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32 Screening, Assessment, and Management of Auditory Disorders in School-Aged Children
Madell et al., Pediatric Audiology: Diagnosis, Technology, and Management, 3rd Ed. (ISBN 978-1-62623-401-7),
copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
Table 32.3 EAA proposed outcomes for school-based audiology services
Person(s) Responsible
Outcome Area Audiologist Audiologist and School Team
Comprehensive educational
audiology services
Assessment 2. Children/youth receive diagnostic audiological
Support and educational
programming
Access and technology 10. Children/youth with auditory disorders have the
Psychosocial development 12. Children/youth with auditory disorders are
1. Children/youth access comprehensive educational
audiology services as part of their developmental and/or
education program.
evaluations within 30 days of referral from screening or
other source.
3. Children/youth receive the necessary medical
management required to habilitate medically treatable
hearing problems.
4. Children/youth receive audiological and auditory skill
assessments that are relevant to the developmental/
educational setting and that accurately identify the
parameters associated with the auditory decit.
opportunity to access appropriate and consistently
functioning hearing instrumentation, including personal
and assistive devices that maximize auditory access within
their environment.
5. Children/youth with auditory decits communicate
eectively with their peers, teachers, and others in their
environment.
6. Families are encouraged and supported to participate
fully in their child/youth’s education.
7. Teachers and other relevant professionals demonstrate
an understanding of the communication, learning, and
social implications of a child/youth’s auditory decit.
8. Children/youth with auditory decits receive instruction
that reects high educational standards that maximize the
child’s learning potential.
9. Children/youth with auditory decits understand and
utilize appropriate services in school and post high school
for education, employment, and life.
11. Children/youth with auditory disorders have full
and equal access to all components of their educational
environment regardless of their communication mode.
self-determined.
Table 32.4 Sample audiology performance standard and evaluation rubric from the Colorado Department of Education
Quality Standard 1. Audiologists demonstrate mastery of and expertise in the domain for which they are responsible.
Basic Partially Procient Procient (Meets State
Element A: Audiologists demonstrate knowledge of current developmental science, the ways in which learning takes place, and the appropriate levels of
intellectual, social, and emotional development of their students.
THE AUDIOLOGIST:
Relates general child and
•
auditory development to
audiological practices.
…and THE AUDIOLOGIST:
Applies knowledge of
•
auditory, language,
and psychosocial
development to practice.
Demonstrates an
•
understanding of
communication systems
and educational options
for children with hearing
loss.
32.4.3 Outcome Measures and
Professional Performance
Standards
Outcome measures address the impact of audiology services and
identify how each service element impacts students’ abilities to
function in school and after high school. Rather than a checklist
of services that are to be provided, this approach focuses on the
results of services. Table 32.3 contains the EAA’s proposed outcomes for school-based audiology services. Indicators for each
outcome are being evaluated in a pilot project through EAA.
Standard)
…and THE AUDIOLOGIST:
Assists others in
•
understanding the
auditory, linguistic, and
developmental factors
that impact student
learning.
Professional performance standards have been developed at
some state departments of education for teachers and related
service providers. These standards include components that are
student performance based, which makes them often challenging
because audiologists are not responsible for direct instruction in
traditional academic areas. Table 32.4 contains an example of
one of the standards and rubric from the Colorado State Model
Evaluation System developed with school-based audiologists
through the Colorado Department of Education.
school-based audiologists, as all special service providers, are
evaluated annually using this model in a self-assessment and
performance review process.
Accomplished Exemplary
…and STUDENTS OR
SIGNIFICANT ADULTS:
Demonstrate
•
understanding of the
impact of hearing
on development and
communication.
…and
STUDENTS:
Make progress toward
•
auditory, language, and
communication goals.
23
In Colorado,
341
IV Educational and Clinical Management of Hearing Loss in Children
Madell et al., Pediatric Audiology: Diagnosis, Technology, and Management, 3rd Ed. (ISBN 978-1-62623-401-7),
copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
32.5 Conclusion
School-based audiology services consist of comprehensive
audiology and related support services that serve children and
their families from the early years through graduation from
high school. The opportunity to inuence the development of
children, each unique in his or her own way, is a responsibility
that yields long-lasting rewards. Reecting on outcomes, the
increased emphasis on the students’ responsibility for self-management of auditory-related needs highlights the importance of
counseling and relevant services to support the development of
identity, self-determination, and self-advocacy.
Audiology provides technology and other tools for improving
hearing and accessing communication and the learning environment. However, those tools must be employed with the goal that
the students will be able to deploy them independently and differentially based on knowledge and understanding of themselves
beginning early on in their education.
References
[1] U.S. Federal Government. Electronic Code of Federal Regulations. Title 34: Edu-
cation. https://www.ecfr.gov/cgi-bin/text-idx?tpl=/ecfrbrowse/Title34/34tab_02.
tpl. January 2, 2018. Accessed January 6, 2018
[2] Americans with Disabilities Act as amended, Titles I–III, 42 USC 126; Title IV, 47
USC 5. https://www.ada.gov/pubs/adastatute08.htm 1990, as amended 2008.
