Approach to Deaf Child -ow to approach and manage a deaf child

how to approach and manage a deaf child

1. Presenter: Maj Bandana Moderator: Lt Col Kamalpreet Singh 24-09-2022 Dept of ENT ‹#› Approach to Deaf Child o

2. 24-09-2022 Dept of ENT ‹#› Introduction Etiology & Risk factors Screening Evaluation Management Recent Advances Lesson Plan

3. Introduction Permanent childhood hearing impairment (PCHI): confirmed permanent bilateral hearing impairment ≥ 40 dBHL (hearing level) averaged over the frequencies of 0.5, 1, 2 and 4 kHz in the better hearing ear Conductive/Sensorineural or Mixed Onset: Pre/Peri/Post natal Aetiology: Congenital/ Acquired 24-09-2022 Dept of ENT ‹#› Chapter 10, Section 1, Volume 2, Scott Brown ORL HNS,8 th Edition

4. Introduction Pre or Post-lingual deafness Distinction: important implications for education and management Early identification 24-09-2022 Dept of ENT ‹#›

5. Introduction Disabling Hearing Loss Adults - Hearing loss greater than 40dB in the better hearing ear Children - Hearing loss greater than 30dB in the better hearing ear 24-09-2022 Dept of ENT ‹#› Chapter 10, Section 1, Volume 2, Scott Brown ORL HNS,8 th Edition

6. Introduction Deaf/Hard of Hearing All children with hearing loss (includes all types & degrees of hearing loss) 24-09-2022 Dept of ENT ‹#› Flint P, Haughey B, Robbins K, Thomas J, Niparko J, Lund V et al. Cummings Otolaryngology - Head and Neck Surgery. 6th ed. London: Elsevier Health Sciences; 2014

7. 24-09-2022 Dept of ENT ‹#› Importance of Early Identification Watkinson J, Clarke R. Scott-Brown's Otorhinolaryngology and Head and Neck Surgery, Eighth Edition. Milton: Chapman and Hall/CRC; 2018. Neural plasticity Auditory deprivation prevents normal development Fades completely between the age of 6 - 8 years (critical period) Neural Scavenging If child deprived of auditory stimulus early in life: nearby visual cortex area will encroach upon the auditory cortex thereby further leading to deprivation

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11. Screening & Surveillance ‹#›

12. JOINT COMMITTEE ON INFANT HEARING 2007 24-09-2022 Dept of ENT ‹#› Include Auditory Neuropathy Spectrum Disorder. Separate protocols for NICU Rescreening - re-evaluation of both ears. Surveillance Genetic counselling

13. Risk Factors Family history of hereditary Childhood Hearing Loss Prenatal infections (TORCH group) Admission of 48 hours or greater in NICU² Craniofacial abnormalities Ototoxic drugs Birth weight < 1500 gms 24-09-2022 Dept of ENT ‹#› ¹US Joint Committee on Infant Hearing. Year 2007 Position Statement: Principles and guidelines for early hearing detection and intervention programs. Pediatrics 2007; 120: 898–921. 15. 16.

14. UNIVERSAL NEONATAL HEARING SCREENING PROGRAMME 24-09-2022 Dept of ENT ‹#› Hospital based Community based School entry screen Unilateral HL, progressive HL Test-Retest-Confirm paradigm followed at time interval of 6-12-24 weeks.

15. Summary 24-09-2022 Dept of ENT ‹#›

16. History Consanguinity Pregnancy Perinatal, birth & postnatal Family history Immunization 24-09-2022 Dept of ENT ‹#›

17. Aetiology 24-09-2022 Dept of ENT ‹#› Congenital Acquired

18. Aetiology 24-09-2022 Dept of ENT ‹#›

19. Aetiology 24-09-2022 Dept of ENT ‹#›

20. Aetiology 24-09-2022 Dept of ENT ‹#›

21. Syndromic Associations 24-09-2022 Dept of ENT ‹#›

22. Syndromic Associations 24-09-2022 Dept of ENT ‹#› Flint P, Haughey B, Robbins K, Thomas J, Niparko J, Lund V et al. Cummings Otolaryngology - Head and Neck Surgery. 6th ed. London: Elsevier Health Sciences; 2014

