A Multidisciplinary Approach to the
Management of Children with ANSD:
The Importance of Collaboration
Shelly Ash, MS, CCC A
Anne Oliver, MA, MEd, CCC SLP
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A Multidisciplinary Approach to the
Management of Children with ANSD:
The Importance of Collaboration
Shelly Ash, MS, CCC A
Anne Oliver, MA, MEd, CCC SLP
Learner Objectives
The attendee will become familiar with the medical testing for management for children
with ANSD.
The attendee will become familiar with the audiological testing, management and
associated challenges for children with ANSD, and the speech and language testing,
management and associated challenges for children with ANSD.
The attendee will become familiar with a team approach to managing the hearing, speech
and language related needs of children with ANSD and when these children may be
considered for cochlear implantation.
What is ANSD?
What is ANSD?
• Auditory Neuropathy Spectrum Disorder
A unique type of hearing loss where sounds aren’t
clearly transmitted to the brain from the ear.
What is ANSD?
• Hearing loss of some degree (can vary
widely from mild to profound loss)
• Difficulty understanding speech,
especially in noise.
What is ANSD?
• Speech understanding difficulties that
are worse than can be predicted from
other tests of hearing function.
• Hearing that appears to fluctuate from
day-to-day and even from hour to hour.
What does ANSD sound like?
Car radio analogy (credit Elaine Blackford)
ANSD Prevalence
• Accounts for 8-15% of new pediatric
hearing losses
• Teagle et al, 2010, Hang et al 2012
ANSD
• Presents a unique challenge to patients,
families and professionals!
The Multidisciplinary Team
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Family
Audiologist
Speech Pathologist
Otolaryngologist
Educators
Other treating professionals
– Neurology, Occupational Therapist, Physical Therapist
Challenges in Diagnosing ANSD
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Diagnosis
Prognosis
What treatments will be of benefit?
What treatments will suit the desires and anticipated outcomes
of the family?
• ??
Evaluative Measures for
Diagnosis
• Medical
• Audiological
• Speech and language
Medical Evaluation
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Otologic
Radiological studies (MRI/CT)
Lab work
Genetics
Neurology
Opthalmologic
Otologic Evaluation
• Medical history
• Ear exam
• Radiologic Studies
Otologic Evaluation
• Medical History
– Evaluation for other health problems
• Developmental
• Vision
• ??
Otologic Evaluation
• Ear exam
Otologic Evaluation
• Radiological studies (MRI/CT)
– Evaluate for cochlear integrity, malformations
– Evaluate cochlear nerve integrity
Role of the Otolaryngologist in the
Management of ANSD
• To provide a thorough medical examination and recommend
testing as needed to diagnose etiology or associated conditions
Role of the Otolaryngologist in the
Management of ANSD
• To provide medical clearance for the use of hearing aids
Role of the Otolaryngologist in the
Management of ANSD
• To make recommendations to the family and/or team regarding
additional specialists that should be consulted relative to
etiology or associated conditions
Audiological Evaluation
• Auditory Brainstem Response testing
• Acoustic Immittance testing to include Tympanometry and
Acoustic Reflex testing
• Otoacoustic Emissions testing
• Behavioral Audiometry
Audiological Evaluation
• Speech Perception Measures
– Infant Toddler Meaningful Auditory Integration Scale or
Meaningful Auditory Integration Scale
– Early Speech Perception Test
– Multilexical and Lexical Neighborhood Word tests
– HINT sentences
Audiological Evaluation:
Challenges
• Pure tone thresholds may not reflect the true degree of auditory
deficit
• Especially critical when making recommendations regarding
consideration for cochlear implantation
Audiological Evaluation:
Challenges
• Poor behavioral test/retest reliability may not reflect an
uncooperative patient
Audiological Evaluation:
Challenges
• For ANSD, behavioral thresholds are NOT a primary
consideration for cochlear implant candidacy.
• Breneman, Gifford and DeJong, 2012
Audiological Evaluation:
Challenges
• Behavioral test methods must be administered in consideration
of the child’s developmental age and skills
Audiological Evaluation:
Necessities
Be thorough in collecting objective measures
Audiological Evaluation:
Necessities
• Give strong consideration to the observations of parents,
teachers and/or other treating professionals
Audiological Evaluation:
Necessities
• It is imperative to obtain a thorough evaluation of expressive
and receptive language skills
Speech and Language Evaluation
• Collaboration with Audiologist
– Review audiological testing results
– Discuss observations
– Prepare and plan
• Objective and Subjective Measures
– Standardized tests
– Parent observations
– Therapist observations
Speech and Language Evaluation
Functional Auditory Skills
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Required tests:
Ling 6 Sound
LittlEars
(Parent Report!)
