Hearing Technology: Current & Future Options
Transcript of Hearing Technology: Current & Future Options
08/10/2016
Carmen Hayman, AuD, CCC-A, CISC 1
PA Great Start Conference
Carmen D. Hayman, AuD, CCC-A, CISC
Penn State Conference Center
11/13/2017
Hearing Technology: Current & Future Options
Children’s Hospital of Philadelphia
08/10/2016
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More Locations
King of Prussia
Brandywine Valley
Plainsboro, NJ 8 Satellite Locations
5 – PA3 - NJ
Center for Childhood Communication
Large Staff• 1 Center Director• 2 Managers (Audiology & Speech)• 1 Academic & Research Program Director• Clinical Staff:
• 43 – Speech-language Pathologists• 38 – Audiologists• 3 – CFY/LEND Fellows• 3 - Aides/Assistants• 1 – Educational Consultant• 2 – Child & Family Therapist
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Balance & Vestibular ProgramNew Program • Care and testing for children with dizziness and
balance disorders. • Evaluation team members:
• Otolaryngologists• Advanced Practice Providers (P.A.)• Audiologists• Physical Therapists• Neurologists• Consult as needed with other specialist
Annual Pediatric Audiology Conference
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Topics for Discussion
• Hearing Aid Technology• Bone Conduction Hearing Devices (BCHD)• Cochlear Implants• Auditory Brainstem Implants (ABI)• Unilateral Hearing Loss & Device Candidacy• Options for Single Sided Deafness (SSD)• Technology in our schools
Overwhelming Numbers
5% of the population have hearing loss 360 million people have a >40 dBHL loss
Less than 5% of those who can benefit from hearing technology have access
Shared by Chris Smith (CEO & President, Cochlear)
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What’s new
Hearing Aid Technology
CHOP Audiology
Generally fitting or recommending three manufacturers: Phonak Oticon ReSound hearing aids
Some new features in hearing aid technology: Rechargeable batteries iPhone and Android direct connectivity Remote microphones direct to hearing aids (no
streamer or boots)
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Rechargeable batteries
Different options: Rechargeable batteries are within in the case
(Phonak) Pro: No more additional tamper-proofing Con: What if you forget to charge?; Hearing aid needs
to be sent to manufacturer for battery to be changed (should last 2-3 years)
Rechargeable batteries can be taken out (Oticon) Pro: If you forget to charge, you can switch to a
regular battery Con: ?
Phone connectivity Previous technology- Near Field Magnetic Induction (needed
streamer) Now using 2.4 GHz (radio frequency transmission)
No audible delay or echo No additional product (straight from device to HA, no streamer
needed) Won’t drain battery like Bluetooth (Bluetooth not an option in HA) Allows ability for Mfi
All manufacturers are going to 2.4 GHz Great for public forums
“MFi” (Made For iPhone) Android and Apple
Only Phonak currently for direct connectivity to BOTH Android and Apple
ReSound and Oticon have Mfi and need extra piece for Andriod
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Remote Microphones
No boots or streamers NOT the same as FM or Roger Available for ReSound, including pediatric line
of hearing aids About to be launched for Oticon (for Opn
hearing aid line, not for pediatric line of hearing aids)
Likely not something Phonak will be recommending as they have Roger technology
Research
Wide range in speech and language skills for children with hearing loss Best when identified early, fit with appropriate
technology and received intervention Key predictors:
Audibility achieved with amplification- the hearing aids are fit well
Hearing aid use: all current hearing aids have datalogging- this should be checked at hearing aid appointments
Cognitive and linguistic factors
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Validation
Outcome measurements: used to evaluate the efficacy of intervention
Aided Speech Perception testing
• Word Recognition Testing in noise• Detection of word-final plurality
(UWO Plurals Test)• Ling 6 Sounds Test
Parents’ report • LittlEARS Auditory Questionnaire• Parents’ Evaluation of Aural/oral
Performance of Children (PEACH)
PMSTB
MINIMUM SPEECH TEST BATTERY (MSTB)
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PURPOSE OF THE MSTB Minimum Speech Test Battery (MSTB) Developed for
pre- and post-operative assessment for adult CI patients Revised in 2011:
AzBio Sentences Test CNC Word Test BKB-SIN Test
Pediatric Minimum Speech Test Battery (PMSTB) Method: Survey was conducted in 2 phases Trends emerged in tests being used Lack of consistency in tests used with children <36 months(Uhler & Gifford, American Journal of Audiology, Vol. 23, September 2014)
PMSTB – ADVANTAGES
More uniform test administration Increase industry’s potential to create standards of performance Create comparison benchmarks Provide age normative data for common speech recognition
metrics taking into consideration demographic variables and hearing configuration To be used with all patients: hearing aids; unilateral CI; bimodal,
bilateral CI Currently – within subject comparison and not year-to-year expected
growth in speech understanding performance in children
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• Bone Conduction Hearing Device (BCHD)• Bone Conduction Hearing Aid (BAHA)• Baha® (Cochlear®)• Processor• Bone conduction hearing implant• Bone anchored hearing system• Bone conduction hearing system• Auditory Osseointegrated Device (AOD)
BCHD
BCHD Candidacy
Mixed
Hearing Loss
Conductive
Hearing Loss
Single Sided
Deafness (SSD)
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Medical Indications
Skin allergies Congenital malformations Draining ears Ear canal stenosis Previous ear surgery
Example: ear canal wall procedure Radical cavity Syndromic hearing losses
BCHD for Pediatrics
