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The Effect of UNHS on Early Cochlear Implantation

Antonia Brancia Maxon, Ph.D.Diane Brackett, Ph.D.

Jennifer Cox, M.A.Alicia Ayles, M.A.

New England Center for Hearing Rehabilitation

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Why is Early Identification of Hearing Loss so Important?

• Congenital hearing loss interferes with the most basic human need to communicate with others

• Hearing loss is the most frequent birth defect

• Undetected hearing loss has serious negative consequences

• There are dramatic benefits associated with early identification of hearing loss

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UNHS lowers age of intervention

• Infants with hearing loss should be fit with hearing aids by four months old

• Infants with hearing loss should be enrolled in early intervention by four months old

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UNHS lowers age of intervention

• When a six month hearing aid trial is used– Benefit from traditional amplification

can be determined by 10 months old

• Infants who do no benefit should not have to wait for the implant.

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UNHS affects the age of cochlear implant candidacy identification

• Until recently the recommended age of implantation was 18 months old.

• Now 12 months is recommended lowest age.

• Surgeons are implanting even younger infants.

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Early Identification Ensures Some Critical Criteria Are Met

• Objective measures are available to make a diagnosis within the first month of life.– will know degree of hearing loss– will know the configuration of hearing loss– will know if there are middle ear problems

• Can begin process of candidacy evaluation

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Early Identification Ensures Some Critical Criteria Are Met

• Parents have accepted the hearing loss and are ready to begin appropriate intervention.

• Parents are aware of communication modality options.

• Parents know the role of and need for sensory devices.

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Candidacy

• Bilateral severe to profound sensorineural hearing loss

• Infant/toddler cannot benefit from traditional amplification

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Diagnosis to Decision - Parent

• Need to understand:– no device is a cure – therapy is long-term– language learning is an ongoing

process– access to acoustic speech cues

• how access relates to child’s progress

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Diagnosis to Decision - Parent

• Need to:– understand the difference between

reacting to cues received through hearing and other senses

– identify auditory responses– learn how to use the home, family,

environment in a positive way for auditory and language learning

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Diagnosis to Decision - Parent

• Need to be comfortable with equipment– know how to use it properly– know how to modify clothing to

accommodate it– accept that people will look, question,

comment on it– able to troubleshoot equipment and

child’s auditory behavior

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Diagnosis to Decision - Parent

• Need to understand the audiogram– know the degree and configuration– know the basics of acoustic cues of

speech– know how speech perception is

affected by their child’s hearing loss– know what their child actually

receives

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Diagnosis to Decision - Child

• Child needs to be fit with best amplification– must get the most auditory

stimulation possible• body-worn FM system • snap-on transducers• power transducers• earmolds - good fit at all times

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Diagnosis to Decision - Child

• Child needs to be enrolled in an EI program that stress auditory function– learning to use hearing– learning to respond to sound– learning to respond to speech– learning the need for communication

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Assessing Auditory Function

• From initial enrollment in EI always assessing auditory function

• Should not make decision about hearing aid or cochlear implant based on pure tone audiogram

• Need to determine if child is able to make maximal use of residual hearing

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Assessing Auditory Function

• Child needs to receive speech at an appropriate level with amplification– detection is not enough– have to look across frequencies for

access to all speech sounds– speech needs to be received at a

useable level

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Assessing Auditory Function

• Child must have good access to speech of others and own speech– at different distances from sound

source

• Child should alert to a variety of speech and non-speech sounds– at different distances from sound

source

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Assessing Auditory Function

• Child’s output– vocalization

• precursor to speech production

– intentional vocalization• precursor to language

– vocalization + gesture• intention to communicate

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Indications the Child is a Candidate

• Child shows limited or no detection of amplified speech

• Comparisons of audiological data and functional use of hearing

• Knowledge of the child’s auditory limitations

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Factors that Facilitate CI Success

• Parents know about hearing loss and accept long-term problems

• Parents understand the implant is not a cure

• Parents are committed to implant use

• Parents are committed to therapy

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Factors that Facilitate CI Success

• Family has access to therapy and mapping facilities

• Family is motivated• One parent at home - minimal day

care• The household is organized• Child is vocalizing• Child has normal cognitive level

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Absence of one or more of those facilitating factors does not rule

out the child’s candidacy.

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Why Implant Early

• Critical period of communication development

• Children implanted below 10 years old– children implanted before age 3 showed

a greater period of auditory plasticity– open-set speech recognition could not

be achieved after 6 years old (Manrique, et al, 1999)

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Why implant early?

• Early is better for children with hearing aids (Apuzzo & Yoshinaga-Itano, 1995)

• Early implantation – allows for extended listening time before

social and school demands start– decreases chance of a large gap between

chronological and communication age– promotes acquisition of speaking and

language

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Why implant early?

