rehabilitation techniques of a deaf child and methods for appropriate screening and management

1. REHABILITATION OF A DEAF
CHILD
PRESENTER: DR ABHIJEET SINGH
MODERATOR: DR DILEEP DUNGALA

2. WHO DEFINITIONS
• HARD OF HEARING - hearing loss ranging from
mild to severe (>20 dB to 90 dB)
• DEAF - profound hearing loss(>90dB)
• DISABLING HEARING LOSS - Hearing loss > 40 db
in the better hearing ear in adults & > 30 db in
the better hearing ear in children.

4. CAUSES OF CHILDHOOD HEARING
IMPAIRMENT

7. BILATERAL PERMANENT CHILDHOOD
HEARING IMPAIRMENT(PCHI)
DEFINITION-
• confirmed permanent bilateral hearing
impairment exceeding 40 dB HL (average of
pure tone thresholds at 0.5, 1, 2 and 4 kHz in
the better hearing ear)

8. • 60 % of these children have a moderate
hearing loss, while the remainder 40% have a
severe or profound loss
• Two-thirds of people with moderate to severe
hearing impairment live in developing
countries

9. HOW TO IDENTIFY PROFOUND CHILDHOOD
HEARING LOSS
• Neonatal screening.
• Infant distraction testing.
• Parental suspicion.
• Pre school screening.

10. • In countries without neonatal screening
– Diagnosis dependent upon parental suspicion,
distraction testing and preschool screening.
• In west: universal neonatal screening
– median age of detection of hearing loss is 10
weeks

11. SCREENING PROTOCOL
• ABR only
• OAE only
• OAE with ABR rescreen only if OAE is failed
• ABR and OAE

12. NEW BORN HEARING SCREENING
• PURPOSE :
1. To identify newborns who are likely to have
hearing loss and who require further
evaluation
2. To identify newborns with medical conditions
that can cause late onset hearing loss and to
establish a plan for ongoing monitoring of
their hearing status

13. • Fail ??
• Refer for a comprehensive audiologic
evaluation, with a goal of having hearing loss
confirmed by 3 months of age.

14. • Pass ??
• Doe s not mean child has normal hearing
• Ongoing surveillance and monitoring
throughout childhood if risk indicators for
late-onset or progressive hearing loss exist
• pass ?? but have risk factors for late-onset
hearing loss??
• require ongoing audiologic monitoring

16. EXPERT PANEL RECOMMENDATIONS ON
NEWBORN HEARING SCREENING (BY ASHA)
• All newborns be screened for hearing loss.
• “1-3-6 time line”
• Hearing screening to be completed no later than
1 month of age
• fail ?? diagnostic audiologic evaluation should be
completed by 3 months of age.

17. • If diagnosed with hearing loss - hearing
technology fitted within 1 month of the
diagnosis and enrol in an early intervention
program by 6 months of age.
• Exception :Newborns in neonatal intensive
care units (NICUs)

18. TARGETED HEARING LEVEL
DURING SCREENING
• The goal of screeing is to identify newborns
who have PROFOUND childhood hearing loss.
• Mild-moderate degree or greater in one or
both ears within the mid-to-high frequency
range (JCIH, 2007).

19. LIMITATION IN SCREENING
• ABR uses a transient, broad-frequency
stimulus with primary contributions from the
mid- to high-frequency range of hearing.
Therefore, newborns with "islands" of normal
to near-normal hearing within those broad
spectra might pass the hearing screen.

22. ADVANTAGES OF ABR COMPARED
WITH OAE
• Accesses more central structures , detects
neurologic involvement.
• Less susceptible to false positives due to ear-
canal debris.

23. DISADVANTAGES OF ABR COMPARED
WITH OAE
• Longer test time.
• Less cost effective than OAE screening,
because of the higher cost of disposables
(electrodes, disposable earphones etc.)
• Increased personnel time.
• False positives for hearing loss, if the infant is
neurologically compromised, but has normal
hearing

25. ADVANTAGES OF OAE AS COMPARED
WITH ABR
• Less expensive than those for ABR.
• Shorter test time.

26. DISADVANTAGES OF OAE AS
COMPARED WITH ABR
• Do not detect neural hearing loss
• Higher refer rate from inpatient screening
compared with the rate for ABRs. (outpatient
re-screening)
• OAEs may be reduced or absent due to outer-
and middle-ear debris.