Can be fit with a softband on children 6 weeks and older
Indications generally the same as adults
In USA and Canada – must be 5 years or older for the implanted BCHD Must have skull bone
at least 2.5 mm thick
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BCHD on Softband
Can be fit at any age > 6 weeks of age
Bilateral BCHD can be used with a softband
Candidacy criteria is the same as with a the BC-implanted device
BCHD on softband does not provide the same benefits as the when connected to an implant due to skin attenuation
BCHD – Direct Connection
Implantation results may be better than results on a softband Patient may perceive
better sound quality and additional loudness
Aided thresholds may better
High frequencies are weakened by passing through skin more than low frequency signals
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Cochlear Surgical OptionsTranscutaneous vs. Percutaneous
Contraindications for Implantation
Patient less than 5 years of age Insufficient temporal bone volume Inability of the patient or parent/guardian to
maintain and clean abutment site (when appropriate)
Significant developmental delays or behavior problems that may jeopardize the abutment/skin interface
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BCHD Device Options
Older BCHD Products
Obsolete- No Longer Repairable Baha Divino, Intenso, Cordelle, BP 100 and
BP 110 Baha BP 110 (repairs until 12/31/17) Baha 4 (Projected date to retire 3/31/19)
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Cochlear & Oticon Medical
Abutment Options
COCHLEAR ATTRACT
ADVANTAGES
No abutment More cosmetically
appealing No issues with skin
infection No special cleaning
required Sound processor easy to
connect Can be converted to
traditional abutment at any time
DISADVANTAGES
Abutment results in better hearing
Attract yields about the same/slightly better hearing than softband
Magnet not as secure as abutment
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Cochlear Connect
ADVANTAGES
Best hearing Secure and
stable connection No retention
issues
DISADVANTAGES
Abutment requires daily cleaning and maintenance
Risk of infection/ surgical intervention
Not as cosmetically appealing
Abutment Complications
Healthy Abutment Common Complications
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Baha® Connect SystemBaha® Attract SystemBaha 5 sound processors on Baha Connect System2
1. Norrman, J, Review of fitting ranges. Cochlear Bone Anchored Solutions AB, D773528, 2015.
2. OFL90 measured on skull simulator TU1000
Baha 5 sound processors on Baha Attract System1
OFL90 measured on Artificial Mastoid IEC 60318-6
Outp
ut
forc
e leveli
n d
B 1
uN
Outp
ut
forc
e leveli
n d
B 1
uN
Cochlear Connect
Better high frequency hearing
Cochlear Processor Candidacy
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Current Cochlear Products Baha 5, Baha 5 Power and Baha 5 Super
Power• Wireless capabilities• No direct connect FM• Programmable• Can stream directly from iPhone®, iPad® or iPod
touch®• Can stream with Android products with app and
phone clip
Wireless Accessories
ReSound technology Can be purchased directly from Cochlear Costco sells Resound Wireless accessories
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Baha 5 Smart App
Baha 5, Baha 5 Power, and Baha 5 SuperPower
The Baha 5 Smart App is verified on iPhone, iPad and iPod touch running iOS 9.1 or latter
Android Smart App• Phone clip needed for streaming• Android Nougat platform (pair with
Bluetooth) Refer to www.cochlear.com for full device
and iOS compatibility
Oticon Medical
Processor Models: Ponto(discontinued) Ponto Pro/Ponto Pro Power (still available)
Ponto Plus and Ponto Plus Power
(will phase out)
Wireless accessories Streamer
Ponto 3 (most current)
All devices are side-specific due to directional microphone
Processors are compatible with Cochlear surgical abutment that was placed prior to 10/2009
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Ponto 3 Series
Push button and Volume Control Tamper-resistant Battery door New software Same chip as Oticon Brain IP 57 classification New Skins New softbands New Connect Line App (Apple and Android) Working on CHOP Pricing
Softbands, Skins And Stickers
New Softbands with 14 color options
Skins
Stickers
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Oticon Medical Wireless Accessories
Ponto Streamer Wirelessly connects Ponto 3 to audio sources Built in telecoil Can connect to computer, cell phone, etc. Can use with a universal FM receiver
BCHD - SUMMARY
More options now than ever before Not all devices may be an
appropriate option for all children Air conduction hearing aids should
always be considered first
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• Candidacy• Hybrid technology• Off-label implantation
COCHLEAR IMPLANTS
CHOP Cochlear Implant TeamMEDICAL TEAM:
Surgeons• Ken Kazahaya, MD, MBA - Medical
Director • John Germiller, MD, PhD• Brian Dunham, MD• Luv Javia, MD• Robert O’Reilly, MD
Advanced Practitioners• Linda Miller Calandra CRNP, MSN• Erin Field, PA
SUPPORT TEAM:
Secretaries • Eileen Kelm (ENT)• Carol Stigale (ENT)• Lindsey Fulton (CCC)• Yaderiah Johnson (CCC)
CLINICAL TEAM:
Audiology• Carmen Hayman, AuD – Coordinator• Michael Jackson, AuD• Melissa Ferrello, AuD
Speech• Paula Barson, MA• Jenna-Leah Duffield, MS, LSLS• Arielle Berne, MS• Kristen Greene, MS
Child & Family Therapist• Rebecca Witmer, MS, LSW
Educational Consultant• Essie Goldsmith, MEd, CEDAudiology Assistants – Colleen Hammil& Jennifer Gadsen-Jones
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The Cochlea: Sound in Motion
HIGH PITCH:
BASE
LOW PITCH:
APEX
Hair Cells
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Damaged Hair Cells
ELECTRODE
Cochlear Implant: Definition An electronic device that
provides improved hearing and improved communication to adults and children with severe to profound sensorineural hearing loss.
