Evaluation of a deaf child for cochlear implantation. complete workup of a bilateral congenital snhl child for cochlear implantation

1. EVALUATION OF DEAF CHILD
Dr.Poongkamali J
MS ENT-HNS, PG 1yr
AIIMS, Bhopal

2. DEFINITION
o Deaf: A term (with a capital D) used by some people who have little or no useful residual hearing to identify
themselves as members of Deaf Culture
o deaf: A term (with a lower-case d) used in this report to describe people who have little or no useful residual
hearing (i.e., severe or profound hearing loss), whether or not they identify themselves as Deaf.
o Hearing loss: Hearing threshold >25 db.
o Disabling hearing loss: > 30 db in children
o Hard of hearing: mild to severe hearing loss
o Deafness: profound hearing loss

3. WHO/INDIAN CONSTITUTION DEFINITION
WHO DEFINITION
o Deafness- refers to the complete loss of hearing ability in one or two ears.
The cases included in this category will be those having hearing loss more than 90dB
in better ear (profound impairment) or total loss of hearing in both the ears, i.e. profound hearing loss
o Hearing impairment- refers to both complete and partial loss of ability to hear.
ACCORDING TO INDIAN CONSTITUTION
o Hearing handicapped –Hearing impairment of 70dB and above, in better ear or
total loss of hearing in both ears (The Rehabilitation Council of India Act, 1992)
o Hearing disability has been redefined as – a hearing disable person is one who has the hearing loss of 60dB
or more in the better ear for conversational range of frequencies & person with disability means a person suffering from not less than 40%
of any disability as certified by a medical authority. (Section 2(i)(iv) of the persons with Disability Act, 1995)

4. Global Burden
 Over 5% of the world’s population – or 466 million people – has disabling hearing loss (432 million adults and
34 million children).
 The prevalence of deafness in South-East Asia ranges from 4.6% to 8.8%.
 In 2005 WHO estimates-India, 63 million people (6.3%) suffer from significant auditory loss. Four in every 1000
children suffer from severe to profound hearing loss. With over 100,000 babies that are born with hearing
deficiency every year
 The National Sample Survey 58th round (2002) surveyed disability in Indian households and found that hearing
disability was the 2nd most common cause of disability and top most cause of sensory deficit.
 World over, hearing loss is the second leading cause for ‘Years lived with Disability (YLD)’
 60 % of childhood hearing loss is preventable with 75% in developing countries.

5. IMPACT OF HEARING LOSS
 Functional impact- delay in development of receptive & expressive communication skills( speech& language)
 Social and emotional impact- communication difficulties leads to social isolation, loneliness, frustration and poor
self regard
 Academic impact – learning problems that results in reduced academic achievement.
 Economic impact- much higher rates of unemployment or very lower grades of employment with adds on to
decreased productivity along with the costs incurred in educational costs, health sector costs and other
vocational supports.

6. A TEAM WORK
ENT
SURGEO
N
AUDIOLOGIST
PEDIATRICIAN
PSYCHOLOGIST
//PSYCHIATRIS
T
RADIOLOGIST
OPHTHALM
-OLOGIST
NEURO/GENETIC
MEDICIEN

7. CAUSES AND CLASSIFICATION

8. WORK UP BY OTORHINOLARYNGOLOGIST
This Photo by Unknown Author is licensed under CC BY-NC

9. HISTORY TAKING
Informant
Parents
Caregivers
Teachers
Pediatricians
Demographics
Age/DOB
Gender
Address
Date of examination
Handedness
Concern
Hearing
Speech defect
Milestone delay

10. NATAL HISTORY
ANTENATAL HISTORY
o Anomaly scans
o Immunization status
o TORCH infections
o Environmental hazards/ototoxic
drugs/teratogenic agents
o Perinatal infections/PROM/
prolonged labour/
hypothyroidism/gestational
diabetes & other medical
conditions
INTRANATAL HISTROY
o Term/pre term
o Place /mode of delivery
o Birth weight/ APGAR
o Congenital anomalies
o Birth weight <1500gms
POSTNATAL HISTORY
o NICU/PICU stay
o Neonatal jaundice required blood
transfusion
o Exanthematous fever
o Encephalitis/ meningitis
o ENT in infections/noise
exposure/ototoxic drugs
o Blood transfusion/ hospital
admissions
o Immunization details

11. FAMILY HISTORY
o Consanguinity
o Birth order
o Status of other siblings
o Similar complaints in the family if any

12. DEVELOPMENTAL MILESTONES
SPEECH & LANGUAGE
Cognitive functions
physical development
Social/ emotional

13. CLINICAL EXAMINATION
 ENT - wax/perforation/canal atresia/adenoids/
tongue tie/cleft lip palate…..
 VISION
 Integumentary changes
 I/Q, D/Q assessment
 PEDIATRIC ASSESSMET- for complete milestone assessment/
neurological development/
associated congenital anomalies
CAUSES OF CHL
CONGENITAL
• Microtia/atresia
• Ossicular malformations
• Cholesteatoma
ACQUIRED
• Infection
• Otitis media with effusion
• Foreign body
• Trauma