Accessed January 6, 2018
[3] U.S. Department of Justice, U.S. Department of Education. Frequently Asked
Questions on Eective Communication for Students with Hearing, Vision, or
Speech Disabilities in Public Elementary and Secondary Schools. http://www2.
ed.gov/about/oces/list/ocr/docs/dcl-faqs-eective-communication-201411.
pdf. Published Nov 12, 2014. Accessed January 6, 2018
[4] Educational Audiology Association. 16 Reasons Why Your Schools Need an
Educational Audiologist. https://www.edaud.org/position-stat/7-position-05-10.
pdf. Published 2010. Accessed January 6, 2018
[5] Educational Audiology Association. School-based Audiology Advocacy Series:
Hearing Screening. http://www.edaud.org/advocacy/3-advocacy-09-09.pdf.
2009. Accessed January 7, 2018
[6] American Speech-Language-Hearing Association. Guidelines for Audiologic
Screening. Rockville, MD: ASHA; 1997 [Superseded for childhood hearing screen-
ing by Childhood Hearing Screening, https://www.asha.org/Practice-Portal/
Professional-Issues/Childhood-Hearing-Screening/. Accessed January 7, 2018]
[7] American Academy of Audiology. Clinical Practice Guidelines: Childhood
Hearing Screening. https://audiology-web.s3.amazonaws.com/migrated/
ChildhoodScreeningGuidelines.pdf_5399751c9ec216.42663963.pdf. Published
2011. Retrieved January 7, 2018
Amer
[8]
ican Academy of Pediatrics Committee on Practice and Ambulatory Medicine and Bright Futures Steering Committee. Recommendations for preventive
pedi
atric health care. Pediatrics 2007;120(6):1376
[9] American Academy of Pediatrics, Joint Committee on Infant Hearing. Year 2007
position statement: Principles and guidelines for early hearing detection and
intervention programs. Pediatrics 2007;120(4):898–921
[10] World Health Organization. Childhood Hearing Loss: Act Now, Here’s How.
http://www.who.int/pbd/deafness/world-hearing-day/WHD2016_Brochure_
EN_2.pdf. Published 2016. Accessed January 7, 2018
[11] Niskar AS, Kieszak SM, Holmes AE, Esteban E, Rubin C, Brody DJ. Estimated prev-
alence of noise-induced hearing threshold shifts among children 6 to 19 years
of age: the Third National Health and Nutrition Examination Survey, 1988-1994,
United States. Pediatrics 2001;108(1):40–43
[12] Shargorodsky J, Curhan SG, Curhan GC, Eavey R. Change in prevalence of hearing
loss in US adolescents. JAMA 2010;304(7):772–778
[13] Meinke DK, Dice N. Comparison of audiometric screening criteria for the
identification of noise-induced hearing loss in adolescents. Am J Audiol
2007;16(2):S190–S202
[14] Meinke DK. School-based hearing screening won’t prevent noise-induced hear-
ing loss. Arch Pediatr Adolesc Med 2011;165(12):1135–1136
[15] Sekhar DL, Rhoades JA, Longenecker AL, et al. Improving detection of adolescent
hearing loss. Arch Pediatr Adolesc Med 2011;165(12):1094–1100
[16] DeConde Johnson C. Making a case for classroom listening assessment. Semin
Hear 2010;31(3):177–187
[17] Johnson CD. Classroom listening assessment: strategies for speech-language
pathologists. Semin Speech Lang 2012;33(4):322–339
[18] Johnson CD, Darr M, Elliott S. Placement And Readiness Checklists (PARC). www
.adevantage.com/resources. Published 2011, 2014. Accessed January 4, 2018
[19] American National Standards Institute. Acoustical Performance Criteria, Design
Requirements, and Guidelines for Schools. New York: Acoustical Society of America; 2010 http://acousticalsociety.org/about_acoustics/acoustics_of_classrooms.
Accessed January 7, 2018
[20] American Academy of Audiology. Clinical Practice Guidelines: Remote Micro-
phone Hearing Assistance Technologies for Children and Youth from Birth to
21 Years, Supplement B: Classroom Audio Distribution Systems—Selection and
Verification. http://audiology-web.s3.amazonaws.com/migrated/20110926_
HAT_GuidelinesSupp_B.pdf_53996ef98259f2.45364934.pdf. Published 2011.
