1. Pediatric Ear Surgery: Cochlear Implants & New Ways to Manage Cholesteatoma
Rick F. Nelson, MD, PhD
Assistant Professor
Department of Otolaryngology—Head and Neck Surgery
Division of Neurotology & Skull Base Surgery

2. Financial Disclosures
None
9/20/2018

3. Outline Hearing Loss & Cochlear Implants Cholesteatoma
Hearing screening/testing
Candidacy
New Implant Technologies
Procedure and Outcomes
Cholesteatoma
Diagnosis
Novel methods of treatment
Case example
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4. Question #1
The newborn hearing screen tests the function of which of the following?
Cochlear Nerve
Outer hair cells
Stria Vascularis
Inner hair cells
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5. Question #2
True or False: The earliest recommended age for deaf children to undergo cochlear implantation is 2 years?
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6. Question #3
Canal wall reconstruction tympanomastoidectomy allows which of the following?
Low rate of recurrent cholesteatoma
Preservation of the ear canal anatomy
Children to participate in water activities
All of the above
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7. Newborn Hearing Screen
All newborns should have bilateral hearing screen prior to discharge
Today 98% of US born children are screened
OAE or Automated ABR
Early Identification
Early Intervention
Improved language development
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8. Hearing—Anatomy
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9. The cochlea has tonotopic mapping
Hearing—Anatomy
The cochlea has tonotopic mapping
Low frequency
High frequency
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10. Hearing—Anatomy Outer hair cells function to augment frequency tuning
Contractile cells
This contraction generates a measurable sound
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11. OAE OAE measure outer hair cell function
OAE measure outer hair cell function
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12. Confirmatory testing—ABR
Goal: before 3 months of age
Auditory brainstem response (ABR)
Tests the entire hearing pathway to the brain
Tests each ear
Can determine hearing level (mild vs. profound)
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13. Hearing Loss in Newborns-Indiana
CDC data 2013
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14. Introduction to Cochlear Implants Cochlear Implant Candidacy
Cochlear Implant Outcomes
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15. Hearing with a Cochlear Implant
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16. Cochlear Implant

17. You Hear with the Brain!
The cochlear implant replaces the function of the cochlea and acts as a mechanism to transmit an auditory signal to the brain.
The brain does all the work for processing and understanding the signal from the CI therefore, auditory adaptation and post-op rehabilitation are integral in order to achieve successful CI use.
The cochlear implant delivers electrical current in the form of digital pulses to the cochlea. It is the brain’s job to decode these pulses into meaningful sound.
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18. Normal Hearing vs. Cochlear Implant
20-20,000 Hz at different locations across the basilar membrane
Normal Pitch Perception:
Conveyed by rate and place of stimulation
Intact Auditory System
Cochlear Implant:
250 – 8,000 Hz at different locations across the electrode array
Cochlear Implant Pitch Perception:
Rate pitch perceived up to only Hz
Place pitch perceived in the relative order of electrodes
Impaired Auditory System (higher level pathways intact?)
The CI does not restore normal hearing!!!
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19. CI Surgery
Mastoidectomy
Facial Recess
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20. CI Surgery
Round Window
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21. Cochlear Implant placed through Round Window
CI Surgery
Cochlear Implant placed through Round Window
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22. Introduction to Cochlear Implants Cochlear Implant Candidacy
Cochlear Implant Outcomes
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23. Audiological Criteria for CI Candidacy
Current Standard Implantation
Current Hybrid Implantation
AGE
Adults
Children (>12 mo)
Adults > 18 years
ONSET of
Hearing Loss
Adults & Children
Pre & Post-lingual
Post-lingual
DEGREE
of SNHL
Severe-Profound: >2 yrs
Profound (>90dB): < 2 yrs
Normal- moderate LFHL (<60dBHL)
Severe- Profound Mid/high freq HL HF ave. 2k, 3k, 4kHz > 75 dBHL
(<60 dBHL in non-implant ear)
ADULT
Open-set Sentences
Pre-op
Best aided
< 50% sentence recognition in implanted ear
< 60% contra ear/bin.
CNC word recognition score >10% and <60%, in the ear to be implanted in the preoperative aided condition
<80% in the non-implant ear
PEDIATRIC
Speech Scores
Lack of auditory progress
< 30% (MLNT/LNT)
Not currently indicated
These current criteria are much more broad than in the early days when patients did not qualify for CI unless they had profound HL and no open-set speech recognition.
UTMOST IMPORTANCE FOR YOU AS the referring physician—patients MUST undergo at least a 3-month trial with appropriately fitted hearing aids before they can be considered for a CI.
Note—these are FDA guidelines; teams may implant off label at their discretion. Insurance companies may deny coverage though based on these guidelines, e.g., won’t allow surgery prior to age 12 months.
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24. Current Standard Implantation Current Hybrid Implantation
Expanding Criteria
Current Standard Implantation
Current Hybrid Implantation
AGE
Adults
Children (>12 mo)
Adults > 18 years
ONSET of
Hearing Loss
Adults & Children
Pre & Post-lingual
Post-lingual
DEGREE
of SNHL
Severe-Profound: >2 yrs
Profound (>90dB): < 2 yrs
Normal- moderate LFHL (<60dBHL)
Severe- Profound Mid/high freq HL HF ave. 2k, 3k, 4kHz > 75 dBHL
(<60 dBHL in non-implant ear)
ADULT
Open-set Sentences
Pre-op
Best aided
< 50% sentence recognition in implanted ear
< 60% contra ear/bin.
CNC word recognition score >10% and <60%, in the ear to be implanted in the preoperative aided condition
<80% in the non-implant ear
PEDIATRIC
Speech Scores
Lack of auditory progress
< 30% (MLNT/LNT)
Not currently indicated
These current criteria are much more broad than in the early days when patients did not qualify for CI unless they had profound HL and no open-set speech recognition.
UTMOST IMPORTANCE FOR YOU AS the referring physician—patients MUST undergo at least a 3-month trial with appropriately fitted hearing aids before they can be considered for a CI.
Note—these are FDA guidelines; teams may implant off label at their discretion. Insurance companies may deny coverage though based on these guidelines, e.g., won’t allow surgery prior to age 12 months.
9/20/2018

