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



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

08/10/2016



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

08/10/2016



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)

08/10/2016



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)

08/10/2016



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

08/10/2016



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

08/10/2016



• 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

08/10/2016



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)

08/10/2016



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

08/10/2016



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

08/10/2016



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

http://www.cochlear.com/

08/10/2016



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

08/10/2016



• 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

08/10/2016



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

08/10/2016



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

08/10/2016



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+

08/10/2016



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

08/10/2016



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

08/10/2016



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

08/10/2016



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)

08/10/2016



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

08/10/2016



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

08/10/2016



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

08/10/2016



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

08/10/2016



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

08/10/2016



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

08/10/2016



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

08/10/2016





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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

08/10/2016



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?

08/10/2016



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

08/10/2016



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

08/10/2016



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.)

08/10/2016



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

08/10/2016



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


Activation: Day One performed in OR (4-6 weeks after surgery)

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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.


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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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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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

http://www.google.com/url?sa=i&rct=j&q=&esrc=s&source=images&cd=&cad=rja&uact=8&ved=0ahUKEwj6hpu5rsbOAhXRsh4KHSChDOgQjRwIBw&url=http://www.clipartkid.com/teacher-cliparts/&bvm=bv.129759880,d.dmo&psig=AFQjCNEiLCzYPZiHVww6JaseRY4YBq0GyQ&ust=1471451576238229

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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

http://www.google.com/url?sa=i&rct=j&q=&esrc=s&source=images&cd=&cad=rja&uact=8&ved=0ahUKEwivqouXpf3OAhWIax4KHXgPAzYQjRwIBw&url=http://www.aamu.edu/admissions/undergraduateadmissions/pages/undergraduate-application-checklist.aspx&bvm=bv.131783435,d.dmo&psig=AFQjCNGLr96qgDUEGFFUL5mja_4SUBpX8A&ust=1473338870060457

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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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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

http://www.google.com/url?sa=i&rct=j&q=&esrc=s&source=images&cd=&cad=rja&uact=8&ved=0ahUKEwj3zsKa4cjOAhWEFR4KHZJvBMgQjRwIBw&url=http://www.forbes.com/sites/jacobmorgan/2015/03/23/the-future-of-work-is-about-choice/&bvm=bv.129759880,d.dmo&psig=AFQjCNGAoPfYSg5cmBC3USixQ3CxVFnfhw&ust=1471533763581126

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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

08/10/2016



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

http://www.google.com/url?sa=i&rct=j&q=&esrc=s&source=images&cd=&cad=rja&uact=8&ved=0ahUKEwj_n9XIssbOAhVHJB4KHXt9C70QjRwIBw&url=http://www.iurc.edu.pk/ranking-research-journals.php&bvm=bv.129759880,d.dmo&psig=AFQjCNEnmtoxuZfOfejzRnNveiwEnc6mtg&ust=1471452684604695

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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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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

https://www.google.com/url?sa=i&rct=j&q=&esrc=s&source=images&cd=&cad=rja&uact=8&ved=0ahUKEwiu2_SK8-PLAhWJth4KHYMdAXoQjRwIBw&url=https://www.phonakpro.com/us/en/solutions/va/va-solutions/va-hearing.html&psig=AFQjCNElT5QUHwz2Z5fj_lrhvG1f0v0RMw&ust=1459272272549939

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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

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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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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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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.

https://www.google.com/url?sa=i&rct=j&q=&esrc=s&source=images&cd=&cad=rja&uact=8&ved=0ahUKEwjYz7KD0_3OAhXK1h4KHb0tC_0QjRwIBw&url=https://www1.toronto.ca/wps/portal/contentonly?vgnextoid%3Dfe27dc273e8cc410VgnVCM10000071d60f89RCRD&psig=AFQjCNFwkMZor1S8BuaqpoFbFRF182z1gA&ust=1473351182169205

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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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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

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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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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

https://www.youtube.com/watch?v=1dhTWVMcpC4

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• 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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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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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

https://www.youtube.com/watch?v=JNzxOJKCUug

https://www.youtube.com/watch?v=ln8NHzVfJkQ

https://www.youtube.com/watch?v=TMv5UuSAsDs

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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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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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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!

mailto:[email protected]

mailto:[email protected]

Documents

Hearing Technology: Current & Future Options