28. ADVANTAGES AS COMPARED WITH
ABR AND OAES ONLY
• Lower Refer rate.
• Cost of program may be lower than using ABR
only.

29. DISADVANTAGES AS COMPARED WITH
ABR AND OAES ONLY
• Two pieces of equipment are needed.
• Personnel must be trained on two procedures.
• Because the tests used currently differ in
detecting different degrees of hearing loss, a
baby may not pass the OAE test but pass the ABR;
hence, the test may miss some mild hearing loss
• Will not detect neural hearing loss.

31. ADVANTAGES AS COMPARED TO ABR
ONLY AND OAES ONLY
• OAEs may be sensitive to mild hearing loss
and ABR screens for neural hearing loss.

32. DISADVANTAGES AS COMPARED WITH
ABR ONLY AND OAES ONLY
• Need to purchase screening equipment that can
perform both measures (purchase and maintain
two pieces of equipment or one device that does
both tests).
• Personnel must be trained on both technologies.
• Takes more time.
• Fail rates may be higher, because two screening
tests must be passed

34. INFANT DISTRACTION TESTING
• Health visitor check at the age of 9 months
within the community.
• Most common mode of detection was a child’s
failure to respond to sound (49%)
• Neither high sensitivity nor specificity

35. INVESTIGATIONS

36. Guidelines by The British Association
of Audiovestibular Physicians (BAAP)
APRIL 2015

37. LEVEL 1 INVESTIGATIONS
• Clinical history
• Clinical examination
• Family audiograms of parents and siblings
• ECG - prolongation of the corrected QT
interval (essential in children with vestibular
hypofunction)
• Ophthalmological assessment - electro-
retinography if evidence of vestibular
hypofunction.

40. • Urine examination - for microscopic haematuria
and proteinuria
• CMV testing
• Blood test for GJB2 [Connexin 26] mutations
• Imaging
– MRI of Internal Auditory Meati and Brain [in SNHL] or
– CT Scan of Petrous Temporal bone [in permanent CHL]
or
– Both MRI and CT in bacterial meningitis.

42. LEVEL 2 INVESTIGATIONS
• Serology [Syphilis, HIV, Rubella, Toxoplasma]
• Haematology/Biochemistry - Not
recommended as routine, (TFT -if Family h/o
thyroid disease, Goitre or Mondini deformity)
• Investigation into autoimmune disease
• Metabolic screen on blood and urine (if
epilepsy,Neuroregression)

43. • Chromosomal studies/CGH microarray (If
developmental delay/dysmorphism)
• Further genetic testing (if Exposure to
aminoglycoside,Progressive hearing
loss,mother/sibling with SNHL,high frequency
SNHL)
• Renal ultrasound (if Preauricular pits/sinuses,
deformity of ear, branchial cleft or cysts,
Mondini defect, permanent conductive or
mixed hearing loss, syndrome with kidney
involvement e.g. CHARGE)

44. • Referral to a Geneticist (if Parental
consanguinity, syndromic child, multiple
abnormalities, parental request, opinion
required on interpretation of genetic mutation
testing, after completion of investigations no
cause has been identified)
• Vestibular investigations (if Delayed motor
milestones, progressive deafness, conditions
associated with vestibular dysfunction e.g.
post-meningitis, vertigo/dizziness, temporal
bone malformations)

45. • The aims of aetiological investigations are as
follows:
– To try to answer parents who ask ‘why is my child
deaf”?
– To identify and treat medical conditions, e.g. 8th
nerve aplasia, congenital infection, Jervell and
Lange–Nielsen syndrome, Alport’s syndrome,
neurofibromatosis type 2, Usher syndrome and
vestibular hypofunction

46. – To assist the family in making decisions about the
most appropriate communication mode,
educational placement and counselling on
cochlear implantation, e.g. in 8th nerve aplasia,
Usher syndrome
– To inform genetic counselling
– To inform epidemiological research

47. GUIDELINES FOR AETIOLOGICAL INVESTIGATION
INTO BILATERAL SEVERE TO
PROFOUND PERMANENT CHILDHOOD HEARING
IMPAIRMENT
• Defined as hearing loss >70 dB HL in the
better ear averaged across 0.5, 1, 2 and 4 KHz.
• Level 1 investigations - all children
• Level 2 – children with specified indications.

