Alexandra D. Costlow, B.S.

Cochlear Implant (CI) AppointmentsGeneral Points

Implantation requires an ongoing commitment of time and resources

The appointment process begins at the candidacy stageHowever, there may have been appointments

with related professionals prior to the candidacy stage

Referral to the audiologist, CI center, etc…

CI AppointmentsGeneral Points

Each appointment should support a successful implantation process as a whole

“Progress is not always a straight line” in reference to the implantation processThe normal series of appointments may be

individualized based on the needs of the patient

CI AppointmentsStages

Candidacy

Surgical Implantation

Follow-Up Care

CI Candidacy AppointmentsReview

Preoperative assessment should be performed at a CI center staffed with clinicians offering experience in postimplantation management.

Clinicians analyze risk-to-benefit ratio through outcome predictors and modifiersFactors that affect performance, perceived

benefit, and long-term use of a CIAn assessment of relative benefit aids in the

decision-making process and helps in aligning the candidate’s expectations with probable outcomes

CI Candidacy AppointmentsReview

Hearing Assessment, including Auditory Skills Assessment

Otologic and Medical AssessmentOpthalmologyPsychological AssessmentLanguage, Educational, and Development of Multimodal Processing Assessment (for the pediatric/school-aged population)

CI Candidacy AppointmentsHearing Assessment

Hearing Assessment by a licensed audiologist Determines a baseline through characterization of

Residual hearing Functional hearing Response to amplification

Changes to Hearing Assessment Movement away from hearing sensitivity alone Instead, consider the patient’s experience with

effectively accessing speech with amplification Note the constraints of hearing aid usage (Niparko,

2009)

CI Candidacy AppointmentsAuditory Skills Assessment

“… Evaluates child’s ability to attend to and integrate sound using conventional amplification” (Niparko, 2009). Speech and environmental sounds over a range of

frequencies Integrate auditory perception with speech

production in order to imitate sounds Make meaningful associations for sounds from

single words to conversation Overall, to integrate hearing into communication

CI Candidacy AppointmentsOtologic Assessment

Determination of the etiology of the hearing loss helps guide the implantation process Ex.: Usher Syndrome patients will also have gradual onset vision loss

Ex.: Patients with Neurofibromatosis II who have bilateral acoustic tumors will benefit more from Auditory Brainstem implants

Medical AssessmentEvaluates patient’s fitness for general anesthetic and

mastoid surgery, as well as device programming and postimplantation rehabilitation

Candidacy AppointmentsOpthalmology

Vision plays a critical role in the deaf child’s development in that it allows the child to associate meaning with auditory inputs (Niparko, 2009).

An opthalmology exam can identify visual abnormalities associated with congential sensorineural hearing loss Refractive errors and cataracts

These visual abnormalities can help to diagnose etiologies such as Usher andWaardenburg syndrome

CI Candidacy AppointmentsPsychological Testing

Screen for psychopathology and cognitive deficits Mood disorders can cause elevations in depression,

social introversion, suspiciousness, social anxiety, and loneliness in adult CI candidates (Knutson, Johnson, & Murray, 2006)

Cognitive defects can indicate the need for unique rehabilitation strategies

CI Candidacy AppointmentsCounseling to ensure suitability and

motivation to participate in the processExpectations of what the CI can and can not doDetermination of family and/or social supportPatient acknowledgement and acceptance of

the time and resources required for care a follow-up service

Device DiscussionTo ensure that the patient and his/her family

understand the motivation and resources required for successful CI implantation and use

May discuss differences between the different implants

May discuss insurance coverage or fundingReiterate that pre-implantation expectations

will shape post-implantation success

CI Candidacy AppointmentsTests performed by the surgeonAnalysis of the brain and middle and inner ear

Computerized Axial Tomography (CAT Scan)/X-rays CT scanner x-rays around the circumference of the patient Detectors in the CT scanner measure how much x-ray is

transmitted through the area of the body of interest CT Scan analyzes soft tissue (including organs), bone, and

blood vessels Is there new bone growth in cochlea due to meningitis?

