CHAPTER 24
INTRODUCTION
Current policy in public health advocacy and primary healthcare delivery has
focused on detecting hearing loss in infants and young children within the first 3
months of life through universal newborn hearing screening and timely diagnostic
follow-up. This emphasis in hearing health care represents a standard of service
delivery that has evolved over the past 45 years of advocacy by the Joint
committee on Infant Hearing (JCIH), and subsequently endorsed by the Centers for
Disease Control and Prevention (CDC-P), the National Institutes of Health (NIH),
and numerous coalitions of professional organizations. It should be noted that all
professional groups involved in the early detection of hearing loss have endorsed
the terminology early hearing detection and intervention (EHDI) programs.
Experience has shown that, to successfully identify and serve infants and young
children (including their families) with hearing loss, professionals had to expand
their emphasis from screening to include comprehensive, coordinated, and
continuous audiologic care, hence the adoption of the terminology EHDI.
To be successful, all components of follow-up from newborn hearing screening
need to be included to meet and serve the childs and familys complex needs,
through coordinated care and collaboration with other professionals, as well as
frequently communicated care plans that include intervention goals and strategies
for hearing loss.
Early referral for suspected hearing loss has evolved into best practice and is
supported by undisputable evidence that the earlier confirmation of hearing loss
occurs, the earlier intervention can begin, thereby increasing the likelihood of
optimizing a childs full potential in all developmental areas. The purpose of this
chapter is to focus on the audiologic assessment and early diagnosis of infants and
young children with hearing loss. Additionally, this chapter is developed with an
emphasis on the following premise: Audiologic procedures must be age
appropriate, outcome based, and cost-effective, and all procedures must have
demonstrated validity and reliability. In addition, audiologic care must be
comprehensive, collaborative, coordinated, culturally sensitive, and continuous.
Evidence Supports Early Detection of Hearing Loss
Undetected hearing loss in infants and young children compromises optimal
language development and personal achievement. Without appropriate
opportunities to learn language, children fall behind their hearing peers in
language, cognition, social-emotional development, and academic achievement.
However, research demonstrates that when hearing loss is identified early (prior to
6 months of age) and followed immediately (within 2 months) with appropriate
intervention services, outcomes in language development, communication
systems and environmental factors which include cultural or linguistic barriers (e.g.,
lack of interpretative services), and fragmentation of healthcare systems; and (2)
discrimination, including biases, stereotyping, and uncertainty. The report suggests
that these disparities might originate from the provider, that is, prejudice against
minorities, clinical uncertainty when interacting with minorities, and stereotypes
held about minorities. Linguistic barriers can minimize effective communication and
compromise rapport with a family, both leading to poor compliance with audiologic
recommendations. Clearly, linguistic barriers degrade the familys expectations of
quality service delivery. Poor communication may also discourage familiarity with
accessing resources and keeping future appointments. A useful skill that
audiologists must master is the effective use of interpreters when dealing with
families who speak other languages. Some techniques to consider for ensuring
successful exchange of information include using short concise sentences and
pausing frequently between them to allow the interpreter to organize and effectively
translate. Additionally, it is necessary to be mindful of nonverbal gestures that may
be interpreted differently by different cultures and certain words that may not have
the same meaning when translated. Appropriate eye contact and appropriate
physical space between individuals are also important considerations in achieving
effective communication. As healthcare professionals, audiologists need to be
respectful of and responsive to the needs of a diverse patient population by
increasing their knowledge and skill in cultural competence. Perceptions regarding
hearing loss differ across cultures. Some cultures may not attribute much
significance or urgency to hearing loss and families may only pursue services
relative to their specific attitudes and beliefs. Additionally, people from different
cultures may not view hearing loss as a disability and may choose to embrace only
those recommendations/ interventions that are consistent with their cultural values,
religious beliefs, or societal norms.