48. GUIDELINES FOR AETIOLOGICAL
INVESTIGATION INTO MILD TO MODERATE
PERMANENT CHILDHOOD HEARING
IMPAIRMENT
• Level 1 investigations
• All except ECG.
• Level 2 investigations
• Same as above.

49. GUIDELINES FOR AETIOLOGICAL INVESTIGATION
INTO UNILATERAL
PERMANENT CHILDHOOD HEARING
IMPAIRMENT
• Level 1 investigations
• All except ECG, urine ex. & blood test for GJB
2.
• Level 2 investigations
• Same as above.

50. SUPPORT AND EDUCATION

51. THE NEWLY DIAGNOSED DEAF CHILD
• Dilemmas and challenges
• Psychosocial stress in the parents and other
family members
– Grieving process akin to a death experience
• Loss of expectations of a ‘perfect’ baby
• Feelings elicited: fear, shock, confusion,
depression, frustration, anger, loneliness,
sadness, blame and even agression.

52. • Risk of disturbing the parent child relationship
by early screening could be minimized by
improved information and rapid and effective
follow up
• Deaf children of deaf parents who use sign
language, language will be acquired naturally
through the visual route.
• For deaf children of deaf parents: language
learning may be easier and more effective.

53. RATIONALE FOR INTERVENTION
• Deafness: different views-
– As a medical condition to be ‘cured’
– As a cultural and linguistic identity to be valued
rather than ‘cured’
• Educational models of deafness and models of
intervention often reflect these differences
• Today’s technology of early diagnosis,
effective hearing aids and cochlear implants
emphasizes the medical model of deafness

54. • Traditional view of the deaf community:
– Threat in which sign language with its own
grammar and culture will no longer have a place
• Newer technology:
– Offers opportunities to move forward from the old
arguments
– Offers deaf children real potential to develop
spoken language, while not denying the value of
sign language

55. MODE OF INTERVENTION

57. MANAGEMENT OF CONDUCTIVE
HEARING LOSS
• 90 – 95 % of childhood HL
• M/C cause – Otitis Media with Effusion
• CONGENITAL CONDUCTIVE HEARING LOSS
1. Major malformations – CAA, EAC stenosis
2. Minor malformations ( normal auricle &
intact EAC & TM )

58. MINOR ANOMALIES

59. • ACQUIRED CHL
1. OME
2. COM ( usually U/L )

60. INVESTIGATIONS
• HRCT temporal bone.
• Exploratory tympanotomy: gold standard.
• Identify conditions not amenable to Sx
(enlarged vestibular aquaduct & SCDS &
Absent oval window & persistent stapedial
artery)

61. TREATMENT
• Amplification & bone conduction technology.
• Preferential seating in class.
• FM systems.
• Individualized education plans

62. • TM perforation – Sx (worse outcome in < 4 yrs
).
• Tympanosclerosis – observation,
amplification, SX.
• Cholesteatoma – Priority is achieving safe ear.

65. MANAGEMENT OF CHILDREN WITH
MILD, MODERATE, AND MODERATELY
SEVERE HEARING LOSS.
• NEW BORN SCREEN targets permanent B/L or
U/L hearing loss > 40dB.
• Evidence shows: even mild degrees of B/L or
U/L permanent HL causes psychological,
educational & behavioural difficulties.