Accessed January 7, 2018
[21] DeConde Johnson C. Functional Listening Evaluation. www.adevantage.com/
resources. Published 2013. Retrieved January 8, 2018
[22] Educational Audiology Association. Supporting Students Who Are Deaf and Hard
of Hearing: Recommended Roles of Educational Audiologists and Teachers of the
Deaf and Hard of Hearing. http://www.edaud.org/position-stat/11-position-08-15
.pdf. Published August 2015. Accessed January 8, 2018
[23] Colorado Department of Education. 2015–16 Rubric for Evaluating Colorado’s
Specialized Service Professionals: Audiologists. http://www.cde.state.co.us/
educatoreectiveness/sspaudiologistrubric. Published 2013. Retrieved January
8, 2018
342
32 Screening, Assessment, and Management of Auditory Disorders in School-Aged Children
Madell et al., Pediatric Audiology: Diagnosis, Technology, and Management, 3rd Ed. (ISBN 978-1-62623-401-7),
copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
32.6 Appendix
Supporting Students who are Deaf and Hard of Hearing: Shared and
Suggested Roles of Educational Audiologists, Teachers of the Deaf
and Hard of Hearing, and Speech-Language Pathologists
(Approved by the Board of Directors of the Educaonal Audiology Associaon February 2018)
Educaonal audiologists, teachers of the deaf and hard of hearing, and speech-language pathologists are crical partners on the
school educaon team. Together, they address the needs of students who are deaf and hard of hearing and promote language and
communicaon access that is essenal for parcipaon and learning in today’s educaonal environments. The Individuals with
Disabilies Educaon Act (IDEA), Secon 504 of the Rehabilitaon Act, and the Americans with Disabilies Act (ADA) all contain
regulaons pernent to the services and accommodaons contained in this guidance document.
Language and Communicaon Regulaons (Title II and IDEA)
Title II of the ADA includes the following requirements for schools:
● Communicaon for students who are deaf and hard of hearing must be “as eecve as communicaon for others” [ADA Title II 28
C.F.R. §35.160 (a)(1)].
● Provision of appropriate aids and services “aording an equal opportunity to obtain the same result, to gain the same benet, or to
reach the same level of achievement as that provided to others” [ADA Title II 28 C.F.R. §35.130 (b)(1)(iii)].
● Students who are deaf and hard of hearing should be able to parcipate in and enjoy the benets of the district’s services, programs,
and acvies” (DOJ-DOE p14)1.
● These requirements apply to all school-related communicaons, and when a public school is deciding what types of auxiliary aids
and services are necessary to ensure eecve communicaon, it must give “primary consideraon” to the parcular auxiliary aid or
service requested by the person with the disability. (DOJ-DOE p27).
IDEA (2004) “Special Factors” regulaons specify that schools must provide the following supports for students who are deaf or hard
of hearing [34 C.F.R. §300.324(a)(2)(iv)]:
● Opportunies for direct communicaon with peers in the student’s language and communicaon mode.
● Opportunies for direct communicaon with professional personnel in the student’s language and communicaon mode.
● Opportunies for direct instrucon in student’s language and communicaon mode.
IDEA (2004) also requires:
● Roune checking of hearing aids and external components of surgically implanted medical devices to ensure they are funconing
properly [34 C.F.R. §300.113(a)(b)(1)]
● Audiology Services [34 C.F.R.§300.34(c)(1)]
● SLP Services [34 C.F.R.§300.34(c)(1)]
● Assisve Technology Devices and Services [34 C.F.R.§300.34(c)(15)]
● Highly Qualied Special Educaon Teachers [34 C.F.R.§300.18]
To assist schools in meeng the language and communicaon requirements above, the following checklist describes supports to be
considered for each student who is deaf or hard of hearing and those with other auditory learning needs. This checklist was developed
and eld-tested with input from all three professional groups via focus group meengs and online surveys. Categories are described
as “student assurances” with acvies and expected outcomes that should be addressed by the student’s team of educaonal
professionals, including educaonal audiologists (Ed. Aud), speech-language pathologists (SLP), and teachers of the deaf and hard of
hearing (TODHH). Because student needs change over me, this checklist should be completed at least annually.
1.
U.S. Department of Justice & U.S. Department of Education (2014, Nov 12). Frequently Asked Questions on Effective Communication for Students with Hearing, Vi-
sion, or Speech Disabilities in Public Elementary and Secondary Schools. http://www2.ed.gov/about/ofces/list/ocr/docs/dcl-faqs-effective-communication-201411.pdf
343
IV Educational and Clinical Management of Hearing Loss in Children
Madell et al., Pediatric Audiology: Diagnosis, Technology, and Management, 3rd Ed. (ISBN 978-1-62623-401-7),
copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
When the student’s team is designang primary responsibility for each acvity listed, the professional scopes of pracce and state
licensure/cercaon requirements, as well as training and experience, should guide consideraons for specifying responsible
personnel. Areas with direct scope of pracce implicaons are checked.
Student Assurances: Audiological and Equipment Needs
Audiological evaluaons that include recommendaons to enhance communicaon
1.
access and learning.
2. Diagnosis of auditory processing disorders (APD) with recommendaons to manage
APD issues provided to school personnel for the classroom and to parents for out of
school consideraon.
