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

Financial Disclosures None 9/20/2018

Outline Hearing Loss & Cochlear Implants Cholesteatoma Hearing screening/testing Candidacy New Implant Technologies Procedure and Outcomes Cholesteatoma Diagnosis Novel methods of treatment Case example 9/20/2018

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

Question #2 True or False: The earliest recommended age for deaf children to undergo cochlear implantation is 2 years? 9/20/2018

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 9/20/2018

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 9/20/2018

Hearing—Anatomy 9/20/2018

The cochlea has tonotopic mapping Hearing—Anatomy The cochlea has tonotopic mapping Low frequency High frequency 9/20/2018

Hearing—Anatomy Outer hair cells function to augment frequency tuning Contractile cells This contraction generates a measurable sound 9/20/2018

OAE OAE measure outer hair cell function http://www.nishconference.com/newborn-screening.html OAE measure outer hair cell function 9/20/2018

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) 9/20/2018

Hearing Loss in Newborns-Indiana CDC data 2013 9/20/2018

Introduction to Cochlear Implants Cochlear Implant Candidacy Cochlear Implant Outcomes 9/20/2018

Hearing with a Cochlear Implant 9/20/2018

Cochlear Implant

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. 9/20/2018

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 300-500 Hz Place pitch perceived in the relative order of electrodes Impaired Auditory System (higher level pathways intact?) The CI does not restore normal hearing!!! 9/20/2018

CI Surgery Mastoidectomy Facial Recess 9/20/2018

CI Surgery Round Window 9/20/2018

Cochlear Implant placed through Round Window CI Surgery Cochlear Implant placed through Round Window 9/20/2018

Introduction to Cochlear Implants Cochlear Implant Candidacy Cochlear Implant Outcomes 9/20/2018

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. 9/20/2018

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

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 9/20/2018

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 9/20/2018 26 26

Evaluation of CI Candidacy Patient History Audiological Evaluation (CIHAE) Radiological Studies (CT, MRI) Medical Evaluation CI Orientation Cognitive Assessment (when appropriate) Inter-Departmental Consultation 9/20/2018

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) 9/20/2018

Introduction to Cochlear Implants Cochlear Implant Candidacy Cochlear Implant Outcomes 9/20/2018

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. 9/20/2018

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) 9/20/2018

Taken from Niparko, et al, JAMA, 2010;303(15):1498-1506 Language Outcomes Taken from Niparko, et al, JAMA, 2010;303(15):1498-1506 9/20/2018

Taken from Niparko, et al, JAMA, 2010;303(15):1498-1506 Language Outcomes Taken from Niparko, et al, JAMA, 2010;303(15):1498-1506 9/20/2018

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

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? 9/20/2018

Cholesteatoma Keratinizing squamous epithelium in the middle ear or mastoid NORMAL Attic Cholesteatoma Perforation with middle ear Cholesteatoma 9/20/2018

Mastoid filled with cholesteatoma Can be very destructive to the following: Ossicles Inner ear Tegmen—bone between ear and brain Mastoid filled with cholesteatoma 9/20/2018

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 9/20/2018

Case CT confirmed mass in middle ear and mastoid Dx: Congenital Cholesteatoma Which surgical approach should be used for this child? 9/20/2018

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 9/20/2018

COM with cholesteatoma Management Issues Gold Standard = Open cavity (CWD) Closed Cavity procedures CWU recidivism rate = 30-70% 9/20/2018

Canal Wall Up (CWU) Combines cortical mastoidectomy & tympanoplasty Avoids open mastoid cavity 9/20/2018

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 9/20/2018

Canal Wall Down (CWD) Creates an open cavity between external auditory canal and mastoid cavity Requires lifelong periodic cleaning (every 6-12 months) 9/20/2018

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 9/20/2018

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 9/20/2018

Canal Wall Up (CWU) view Mastoidectomy Cholesteatoma Canal wall 9/20/2018

Canal Wall Reconstruction (CWR) view Middle ear/stapes Canal wall cuts 9/20/2018

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 9/20/2018

Exposure Large Anterior Based Palva Flap Incision in hairline provides wide access to mastoid cortex Large Anterior Based Palva Flap 9/20/2018

Collect Bone Pate 9/20/2018

Bone Chips from Outer Cortex Bone Chips from Cortex 9/20/2018

Elevate canal skin 9/20/2018

Canal Cuts 9/20/2018

Remove Posterior Canal 9/20/2018

Exposure of Zygomatic Root and Epitympanum Open Cavity View Canal cut Canal cut Exposure of Zygomatic Root and Epitympanum 9/20/2018

Temporalis Fascia for Tympanoplasty 9/20/2018

Reconstruction 9/20/2018

Reconstruction 9/20/2018

Reconstruction Replace Canal Wall Mastoid tip bone- Scutum to Facial Ridge Bone Chips Facial Recess 9/20/2018

Reconstruction Bone pate filling mastoid 9/20/2018

Pack External Canal 9/20/2018

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 9/20/2018

Previous CWU Tympanomastoidectomy Results 1997-2012 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 1997-2012 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. 2014 Jul;35(6):954-60 9/20/2018

Walker, et al, Otol Neurotol. 2014 Jul;35(6):954-60 Results 1997-2012 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. 2014 Jul;35(6):954-60 9/20/2018

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. 2014 Jul;35(6):954-60 9/20/2018

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 9/20/2018

Case Audiogram Pre-op Post-op 9/20/2018

Reconstructed Ear Canal and New Ear Drum Case Reconstructed Ear Canal and New Ear Drum Cartilage graft 9/20/2018

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 9/20/2018

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

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

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 9/20/2018

Thank You Rick F. Nelson MD PhD rnelson10@iuhealth.org Office: 317-963-7073 Any type of hearing loss Ossicular chain reconstruction TM perforation Cholesteatoma Facial paralysis Skull base tumors (Acoustic neuroma, Meningioma) 9/20/2018