3D images of organs are created by stacking slices of images

New, multidetector scanners scan the body in about 30 seconds

CT Scan of Cochlea

http://www.sciencedaily.com/releases/2008/04/080425151819.htm

CI Candidacy AppointmentsMagnetic Resonance Imaging (MRI)

Uses powerful magnets and radio waves The scanner contains a magnet whose field is ~10,000x

greater than the earth’s magnetic field Magnetic field causes hydrogen atoms in the body to align in

a specific configuration Radio waves are projected onto the hydrogen atoms, and

bounce back Computer records the signal, which is specific to the type of

tissue MRI more effectively images soft tissues (brain) than does CT Single MRI images are also called slices The exam usually takes 1 hour or longer, depending on the

number of slice being analyzed

MRI (at 3 tesla) of Cochlea

http://www.vanderveer.org.nz/research/labs/mri.php

CI Candidacy AppointmentsCAT Scan and MRI

Analyze the structure (and presence or absence) of the 8th Cranial Nerve (Auditory)

Can identify structural abnormalities of the cochlea and/or mastoid bone that could compromise the surgery

Can identify space-occupying lesions that result in hearing loss

SurgeryThe patient is deemed a candidate and

decides to go forth with implantation.Surgery may involve an overnight stay at a

hospital, but generally, it can be performed on an out-patient basis.

It takes place under general anesthesia and takes 1 to 2 hours.

Review of CI Components Microphone- picks up sound from the environment Speech Processor- placed behind the ear or worn on the body, it selects and arranges sounds detected by the microphone Coil- held in place by a magnet, radio waves transmit the coded signal to the transmitter Transmitter- placed internally, it receivessignals from coil and converts them into electrical impulses Electrode array- placed in the cochlea, it collects impulses from

the transmitter and stimulates portions of the auditory nerve (NIDCD, 2009)

SurgeryThe surgeon makes a trans-mastoid facial recess

incisionThe surgeon drills into the mastoid bone in order to

access the inner earA depression is created in bone behind the mastoid

to accommodate the transmitter (receiver-stimulator) internal device

The electrode array is threaded into the scala tympani through the round window membrane or cochleostomy (25-30 mm is full insertion)

The cochleostomy is sealed around the electrode with fibrous tissue

SurgeryThe outer end of the electrode array is

fastened to the skullThe scalp should be thinned to no more

than 1cm to enable stable retention of the magnet

The incision is closedThe patient returns in about one week for

suture removal

Surgery Part Ihttp://www.youtube.com/watch?

v=x7ltzA0B2X8&feature=related

Surgery Part IIhttp://www.youtube.com/watch?

v=I0Z3eKNw2vc&feature=related

Activation and Initial FittingDays after the surgical implantation of the

receiver (just behind the ear) and the electrodes (in the cochlea), the patient returns to the CI center.

An audiologist fits the patient with:A microphone (resembles a BTE hearing aid)A speech processor (may behoused with the microphone or worn at chest-level)

Activation and Initial FittingAudiologist runs standard check of the speech processorInitial activation and programming (mapping) of the implant

Mapping- a set of parameters of electrode stimulation that gives the patient maximum hearing

Establishment of electrical dynamic range (Level of Comfort* – Threshold** for each electrode)

May occur over several appointments because the patient will adjust to sound as s/he gains experience with the implant

Appointments are generally 2 hours, of which 20-30 minutes are spent obtaining T and C Levels (Craver, 2010).

Activation and Initial FittingHow is mapping conducted?Using speech (subjective)Using tones/beeps/bursts (subjective)Neural Response Telemetry (objective)

Telemetry is the remote measurement of various electrical parameters (in our case, through implant feedback)

Neural Response Telemetry measures the response of the auditory nerve to electrical stimulation via a cochlear implant (The Hearing House).

NRT takes about 5 minutes to complete (Craver, 2010).