3. Management of auditory access in all educaonal environments
4. Assessment of classroom acouscs with recommendaons made to improve
classroom listening environments where necessary.
5. Evaluaon and ng for personal hearing instruments, classroom, and other hearing
assisve technology.
6. Management of hearing assisve devices including maintenance and
troubleshoong.
7. Provision of training for school personnel and students, when appropriate, to
perform listening checks and basic troubleshoong to maintain proper funconing of
personal hearing instruments and hearing assistance technology.
8. Provision of hearing assisve technology services including educang students,
teachers of the deaf/hard of hearing, and other school personnel regarding
technology performance and expectaons.
9. Use of daily listening checks to monitor funconing of hearing technology used by
students.
10. Other:
Ed
Aud
TODHH SLP Other
Student Assurances: Communicaon - Speech, Language, Auditory, Visual Needs
11.
Evaluaon of current speech producon skills including arculaon, uency,
voice, and resonance, as appropriate for the student’s preferred language and
communicaon mode.
12. Evaluaon of current language skills in the student’s preferred language and
communicaon mode, including:
● Comprehension, expression, and language processing in oral wrien, graphic
and manual modalies
● Phonology, semancs, syntax, morphology and pragmacs/social aspects of
communicaon
● Pre-literacy and language-based literacy skills, including phonological awareness
● Descripon and interpretaon of specic language communicaon skills and
needs idened through appropriate formal and informal, standardized and
non-standardized assessments.
13. Evaluaon of communicaon-related visual and/or auditory skills and needs as
appropriate in the student’s preferred language and communicaon mode.
Ed
Aud
TODHH SLP Other
344
32 Screening, Assessment, and Management of Auditory Disorders in School-Aged Children
When the student’s team is designang primary responsibility for each acvity listed, the professional scopes of pracce and state
licensure/cercaon requirements, as well as training and experience, should guide consideraons for specifying responsible
personnel. Areas with direct scope of pracce implicaons are checked.
Student Assurances: Audiological and Equipment Needs
Ed
Aud
TODHH SLP Other
1.
Audiological evaluaons that include recommendaons to enhance communicaon
access and learning.
2. Diagnosis of auditory processing disorders (APD) with recommendaons to manage
APD issues provided to school personnel for the classroom and to parents for out of
school consideraon.
3. Management of auditory access in all educaonal environments
4. Assessment of classroom acouscs with recommendaons made to improve
classroom listening environments where necessary.
5. Evaluaon and ng for personal hearing instruments, classroom, and other hearing
assisve technology.
6. Management of hearing assisve devices including maintenance and
troubleshoong.
7. Provision of training for school personnel and students, when appropriate, to
perform listening checks and basic troubleshoong to maintain proper funconing of
personal hearing instruments and hearing assistance technology.
8. Provision of hearing assisve technology services including educang students,
teachers of the deaf/hard of hearing, and other school personnel regarding
technology performance and expectaons.
9. Use of daily listening checks to monitor funconing of hearing technology used by
students.
10. Other:
Student Assurances: Communicaon - Speech, Language, Auditory, Visual Needs
Ed
Aud
TODHH SLP Other
11.
Evaluaon of current speech producon skills including arculaon, uency,
voice, and resonance, as appropriate for the student’s preferred language and
communicaon mode.
12. Evaluaon of current language skills in the student’s preferred language and
communicaon mode, including:
● Comprehension, expression, and language processing in oral wrien, graphic
and manual modalies
● Phonology, semancs, syntax, morphology and pragmacs/social aspects of
communicaon
● Pre-literacy and language-based literacy skills, including phonological awareness
● Descripon and interpretaon of specic language communicaon skills and
needs idened through appropriate formal and informal, standardized and
non-standardized assessments.
13. Evaluaon of communicaon-related visual and/or auditory skills and needs as
appropriate in the student’s preferred language and communicaon mode.
Madell et al., Pediatric Audiology: Diagnosis, Technology, and Management, 3rd Ed. (ISBN 978-1-62623-401-7),
copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
Student Assurances: Communicaon - Speech, Language, Auditory, Visual Needs
Ed
Aud
TODHH SLP Other
●
●
●
●
●
●
Student Assurances: Academic Needs
•
•
•
Ed
Aud
TODHH SLP Other
345
IV Educational and Clinical Management of Hearing Loss in Children
●
●
●
●
●
●
Student Assurances: Academic Needs
Ed
Aud
TODHH SLP Other
•
•
•
Madell et al., Pediatric Audiology: Diagnosis, Technology, and Management, 3rd Ed. (ISBN 978-1-62623-401-7),
copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
Student Assurances: Academic Needs
22. Assessment of literacy skills conducted in the child’s preferred language and
communicaon mode.
23. Literacy development plans designed and implemented according to the individual
student’s needs.
