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EARLY DETECTION OF DEAFNESS IN CHILDREN AND ROLE OF COCHLEAR IMPLANTATION

EARLY DETECTION OF DEAFNESS IN CHILDREN AND ROLE OF COCHLEAR IMPLANTATION. Dr. Bayya Srinivasa Rao MS(PGIMER). Introduction. Childhood deafness presents special problems:. Need for evaluation & treatment. Anatomy Scalatympani Scalavestibuli Cochlear duct Basilar membrane

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EARLY DETECTION OF DEAFNESS IN CHILDREN AND ROLE OF COCHLEAR IMPLANTATION

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  1. EARLY DETECTION OF DEAFNESS IN CHILDREN AND ROLE OF COCHLEAR IMPLANTATION Dr. Bayya Srinivasa Rao MS(PGIMER)

  2. Introduction • Childhood deafness presents special problems:

  3. Need for evaluation & treatment

  4. Anatomy • Scalatympani • Scalavestibuli • Cochlear duct • Basilar membrane • Tectoralmembrane • Hair cells (outer/inner) • Cochlear nerve fibers

  5. Physiology of Hearing

  6. Neural plasticity For speech articulation: 2-3 years For Listening : 6-8 years.. 2-3 per 1000 newborns will have permanent childhood hearing loss .

  7. Causes of hearing impairment in childhood • Congenital - Genetic {syndromic or non syndromic / recessive or dominant} - Non genetic • Acquired

  8. GENETIC NON SYNDROMIC MEMBRANOUS LABYRINTH DEFECTS BING SIEBENMANN SCHEIBE ALEXANDER FAMILIAL PERCEPTIVE DEAFNESS(HEREDODEGENERATIVE DEAFNESS) MEMBRANOUS &OSSEOUS LABYRINTH DEFECTS MICHEL MONDINI COCHLEAR APLASIA COCHLEAR HYPOPLASIA

  9. Syndromes associated with genetic hearing loss

  10. CONGENITAL • DEVELOPMENTAL:NON GENETIC: • PRE NATAL • INFECTIONS –Toxoplasmosis, rubella, cytomegalovirus, herpes1&2, syphilis, hiv, measles, chickenpox, parvo • Environmental hazards, radiation, diabetes,toxaemia,ototoxicdrugs,teratogenic chemicals • PERINATAL: • Low birth weight, hyperbilirubinaemia, anoxia • NEONATAL: • INFECTIONS – Meningitis,encephalitis,septicemia • Delayed auditory maturation • MISCELLANEOUS- Congenital cholesteatoma

  11. Acquired • Meningitis • Encephalitis • Infantile measles and mumps • Ototoxic drugs • Erythroblastosisfoetalis • trauma • Hypoxia • Infections of inner ear • Noiceinduced • Otitis media • Functional

  12. speech and language milestones • Birth to 3 months • Startles to loud noise (Moros Reflex) • Awakens to sounds(Arousal Test) • Blinks or widens eyes in response to noises(Cochleo Palpebral Reflex) • 3-4 months • Quiets to mother's voice • Stops playing, listens to new sounds • Looks for source of new sounds that are not in sight

  13. 6-9 months Enjoys musical toys Coos and gurgles with inflection Says "mama" 12-15 months Responds to his or her name and the word "no" Follows simple requests Uses expressive vocabulary of 3-5 words Imitates some sounds

  14. 18-24 months Knows body parts Uses expressive vocabulary with 2-word phrases (minimum of 20-50 words) 50% of speech intelligible to strangers By 36 months Uses expressive vocabulary of 4- to 5-word sentences (approximately 500 words) 80% of speech intelligible to strangers Understands some verbs

  15. Failure to achieve these speech and language milestones may indicate hearing loss and necessitate a hearing evaluation

  16. Hearing assessment in children • A) Neonatal screening procedures: Arousal test Auditory response cradle Electric response audiometry B) Behaviour observation audiometry: Moro’s reflex, Cochleo-palpebral reflex, Cessation reflex C) Distraction techniques: D) Conditioning techniques: Play audiometry ,Visual reorientation audiometry E) Objective tests: Evoked response audiometry Impedance audiometry, Otoaoustic emissions Heart rate audiometry

  17. DPOAE

  18. Why Intervene Early? Early experiences have a decisive impact on the architecture of the brain • In the first year of life, neurons in the auditory brainstem are developing • Billions of major neural connections are being formed(number of synapses increases 20 fold to 1,000 trillion). • Newborn brain is in a subcortical state; Areas of cortex responsible for language are well developed by 12 months of age.