Neural Response Telemetry

http://www.uzh.ch/orl/lea/nrt.jpg

Map ParametersStrategy- Method by which sound is analyzed

and presented, which varies by manufacturerStimulation- Specifies the difference between

active and indifferent electrode modeRate- Pulses per second (frequency) of

electrical currentPulse Width- Amount of time the electrical

current (pulse) is delivered in microseconds (Carver, 2007)

ActivationCochlear Implant Activation – Captured

http://www.youtube.com/watch?v=YFBUNJtT39Y&feature=related

Follow-Up to Initial FittingMay include several visits over the span of weeks or monthsWhy is this such a lengthy process?

Each electrode in the cochlea is activatedEach electrode must be programmed and adjusted into

the speech processorCan create programs for special listening situationsThe patient develops more skill from using the implant,

thus more adjustments must be made as skill improvesOver time, less adjustments are necessary and the

patient will return to the CI center every 6 months or annually

Appointment time can be spent on education and rehabilitation (Craver, 2010).

Follow-Up to Initial FittingSample Programming Schedule

Days 1, 2: Activation of the external equipment, which occurs approximately 4 weeks after surgery

1 week: Audiogram and reprogramming1 month: Audiogram and reprogramming3, 6, 9 months: Audiogram, Speech Perception

Testing and reprogramming1 year: Audiogram, Speech Perception Testing and

reprogrammingEvery 6-12 months thereafter: Audiogram, Speech

Perception Testing and reprogramming (Carver, 2007).

Aural RehabilitationPrevious vs. New Listeners

Patients with previous experience hearing and listening should be counseled that speech will have a different sound quality.

They may report that CI speech sounds unnatural.

Patients who are new to hearing sound and speech will need to learn perceive sound and how to respond to it.

Aural RehabilitationTeaches the patient how to use the CI and respond to auditory input

Listen to an array of auditory stimuliImprove speech (expressive and receptive)Use speech-reading

AR Programs DASL II Speech that Works Cottage Acquisition Scales for Listening, Language, &

Speech The Miami Cochlear Implant, Auditory & Tactile Skills

Curriculum (CHATS)

Aural Rehabilitation ActivitiesAdults ChildrenMay focus on social or

occupational skills and/or demands

Occupation/trade specific vocabulary or communication skills

Practice communication skills using current events, popular television shows, etc…

Alerts: car horn, microwave, etc…

“Mother May I?” Board games: The child

advances if he successfully speechreads or can hear and/or repeat stimuli correctly

Ability to follow directions common to school

Ability to function in a social setting (ex: playground, cafeteria, sports game)

Alerts: fire alarm, whistle, etc…

Outcomes of CI PerformanceHearing Aid + CI

Ching, Incerti, and Hill (2004) tested 21 adults who used a Nucleus CI-22 or CI-24 and either unilaterally or with a Ha set to NAL-NL1 and loudness-balanced to the CI. They found that CI+HA users, compared to unilateral CI or HA users, had better:Speech perception in noise on dichotic and diotic listening

conditions Subjects who performed better in speech perception also had

benefit in localization and functional performance in everyday lifeHorizontal localizationFunctional performance in everyday life evaluated by a

functional performance questionnaireAll subjects showed binaural benefit in at least one

measure

Hearing Aid + CILitovsky, Parkinson, Arcaroli, Peters, Lake,

Johnstone, and Gonqiang (2004) tested 17 adults and 3 children with one CI and then bilaterally.