Consists of a surgically implanted internal component and externally worn headset with speech processor.
External power source
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How a Cochlear Implant Works
CHOP CI Program Updates Our numbers
Calendar year 2016 = 90+ ears Increase in simultaneous implants
CI Services in the satellites Programming; info meetings; annual audiology
and speech evaluations Plainsboro, NJ (Started in September) King of Prussia, PA (Started in October 2015)
ENT Services - Lancaster
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Memory Lane
House Ear Institute 1st pediatric CI program – 1980 Implanted the first child – 9 year old boy with the
House/3M device By 1982 – 12 children (3.5 – 17 years) FDA approved: 1984 in adults and 1986 in
children
Memory Lane
Cochlear Corporation Nucleus-22 channel implant 1985 – FDA approval for adults 1990 – FDA approval for children 2 years+
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Memory Lane
Cochlear Implants In Younger Children Mid-to-late 1990s – UNHS
Earlier diagnosis = earlier implantation
Clarion clinical trial – 18 months and older
2000 – FDA approves cochlear implants for children 12-months+
Pediatric Candidacy Guidelines
Severe to profound sensorineural hearing loss in both ears
> 12 months of age Lack of progress in development of auditory skill
with hearing aid or other amplification ▪ For younger children
▪ As demonstrated on the IT-MAIS or MAIS▪ Therapist and or teacher reports
Receive little or no benefit from hearing aids ▪ For older children
▪ Score < 30% correct on word recognition test
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Adult Candidacy Guidelines
Moderate to Profound SNHL, bilaterally 50% or less - sentence recognition
in the ear to be implanted 60% or less - sentence recognition
in the opposite ear or binaurally Pre-linguistic or post-linguistic onset of
moderate-to profound SNHL No medical contraindications A desire to be a part of the hearing world
Candidacy of School Age Children
Child must: Be an active participant in the evaluation process
8+ years – part of CI information meeting Want the cochlear implant Understand that there is a surgery involved Understand that there may be time post-implant
that they do not wear the hearing aid Considerations:
Timing of surgery During the school year vs. over a school break
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Age of Implantation
Congenitally Deaf ChildrenCritical Period for Acquisition of Verbal
Language
Optimal age for Implant
Suitable for Implant
Benefit from implant may be limited
0-----1-----2-----3-----4-----5-----6-----7-----8-----9 Age in yrs.
Hammes D. et al. “Early Identification and Implantation: Critical Factors for Spoken Language” Annals of Otology,
Rhinology and Laryngology, Supplement 2003: May: 189:79-84
Karen Iler Kirk, Ph.D. et al. The Volta Review, Vol. 102(4): (monograph) 127-144, Indiana University)
It All Starts With The Brain
Speech understanding is a cognitive process Having access to sound does not guarantee
spoken language will be the primary mode of communication
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Pediatric CI Candidacy Challenges
Children with additional disabilities or suspected but not formally diagnosed
40% of children with SNHL have other medical and / or developmental diagnoses.
(Fortnum, Marshall & Summerfield, 2002; Gallaudet Research Institute, 2008; Roberts & Hindley, 1999; Van Naarden et al., 1999)
No professional agreement regarding implantation in children with special needs.