24. Provision of interpreng, notetaking, caponing, transliteraon, and/or voice-to-text
services to opmize access to instrucon for those who require these supports.
25. Provision of opmal visual and auditory access for both assessment and instrucon.
26. Other:
Collaboraon Program Management Needs for Students who are Deaf/Hard of
Hearing: Idenfy Individual Responsible for Coordinaon
Educaonal plans developed, reviewed, and implemented in a mely manner by
27.
team members who have knowledge, skills, and resources related to the impact
of hearing loss/deafness on communicaon, access to classroom instrucon and
academic performance.
28. Communicaon that is consistent between school-based instruconal sta and other
specialized personnel (e.g., private SLPs, interpreters, audiologists).
29. IEP and 504 development and meeng parcipaon by one or more specialists in
hearing loss/deafness to address student communicaon, educaon, access needs
and to develop a transion plan for post-secondary educaon/training/employment.
30. Educaon of students and their families regarding hearing status, communicaon
approaches, associated accommodaons, technology opons, and self-advocacy.
31. Observaon of classroom and school environments that connuously evaluates
and monitors communicaon access, classroom acouscs, and how children are
funconing in these sengs.
32. Consultaon acvies that ensure school personnel understand the language,
communicaon, social, and educaonal eects of hearing loss/deafness, technology
opons and associated accommodaons.
33. Educaon of students and their families about resources in the community,
nancial resources (for personal hearing technology), educaonal resources and
opportunies to connect with other students who are deaf and hard of hearing and
their families.
34. Other:
Ed
Aud
Aud
TODHH SLP Other
Ed
TODHH SLP Other
346
33 Managing Infants and Children with Auditory Neuropathy Spectrum Disorder
Madell et al., Pediatric Audiology: Diagnosis, Technology, and Management, 3rd Ed. (ISBN 978-1-62623-401-7),
copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
33 Managing Infants and Children with Auditory Neuropathy
Spectrum Disorder
Kirsty Gardner-Berry, Sanna Y. L. Hou, and Teresa Y. C. Ching
Summary
With the implementation of newborn hearing screening, infants
with auditory neuropathy spectrum disorder (ANSD) are being
identified within the first months after birth. Current evidence
shows that the provision of early, prescriptive amplification and/
or cochlear implants (CIs) is associated with better speech-language outcomes in infants with hearing loss. However, clinicians
are unable to use auditory brainstem response (ABR) testing to
estimate the audiogram for infants with ANSD, so determining
whether amplification is required, and how much amplification
to apply, has been problematic.
Over recent years, new testing methods have become available
that can assist clinicians in the early management of infants with
ANSD. This chapter describes how the combination of cortical
auditory evoked potentials (CAEPs) and measures of functional
auditory behavior such as the Parents’ Evaluation of Aural/oral
performance of Children (PEACH) can provide valuable information to help guide the management of these infants shortly
after diagnosis. This includes their use in the decision of whether
to fit amplification; assessing the eectiveness of the chosen
amplification settings; discussions around the need for signed
communication; and deciding whether to refer for CI candidacy
evaluations. These tools, together with results from medical
imaging and multidisciplinary input, place clinicians in a better
position to advise families on the most appropriate way forward
for each individual child.
Keywords
auditory neuropathy spectrum disorder (ansd), cortical auditory evoked potentials (caep), cochlear implants, hearing aids,
functional auditory behavior, magnetic resonance imaging
(mri), speech and language development, speech discrimination,
multi-disciplinary team, newborn hearing screening
t hearing aids and the evaluation of the eectiveness of
amplication.
Medical imaging and electrical auditory brainstem reponse
•
testing through the cochlear implant can help explain variations in outcomes for children with auditory neuropathy
spectrum disorder who use cochlear implants.
The early intervention team ne eds to be multidisciplinary to t ake
•
into consideration both the hearing- and non-hearing-related
needs of each child, particularly for those with additional
disabilities.
Parental involvement and consultation is vital during the
•
assessment and management process, and particular care
needs to be taken to minimize parental stress relating to
conicting information and advice.
33.1 Overview of Auditory
Neuropathy Spectrum
Disorder
ANSD is now a widely used term to describe a specific hearing
disorder characterized by abnormal function of the auditory
pathway in the presence of cochlear outer hair cell function. The
audiologic characteristics are demonstrated by the presence of
evoked otoacoustic emissions (OAEs) or cochlear microphonic
(CM) with absent or abnormal morphology of ABR waveforms.1
Up until recently there has been conicting information about
appropriate management and expected outcomes for children
diagnosed with ANSD. Chapter 28 provides more detailed
population-based information about spoken language outcomes
in children with ANSD wearing hearing aids or CIs. This chapter
will describe the new techniques being used to assist in the early
fitting and verification of amplification in infants with ANSD,
together with information from medical imaging and electrical
ABR testing that help to explain why variations in outcomes may
be observed.