23. 24-09-2022 Dept of ENT ‹#› Syndromic Associations Watkinson J, Clarke R. Scott-Brown's Otorhinolaryngology and Head and Neck Surgery, Eighth Edition. Milton: Chapman and Hall/CRC; 2018

24. Clinical Examination Complete head to toe ENT Examination Any dysmorphism in immediate family 24-09-2022 Dept of ENT ‹#›

25. Audiological Tests in Children Age-appropriate Specific Valid Adaptable Fun 24-09-2022 Dept of ENT ‹#›

26. 24-09-2022 Dept of ENT ‹#› Behavioral observation audiometry : 0-6 months The distraction test : 6-18 months Visual Reinforcement audiometry : 5-36 months. Audiological Tests in Children

27. 24-09-2022 Dept of ENT ‹#› Performance testing( Play Audiometry) : 2-5 yrs Pure tone audiometry : 3yrs and above Auditory speech discrimination test : 2.5-3 yrs and above Audiological Tests in Children

28. 24-09-2022 Dept of ENT ‹#› Classification of Hearing Loss Chapter 10, Section 1, Volume 2, Scott Brown ORL HNS,8 th Edition

29. 24-09-2022 Dept of ENT ‹#› Distraction Test Principle - normal response observed when sound is presented to a baby: a head turn to locate the source of sound Not done nowadays due to poor responses

30. 24-09-2022 Dept of ENT ‹#› Visual Reinforcement Audiometry Principle: children can be trained by operant conditioning to produce localizing turn to a visual stimulus in response to a sound stimulus Steps Establish conditioned response Reduce habituation Establish MRL( minimum response level) for each frequency

31. 24-09-2022 Dept of ENT ‹#› Performance testing/ Play Audiometry Till cooperation with PTA Conditioned to wait for sound & then respond with a play activity Minimal threshold response

32. 24-09-2022 Dept of ENT ‹#› Pure Tone Audiometry > 3yrs raise the corresponding hand when sound is heard CHL and SNHL can be differentiated Quick and easy screening test Disadvantages

33. 24-09-2022 Dept of ENT ‹#› Impedance Audiometry Rule out conductive causes- OME

34. Otoacoustic Emission Principle:- Response of outer hair cells to acoustic stimuli Limitations 24-09-2022 Dept of ENT ‹#›

35. 24-09-2022 Dept of ENT ‹#› Automated Auditory Brainstem Response Advantages: Can be done – with background noise. Screening Results- Pass/Refer Disadvantages: Lacks frequency specific information Can’t differentiate type/degree of hearing loss Requires increased preparation time prior to test

36. Brainstem Evoked Response Audiometry 24-09-2022 Dept of ENT ‹#›

37. ASSR Continuous tonal stimulus Multiple frequencies can be assessed simultaneously Response is @ modulation response of stimulus Phase locked response for that carrier frequency 24-09-2022 Dept of ENT ‹#›

38. Imaging- MRI Aim: Look for inner ear bony anomaly Cochleo vestibular nerve deficiency Luminal obstruction (Labyrinthitis ossificans) 24-09-2022 Dept of ENT ‹#›

39. Imaging-CT 24-09-2022 Dept of ENT ‹#› Inner ear bony dysplasia Semi-circular canal absence or dysplasia Internal auditory canal narrowing or dilation Cochlear aperture / bony canal for cochlear nerve Facial nerve anatomy Sigmoid sinus and tegmen plate(surgical planning) Ossicular chain Dilated vestibular aqueduct*(>1.5mm at midpoint or >2mm anywhere along its length)

40. 24-09-2022 Dept of ENT ‹#› Normal Cochlea

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42. Incomplete Partition ‹#› Type I: cochlea without modiolus and interscalar septa, large dilated vestibule. Exter nal dimensions ≈ normal cochlea Type II: Only basal part of modiolus is present. Minimally dilated vestibule, large vestibular aqueduct. “Mondini deformity”. External dimensions ≈ normal cochlea Type III: Interscalar septum present, modiolus completely absent. Cochlea placed directly at lateral end of IAC . External dimensions ≈ normal cochlea.