CI Candidacy Guidelines
Pediatric candidacy criteria has notexpanded/changed in 17 years
Many centers perform “off-label” implantation More hearing Better than 30% word recognition abilities
(pediatric criteria) Follow adult candidacy guidelines
Moderate to profound hearing loss 50% sentence recognition in the ear to be implanted 60% sentences recognition in the non-implanted ear
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Current Options We Can Offer
Under Current FDA Guidelines: Unilateral implantation
Standard of care from 1990 to ~2006 Bimodal stimulation Sequential bilateral implantation
CHOP began in 2006 Simultaneous bilateral implantation
Steady increase over the last 3 years
What We Know…… Sequential bilateral cochlear implantation
allows asymmetric auditory development if large gap between 1st & 2nd ear
compromises binaural processing in the auditory cortex Aggressive bilateral cochlear stimulation promotes:
Shorter duration between 1st & 2nd ear symmetric auditory brainstem development spatial unmasking binaural processing
Simultaneous bilateral cochlear implantation promotes symmetric auditory brainstem development protects the auditory cortex from reorganized lateralization
Blake Papsin, MD ( 6th International Pediatric Audiology Conference, 12/9/2013)
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What We Offer…..
Management of hearing loss is a continuum Provide the best technology for each ear
No longer requiring bilateral severe to profound hearing loss in children
Expanding what we offer: Implants under 12 months of age Hearing preservation & EAS devices Asymmetrical hearing loss Single Sided Deafness
• Cochlear Implantation < 12 Months• Hearing Preservation• Electro-acoustic Stimulation
Frequently Asked Questions
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Implants Under 12 Months
Challenge: Reliable diagnosis to confirm profound hearing loss
Recommending: attempt behavioral audiometry; bilateral OAEs; ear specific and frequency specific ABR or ASSR; bilateral tympanometry and acoustic reflexes
Concerns: Surgical risk – minimal
Research supports minimal anesthetic, surgical and long-term complications
Programming Use more objective measures: ECAPs and ESRT
Outcomes: Eliminate the need for “catch-up” Multiple studies suggest benefit in language development
& speech perception
Implants Under 12 Months
Challenge: Reliable diagnosis to confirm profound hearing loss
Recommending: attempt behavioral audiometry; bilateral OAEs; ear specific and frequency specific ABR or ASSR; bilateral tympanometry and acoustic reflexes
Concerns: Surgical risk – minimal
Research supports minimal anesthetic, surgical and long-term complications
Programming Use more objective measures: ECAPs and ESRT
Outcomes: Eliminate the need for “catch-up” Multiple studies suggest benefit in language development
& speech perception
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Hearing Preservation
Change in counseling Preservation of residual hearing Two factors:
Minimally traumatic surgery Cochleostomy or round window insertion
Atraumatic electrodes Both short and long electrodes Thin electrode array
Electro-Acoustic Stimulation Devices
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Electro-Acoustic Stimulation Devices
EAS Devices – Cochlear & Med-EL
Nucleus HybridM
Synchrony EAS
Med-EL EAS
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EAS Devices – Cochlear & Med-EL
Nucleus Hybrid
Synchrony EAS
Med-EL EAS
Cochlear – Nucleus Hybrid Implant
Hybrid implant – shorter in length (15 mm)
High frequency hearing loss
FDA approved for adults in 2014
EAS accessory for N6 Processor Can be used with children
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Med-El – Synchrony EAS (09/2016)
18 years and over Hearing loss that falls within the
shaded area on the audiogram Normal to moderate SNHL in the
low frequencies, sloping to severe-to-profound hearing loss in the high frequencies.
Pre-operative scores for speech understanding for single words in quiet is 60% or less.
Different length electrode arrays available
Off-label implantation
Case Studies
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J.C. Pre-implant
Diagnosed at 3y and fit with hearing aids at 3y-2m.
J.C. Post-implant
Left ear: 01/2009Right ear: 03/2012
Hearing preservation of left ear 5+ years post -implant
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L.R. Pre-Implant
Diagnosed @ 10 months with severe to profound hearing loss
Fit with hearing aids @ 11 months
Hearing remained stable; auditory-oral patient. Met someone with a cochlear implant at 15 years and began to do her own research.
L.R. Post-implant
Right ear implanted in 10/2005
Testing completed 2 months post activation
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Hearing Preservation - DB
ADD PRE-
SPEECH
Asymmetrical Hearing Loss - BD
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Asymmetrical Hearing Loss - BD
Asymmetrical Hearing Loss - ZZ
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Asymmetrical Hearing Loss - ZZ
Asymmetrical Hearing Loss - ZS
Failed NBHS Hearing loss
managed locally At 5 years saw Dr.
Kazahaya EVA
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Asymmetrical Hearing Loss - ZS
Asymmetrical Hearing Loss - ZS
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CI Candidacy - Summary
Pediatric candidacy has not expanded
More experienced centers are comfortable with off-label implantation of their pediatric patients
Fit most appropriate technology for each ear Consideration for more bimodal
patients
Difference between CI and ABI.Pediatric Candidacy Studies
Auditory Brainstem Implants
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Auditory Brainstem Implants
The Auditory Brainstem Implant (ABI) system is designed to restore a degree of hearing
sensation to patients who have bilateral
dysfunction of the auditory nerve or
cochlear nerve deficiency (CND)
This is achieved by direct electrical stimulation of the cochlear nucleus complex in the brainstem
Options for CND
Patient with Questionable Auditory Nerves
Hearing Aid?
