling method but has the disadvantage

that abstract ideas are difficult to express and

general public does not understand it.

(c) Total communication. It uses all modalities of sensory

input, i.e. auditory, visual, tactile and kinaesthetic.

Such children are taught to develop oral speech, lipreading

and sign language. All children with prelingual

severe to profound deafness, should undergo

training in this form of communication. Vibrotactile

aids are useful for those who are totally deaf and also

blind. These aids are attached to the child’s hand or

sternum and the vibrations of speech are perceived

through tactile sensation.

5. EDUCATION OF THE DEAF. There are residential and day

schools for the hearing impaired. Some children with moderate

hearing loss can be integrated into schools for the normal

hearing children with preferential seating in the class.

Radio hearing aids have revolutionized education of

the deaf. In this device, the microphone and transmitter

are worn by the teacher and the receiver and amplifier

by the child. With this system, the child can hear the

teacher’s voice better, without being disturbed by environmental

noises.

6. VOCATIONAL GUIDANCE. The deaf are sincere and good

workers. Given the opportunity, commensurate with their

ability, they can be usefully employed in several vocations.