Continued Surveillance
Concern for hearing loss must not stop at birth. It must be recognized that limiting
hearing screening to the neonatal period will result in a significant number of
children with hearing loss excluded from the benefits of early detection. Some
newborns and infants may pass initial hearing screening but require periodic
monitoring of hearing to detect delayed-onset hearing loss. The JCIH (2007)
identified 11 risk indicators associated with either congenital or delayedonset
hearing
loss,
including
family
history
and
maternal
illness
(www.jcih.org/ExecSummFINAL.pdf). Therefore, heightened surveillance of all
infants with risk indicators is recommended. Unlike the newborn and school-age
populations when nearly all of the children can be evaluated in hospitals or
schools, preschoolers are generally not available in large, organized groups that
lend themselves to universal detection of hearing loss. For this reason, an
interdisciplinary, collaborative effort is particularly important for this age group.
Physicians and other professionals who make up the childs medical home and
other professionals who specialize in child development should be included in the
planning and implementation of hearing screening programs to increase the
likelihood of prompt referral of children suspected of hearing loss.
PEDIATRIC AUDIOLOGIC
PRACTICES
Audiologic assessment of infants and young children includes a thorough patient
history, otoscopic inspection, and both physiological and behavioral measures. As
stated earlier, the need for a battery of tests in pediatric assessment is essential to
optimally plan for and meet the diverse needs of the pediatric population. During
the diagnostic process, the integrity of the auditory system is evaluated for each
ear, and the status of hearing sensitivity across the frequencies important for
speech understanding is described, as well as the type and configuration of
hearing loss. In turn, these data provide essential information for medical
management when indicated and the data required to begin the amplification
process. Finally, these data are further used as a baseline for continued audiologic
monitoring.
Patient History
The case history is a component of the audiologic assessment that guides the
audiologist in constructing an initial developmental profile based on the childs
physical, developmental, and behavioral status. The outcome of the patient history
is particularly important because it will often guide the strategy for the audiologic
assessment and for making subsequent recommendations and referrals. It can
also serve as the first cross-check on the audiologic test outcome. The patient
history process begins as early as calling the childs name in the waiting room.
Time spent observing the child from the waiting room to the assessment area can
provide valuable information about the childs physical and developmental status.
Some background health-related and developmental information (American
Speech-Language-Hearing Association, 2004) can be obtained prior to the initial
evaluation (by telephone and/or mail). The case history questionnaires are used to
gather information regarding the family history, birth history, developmental history
(including hearing and speech/language development), and medical history. This
information guides the clinician to generate questions as each childs status may
require a meaningful history intake process. See Food for Thought about the
kinds of questions an audiologist should consider while taking a case history. It is
recommended that time for obtaining a patient history be viewed as a valuable
opportunity to observe and interact with the child and family, build rapport, and
instill confidence and comfort in the child and family, while acquiring patientspecific history information. The single most important thing that may determine the
comfort level of the child is the communication between the family
(parent/caregiver) and the audiologist.
Otoscopic Inspection
Otoscopy is intended as a general inspection of the external ear and tympanic
membrane for obvious signs of disease, malformations, or blockage from atresia,
stenosis, foreign bodies, cerumen, or other debris. To promote health and prevent
disease it is appropriate to change specula before examining each ear to avoid
reinforcers on both the right and left of the child increases the complexity and
novelty of the VRA procedure. From an instrumentation standpoint, it is a simple
and relatively inexpensive matter to use several visual reinforcers on each side, in
a stacked configuration.
The application of multiple visual reinforcers serves to postpone habituation
(Thompson et al., 1992), increasing the number of VRA responses that can be
obtained in a given clinical visit.
The second examiner, seated in front of the infant, maintains the infants head in a
midline position by quietly encouraging the child to observe passively or to
casually, but quietly, play with colorful, nonnoisy toys (Figure 24.1). An appealing
toy is manipulated by the examiner in front of the infant as a distracter. The
examiners role is to maintain the infants attention at midline and return the infant
to this position once a response is made and reinforcement is completed.