Adults localize better with 2 bilateral CIsAdults perform better hearing speech in noise with

bilateral CIs when the noise is in the poorer ear Children perform better on localization and speech

recognition with bilateral CIs, but not remarkablyOne child did better with a unilateral CI and two

children had better speech recognition when noise was presented to the ear that was implanted first

Bilateral CIs vs. Unilateral CITyler, Gantz, Rubenstein, Wilson, Parkinson, Wolaver,

Preece, and Lowder (2002) evaluated 9 post-lingually deafened adults using the Cochlear Corporation C24M implant for speech in quiet, speech in noise, and localization ability.Bilateral CIs showed a significant advantage over CI in th

better ear for speech in quietand for speech in noise (located at 0 degrees azimuth) for 4/9 subjects

When CI was added to ear ipsilateral to noise, a significant advantage was noted for 4/7 subjects

3/7 subjects could discriminate noise at 45 degrees with one CI, but 7/7 could with bilateral CIs

Conclusion: Bilateral CIs offer advantage, especially when for ear contralateral to noise

Bilateral CIs vs. CI+HALitovsky, Johnstone, and Godar (2006)

evaluated 20 children (20 use bilateral CIs, 10 use CI+HA) in their ability to hearing in quiet and in noise, and to localize sound. Both groups have similar speech reception

thresholdsImproved localization and speech thresholds

with bilateral CI group compared to CIHA groupIndividual variability suggests that some

children perform as well as normal hearing children while others do not

Where to Go from Here?CI+HA and bilateral CIs offer benefit over unilateral CIBilateral CIs offer benefit over CIHA (Litovsky, Johnstone, &

Godar, 2006).HA should be programmed to NAL-NL1 Rx for adults and then

adjusted on an individual basis (Ching, Incerti, & Hill, 2004).Facilitate bimodal amplification by setting stable maps and

then adjust HAs (Ching, Psarros, Dillon, & Incerti, 2001).Should further investigate the differences in success with

bilateral CIs between adults and children (Litovsky, Parkinson, Arcaroli, et al., 2004)

Should determine why some children are more successful with CIs than are others (Litovsky, Johnstone, & Godar, 2006).

Many complicating factors to CI success

Deaf CultureThe Basics

Deaf Culture is linguistically unified through American Sign Language (ASL)ASL is not a manual translation of EnglishASL has its own syntax, morphology, and vocabularyASL does not have a written correlate

Deaf individuals share an identity based on a culture rather than a medical diagnosis

“Deafness, particularly when early in onset, confers a life experience that is radically different owing to a systematically different language base not shared by the majority of hearing culture” (Niparko, 2009).

Deaf Culture and Cochlear ImplantsConflict of Cross-Cultural Values

Child as Recipient Impact on Deaf CultureParental Authority-

Hearing parents who implant deaf children are viewed as “ill-founded” and “ill-fated” in their decision

Deaf do not view deafness as a disease, and it is unethical to operate on a healthy child

CIs fail to foster language acquisition in children born deaf (Lane & Bahan, 1998).

Socio-cultural genocideUndermines the survival

of Deaf culture

Deaf Culture and Cochlear ImplantsAdvocates for Deaf individuals

The National Association of the DeafThe World Federation of the DeafThis cross-cultural conflict is without a resolution that is

morally valid (Lane & Bahan, 1998)Research is needed to determine why some children with

CIs are successful in the hearing world and other are notClinical trialsHigh quality, generalizable results

References American Speech-Language Hearing Association (ASHA). Cochlear implants. Retrieved from

http://www.asha.org/public/hearing/treatment/cochlear_implant.htm

Carver, C.L. (2007). Cochlear implant mapping: What every CI user and candidate should know.

Children’s Hospital and Health System. (2010). Cochlear implantation clinic. Retrieved from http://www.chw.org/display/PPF/DocID/25735/Nav/1/router.asp

Ching, T.Y., Incerti, P., & Hill, M. (2004). Binaural benefits for adults who use hearing aids and cochlear implants in opposite ear. Ear and Hearing, 25(1), 9-21.

Ching, T.Y.C., Psarros, C., Hill, M., Dillon, H.,& Incerti, P. (2001). Should children who use cochlear implants wear hearing aids in the opposite ear? Ear and Hearing, 22(5), 365-380.