  19. Organ of Corti Cochlea Cross-Section No Hair Cells!

  20. What is a Cochlear Implant? • A cochlear implant is an electronic device, that bypasses the damaged hair cells of the cochlea and stimulates the auditory nerve directly. • It can provide the child with useful “hearing” and improved communication abilities in the implant user. • It is a safe, reliable, and an effective treatment for profound hearing loss in children (and adults).

  21. Components of the Implant System Implant body(receiver/stimulator) RF Transmission Coil Speech Processor(BTE shown) Electrode array(inserted to cochlea)

  22. Cochlear: E.N.T. NURSING HOME

  23. MEDEL: E.N.T. NURSING HOME

  24. Advanced Bionics: E.N.T. NURSING HOME

  25. Selection criteria - Children • Twelve months of age or older • Bilateral severe to profound sensory neural hearing loss- congenital or acquired • No or less benefit with the most optimised hearing aid • Aided audiometric thresholds that fall outside speech range at 2kHz • No medical or radiological contraindications • Motivation and good family support.

  26. SELECTION CRITERIA - ADULTS • 18 years old and older (no limitation by age) • Bilateral severe-to-profound sensorineural hearing loss (70 dB hearing loss or greater with little or no benefit from hearing aids for 6 months) • Psychologically suitable • No anatomic contraindications • Medically not contraindicated

  27. CandidacyGuideline Audiograms

  28. Pre surgical Evaluation: • Ear examination to determine cause of hearing loss and absence of infection. • Medical examination for eligibility for surgery and anesthesia. • Hearing ability testing, with and without best-fit hearing aid. • Evaluation of communication skills. • CT scan to determine patency of the cochlea. • MRI • Internal auditory canal contents • Presence of auditory nerve. • Detection of endocochlear obstruction.

  29. The Cochlear Implant Team“Cochlear Implant Program is a Team Approach Program” • The Parents • Need to be involved in all evaluation processes and decisions. Must have realistic expectations and fully understand all aspects of process. • The Surgeon • Full medical and surgical evaluation, surgery and post surgical care • The Audiologist • Full audiological evaluation, hearing aid fitting, Pre implantation counseling, mapping, Hearing habilitation Planning and ongoing follow up • The Auditory Verbal Therapist • Pre and post surgery listening training, Auditory Verbal Practice for individual and full home training program for parents

  30. Normal Cochlea-CT Findings: E.N.T. NURSING HOME

  31. Hi Resolution 3D MR Imaging Technology

  32. Abnormal cochlea Abnormal Cochlear aqueduct

  33. Ossified Cochlea Common Cavity E.N.T. NURSING HOME

  34. Common Cavity:

  35. Mondini Dysplasia:

  36. Michale Deformity:

  37. Posterior tympanotomy E.N.T. NURSING HOME

  38. Cochleostomy

  39. Electrode Array in the Cochlea

  40. Scala Tympani is Good Modiolar Wall Lateral Wall 1 Scala Vestibuli Reissner’s Membrane Scala Media Hair Cells Spiral Ganglion nerve cells Basilar Membrane Scala Tympani To auditory nerve

  41. Scala Tympani isGood Modiolar Wall Lateral Wall 1 Scala Vestibuli Scala Media Scala Tympani

  42. Scala Tympani isGood Electrode 1 Scala Vestibuli Scala Media Electrode Scala Tympani Modiolar Wall Lateral Wall

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