Speech and Language Evaluation:
Functional Auditory Skills
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Probe and observe:
Responses to noisemakers
Responses to environmental sounds
Responses to Learning to Listen Toys
• Responses to speech (calling name, simple phrases)
Speech and Language Evaluation:
Functional Auditory Skills
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Optional:
Auditory Learning Guide
SPICE
Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS)
Listening Skills Scale for Kids with Cochlear Implants
Auditory Skills Hierarchy (Erber)
St. Gabriel’s Curriculum (Audition)
ELF
CHILD
Auditory Processing Abilities Test (APAT)
APT-HI
Speech and Language Evaluation:
Language Skills
• Required tests:
• Rossetti Infant-Toddler Language Scale (Language
Comprehension/Expression)
• Preschool Language Scale
• Clinical Evaluation of Language Fundamentals (CELF)
Speech and Language Evaluation:
Language Skills
• Optional:
• St. Gabriel’s Curriculum (Language)
• SKI-HI Language Development Scale (if school system hasn’t
already administered it)
• Test of Oral Language Development (TOLD)
• Expressive One Word Picture Vocabulary Test (EOWPVT)
• Receptive One Word Picture Vocabulary Test (ROWPVT)
Speech and Language Evaluation:
Language Skills
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Observe and Document:
Intentionality
Communicative Functions (and how they are met)
Parent’s primary mode of communication (speech/sign/natural
gestures)
Speech and Language Evaluation:
Speech Skills
• Vocal and Phonemic Repertoire form
• St. Gabriel’s Curriculum (See Speech)
• Goldman Fristoe Test of Articulation
Speech and Language Evaluation:
Speech Skills
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Observe and Document:
Phonemic repertoire
Spontaneous vocalizations
Imitative vocalizations (pitch/duration/intensity)
Frequency of use of voice
Ease of vocalizations, spontaneous and imitative
Ease of imitation of vowels and consonants
Stimulability of vowels and consonants
Intelligibility of word approximations
Intelligibility of connected speech
Resonance (if concerns, give Bzock)
Speech and Language Evaluation:
Other Areas of Development
• Oral Mechanism Exam
• Voice/Fluency
• Rossetti Infant-Toddler Language Scale
(Interaction/Attachment, Pragmatics, Gesture, Play)
• St. Gabriel’s Curriculum (Cognition, starting at 6 months)
Challenges in Managing ANSD
Patients
• Auditory responses are variable. Parents describe “good hearing
days” and “bad hearing days”. This variability makes it difficult
to judge child’s listening skills.
• Significant health issues and challenges often accompany ANSD.
(Many infants with this diagnosis have had a difficult and
complicated neonatal course).
• Due to the variable presentations of ANSD, and the variety of
outcomes, parents often express feelings of confusion regarding
the diagnosis and anxiety about their child’s future.
Challenges in Managing ANSD
Patients
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Decisions regarding cochlear implantation are complicated by
fluctuating auditory functioning and fluctuating speech and language
performance.
Cochlear implant candidacy considerations are different for children
with ANSD diagnosis. (If insufficient speech and language progress,
they are considered CI candidates regardless of audiometric
thresholds).
Auditory functioning may change over time and future outcomes with
CI cannot be predicted.
Challenges in Managing ANSD
Patients
• Professionals and interventionists may be unfamiliar with the
diagnosis of ANSD and its implications. This may make finding
the appropriate interventionist difficult.
• Evidence-based data comparing outcomes of visually based
intervention and auditory based intervention with children who
have ANSD is lacking.
Challenges in Managing ANSD
Patients
• The SLP can expect to observe auditory behaviors that are
different from both hearing children and children with hearing
loss.
Making Decisions as a Team:
Considerations
• Language acquisition and therapeutic intervention
• Trial period with hearing aids and/or FM system
• Cochlear implantation
Making Decisions as a Team:
Considerations
• The benchmark for children with ANSD is language acquisition
and development.
• The goal is for the child to make a month of progress in
language skills for every month enrolled in appropriate
intervention
Making Decisions as a Team:
SLP Role in Language Acquisition
• To develop the child’s language and listening skills
• To coach the family in how to incorporate language and
listening into daily routines
• To help the team (Family, School, Audiology,) determine if the
child is benefiting from hearing aids
Making Decisions as a Team:
SLP Role
• To monitor (through systematic observations and analysis) and
report progress/lack of progress in speech, language and
listening to the team
• To collaborate with the treating audiologist and/or the cochlear
implant team on a regular basis
• To provide ongoing counseling and education to the family
regarding ANSD and its impact on speech and language learning
Making Decisions as a Team:
• The primary goal of auditory intervention is to give the child
hearing sufficient for him/her to develop receptive and spoken
language. It is for this reason that speech recognition ability is
considered a primary outcome measure for assessing the
benefits of amplification.
• Humphries et al, 2013
Auditory Based Care for children with ANSD:
Monitoring Progress
• Auditory functioning and speech and language performance is
monitored and documented as part of every treatment
session.
• A tracking form is used to document the child’s progress
across sessions.