Cochlear implant?
Auditory Brainstem Implant?
Nothing/ No Technology?
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PA Great Start Conference
Try Amplification First
Differences Between CI and ABI
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Differences Between CI and ABI
ABI and Neurofibromatosis Type 2
Approved in 2000 for individuals with NF2 age 12 years and older
Provides sound awareness, help in speech reading
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Pediatric Non-NF2 ABI Feasibility Study Centers (4)
NYU
MEEI
UNC
House EI
CHLA
Keck-USC
Candidacy for an ABI
Imaging/MRI Behavioral Audiometry eABR/Prom Stim
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Promontory Stimulation (Prom Stim)
Electrically evoked ABR (eABR) Stimulus is not acoustic like traditional ABR Stimulation site may be promontory, but round
window often used as well Stimulation delivered via isolated current
stimulator (“black box”) that delivers biphasic rectangular pulses
Used to determine if the auditory nerve is electrically stimulable
Background
Developed by House and Brackmann in 1974 to predict how well surviving spiral ganglion nerve fibers would respond to cochlear implantation
Over time, most patients had positive eABR responses and this was no longer considered a prognostic factor
Most studies found no correlation between eABR thresholds and outcome with CI so the test fell out of favor
However, eABR responses confirm the existence of intact auditory neurons in cases where this is in question
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Prom Stim History at CHOP
Early 1990s: CHOP Audiologists performed prom stim testing
2008: ENT hired an outside consultant (Paul Kileny, PhD) to perform prom stim testing
In 2011, a decision was made to provide this service in our department
We worked with the consultant for training purposes CHOP Audiologists have being doing prom stim
testing since then
Who Gets This Evaluation?
Patient whose imaging shows abnormalities such as cochlear nerve dysplasia or possible aplasia, congenital malformations, narrow internal auditory canal, etc.
Patient should otherwise be an appropriate candidate for CI
Ideally, patient will have initial CI evaluation prior to testing. The process is ever-evolving (patients from other sites, infants, etc.)
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What Do Results Indicate?
No response: CI not an option but may be candidate for ABI
Response obtained: Patient may be a candidate for CI pending other evaluations. Counseling stresses guarded expectations
Test Environment
Completed in operating room under general anesthesia 2 Audiologists + ENT
Needle electrodes used with various types of stimulator electrodes (ball/needle)
Electrode sites (5): Forehead, tragus (2), nape, transtympanic
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Needle electrodeBall tip probe
Stimulation Sites
Order of testing based on responses:First, PromontoryThen, Round Window (if no response on promontory)
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Electrode On Promontory
Round Window Before Stimulation
Round Window
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Responses
Positive Response No Response
Data from March 2011 to Present
912
Patients who received Prom Stim
ResponseNo
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Of 9 With A Response…..
4
4
1
Device
CI @ CHOPNon-
CHOP Patients
ABI
Of 12 patients who had no response...
1
10
1
DeviceABI
No technology
CI @ outside facility
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ABI Operation & Initial Activation
ABI Surgery Longer
procedure than a CI, higher risk of complications
Electrode pairs stimulated with ABR and nerve integrity monitoring Picture courtesy of NYU
ABI Operation & Initial Activation
Activation: Day One performed in OR (4-6 weeks after surgery)
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ABI Operation & Initial Activation
Activation: Day Two performed in surgeons’ office Blood pressure and heart
rate are monitored. Electrode pairs are activated
that provided good electrophysiological responses, without NASE.
ABI Operation & Initial Activation
Activation: Day Three performed in CI programming room Continue to search for
electrode pairs that provide good auditory access without NASE.
Patient returns monthly for first year for continued programming
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Programming CI vs. ABI
ABI Programming for Adults
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Categories of Auditory Performance
0 = No awareness of environmental sounds1 = Awareness of environmental sounds2 = Response to speech sounds3 = Identification of environmental sounds4 = Discrimination of speech sounds5 = Understanding common phrases without lip reading6 = Understand conversation without lip reading7 = Use of telephone with known speaker
Archbold et al, 1995
Mor
e di
fficu
lt
Colletti et al. (2014) Audiol Neurotol
0 = No awareness of environmental sounds1 = Awareness of environmental sounds2 = Response to speech sounds
3 = Identification of environmental sounds4 = Discrimination of speech sounds5 = Understanding common phrases
without lip reading
6 = Understand conversation without lip reading7 = Use of telephone with known speaker
CAP Scores
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Wilkinson et al (2017) Otol Neurotol
Managing Patients with CND
Standard Practice; Fit
with amplification
Diagnosis of cochlear
nerve deficiency
Refer to Cochlear Implant Team
CI Candidate?
Yes
No Continued monitoring,
possible later referral back to CI
Team
Promontory Stimulation?
Positive
Response
Absent
Response
Cochlear Implant
Refer out for ABI, CI team
will consult with ABI center
Sign Language
Discontinuehearing aid?