43. Genetic testing Connexin 26 (GJB-2) Connexin 30 (GJB-6) A1555G 24-09-2022 Dept of ENT ‹#›

44. Other Consultations Paediatric Ophthalmology Clinical Psychology Speech therapist 24-09-2022 Dept of ENT ‹#›

45. Special Tests CMV DNA PCR Urine for microscopic haematuria Renal USG Serological tests for syphilis 24-09-2022 Dept of ENT ‹#›

46. Diagnosis Congenital SNHL Bilateral or unilateral Severity Likely cause if any Syndromic vs Non syndromic ANSD Associated co morbidities 24-09-2022 Dept of ENT ‹#›

47. Management 24-09-2022 Dept of ENT ‹#›

48. Parents Counselling Empathic attitude Assist early decision making Education & genetic counselling 24-09-2022 Dept of ENT ‹#›

49. Interventions Auditory rehabilitation Amplification Implantable devices Speech/language/AVT 24-09-2022 Dept of ENT ‹#›

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52. Hearing Aid Sound amplifying device Mechanism Pick up sound Convert to electrical signals Amplify the sound Convert electrical signals back to sound 9/24/2022 Dept Of ENT

53. Cochlear Implant Severe to profound hearing loss Louder than 90dBHL at 2kHz and 4kHz without hearing aids. At least 3 months of hearing aid use Adequate benefit with hearing aids Multidisciplinary team Simultaneous bilateral CI 24-09-2022 Dept of ENT ‹#›

54. 24-09-2022 Dept of ENT ‹#› ABI Sennaroğlu L, Bajin MD. Classification and current management of inner ear malformations. Balkan medical journal. 2017 Sep 29;34(5):397-411

55. Recent Advances Stem cell therapy 24-09-2022 Dept of ENT ‹#›

56. 24-09-2022 Dept of ENT ‹#› TEAM EYE Spl Speech Therapist Paediatrician Audiologist Clinical Psychologist Parents ENT Spl Team & Holistic Approach

57. Approach 24-09-2022 Dept of ENT ‹#›

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59. Service Aspect CI Policy in AFMS DGAFMS letter no 6022/Cochlear implant/DGAFMS/ DG-3A dt 27 Sep 2016 DGAFMS letter no 6022/Cochlear implant/DGAFMS/ DG-3A dt 08 Jul 2019 9/24/2022 Dept Of ENT

60. Pre-lingual deaf child 2016 Bilateral severe to profound hearing loss. PTA showing hearing threshold of > 90dB at 1,2,& 4 kHz or no identifiable waves at 90dB nHL in ABR 2019 Bilateral severe to profound hearing loss with no identifiable waves on ABR/BERA upto 70dB nHL (s/o hearing threshold of >60dB) in better ear 9/24/2022 Dept Of ENT

61. Post-lingual deafness 2016 Bilateral severe to profound hearing loss with PTA showing hearing threshold of > 90dB at 1, 2 & 4 kHz 2019 Bilateral severe to profound hearing loss with PTA showing hearing threshold of >60dB in better ear at frequencies 0.5 , 1, 2 & 3 kHz 9/24/2022 Dept Of ENT

62. Contraindications 2016 Prelingual deaf adults 2019 Pre-lingual bilateral severe to profound hearing loss in patients >5 years of age who have not undergone auditory habilitation & haven’t developed adequate speech & language 9/24/2022 Dept Of ENT

63. Take Home Message Early Identification Early Assessment Early Rehabilitation 1-3-6 goal 24-09-2022 Dept of ENT ‹#›

64. References 1. Watkinson J, Clarke R. Scott-Brown's Otorhinolaryngology and Head and Neck Surgery, Eighth Edition. Milton: Chapman and Hall/CRC; 2018. 2. Flint P, Haughey B, Robbins K, Thomas J, Niparko J, Lund V et al. Cummings Otolaryngology - Head and Neck Surgery. 6th ed. London: Elsevier Health Sciences; 2014. 3. Sennaroğlu L, Bajin MD. Classification and current management of inner ear malformations. Balkan medical journal. 2017 Sep 29;34(5):397-411. 24-09-2022 Dept of ENT ‹#›

65. References 24-09-2022 Dept of ENT ‹#› 4. US Joint Committee on Infant Hearing. Year 2007 Position Statement: Principles and guidelines for early hearing detection and intervention programs. Pediatrics 2007; 120: 898–921.

66. THANK YOU 24-09-2022 Dept of ENT ‹#›