Key Points
Auditory neuropathy spectrum disorder is a heterogeneous
•
disorder and has a number of potential sites of lesion, depending on the underlying cause. Hence, the hearing conguration, speech perception, and functional and speech-language
outcomes are highly variable.
Specic assessment techniques need to be used to diagnose
•
auditory neuropathy spectrum disorder correctly using auditory brainstem response testing.
Clinicians need to be aware of undiagnosed and late-onset
•
auditory neuropathy spectrum disorder.
Cortical auditory evoked potentials and assessments of
•
functional auditory behavior can assist with the decision to
33.1.1 Denition and Terminology
The condition of ANSD has been referred to in the literature as
auditory neuropathy,1 auditory dys-synchrony,2 and Type I aerent neuron dysfunction.3 The reason behind the use of dierent
terminology relates to a number of possible sites of lesion, which
are not limited to the auditory (cochlear) nerve and/or neural
pathways,4 such as the inner hair cells and synapses.5 The term
“ANSD” has now been adopted to acknowledge the heterogeneous and multifaceted nature of this disorder.
6
347
IV Educational and Clinical Management of Hearing Loss in Children
Madell et al., Pediatric Audiology: Diagnosis, Technology, and Management, 3rd Ed. (ISBN 978-1-62623-401-7),
copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
33.1.2 Prevalence and Etiology
The prevalence of ANSD has been estimated to range from 7 to
10% of all children with permanent hearing loss.
neonatal intensive care (NICU) population, 25% of those diag-
nosed with a permanent hearing loss have been reported to have
ANSD.9 Medically related risk factors in the NICU population
include prematurity, low birth weight, mechanical ventilation,
hyperbilirubinemia, respiratory distress, meningitis, and ototoxic medication such as vancomycin.
Approximately 40% of cases of ANSD are believed to have a
genetic basis.11 Genetic mutations associated with congenital
ANSD include nonsyndromic genes, such as OTOF (coding for
otoferlin)12 and GJB2 (connexin 26),13 and syndromic conditions,
such as some cases of CHARGE14 and Waardenburg syndrome.15
Genetic mutations associated with late-onset ANSD include those
associated with Friedreich ataxia,
ropathy,17 Charcot-Marie-Tooth disease,18 riboavin transporter
deficiency,19 and Mohr-Tranebjærg syndrome.
ANSD can also develop during infancy as a result of dietary
thiamine deficiency,
present transiently.22 For these reasons, clinicians need to keep in
mind the possibility of ANSD when a child who has passed their
newborn hearing screening, or other hearing assessments during
infancy and early childhood, later presents with a hearing loss.
21
and temperature-sensitive ANSD can
10
16
Leber hereditary optic neu-
20
7,8
Within the
Fi g . 33.1 Chart shows the better-ear four-frequency average (4FA)
results for 3-year-old children with auditory neuropathy spectrum
disorder (ANSD) involved in the Longitudinal Outcomes of Children
with Hearing Impairment (LOCHI) study. Half of the children have pure
tone thresholds in the mild to moderate range, and half in the severe
to profound range.
pure tone thresholds in the mild to moderate range, and 50% in
the severe to profound range.
33.1.3 Site of Lesion
Variations in outcomes in patients with ANSD are likely to be
related to dierences in the underlying pathophysiology. For
example, the inner hair cell (IHC) is responsible for the synchronous release of neurotransmitter and triggering of the cochlear
nerve to fire. A history of hypoxia during the newborn period or
a mutation of the OTOF gene can cause the impairment of IHC
function. Accurate transfer of temporal information for speech
understanding is also dependent on good neural conduction.
Late-onset ANSD is frequently associated with neurologic
conditions that can aect the peripheral dendrites, spiral ganglion cells, axons, or myelin. These conditions include MohrTranebjærg syndrome,20 Friedreich ataxia,23 mutations of the
OPA1 gene (optic atrophy type 1),24 Brown-Vialetto–Van Laere
syndrome,19 and Charcot-Marie-Tooth.25 It is important to note
that some of these conditions have associated problems, such as
vision loss, balance disturbance, and other peripheral neuropathies. Therefore, referral for vision and neurologic assessment is
p
articularly important if late-onset ANSD has been identified.
33.1.4 Audiologic Characteristics
The degree of hearing loss can range anywhere from normal
to profound, and the audiometric configuration is variable.26
Fluctuations in hearing and perceptual abilities have also been
reported in some individuals.
of degree of hearing loss for a population of children with ANSD
identified through newborn hearing screening participating in
the Longitudinal Outcomes of Children with Hearing Impairment
(LOCHI) study.8 At the age of 3 years, approximately 50% have
27
Fig. 33.1 shows the breakdown
33.1.5 Speech Perception Characteristics
Like behavioral pure tone thresholds, speech perception ability
can be varied. Speech recognition scores have been reported
to be worse than the lowest score expected for the behavioral
audiogram in almost 50% of children with ANSD.28 The most
consistent abnormalities found in subjects with ANSD have been
in the processing of timing information, such as pitch discrimination at low frequencies, gap detection, temporal integration,
signal detection in noise, and sound localization using interaural
time dierences.29 The severity of the temporal processing deficits is correlated to speech perception performance, with better
temporal resolution relating to better performance for speech
recognition in quiet. In some cases speech recognition ability
may be similar to that in children with sensorineural hearing loss
(SNHL) in quiet listening conditions, but worse in the presence of
background noise.