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PA Great Start Conference
In Summary
ABI ≠ CI Research is ongoing. The procedure is reasonable and safe for children
with CND. ABI’s show great potential, but significant limitations. The most optimistic outcomes are < half that of
cochlear implant users. If children have other diagnoses, the ABI has not been
shown to be beneficial beyond sound awareness. Some children do not achieve consistent sound
awareness.
Establishing Best Practices
Unilateral Hearing Loss
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PA Great Start Conference
Summary of Difficulties Encountered by Children with UHL
• Academic Performance– Grade failure – Resource room help – Lower teacher perceptions
• Cognition– RE loss- Lower verbal Score – LE loss- Lower performance Score– Compromised executive functions
• Speech/Language– Language delays
• Behavior/Emotion– Behavioral problems– Fatigue– Stress
Importance of Binaural Hearing:Practical Considerations
Head shadow effect Localization Binaural summation Binaural release from masking
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PA Great Start Conference
Referrals for Children Diagnosed with UHL
Otolaryngologist to investigate etiology Early Intervention / Education Program for
evaluation and service determination Ophthalmologist for complete examination of vision Speech/Language Pathologist for speech and
language evaluation Geneticist/Genetic Counselor for genetic evaluation
Joint Committee on Infant Hearing (2007)
Additional Referrals for Children Diagnosed with UHL at CHOP
Child and Family Therapist for adjustment to hearing loss consultation and/or support group opportunities
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PA Great Start Conference
Rule out outer or middle ear condition contributing to hearing loss
Order additional testing to determine etiology and/or associated problems (i.e., imaging, renal studies)
Provide medical clearance for amplification Establish relationship for expeditious assessment
and treatment of middle ear problems and/or sudden hearing loss
Importance of Otolaryngology Visit
Anatomical ConsiderationsEtiology of Unilateral SNHL
Finding Number Percent
Normal Imaging 17 20.5Enlarged Vestibular Aqueduct (EVA)
Unilateral EVABilateral EVANormal, but asymmetrical
2747
32.54.88.4
Cochlear Nerve DeficiencyUnilateral (isolated 19; w/other anomaly 6)Bilateral
250
30.10
Cochlear/vestibular dysplasiaUnilateralBilateral
30
3.60
Total 83 100
Germiller et al. (2008)
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Carmen Hayman, AuD, CCC-A, CISC 65
PA Great Start Conference
Anatomical ConsiderationsEtiology of Unilateral Neural Hearing Loss
MRI results were evaluated on 11 children with audiologic results consistent with unilateral auditory neuropathy
Over half were identified at newborn screening Etiology identified in 90% from MRI results
Eight (73%) had absent cochlear nerves Two (18%) patients had tumors Only one patient was idiopathic
Laurey et al. (2009)
How Anatomical Findings May
Impact Recommendations
Significant percentage of children with UHL may have abnormal findings on imaging
Findings such as unilateral or bilateral EVA, cholesteatoma, or ossicular malformations, may warrant a proactive approach to the fitting of amplification
Findings such as abnormal cochlear or neural anatomy may warrant a more cautious approach to the fitting of amplification, even in cases where the loss appears “aidable”.
Etiology of hearing loss (conductive and sensorineural) may be determined by imaging, and results are valuable for counseling and in guiding next steps
08/10/2016
Carmen Hayman, AuD, CCC-A, CISC 66
PA Great Start Conference
Limited Research to Guide Us
Professional perspectives vary Patient age, degree of hearing loss & etiology
impact management decisions Variable outcomes with hearing aid fittings
Device chosen on a case-by-case basis Published research has demonstrated that
children with UHL, fit with appropriate device, can perceive benefit
Why not fit an infant immediately following diagnosis of UHL?