The audiologist must be creative in keeping the child alert and in a listening
posture without the child becoming so focused on the activity. The toys used for
this purpose should be appealing but not so attractive as to overly occupy the
infants attention. The closer the audiologist is to the child, the more easily the child
is engaged in the activity. If the infant under test shows too much interest in the
colorful, nonnoisy toys, then the potential exists for reduced responding during
testing or for elevated response levels because of decreased attention.
Conversely, if the examiner and toys are not sufficiently interesting, the likelihood of
false responses (random head-turning toward the visual reinforcer) will be high.
Often, a touch of the hand on a childs shoe or leg will quietly redirect the child to a
midline position. Actually sitting on the floor in front of the child allows for a totally
unobstructed view of the child for the control room audiologist and being located
slightly below the level of the child is a nonthreatening position. Clearly, the
challenge for the second examiner is to balance between distracting the child
without overly entertaining the child and consuming too much of the childs
attention.
A single examiner approach from the control room can be accomplished by using a
distracting centering toy positioned at midline in the test room for maintaining a
childs midline gaze. Although the use of a centering toy is a frequent alternative
to the more classical approach of using two examiners, this approach distances the
examiner (in the control room) from the infant (in the exam room).
Although a single examiner may be more cost-effective and practical in busy
clinical settings, eliminating the second examiner reduces the ability to maintain the
infant at midline with multiple distraction toys, that is, the centering toy is always
the same toy, is somewhat noisy, and may be overly distracting for some infants.
The fact that the centering toy is usually not housed in a dark plexiglass box
raises the concern that the constant viewing of the colorful toy may eventually
compromise the infants interest in the visual reinforcement.
Greenberg et al. (1978) found that individuals with Down syndrome did not achieve
a high rate until 10 to 12 months BSID mental age equivalent. These investigators
further pointed out that when one is predicting potential success with the VRA
procedure for children with Down syndrome, the BSID mental age equivalent score
provides the most distinct distribution between successful and unsuccessful tests,
with the dividing point being a BSID mental age equivalent of at least 10 months.
Similarly, Wilson et al. (1983) reported that 80% of the children in their study with
Down syndrome were testable by 12 months of age using VRA.
Although these data provide guidance, attempting behavioral audiologic evaluation
on children presenting with any neurologic or development condition, including
Down syndrome, under the age of 10 months is encouraged. In a clinical setting
with a diverse and complex patient population, it is helpful to obtain any behavioral
information that leads to more informed decision making and recommendations.
In summary, when VRA is implemented audiologists must consider (1) corrected
age adjusted for prematurity
rather than chronologic age or (2) mental age/developmental age when disparities
exist between corrected age and the childs developmental status. Of the two
predictors of VRA performance (corrected age and mental age), corrected age may
be the more practical one to use in most cases because it can be obtained from
parental report, case history information, and/or hospital records and does not
require testing to determine mental age.
CONDITIONING IN VRA
In clinical assessment, the first phase of VRA is the conditioning process. An
examiner must be skilled in response training and sensitive to the various stages of
response acquisition. Evidence suggests that different signals (e.g.,tones, filtered
noise, speech) are equally effective during the conditioning phase (Primus and
Thompson, 1985).
Response shaping is critical to the success of the operant procedure. Two different,
but commonly used approaches can be implemented to condition the head-turn
response: (1) Pairing a suprathreshold auditory stimulus with the visual reinforcer
or (2) presenting a suprathreshold auditory stimulus and observing a spontaneous
response from the infant, followed by activation of the visual reinforcer. Successful
completion of the training phase is the achievement of a pre-established criterion of
consecutive headturn responses (usually two, but no more than three consecutive
responses following the response shaping trials).
If the criterion is not reached, retraining is necessary until the criterion is met. The
number of training trials needed before phase 2 trials begin varies, but the training
phase is usually brief.