30
33.1.6 Functional Auditory Behavior and
Language Outcomes
The majority of studies, to date, have focused on sound detection
and speech perception, with very limited information on functional ability.31 Parent questionnaires, such as PEACH, LittlEARS
auditory questionnaire, and the Infant-Toddler: Meaningful
Auditory Integration Scale (IT-MAIS), provide a valuable way of
assessing infants’ auditory behavior, monitoring progress over
time, and engaging parents in the observation process.
More recently, Ching et al reported that there were no signifi-
cant group dierence in the speech and language outcomes of a
348
33 Managing Infants and Children with Auditory Neuropathy Spectrum Disorder
Madell et al., Pediatric Audiology: Diagnosis, Technology, and Management, 3rd Ed. (ISBN 978-1-62623-401-7),
copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
population-based sample of 3-year-old children with ANSD wearing hearing aids compared to those with SNHL, after controlling
for the degree of hearing loss and other factors known to aect
speech and language outcomes.8 Systematic reviews on children
with ANSD following a CI generally show an improvement in
sound detection, speech discrimination, speech recognition, and
similar appearance as children with SNHL.
32,33
33.2 Diagnosing ANSD
33.2.1 Clinical Test Battery
Diagnosing ANSD is accomplished on the basis of a combination
of electrophysiologic assessments. The key features identified
include evidence of surviving outer hair cell function, shown by
the presence of OAEs or CM on ABR testing, together with absent
or abnormal A BR waveforms. The absence of OA Es, therefore, does
not preclude a diagnosis of ANSD, as OAEs have been reported to
be absent in around 50% of children with ANSD.27 Tympanometry
or high-frequency tympanometry (for infants ≤ 6 months) should
also be included to rule out any middle ear involvement, as the
OAEs and CMs can be diminished by a conductive component.
Auditory steady-state response (ASSR) results can be misleading
in cases of ANSD, with reports of both significant over- and
underestimation of hearing thresholds.
testing should not be used in isolation when assessing infants
and children with ANSD.
27
For this reason ASSR
Table 33.1 and Fig. 33.2 describe the key components to ABR
testing and the potential pitfalls if these steps are not taken. The
presence of a CM is determined by performing click-evoked ABR
testing using a high-intensity click stimulus (≥ 70 dB HL). The CM
follows the polarity of the stimulus and, therefore, reverses in
polarity when changing from a condensation to rarefaction click
stimulus (Fig. 33.3). Insert earphones rather than supra-aural
headphones must be used. Also, a control run with clamped insert
earphone tubing (blocked stimulus) should be conducted to eliminate the possibility of stimulus or transducer artifacts.34 ANSD can
be diagnosed if the ABR assessment conducted in this way shows
an absent or abnormal morphology of ABR waveforms following
the presence of CM with normal middle ear function.
Pearl
Performing ABR testing using separate polarity click stimuli via
insert earphones is important in correctly diagnosing ANSD.
Middle ear muscle reexes (MEMRs) are elevated or absent in
the majority of children with ANSD.
thresholds for infants ≤ 6 months using a high-frequency probe
tone is now available.36 However, the application of MEMR in
the clinical test battery of infants with ANSD remains to be
investigated.
35
Normative data on MEMR
Pitfall
ABR and ASSR testing do not provide accurate estimates of
auditory thresholds in infants with ANSD.
Table 33.1 Key ABR procedures required for the correct diagnosis of ANSD
Description and diagnostic risk ABR test procedure for correct interpretation
1. Stimulus artifact and
response interpretation
2. Alternating click and
missed ANSD
3. Single-polarity click and
incorrect ABR interpretation
Abbreviation: ABR, auditory brainstem response; ANSD, auditory neuropathy spectrum disorder; CM, cochlear microphonic.
The electrical stimulus artifact generated from the
earphone transducer changes in polarity with the stimulus
and can be mistaken for the CM.
When an alternating click stimulus is used, the CM is
canceled out and the diagnosis of ANSD can be missed
(Fig. 33.2).
If only one polarity click is used, the CM can be mistakenly
interpreted as an ABR, and ANSD can be missed.
33.2.2 Recovery
Another challenge clinicians face when managing infants with
ANSD is the possibility that the child may recover or improve
over time. Improvements in the ABR can be seen over the first
Use insert earphones rather than supra-aural
•
headphones to separate the stimulus artifact from
the CM. The stimulus artifact occurs immediately
following the onset of the stimulus, whereas the CM
(if present) occurs approximately 0.8 ms later when
insert earphones are used.