25. Expanding Criteria—Hybrid CI
Adults > 18 years
Normal- moderate LFHL (<60dBHL)
Severe- Profound HL in the Mid/high freq (HF ave. 2k, 3k, 4kHz) > 75 dBHL in ear to be implanted AND <60 dBHL in non-implant ear
Pre-operative CNC word recognition scores >10% and <60%, in the ear to be implanted and <80% in the non-implant ear (best aided condition
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26. Expanding Criteria—Single-Sided Deafness
CI for Single sided deafness
Improvements in:
Hearing on CI side
Sound localization
Speech-in-noise understanding
Realistic Expectations
Involves great deal of counseling
Normal/near-normal hearing in contralateral ear
Costs/Insurance Approval
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27. Evaluation of CI Candidacy
Patient History
Audiological Evaluation (CIHAE)
Radiological Studies (CT, MRI)
Medical Evaluation
CI Orientation
Cognitive Assessment (when appropriate)
Inter-Departmental Consultation
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28. Vaccination All children with CI should be vaccinated for S. Pneumo
<2 years:
Prevnar (PCV13)
2-5 years:
Prevnar (PCV13) + PNEUMOVAX (PPV23) 2 months later
>5 years:
PNEUMOVAX (PPV23)
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29. Introduction to Cochlear Implants Cochlear Implant Candidacy
Cochlear Implant Outcomes
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30. Minimum Expected Benefits of CI
Awareness of environmental sounds
Detection of speech sounds
Awareness of music
Improved speech reading abilities
Awareness of own voice
Potential for improvement in speech intelligibility
Potential for telephone use
Note that we chose our words carefully, i.e., ‘awareness, detection, improved, potential’. We never promise any patient that they will understand speech without the need for lipreading or that they will hear better on the phone, in noisy environments or will enjoy music. Many patients do achieve these goals, but we cannot reliably predict which patients will be able to do so.
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31. Cochlear Implant Outcomes
Variables that affect level of performance:
Duration of deafness/age at onset of deafness
Age of implantation
Residual hearing prior to implantation/Previous auditory experience
Etiology (ossification and cochlear anatomy)
Presence of other learning difficulties or cognitive deficits
Family involvement
Therapy, education, and emphasis on auditory learning
Consistency of device use
Variables that do not appear to affect performance:
Device
Number of functional electrodes (if greater than 6-8)
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32. Taken from Niparko, et al, JAMA, 2010;303(15):1498-1506
Language Outcomes
Taken from Niparko, et al, JAMA, 2010;303(15):
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33. Taken from Niparko, et al, JAMA, 2010;303(15):1498-1506
Language Outcomes
Taken from Niparko, et al, JAMA, 2010;303(15):
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34. CI Outcomes – Audiometric Results
The detection levels for a cochlear implant user typically fall between 25dB to 35dBHL, which is equivalent to a mild hearing loss.
Sound detection does not guarantee comprehension.
An implant user will not necessarily understand all the sounds that they detect!
Significant auditory training and speech therapy are necessary for a cochlear implant user to develop new listening and speaking skills with their cochlear implant. CI CI CI CI CI CI 34