Contradica. (2007, November 26). Cochlear implant activation – captioned [Video file]. From http://www.youtube.com/watch?v=YFBUNJtT39Y&feature=related

Dillier, N. (2010). Illustration of the setup for intracochlear recordings of electrically evoked compound action potentials (NRT, Neural Response Telemetry). From http://www.neuroscience.ethz.ch/research/sensory_systems/dillier/index

References Kim, H.J. (1993). Congenital inner ear malformations. Retrieved from Baylor College of Medicine’s

website http://www.bcm.edu/oto/grand/111193.html

Knutson, J.F., Johnson, A., & Murray, K.T. (2006) Social and emotional characteristics of adults seeking a cochlear implant and their spouses. British Journal of Health Psychology, 11(2), 279-292.

Lane, H, & Bahan, B. (1998). Ethics of cochlear implantation in young children: A review and reply from a Deaf-World perspective. Otolaryngology and Head Neck Surgery, 119, 297-313.

Litovsky, R.Y., Johnstone, P.M., & Godar, S.P. (2006). Benefits of bilateral cochlear implants and/or hearing aids in children. International Journal of Audiology, 45(S1), 78-91.

Litovsky, R.Y., Parkinson, A., Arcaroli, J., Peters, R., Lake, J., Johnstone, P, & Gonquiang, Y. (2004). Bilateral cochlear implants in adults and children. Archives of Otolaryngology Head and Neck Surgery, 130(5), 648-655.

MedLine Plus. (2008). CT scan. Retrieved from http://www.nlm.nih.gov/medlineplus/ency/article/003330.htm

MedLine Plus. (2008). Head MRI. Retrieved from http://www.nlm.nih.gov/medlineplus/ency/article/003791.htm

References National Institute on Deafness and Other Communication Disorders (NIDCD). (2009). Cochlear

implants. From http://www.nidcd.nih.gov/health/hearing/coch.asp

Niparko, J. (2009). Cochlear implants. Baltimore, MD: Lippincott, Williams, and Wilkins.

Nussbaum, D. (2003). Cochlear implants: Navigating a forest of information … one tree at a time. From Gallaudet University Laurent Clerc National Deaf Education Center’s website http://clerccenter.gallaudet.edu/Clerc_Center/Information_and_Resources/Cochlear_Implant_Education_Center/CI_Navigating_a_Forest/What_is_a_Cochlear_Implant/Cochlear_Implant_Candidacy.html

Science Daily. (2009). Low frequency hearing linked to shape of cochlea. Retrieved from http://www.sciencedaily.com/releases/2008/04/080425151819.htm

Tbhbc. (2009, April 14). Cochlear implant surgery part 1 (without sound) [Video file]. From http://www.youtube.com/watch?v=x7ltzA0B2X8&feature=related

Tbhbc. (2009, April 14). Cochlear implant surgery part 2 (without sound) [Video file]. From http://www.youtube.com/watch?v=I0Z3eKNw2vc&feature=related

The Hearing House. Glossary of terms. From http://www.hearinghouse.co.nz/information+for+parents/glossary+of+terms

References Tyler, R.S., Gantz, B.J., Rubenstein, J.T., Wilson, B.S., Parkinson, A.J.,

Wolaver, A., Preece, J.P., Witt, S., & Lowder, M.W. (2002). Three-month results with bilateral cochlear implants. Ear and Hearing, 23(1), 80S-89S.

University of Maryland Medical Center (UMMC). (2010). Maryland hearing and balance center: Cochlear implant program. Retrieved from http://www.umm.edu/otolaryngology/cochlear.htm#noE

U.S. Food and Drug Administration (FDA). (2009). Medical devices: Cochlear implants. Retrieved from http://www.fda.gov/MedicalDevices/ProductsandMedicalProcedures/ImplantsandProsthetics/CochlearImplants/default.htm

Van Der Veer Institute for Parkinson’s and Brain Research. (2007). Magnetic resonance Imaging at 3 tesla – The first in New England. Retrieved from http://www.vanderveer.org.nz/research/labs/mri.php