Maintain the transducer of the insert earphone away
•
from the electrode sites to reduce electrical artifact
interference.
Perform an additional recording with the insert tubing
•
clamped to prevent sound reaching the ear. The
stimulus artifact will remain, but the CM (if present)
will disappear.
Do not use an alternating click stimulus alone unless your
recording system is capable of separating rarefaction and
condensation clicks into separate buers for viewing and
interpretation.
Perform both rarefaction and condensation clicks in
separate traces. If the response to one polarity is a mirror
image of the response to the opposite polarity, it is a CM
rather than an ABR waveform.
349
IV Educational and Clinical Management of Hearing Loss in Children
Madell et al., Pediatric Audiology: Diagnosis, Technology, and Management, 3rd Ed. (ISBN 978-1-62623-401-7),
copyright © 2019 Thieme Medical Publishers. All rights reserved. Usage subject to terms and conditions of license.
Fig. 33.2 Auditory brainstem response (ABR) waveforms for children.
(a) Normal hearing: The top traces show the condensation, rarefaction,
and alternating ABR waveforms for a child with normal hearing. A
cochlear microphonic (CM) is seen prior to wave I when the condensation and rarefaction traces are superimposed, and ABR waveforms
re present. (b) Sensorineural hearing loss (SNHL): The middle traces
a
show ABRs to dierent polarity stimuli for a child with a moderate
SNHL. No CM is seen, but reproducible ABR waveforms are present. (c)
Auditory neuropathy spectrum disorder (ANSD): The bottom traces
show ABRs for a child with ANSD. A CM is seen when the responses to
condensation and rarefaction stimuli are superimposed; however, no
ABR waveforms are present.
few months after birth in premature infants where myelination
may not have reached an equivalent level to that of a full-term
baby.37 Improvements in behavioral thresholds have also been
reported in some infants with a history of hyperbilirubinemia.38
These highlight the importance of repeating ABR and behavioral
threshold assessments over time to monitor for any changes. It
is important to dierentiate between improvements in hearing
thresholds together with normalization of the ABR and an
improvement in hearing thresholds with persisting abnormality
of the ABR. An improvement in auditory thresholds does not
necessarily translate to an improvement in functional auditory
performance if the ABR is still abnormal.
33.2.3 Late Diagnosis
Given that the onset of ANSD can occur later in childhood, and
that newborns with ANSD screened using OAEs can pass their
screen, clinicians need to be alert to the possibility of undiagnosed ANSD in the paediatric population. Concerns are raised if
children demonstrate significantly poorer speech discrimination
than expected given their pure tone audiogram or if they exhibit
inconsistent auditory behavior that is not explained by intermittent middle ear pathology. If OAEs are detected in the presence
of a significant pure tone loss, or if MEMRs are absent for degrees
of loss where they are expected to be present, referral for ABR
testing should be initiated to check for ANSD.
Fig. 33.3 A recording of cochlear microphonic (CM) to a single-polar-
ity click stimulus (top, rarefaction trace a)
to a typical auditory brainstem response (ABR) waveform; however,
when the same response is superimposed with a condensation click
recording (middle traces), the responses are mirror images of one
another. Had only one-polarity recording been used, the waveforms
could have been misinterpreted as a present ABR rather than a CM.
. The response appears similar
Pitfall
Clinicians need to be aware of the possibility for children to
develop late-onset ANSD and for children with ANSD to pass
their newborn hearing screen using OAEs. An ABR should be
arranged if auditory performance is poorer than expected given
the degree of the hearing loss.
33.3 Postdiagnostic Management
It has been genera lly accepted that childr en diagnosed with A NSD
should be fitted with appropriate hearing aids when behavioral
thresholds show a significant hearing loss6 and CIs when poor
progress in speech and language development is found in spite of
appropriate amplification.7 When infants with ANSD are considered, it is more controversial to
thresholds can be obtained, for fear of overamplification causing
damage to surviving cochlear structures.
Unfortunately, infants are not typically able to perform visual
reinforcement audiometry (VRA) until approximately 6 months
developmental age,39 and the abnormal or absent brainstem activity to sound means that ABR and ASSR testing cannot be used to
stimate hearing thresholds in infants with ANSD in the same way
e
as they can for infants with SNHL. This delay in obtaining behav-
ioral thresholds means that an infant identified from newborn
hearing screening may not be fitted with amplification until the
child is 1 year old. The delay in treatment can be even longer for
infants born prematurely or with additional disabilities.
Current evidence from the LOCHI study indicates that positive
advantages for language development are associated with early
intervention for both children with SNHL and those with ANSD
(see Chapter 28). For this reason, CAEP assessment is being used
fit hearing aids before behavioral
350
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