35. The Indiana University Cochlear Implant Team
Clinical Team
Rick F. Nelson, MD, PhD
Charles W. Yates, MD
Jake P. Dahl, MD, PhD
Wendy Myres, MAT, CCC-A
Kelly Lormore, MS, CCC-A
Katie Jones, AuD, CCC-A
Kim Wolfert, MS, CCC-A
Ann Kalberer, MS, CCC-A
Kimberly Cave
Francia Ware
Carol Fulford, RN
Emily Minniear, RN
Heather Humphery, RN
Research Team
David Pisoni, PhD
Shirley Henning, MA, CCC-SLP
Mary Sanders, PhD
Questions So Far?
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36. Cholesteatoma
Keratinizing squamous epithelium in the middle ear or mastoid
NORMAL
Attic Cholesteatoma
Perforation with middle ear Cholesteatoma
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37. Mastoid filled with cholesteatoma
Can be very destructive to the following:
Ossicles
Inner ear
Tegmen—bone between ear and brain
Mastoid filled with cholesteatoma
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38. Case
Audiogram
17 yo F with progressive hearing loss in right ear for >3 years.
No history of ear surgery
Exam shows intact ear drum with mass in middle ear
Pre-op
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39. Case CT confirmed mass in middle ear and mastoid
Dx: Congenital Cholesteatoma
Which surgical approach should be used for this child?
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40. Surgical Management of Cholesteatoma
Atticotomy- Limited disease
Canal up or canal wall reconstruction- default procedure.
Canal wall down- only hearing ear, complicated disease, or posterior canal wall erosion
Subtotal petrosectomy- (Removal of all air cells and closure of Eustachian tube and external auditory canal)
Dead ear
CSF leak in CWD cavity
Severe, recalcitrant otorrhea- Use BAHA for hearing rehabilitation
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41. COM with cholesteatoma
Management Issues
Gold Standard = Open cavity (CWD)
Closed Cavity procedures
CWU recidivism rate = 30-70%
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42. Canal Wall Up (CWU) Combines cortical mastoidectomy & tympanoplasty
Avoids open mastoid cavity
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43. COM with Cholesteatoma
Intact Canal-wall Mastoidectomy (CWU)
Advantages:
Better hearing results???
Keep ear canal intact—Allows for swimming
No long term yearly maintenance
Disadvantages:
2-Stage procedure
High recidivism (30-70%)
Poor visualization of anterior tympanum and attic
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44. Canal Wall Down (CWD)
Creates an open cavity between external auditory canal and mastoid cavity
Requires lifelong periodic cleaning (every 6-12 months)
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45. COM with Cholesteatoma
Open Cavity Mastoidectomy (CWD)
Advantages:
1 stage procedure
Wide exposure of tympanum, attic, mastoid
Reduced recidivism
Disadvantages:
Hearing results???
Long term debridement
More inconvenient for patient
No swimming
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46. Canal Wall Recon Tympanomastoidectomy with Mastoid Obliteration
Improves intra-operative exposure
Preserves posterior canal wall post-operatively
Reduced recidivism rate
Blockage of epitympanum/attic and facial recess to prevent re-retraction of TM
Mastoid obliteration reduced N2 resorptive capacity
Historic increased risk of infection
Requires 2nd look procedure
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47. Canal Wall Up (CWU) view
Mastoidectomy
Cholesteatoma
Canal wall
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48. Canal Wall Reconstruction (CWR) view
Middle ear/stapes
Canal wall cuts
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49. CWR Surgical Technique Complete Mastoidectomy, collect bone pate
Extended facial recess
Elevate posterior canal skin forward- no incisions
Remove posterior canal wall with microsaw
Silastic spacer and fascia for reconstruction
Replace posterior canal wall
Block attic with cortical bone chips
Fill mastoid with bone pate
Pack external canal with iodoform/bacitracin gauze
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50. Exposure Large Anterior Based Palva Flap
Incision in hairline provides wide access to mastoid cortex
Large Anterior Based Palva Flap
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51. Collect Bone Pate