66. MINIMAL OR MILD B/L HEARING LOSS
• Pure Tone Audiogram (0.5,1 & 2 kHZ) between
20 and 40 db HL or
• High frequency (> 2 kHZ) HL with PTA
thresholds > 25 db HL at 2 or more
frequencies

67. COMMUNICATON MODE
1. Listening & spoken language.
2. Visual or manual communication.
3. Combination of oral & manual
communication (total communication).

68. HEARING ASSISTIVE TECHNOLOGY
1. Hearing aids.
2. Remote microphone technology.
3. Cochlear implantation.

69. HEARING AIDS
• Behind The Ear Hearing Aid ( M/C type used
by children ).
• In The Ear Hearing Aid
1. Fits in the canal or concha.
2. American academy of audiology doesn’t
recommend in young
3. Swallowing risk because of small size.

70. • For B/L HL - B/L Hearing Aid ( American academy
of audiology recommendation ).
• F/U – every 3 months during first 2 years of
amplification use (the paediatric working group
recommendation ).
• Monitoring & ear mould replacement in growing
child.
• Aim Full time use – maximum auditory
experience.

72. • Clinician involved in the fitting of hearing aids
or implants should ensure that the child’s
teacher of the deaf
– understands the technology
– can carry out simple trouble-shooting
– has access to spares to ensure that the child is not
without their amplification during the day
• Current annual production of hearing aids is
estimated to meet less than 10 % of global
need

73. REMOTE MICROPHONE TECHNOLOGY
• To counter difficulty in undetrstanding speech
in suboptimal conditions (noisy).
• Optimal learning require improved signal
noise ratio (20 dB).
• Microphone near speakers mouth
• Wireless head set (personal system) or
loudspeaker positioned near listener (audio
distribution system)
• Candidate ?? Any child who wear HA.

74. COCHLEAR IMPLANTATION
• Fall below FDA guidelines for paediatric
Implantation criteria in mild to severe
hearing loss.
• Using HA on full time basis but not making
expected progress –may be a candidate*
*Carlson ML, Sladen DP, Haynes DS, et al. Evidence for the expansion of pediatric cochlear implant candidacy.
Otol Neurotol 2014;36(1):43–50.

75. ONGOING SURVEILLANCE
(The JCIH recommendation )
• Monitoring for auditory skills, middle-ear
status, and developmental milestones at each
visit
• A validated global screening tool should be
administered - at 9, 18, and 24 to 30 months
or at any time if there is health care
professional or family concern about hearing
or language.
• Do not pass or if there is physician or caregiver
concern - referred immediately for further
evaluation.

76. • Siblings of an infant diagnosed with HL - referred
for audiological evaluation.
• All infants with risk indicators for hearing loss,
regardless of surveillance findings, should be
referred for an audiological assessment at least
once by 24 to 30 months of age.

78. MANAGEMENT OF CHILDREN WITH
SEVERE – PROFOUND HEARING LOSS
GOAL :
1. Introduce adequate sound for auditory –
verbal speech development or
2. Teach child & caregivers alternative
communication strategies.

79. COMMUNICATION OPTIONS &
INTERVENTION :
• Verbal strategies
1. Auditory (Amplification or Cochlear implantation
or Auditory brain stem implantation)
2. Non auditory [ Lip reading ].
• Non verbal strategies [ sign language ].

80. • Amplification/Cochlear Implantation – highest
level of educational achievement &
employment opportunities.
• 95% - Born to hearing parents & auditory –
oral strategy is the dominant choice.
• Fitting HA – before 3 months of age &
intervention program by 6 months.

81. • Close observation for speech & language
progress (by well trained profesionals,
audiologists, speech – language pathologists,
ENT)
• Watch for OME
– Prolonged waiting is inappropriate
– Early tympanostomy tube

82. COCHLEAR IMPLANTATION
• Adequate progress with Hearing Aid alone –
observe.
• No age appropriate gains – Cochlear
Implantation.
• If implanted around 12 months – language
acquisition rates match normal hearing peers.
• Worse outcomes – beyond 2 years.

83. SIMULTANEOUS OR CLOSE B/L
SEQUENTIAL IMPLANTATION
1. Improved sound localization & hearing in
noise.
2. Improved speech & language acquisition.
3. Enhanced incidental learning.
4. Built – in backup if one device fails.