For the child with special needs, conditioning may take longer to establish the
conditioned behavior. Successful completion of the conditioning phase occurs
Following successful response shaping, the test phase of VRA begins (Figure
24.2). Signal intensity is attenuated 10 dB after every yes response or increased
5 dB after every no response (descending and ascending technique). Testing
progresses until the stopping criterion (four reversals in the threshold search) has
been achieved. Thresholds are defined as the mean of the four reversal points.
Note: Thresholds are often called minimum response levels (MRLs) in VRA
because the term MRLs serves as a reminder that improvement in response
behavior at lower hearing levels should be anticipated with maturation (Matkin,
1977, p. 130).
PROCEDURAL GUIDELINES
The recommended trial duration (incorporating the signal and response interval) is
approximately 4 seconds (Primus, 1992). That is, although the signal duration is
approximately 4 seconds, this 4-second duration also defines the interval of time
during which a response should be judged to be present or not. Head-turn
responses outside of the 4-second interval are typically not interpreted as valid
responses, and therefore not reinforced. For some children with developmental
and/or physical challenges, it may be necessary to increase the trial duration
(beyond 4 seconds) during which a head-turn response is acceptable. However, by
increasing trial duration beyond 4 seconds, audiologists also risk an increase in
judging false responses as valid responses.
Conditioned head-turns are visually reinforced only for correct responses that
occur during signal trials. Between the ages of 6 and 17 months, Lowery et al.
(2009) demonstrated that infants are not more or less attentive to a dynamic video
than to the three-dimensional animated toy as effective visual reinforcement.
Therefore, when using colorful, threedimensional toys as visual reinforcers it is
sometimes judicious to use the light initially (showing the toy), eventually adding
animation. By starting with the light only, the audiologist can gauge any potential
for a fearful response to the three-dimensional toy. The novelty and strength of
visual reinforcement may be preserved longer by introducing more complex
reinforcement (light plus animation) as the testing progresses. The novelty of VRA
is clearly strengthened with somewhat older children by using moving images
generated by a digital video disc (DVD) player/monitor. Schmida et al. (2003) used
digital video with 19- to 24-month-old children. Their results demonstrated a
greater number of head-turn responses before habituation when viewing video
reinforcement than when viewing conventional animated toy reinforcement. These
results support the hypothesis that the complex and dynamic nature of the video
reinforcement would be more effective in achieving a greater number of responses
than the conventional toy reinforcer prior to habituation in the 2-year-old age group.
In general, a 100% reinforcement schedule (reinforcement for every correct
response) results in more rapid conditioning, yet more rapid habituation.
Conversely, an intermittent reinforcement schedule produces slower conditioning
but also a slower rate of habituation. Consequently, most clinicians recommend a
protocol that begins with a 100% reinforcement schedule and then gradually shifts
to an intermittent reinforcement schedule.
Primus and Thompson (1985) compared a 100% reinforcement schedule to an
intermittent reinforcement schedule with 2-year-old children. The two reinforcement
schedules resulted in no differences in the infants rate of habituation or the
number of infant responses to stimulus trials. These findings provide an excellent
guideline for delivering reinforcement. Since Primus and Thompsons data suggest
that withholding reinforcement should not affect the amount of response behavior,
reinforcement should only be provided when the audiologist is certain about the
validity of an infants head-turn response. The risk of reinforcing a random headturn is that it may lead to confusion for a child during the test session and increase
the childs rate of false responding. Conversely, failure to reinforce a valid headturn will not degrade subsequent responding.
In this scenario, withholding reinforcement for a valid but ambiguous response is
simply viewed as intermittent reinforcement. Reinforcement duration is also a
factor influencing response outcome from children around the age of 2 years
(Culpepper and Thompson, 1994). Decreasing the duration of a childs exposure to
the visual reinforcer (e.g., 4 to 0.5 seconds) results in an increase in response
behavior and a decrease in habituation. Audiologists may increase the amount of
audiometric information obtained from children by decreasing their exposure to the
visual reinforcer. For the child with special needs who may have a slower
response, the visual reinforcer should be activated for a sufficient length of time for
the child to very briefly observe it, but prolonged visual reinforcement should be
avoided.