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52. Bone Chips from Outer Cortex
Bone Chips from Cortex
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53. Elevate canal skin
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54. Canal Cuts
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55. Remove Posterior Canal
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56. Exposure of Zygomatic Root and Epitympanum
Open Cavity View
Canal cut
Canal cut
Exposure of Zygomatic Root and Epitympanum
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57. Temporalis Fascia for Tympanoplasty
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58. Reconstruction
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59. Reconstruction
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60. Reconstruction Replace Canal Wall Mastoid tip bone-
Scutum to Facial Ridge
Bone Chips
Facial Recess
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61. Reconstruction
Bone pate
filling mastoid
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62. Pack External Canal
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63. The “2nd look”
Generally performed 6-9 months following initial CWR procedure
Goals:
Rule out recurrent middle ear cholesteatoma
Ossicular reconstruction after middle ear has become well-mucosalized
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64. Previous CWU Tympanomastoidectomy
Results
Total Ears
285
Mean Age
35±18
(2-80)
Male
175
(64%)
Female 98
(36%)
<18 years 70
(25%)
Previous CWU Tympanomastoidectomy 57
(20%)
Previous atticotomy 9
Study period
285 ears in 273 patients
Mean F/u = 4.3 yrs (range 1-15 years)
37 pts followed 9.5yrs
OCR performed in 253 ears (89%)
Walker, et al, Otol Neurotol Jul;35(6):954-60
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65. Walker, et al, Otol Neurotol. 2014 Jul;35(6):954-60
Results
Residual Disease
30/253 (12%) had small cholesteatoma in ME at 2nd look.
All successfully removed at that time.
No cases of residual disease in obliterated mastoid
Failure rate (Required CWD or Petrousectomy) (2.6%)
98% had dry ears
Walker, et al, Otol Neurotol Jul;35(6):954-60
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66. Walker, et al, Otol Neurotol. 2014 Jul;35(6):954-60
Results Children
Children as young as 2 years old have had CWR
2.8% failure rate in children
Walker, et al, Otol Neurotol Jul;35(6):954-60
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67. Case Had CWR with cholesteatoma removal from mastoid and middle ear
6 months later had transcanal 2nd look with no residual cholesteatoma
Ossicular chain reconstruction performed
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68. Case
Audiogram
Pre-op
Post-op
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69. Reconstructed Ear Canal and New Ear Drum
Case
Reconstructed Ear Canal and New Ear Drum
Cartilage graft
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70. CWR for Cholesteatoma Advantages Improved access for disease removal
Reduced recurrence of retraction pocket
Due to reduction of gas resorbing mastoid epithelium and attic block???
If ETD persists, medialization of TM does not result in recurrent retraction pocket
No long term debridement necessary
One procedure for nearly all cholesteatomas regardless of age
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71. Question #1
The newborn hearing screen tests the function of which of the following?
Cochlear Nerve
Outer hair cells
Stria Vascularis
Inner hair cells
9/20/2018

72. Question #2
True or False: The earliest recommended age for deaf children to undergo cochlear implantation is 2 years? CI in deaf children at 1 year of age offers the best chance to achieve normal or near normal language development
9/20/2018

73. Question #3
Canal wall reconstruction tympanomastoidectomy allows which of the following?
Low rate of recurrent cholesteatoma
Preservation of the ear canal anatomy
Children to participate in water activities
All of the above
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74. Thank You
Rick F. Nelson MD PhD Office:
Any type of hearing loss
Ossicular chain reconstruction
TM perforation
Cholesteatoma
Facial paralysis
Skull base tumors (Acoustic neuroma, Meningioma)
9/20/2018