14. AUDIOLOGICAL TESTS
BEHAVIOURAL RESPONSE AUDIOMETRY(0-6 MONTHS)
1. Moro’s reflex
2. Cochleopalpebral reflex
3. Cessation reflex
Has been largely superseded by availability of electrophysiological techniques but, may be useful in auditory spectrum disorder or retro cochlear lesion
where ABR will be of little use.
DISTRACTION TEST (6 – 18 months)
CONDITIONING TECHNIQUES (7months- 2 years)
1. Visual reinforcement audiometry
2. Play audiometry
OBJECTIVE TESTS
1. ABR
2. OAE
3. Impedance audiometry
SPEECH DISCRIMINATION TEST

15. INVESTIGATIONS CONTD….
OTHER TESTS
o Echocardiography/ ECG
o USG KUB & abdomen
o TFT
o Ophthalmic assessment of
vision and fundus
o Serological tests
o Immunological tests
o Metabolic and
chromosomal studies
RADIOLOGICAL TESTS
o HRCT temporal bone
o MRI brain with 3D
reconstruction with 8 th nerve
complex

16. DIAGNOSIS
 Congenital/acquired
 Unilateral/bilateral
 SNHL/CHL/MHL with degree of hearing loss
 Probable etiology
 Associated with speech and language delay
 Associated conditions

17. SCREENING OF HEARING
HNHS
 “High risk Neonatal Hearing Screening”
 Babies born out of high risk pregnancies are
screened.
 Specificity is 50% only
 Cost effective protocol
UNHS
 “Universal neonatal hearing screening”
 All births are screened for hearing impairment
within 48 hrs of birth
 Almost specificity is 100%
 High cost expenditure.

18. HIGH RISK REGISTER
 H/O inutero infections
 H/O ototoxic drug use by mother during pregnancy
 Alcohol intake
 Prolonged/hazardous labour
 NICU admission >/- 48 hrs in first 4 weeks of life
 Birth weight <1500 grams
 APGAR below 4 at 1 minute and below 6 at 5 minutes of
birth
 Any recognizable syndrome
 Family h/o permanent marked SNHL
 Craniofacial anomalies
 Born out of consanguineous marriage
 Parental concern regarding speech, language and hearing
 Delayed developmental milestones
 Identification of any syndrome which has late onset hearing loss
 H/O postnatal infection, neonatal conditions like hyperbilirubinemia
 H/O head trauma
 Recurrent and persistent otitis media with effusion for 3 months
 Ambiguous neonatal screening tests results
Joint Committee on Infant Hearing 1994 Position Statement – Joint Committee on Infant Hearing, American Academy Of
Pediatrics January 1995, 95 (1) 152-156;

19. SCREENING FLOWCHART
SCREENING (Universal+ High risk)- OAE with 48 hrs.
If normal – normal hearing
If abnormal – recheck after 5-7 days
If abnormal- BERA
BERA normal- middle ear pathology
BERA Abnormal- Intervention.

20. REFERENCES:
 https://www.who.int/news-room/fact-sheets/detail/deafness-and-hearing-loss
 https://www.who.int/data/gho/publications/world-health-statistics
 Varshney S. Deafness in India. Indian J Otol 2016;22:73-6
 National Research Council (US) Committee on Disability Determination for Individuals with Hearing Impairments; Dobie RA, Van Hemel S,
editors. Hearing Loss: Determining Eligibility for Social Security Benefits. Washington (DC): National Academies Press (US); 2004. Appendix A,
Definitions and Technical Terms. Available from: https://www.ncbi.nlm.nih.gov/books/NBK207847/
 Garg S, Singh R, Khurana D. Infant Hearing Screening in India: Current Status and Way Forward. Int J Prev Med. 2015;6:113. Published 2015
Nov 19. doi:10.4103/2008-7802.170027
 Consensus Statement of the Indian Academy of Pediatrics on Newborn Hearing Screening- 2017
 Principles and Guidelines for Early Hearing Detection and Intervention Programs .Joint Committee on Infant Hearing .Pediatrics October
2007, 120 (4) 898-921; DOI: https://doi.org/10.1542/peds.2007-2333
 Clinical Audio-Vestibulometry for Otologist and Neurologists- Anirban Biswas
 Scott’s Brown Otorhinolaryngology Head and Neck Surgery.
 https://ghr.nlm.nih.gov/ https://hereditaryhearingloss.org/pds

21. “NO CHILD IS TOO YOUNG TO BE EVALUATED FOR HEARING”
“SHOULD BE SUSPECTED WITHIN 1 MONTH, DIAGNOSED WITHIN 3 MONTH AND INTERVENED WITHIN
6 MONTHS OF AGE”
“EARLY IDENTIFICATION OF HEARING LOSS IS CRITICAL, NOT JUST FOR COMMUNICATION PURPOSES,
BUT FOR A CHILD TO REACH THEIR MAXIMUM HUMAN DEVELOPMENT AND SELF-ACTUALIZATION”
Thank you