• Children implanted early (2-3 years) make greater growth than later implanted (3-5 years) children– expressive vocabulary– receptive vocabulary– dramatic improvements first year post

implant (Brackett and Zara, 1998)

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Why implant early?

• Surgical risks are minimal• Complications are infrequent• Objective testing can be used to

predict appropriate programming• Can use audiological behavioral

measures for programming (Cohen, Drous, Shapiro & Waltzman,

2003)

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Information Needed by Parents• Parents wanted most information prior

to surgery, but wanted continued informational support post-implant

• Parents felt emotional support was most lacking

• Majority of parents felt there needed to be a professional liason between CI center and educational program (Most and Zaidman-Zait, 2003)

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Cochlear Implant Surgeon Selection

• Competent and experienced with infants

• Familiar with surgical placement of receiver/stimulator package that reduces potential damage from head impact

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Cochlear Implant Surgeon Selection

• Familiar with techniques that reduce intracranial complications

• Willing to make accommodations for child and family regarding hospitalization, pre- and post-surgery conditions

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Mapping Audiologist Selection

• Competent and experienced with infants

• Familiar with VRA• Availability of multiple testers• Availability of appropriate

equipment and reinforcement

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Mapping Audiologist Selection

• Knows about speech acoustics• Uses functional measures to fine

tune the map• Accepts input from parents and

rehabilitation professional• Maps the implant “on demand”• Knows about aural rehabilitation

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Surgery to Stimulation

• Child – becomes familiar with and gets used

to equipment– uses hearing aid on non-implanted ear

• Aural rehabilitation – communication focus continues– conditioning sessions to facilitate

mapping

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Initial Stimulation

• Short term goal is good access to speech across frequencies

• Weekly sessions until have a good map

• Use input from parent(s) and rehabilitation professional

• Use functional responses to modify maps

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Rehabilitation Professional Selection

• Knowledgeable about:– infant behavior– infant development– infant communication– auditory development– activities that facilitate auditory-vocal

behavior

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Rehabilitation Professional Selection

• Knowledgeable about:– infant adaptation to equipment– home therapy suggestions– troubleshooting the equipment– troubleshooting the child’s behaviors

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Rehabilitation Programming

• Keeping external components on head• Keeping body processor comfortable• Continued conditioning for mapping

when necessary• Mapping as required

– parent request– rehabilitation professional request

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Case Study - Stevie

• UNHS - failed bilaterally (before mandate in CT)

• Bilateral profound SNHL diagnosed - 5 mos

• Hearing aids fit - 5 mos

• Enrolled EI (2x/week) - 6 mos

• FM fit - 10 months

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Case Study - Stevie

• Aided response levels - 50-60 dB HL

• Aided SAT - 45 dB HL

• CI evaluation - 10 mos

• CI surgery - 13 mos

• Initial stimulation - 14 months

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Case Study - Stevie(evaluation at 32 months)

• Speech perception: 100% spondees, 83% monosyllables

• Speech production: 75% consonants, 97% vowels

• Vocabulary: Receptive - not basal, Expressive - <1.9 years

• Utterances: 4-5 words, not many verbs, rising inflection for questions

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Case Study - Stevie(evaluation at 46 months)

• Speech perception: 100% consonants, 100% vowels

• Speech production: 90% consonants, 100% vowels

• Vocabulary: Receptive - 10 month delay, Expressive - 8 month delay

• Sentences: 5-7 words, nouns and verbs some adjectives, possessive, plural markers emerging, some pronouns.

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Case Study - Anna

• UNHS - failed bilaterally (post mandate)

• Bilateral profound SNHL diagnosed - 3.5 weeks

• Hearing aids fit - 4 weeks

• Enrolled EI (2x/week) - 4 weeks

• Hearing aids and FM fit - 4 weeks

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Case Study - Anna

• Aided response levels - 40-50 dB HL

• Aided SAT - 35 dB HL

• CI evaluation - 10 mos

• CI surgery - 11 mos

• Initial stimulation - 12 months

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Case Study - Anna(evaluation 28 months)

• Speech perception: excellent• Speech production: errors - m/n, g/d,

omits ch, t, m, z• Vocabulary: Receptive - 21 months,

Expressive - 21 months• PLS: Auditory Comp - 31 months,

Expressive Comp - 28 months, Total Language - 30 months

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Universal Newborn Hearing Screening

• Lowers the age of hearing loss diagnosis

• Lowers the age amplification is provided

• Lowers the age of enrollment in early intervention

• POTENTIALLY lowers the age of cochlear implantation

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Benefits of Early Cochlear Implantation

• Minimizes the effects of auditory deprivation

• Electrical stimulation provides necessary information to auditory system at critical period for speech and language acquisition.

• Provides child with a good chance to develop normal speech and language