CONTROL TRIALS
Ensuring valid behavioral assessment outcomes depends on separating true
responses from false responses during threshold acquisition. Infants are likely to
produce a number of false responses during clinical assessment. To quantify false
responding behavior in VRA, the use of control trials are necessary.
False responses are monitored by inserting control trials in the staircase algorithm
(see Figure 24.2). A head-turn observed during a control trial is evidence of false
responding. Thus, it is possible to systematically estimate errors or chance
responding (false responses during signal trials) by calculating the number of false
responses during control trials.
Moore (1995) recommended that one out of four presentations should be a control
trial and that test results are questionable if the false-positive rate exceeds 25%.
Eilers et al. (1991) suggest that a false alarm rate of 30% to 40% is acceptable and
adopting such a rate as acceptable does not compromise the accuracy of
thresholds for clinical assessment. Clearly, high false alarm rates (>50%) require
the audiologist to further consider that test results may be inaccurate.
Excessive false responses suggest that the infant is not under stimulus control. In
this situation, audiologists should focus on two factors to rectify clinical outcomes:
(1) Reinstituting phase 1 shaping and conditioning and (2) increasing the
entertainment level of the distraction activity to engage the childs interest and
attention at a midline position before starting a test trial. When in the test room, an
examiner must be able to choose from a variety of toys available and judge when a
toy change in either direction (enhanced distraction and more entertaining, or
reduced distraction and less entertaining) is necessary to maintain the childs
midline focus and optimum response readiness.
Occasionally, overactive parents can bias their children to respond, thereby
resulting in excessive false responses. Therefore, parents may need to wear
headphones through which music or noise is delivered.
Threshold determination in audiometry is based on the lowest intensity level where
responses are obtained approximately 50% of the time. In VRA, as the staircase
algorithm is executed, how many reversals should be required before identifying
the hearing threshold? Too few reversals may sacrifice response accuracy.
However, too many will increase test time, in turn reducing the number of stimulus
presentations that could be spent obtaining thresholds to other stimuli. Assessing a
desired stimulus may be stopped once the infant has exhibited between three and
four response reversals (Eilers et al., 1991). Eilers and her colleagues found that
using six rather than three response reversals before discontinuing the threshold
search had minimal effect on threshold. Yet, tests with a three-reversal stopping
rule were significantly shorter than those with six reversals. As stopping rules are
increased from three to six, there is about a 50% increase in the number of test
trials, with no improvement in response accuracy. These results suggest that, by
using relatively few reversals to estimate threshold, a staircase algorithm may be
shortened to increase efficiency without sacrificing accuracy. Thus, there is no
need to continue testing beyond three or four reversals since the results obtained
are not substantially better.
Thresholds obtained with the VRA procedure for infants 6 to 12 months of age
have been shown to be within
10 to 15 dB of those obtained from older children and adults (Nozza and Wilson,
1984). In addition, VRA thresholds are similar across the age span (6 to 24
months) and show good reliability when compared to thresholds obtained from the
same child at older ages.
Throughout testing, audiologists must consider that the next response to a test
stimulus may be the childs last response. However, audiologists can influence
attention and motivation by being flexible with their clinical decision making. Often,
if a child begins to habituate to a specific stimulus, response behavior can be
increased by using a different stimulus, a different transducer, or moving to the
other ear. This approach to clinical assessment can optimize air conduction/bone
conduction, another puretone versus speech, and switching ears. Thompson et al.
(1992) also demonstrated that when 1-year-old children habituate to testing and
are given a 10-minute break, the children return and are likely to provide a
significant amount of additional information. Even a few additional responses in the
same session may provide just enough additional information to be confident that
hearing loss is not a factor concerning speech/language development and
communicative functioning, thereby eliminating the need for a costly follow-up clinic
visit.