Babies are usually at a close distance to the speaker allowing for an optimal signal-to-noise ratio
There is no evidence that early fitting of amplification to infants with UHL is beneficial or more importantly, that it is not detrimental
The added time taken to obtain behavioral results, rule out medical contraindications and confirm that speech and language surveillance services are in place is time well spent to insure best patient care
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PA Great Start Conference
Evidence Supporting The Early Fitting Of Conventional Amplification
Existing evidence
▪ Improvement in localization skills Johnstone et al. (2000)
Reports of subjective benefit from older children
Briggs, Davidson, Lieu (2011); Priwin et al, (2007); Kiese-Himell (2002); McKay (2002); Davis et al. (2001)
Auditory deprivationPlasticity
Sharma, Dorman & Spahr (2002)
Reorganization Gordon, Henkin & Kral (2015)
Evidence not yet available
Speech understanding Speech understanding in
noise Speech and language
development Academic achievement Social-emotional impact Cognitive impact
Device Options for Children w/UHL
Conventional amplification Air conduction hearing aids Bone Conduction Hearing Devices (BCHD)
Conductive or mixed hearing loss
Unconventional amplification BCHDs CROS & TransEar for SSD FM/remote microphone technology Cochlear implants
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PA Great Start Conference
Conventional Amplification
Mild to moderately-severe UHL Usable word recognition abilities in the impaired ear
(if age-appropriate) Otologic work-up indicating no transient or
permanent medical contraindications Enrollment in a program designed to monitor
speech, language and auditory development as well academic achievement
Unconventional Amplification for Children w/ SSD
Non-conventional amplification, such as CROS or transcranial systems, may be beneficial for some children with SSD. Non-conventional Amplification Options may be considered for patients who meet all of the following criteria:
Severe-to-profound sensorineural hearing loss in the impaired ear
Otologic work un indicating no medical contraindications Older children (~eight years of age) who clearly demonstrate
the aptitude to manipulate hearing aid programs when listening environments change
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Carmen Hayman, AuD, CCC-A, CISC 69
PA Great Start Conference
Unconventional Amplification for Children w/ SSD
Contralateral Routing of Signal (CROS)
Beneficial when the speech signal originates on the impaired side
Detrimental when noise originating on the impaired side and is sent to the normal hearing ear
Only appropriate for children who can determine which listening environment would benefit using a CROS
Bone Conduction Device
Improved speech understanding in noise in older children
Subjective benefit reported by older children
Updike (1994); Kenworthy, Klee, & Tharpe (1990)
Christensen & Dornhoffer, 2008; Christensen, Richter, & Dornhoffer, 2010)
Remote Microphone/FM Systems
A remote microphone or FM system is considered beneficial for children with UHL and the following candidacy criteria should be considered: School-aged children with permanent/chronic UHL Children under age five years considered on a case-by-
case basis Choice of remote microphone/FM style should be made on
a case-by-case basis The child’s educational audiologist/hearing support
professional should be consulted regarding remote microphone/FM system selection and settings
08/10/2016
Carmen Hayman, AuD, CCC-A, CISC 70
PA Great Start Conference
Remote Microphone/FM Systems
There is no “one size fits all” for UHL Ear-level FM provides best signal-to-noise ratio Considerations for choice of FM delivery to normal
hearing ear, impaired ear, or both? Word recognition abilities of impaired ear Dexterity, maturity, activity level Potential occlusion of normal hearing ear Educational setting (e.g. desk-top may work in
elementary setting but not in middle school setting)
CHOP’s Selection and Evaluation for UHL
Offer/provision of four week trial with conventional hearing aid, bone conduction device or CROS system
Verification of fitting using appropriate, device-specific method
Speech-in-noise testing (unaided), under three test conditions (if possible) using age-appropriate recorded word recognition lists (> 25 words) with multi-talker babble as noise. Word list should be presented at 50dB HL with a +5 SNR
08/10/2016
Carmen Hayman, AuD, CCC-A, CISC 71
PA Great Start Conference
CHOP’s Selection and Evaluation for UHL
Three test conditions (using age-appropriate recorded word recognition lists (> 25 words) with multi-talker babble as noise.
Hearing Instrument Validation
Aided speech in noise testing using three conditions consistent with unaided testing
Administration or provision of age-appropriate functional auditory measures (e.g. CHILD, SIFTER, PreSchool SIFTER, PEACH)
Administration of CHOP Unilateral Hearing Loss Questionnaire toparent/guardian and /or child
Documentation of other anecdotal information regarding perceived benefit and auditory function
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PA Great Start Conference
Unilateral Amplification Questionnaire
Post-amplification questionnaire comparing current amplification to no amplification
Option for parent and child to complete
Information obtained can be used to guide counseling
Unilateral Amplification JournalFour week daily journal Hours worn at school Hours worn at home Situation where
listening was easier Situations where
listening was difficultFinal week Comfort Sound quality Ease of use “Would you use this device?”
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PA Great Start Conference
Written Information for Families & Teachers
CHOP’s Hearing Loss Parent Information materials Children with Unilateral Hearing Loss: a Parent’s Guide Children with Unilateral Hearing Loss: a Teacher’s Guide CHOP‘s parent and education support services, and state
medical assistance programs (if appropriate). Additional written information (e.g. Educational Audiology
Association handouts)
Summary – UHL EBP
Even with limited evidence, Audiologists can use an EBP model to develop best practices for their patients with UHL.
Determination for amplification for children with UHL must be made on a case-by-case basis considering audiological, medical, developmental, educational, and social factors.
Families should be provided with information about what is both known and unknown regarding the fitting amplification to young children with UHL. This insures informed decisions.
Child caregivers, managing clinical audiologists and educational/ hearing support professionals should work collaboratively to develop plans with the best possible outcomes for each child.