• Parents provide ongoing information about how the child is
functioning in different environments and are encouraged to
keep a log of auditory functioning
Add SLP Tracking Form here
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add
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Auditory Based Care for children with ANSD:
Progress Testing
• Documentation is completed, minimally, every three months.
This includes re-administration of formal tests (usually the PLS
and GFTA).
Auditory Based Care for children with ANSD:
Progress Testing
• Progress testing should include info on:
– Level and consistency of child’s comprehension of
spoken language
– Level and consistency of child’s expressive spoken
language
– Articulation abilities and intelligibility
• A comprehensive summary of child’s Auditory Functioning
including consistency and nature of child’s responses,
dependency on visual information *
Auditory Based Care for children with ANSD:
Progress Testing
• The progress report includes documentation of observations in
therapy as well as the observations of parents and others
(teachers, day care providers, grandparents etc.).
• Information is gathered from formal testing, tracking chart,
parent report/journal, and other resources such as SIFTER,
preschool SIFTER or FISHER.
Auditory Based Care for children with ANSD:
Progress Testing
• This information is then shared with the family and other team
members following the child’s progress in order for the team to
recommend future directions for intervention.
Making Decisions as a Team:
SLP Role
• To discuss parent’s goals for their child and provide guidance on
how to reach those goals, including education regarding
different habilitative options and available community services
• To guide family in accessing resources for education and
support
• To support the unique strengths and abilities of the child and
family
Hearing Aids and FM Systems
• Fitting challenges
– Functional test limitations
– ANSD patients have increased difficulties listening in noise
– Fluctuating hearing levels
Hearing Aids and FM Systems
• Fitting challenges
– ANSD identified in children with limited language creates
challenges in determining speech perception abilities with
hearing aids
Hearing Aids and FM Systems:
Audiologist Role
• To utilize all measures (behavioral and objective) to assist in
choosing and fitting hearing aids
Hearing Aids and FM Systems:
Audiologist Role
• To monitor and report progress regarding hearing aid use to the
team
Hearing Aids and FM Systems:
Audiologist Role
• To closely monitor the patient for any necessary adjustments or
modifications as needed
– Query SLP regarding child’s responses and use of sound.
– Discuss with parents what sounds child is responding to at
home, with and without the hearing aids on
Hearing Aids and FM Systems:
Audiologist Role
• To discuss with the family their goals for the child and provide
guidance on how to reach those goals.
– Collaborate with team SLP regarding habilitation options,
community resources and educational choices that support
the goals of the family and needs of the child
Hearing Aids and FM Systems:
Audiologist Role
• To discuss with families that ANSD presents a challenge in
making a prognosis regarding potential for success with
traditional hearing aids
– Pure tone audiogram is not a reliable measure
– Limited clinical tools available relative to making predictions
for success
Hearing Aids and FM Systems:
Audiologist Role
• To advise the family that consistency of hearing aid use and
participation in language therapy will be key to determining
success of the hearing aid fitting
Hearing Aids and FM Systems:
Audiologist Role
• Provide the family with recommendations and information
regarding considering cochlear implantation
Cochlear Implantation:
When to Consider?
• Is child capable of using auditory information as provided by
hearing aids (or without sensory devices) for language learning
purposes??
Cochlear Implantation:
When to Consider?
• Has the child made a month of progress in language
development for every month of therapy?
• Discuss with SLP the established auditory based goals for
language development and whether the goals are being
attained
• Discuss parental expectations and desired outcome in
language learning
Cochlear Implantation
• To best evaluate effectiveness of hearing aids, the child should
be enrolled in language therapy that emphasizes auditory based
goals.
Cochlear Implantation
• Prior to approving cochlear implantation, 3rd party payers may
require a hearing aid trial for a minimum of 3 months
Cochlear Implantation
• It is important to establish that the cochlear implant is of benefit
to children with ANSD. This further supports bypassing of
traditional audiological candidacy guidelines.
• Humphries et al, 2013
The All Children’s Hospital
ANSD Experience
• 23 children identified with ANSD
• 8 children implanted at ACH
• 6 implanted at other facilities
The All Children’s Hospital
ANSD Experience
• 11 currently enrolled in language
therapy at ACH
– 4 using cochlear implants
– 7 using hearing aids
– 2 in CI candidacy process
The All Children’s Hospital
ANSD Experience
• Breakdown of patients enrolled at ACH in therapy:
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0-3 year olds=
4-5 year olds=
6-8 year olds=
9-11 year olds=
0
7
4
0
The All Children’s Hospital
ANSD Experience – Multidisciplinary Approach
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Audiological evaluation and diagnosis of ANSD
Medical evaluation with otolaryngologist
Hearing aid trial
Enrollment in Auditory Based Care (ABC) Therapy
Consultation with educators
Recommendations regarding cochlear implant candidacy
The All Children’s Hospital ANSD
Experience
It Takes a Village!