Conditioned Play Audiometry
Operant conditioning of behavioral responses to sound continues to be an effective
approach for older children. What changes as children age, however, are the
operant behaviors and the reinforcement that is used. Similar to operant
conditioning in VRA, CPA uses positive reinforcement to increase response
behavior. In CPA, children learn to engage in an activity (e.g., putting rings on a
spindle, putting pegs in a board, dropping or stacking blocks, putting together
simple puzzles) each time they hear a test signal. These activities are usually fun
and appealing to children, are within their motor capability, and represent a specific
behavior that is used to denote a deliberate response to a stimulus.
When teaching children to perform CPA, it is usually not difficult to select a
response behavior that children are capable of performing, as long as the
audiologist is intuitive in matching the childs motor skill with an appropriate play
activity. CPA follows the traditional operant conditioning paradigm of stimulus
response reinforcement, in which the play activity/motor activity is the response
behavior, and social praise or other positive reward is the reinforcement. Three
decisions are needed in play audiometry: First, the audiologist must select a
response behavior that the child is capable of performing. Second, the audiologist
must consider how to teach the child to wait, listen, and respond only when the
auditory signal is presented. The third decision is what social reinforcement (the
most common reinforcement with young children) the audiologist should give that
is natural and genuine at the appropriate time and interval.
Separation of response behavior and reinforcement is essential in CPA. Although
the play activity is fun for the child, it is not the reinforcement. A separate
reinforcement is essential to minimize habituation and maximize repeated
response behavior. In addition to social praise, other forms of reinforcement have
been suggested. Tokens that can be traded for small toys at the end of the test
session, unsweetened cereal, and a changing computer display screen all have
been used successfully with play audiometry.
Children with multiple health concerns present unique challenges during
audiometric evaluation using CPA. Challenges to consider include obtaining
verifiable responses, choosing reinforcements that will interest the child, and
response time. For children who have visual and hearing impairments, Holte et al.
(2006) suggest using tactile cues (bone oscillator or simple touch) to train a child to
the CPA task. For youngsters with limited gross motor/fine motor skills, a variety of
responses (e.g., finger swing, hand motion, arm motion, eye motion, visual gaze)
measuring spoken word recognition with greater accuracy in children with hearing
loss.
The Lexical Neighborhood tests (LNTs) (Kirk et al. 1995) assess word recognition
and lexical discrimination in children with hearing loss. A primary goal in the
development of these perceptual tests was to select words that were likely to be
within the vocabulary of children with profound hearing losses. These tests
approach speech perception from the perspective that word recognition
performance is influenced by the lexical properties of the stimulus words.
Kirk et al. (1995) examined the effect of lexical characteristics on a group of
pediatric cochlear implant users spoken word recognition and compared their
performance on the LNT and Multisyllabic Lexical Neighborhood Test (MLNT) with
scores on the traditional, PB-K. Word recognition was significantly higher on the
lexically controlled lists than on the PB-K. In fact, only 30% of the words on the PBK were contained within the childhood language database from where the words
for the LNT and MLNT were derived. It may be that the restrictions imposed by
creating a PB word list result in the selection of test items that are unfamiliar to
children with hearing loss.
SUMMARY
The standard of care in the United States for EHDI is founded on hearing
screening by 1 month of age, audiologic diagnosis by 3 months of age, and
intervention by 6 months of age. The accuracy and precision of our audiologic test
battery is critical in achieving valid diagnostic outcomes in a timely manner.
Important and fundamental decisions in management and intervention depend on
the audiometric outcomes and diagnosis provided by audiologists.
Clearly, information must be accurate, precise, timely, and cost-effective to provide
optimal service and help families move forward with intervention. When achieved,
the goal of optimizing a childs communication behavior and global development is
positively influenced, and a familys empowerment is significantly enhanced.
FOOD FOR THOUGHT
1. What kind of information should be collected for a childs case history?
2. What is an age-appropriate, cost-effective, and efficient diagnostic protocol for a
12-month-old normal developing and cooperative child referred for suspected
hearing loss?
3. What is the impact of receptive vocabulary on the measurement of speech
perception with the pediatric population?