08/10/2016
Carmen Hayman, AuD, CCC-A, CISC 74
PA Great Start Conference
Single Sided Deafness
CI for Children with SSD
Not FDA approved for individuals with UHL-must be done “off label”
Goal of restoring some binaural advantages Initial objective and/or subjective findings suggest
improved speech perception abilities in noise and localization abilities Friedmann et al. (2016); Arndt et al. (2015)
Considerations for implantation of SSD Age Duration of hearing loss (congenital, progressive/acquired) Etiology Risk of progressive hearing loss in normal hearing ear
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PA Great Start Conference
Possible Causes SSD
Acoustic neuroma tumors Congenital factors Genetic factors Meniere’s disease Neurological degenerative disease Ototoxic drugs Sudden deafness Surgical interventions Trauma
BCHD Candidacy
Normal hearing (air and bone conduction thresholds) in one ear.
Need realistic expectations Will not perceive hearing
from their deaf side > 8 years of age (CHOP
criteria) 4 week trial is suggested
using SSD testing paradigm
Single-sided Deafness Selection Criteria
08/10/2016
Carmen Hayman, AuD, CCC-A, CISC 76
PA Great Start Conference
Device Options for SSD in Kids
BCHD on Softband Implanted BCHD
CROS Aid BCHD Benefits over CROS No occlusion of the hearing
ear Proven solution – long term
studies No need to wearing hearing
devices on both ears
SSD Fitting Challenges
Introduction of devices into clinical practice without the evidence to support their benefit
Initial skepticism of audiologists about potential benefit
Age cutoffs during initial fittings Potential detrimental effects of CROS hearing aids Large variations in patient maturity
Patient reluctance/refusal to try any transcranial device
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PA Great Start Conference
Single Sided Deafness & CIs
Michael Dorman, PhD and Colleagues: The Sound of a Cochlear Implant: Insights from Studies of
Patients with Single Sided Deafness Reorganization of the cortex following unilateral auditory
deprivation occurs with electrical stimulation. Reorganization takes anywhere from 3 – 8 months
Binaural Localization Normal hearing – excellent performance Bilateral CI – scattered performance SSD-CI – looks like distribution of bilateral CI patients
Comparing sounds of SSD-CI to normal hearing ear
https://www.youtube.com/watch?v=1dhTWVMcpC4
•Advanced Bionics•Cochlear Americas•Med-El
Note: Many slides in this portion were provided by the manufacturers represented.
Cochlear Implant Manufacturers
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PA Great Start Conference
• FM Technology• Phonak Roger System• Wireless options
Note: Some content in this portion of presentation was provided by the manufacturer represented.
CONNECTIVITY
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Carmen Hayman, AuD, CCC-A, CISC 79
PA Great Start Conference
Purpose of an FM System
Make it easier to identify and understand speech in noisy situations or over distances of up to 50 feet
Used to reduce the effects of: Background Noise Reverberation Distance of the speaker
Transmitter (microphone) picks up speech and sends the signal via radio wave to an FM receiver.
Ear-Level Receivers
Direct connect with sound processor or Integrated with the hearing aid
FM transmitter worn by speaker FM receiver connected directly to the sound
processor using an adaptor or a cable FM receiver picks up signal from transmitter and
sends it to the processor or hearing aid
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Carmen Hayman, AuD, CCC-A, CISC 80
PA Great Start Conference
Use FM … Don’t Use FM…
Instructing entire class Repeating what other
students say Adult-directed small
group discussions Giving oral instructions
/exams Watching a video Student giving oral
presentation
Independent seat-work
Addressing another student individually
Whenever speech is not directed toward the student…. teachers lounge, another teacher, restroom, etc.
FM Simulations & Testimonial
Vermont Center for Deaf and Hard of Hearinghttps://www.youtube.com/watch?v=JNzxOJKCUug
Pediatric Audiology Projecthttps://www.youtube.com/watch?v=ln8NHzVfJkQ
Ethan’s Testimonialhttps://www.youtube.com/watch?v=TMv5UuSAsDs
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PA Great Start Conference
FM Recommendations @ CHOP
Map should be stable Recommendations vary depending on where
the child lives - how it is handled PA – varying preferences
Direct input FM prior to Kindergarten Collaboration between hearing aid/CI
audiologist and educational audiologist is essential
• More technology options • Who is recommending?• Who is paying?• The important role of educational
audiologist
Educational Impacts
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PA Great Start Conference
Who Pays?
✓ School
✓ Medical Insurance
✓ Family
✓ Other
Practical Considerations
FM must be set by audiologist familiar with FM’s and cochlear implants
Equipment is only as good as how comfortable the personnel are with it.
Communication among teachers, therapists, educational audiologist, and cochlear implant audiologist is essential.
Have copies of user manuals/videos for speech processor and FM system on site.
Identify an individual to perform daily listening check and troubleshooting.
Don’t use FMs where they are not needed. Don’t forget about FMs when you have a “superstar”.
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PA Great Start Conference
Contacting the CHOP CI Team
Carmen Hayman, AuD, CCC-ACoordinator, CI Program(215) [email protected] (best way to reach me!)
[email protected] Any question you, a student or parent has – we’ll
be directed to the correct person Appointments: 800-551-5480, option #1
CI Packets; scheduling post-implant appointments
THANK YOU FOR JOINING US TODAY!
Thank You For Listening Today!