84. REHABILITATION & RECOVERY
• Intense period of work with CI audiologists &
speech therapists.
• Deep commitment from families
• Regular visits to achieve stable & appropriate
implant map
• Education sessions for child and family

85. OUTCOMES
• Sound awareness & open set speech – within
1 to 2 years.
• Realistic individualized expectations

86. • Rate of acquisition & max. achieved level –
vary with
– Age at implantation
– presence & type of cochlear malformations
– degree of parental involvement
– perinatal CMV
– post meningitis
– other medical comorbidities or developmental
delays.

87. ELECTROCOCHLEOGRAPHY
• Intra operative electrocochleography – for
measuring cochlear health during surgery
• As a way to predict speech perception scores
& explain previously unexplained outcome
post implantation

89. • Stable map achieved – regular audiometric &
speech & language evaluations.
• Underperformance / regression : look for
electrode – neural interface problems
– patient factors [poor compliance]
– audiologic [incorrectly assigned mapping], device
issues.
• When all reversible are excluded – consider
soft failures.

90. AUDITORY NEUROPATHY SPECTRUM
DISORDER
• Intact outer hair cell function (presence of
otoacoustic emissions) and/or cochlear
microphonics
• Poor eighth nerve brainstem response (absent
or highly abnormal auditory brainstem
responses)
• Absent or elevated middle ear muscle reflexes
• Abnormal medial olivocochlear reflexes

93. GUIDELINES FOR AETIOLOGICAL INVESTIGATION
INTO UNILATERAL
PERMANENT CHILDHOOD HEARING
IMPAIRMENT
• Level 1 investigations
• All except ECG, urine ex. & blood test for GJB
2.
• Level 2 investigations
• Same as above.

95. THE EDUCATION OF DEAF CHILDREN
• Subject of controversy
• Where and how deaf children should be
taught
• Traditionally, deaf children were educated in
special residential schools
• Second half of the twentieth century: more
deaf children were educated in integrated
settings eg: FM system using microphone

96. • INCLUSIVE EDUCATION and LEAST
RESTRICTIVE ENVIRONMENT: used
internationally and supported by the legal
requirement in many developed countries for
deaf children to have an individual education
program with their needs identified
• Major decision is communication mode:
whether to use a signed or oral approach

97. • Milan conference,1860
– Uncontestable superiority of speech over sign
• DRAWBACKS: speech over sign language-
• Poor linguistic and educational outcomes
• Increasing voice of the deaf community,
promoting its own culture and language
• Sign language has a grammar of its own and
cannot be used with spoken language

98. • Spoken language is a realistic outcome from
cochlear implantation
• Interestingly, use of sign language appears to
be valued by both parents and young people
with implants and it does not appear to be the
case that early signing will impede the
development of spoken language
• Rather, there is early development of effective
communication which is important in early
intervention, whether it is oral or signed

99. • Implantation is improving access to spoken
language, there remain educational challenges
to face
• Recent evidence: children with implants
– Did better than those without, particularly in
mathematics
– Better in reading than those without
– Not doing as well as their hearing peers in
mainstream schools

100. NATIONAL PROGRAMME FOR
PREVENTION AND CONTROL OF
DEAFNESS
[NPPCD]

101. • The MOHFW, Govt. of India - launched the
pilot phase from 2006 to 2008 in 10 States
and 1 Union territory during 11th Five Year
Plan.
• In the 12th Plan - proposed to expand the
Programme to additional 200 districts in a
phased manner probably covering all the
States and Union territories by March, 2017.

102. OBJECTIVES
• To prevent the avoidable hearing loss on
account of disease or injury.
• Early identification, diagnosis and treatment
of ear problems responsible for hearing loss
and deafness
• To medically rehabilitate persons of all age
groups, suffering with deafness.
• To strengthen the existing inter-sectoral
linkages for continuity of the rehabilitation
Program, for persons with deafness

103. • To develop institutional capacity for ear care
services by providing support for equipment and
material and training personnel.
• Long term objective: To prevent and control
major causes of hearing impairment and
deafness, so as to reduce the total disease
burden by 25% of the existing burden by the end
of 12th Five Year Plan.

104. COMPONENTS OF THE PROGRAMME
1. MANPOWER TRAINING AND DEVELOPMENT– For prevention,
early identification and management of hearing impaired and
deafness cases, training would be provided from medical college
level specialists (ENT and Audiology) to grass root level workers.
2. CAPACITY BUILDING – For the district hospital, community health
centers and primary health center in respect of ENT/ Audiology
infrastructure.
3. SERVICE PROVISION – Early detection and management of
hearing and speech impaired cases and rehabilitation, at
different levels of health care delivery system.
4. AWARENESS GENERATION THROUGH IEC/BCC ACTIVITIES – For
early identification of hearing impaired, especially children so
that timely management of such cases is possible and to remove
the stigma attached to deafness.– For the district hospital,
community health centers and primary health center in respect
of ENT/ Audiology infrastructure.

105. EXPECTED BENEFITS OF THE
PROGRAMME
1. Availability of various services like prevention, early
identification, treatment, referral, rehabilitation etc. for hearing
impairment and deafness as the primary health center /
community health centers / district hospitals largely cater to their
need.
2. Decrease in the magnitude of hearing impaired persons.
3. Decrease in the severity/ extent of ear morbidity or hearing
impairment.
4. Improved service network/referral system for the persons with
ear morbidity/hearing impairment.
5. Awareness creation among the health workers/grass root
level workers through the primary health centre medical
officers and district health officers, which will percolate to the
lower level health workers functioning within the community.
6. Capacity building at the district hospitals to ensure better care

106. GUIDELINES TO PRESCRIBE BTE HEARING AIDS UNDER
NPPCD
FOLLOWED BY THE ENT SURGEONS
• Family income should be less than Rs. 6,500/- per
month to obtain the hearing aid free of cost. However,
testing for hearing aids prescription for hearing
aidsshall be provided to all irrespective of income
• The Hearing Aids will be provided only to the Hearing
Impaired children (upto theage of 14 years)
• Hearing aids are to be given only after an ENT
clearance and not to be given incase there is active ear
discharge or any external ear infection
• A person is a candidate only if he has a SNHL or a CHL
which cannot be treated medically or surgically.

107. • Separate guidelines are provided to fit the adults,
children who cannot be conditioned to respond,
and children who have limited or no speech
• All the cases who have been prescribed and
issued a hearing aid have to be counselled
regarding the following
– Optimum use, care and maintenance of the hearing
aid including the ear mould
– Working on acceptance of amplification
– Expectations from the device
– Trouble shooting of the device
– Utilizing the monthly service provision by the
manufacturer of the hearing aid as made under
NPPCD
– Reporting back to the Audiologist / ENT surgeon in
case of any problems with the hearing aid.

108. • Replacement / reissue of a hearing aid will be
done only after 3 years of usage.
• The aided score should show an improvement
of 30% over the unaided score.

109. SOUND HEARING 2030

110. • Started in 2006, for the prevention and
elimination of hearing impairment in the south
east asia region.
• Permanent organization with representations
from IFOS, SAARC ent society, ASEAN ORL-HNS
society, Governments, WHO, INGOS & NGO and
active individuals.
• The scope of work spreads to 11 WHO south east
asian countries, with national committees already
formed in india, indonesia and bangladesh.

111. • The “goal” is to reduce avoidable hearing
impairment to 50% by 2015 and to 90% by 2030.
• The objective of the project is to promote the
right to better hearing through Inclusive
development and sustainable ear and hearing
care programmes with National efforts in India,
Indonesia, Bangladesh, Nepal, Sri Lanka, Thailand
and Myanmar in the five years (2010-2014)

112. REFERENCES
• Scott brown 7 th edition
• Cummings 6 th edition
• Asha guidelines for neonatal screening
• Baap guidelines for evaluation of deaf child
• Ocna 2015
• Mohfw website

113. THANK YOU