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Audiology Review: Preparing for the Praxis and Comprehensive Examinations
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designed for children and adolescents who have hearing loss. The HEAR QL examines how the patient experiences their hearing loss and identifies potential areas of the patient’s life that could use additional hearing support.
n
Surveys assessing functional outcomes
Functional validation measures provide insight into everyday listening experiences and skill
development. Input can be received by the patient as well as their communication partners. Some examples include International Outcome Inventory for Hearing Aids (IOI-HA), Client Oriented Scale of Improvement (COSI), and the Abbreviated Profile of Hearing Aid Benefit (APHAB). For the pediatric population, these measures can include feedback from parents and teachers.
Special Populations
Pediatric Minimal or Mild Hearing Loss
The recommendation of amplification as a means for auditory habilitation or rehabilitation is not always straightforward when one considers the degree of hearing loss. For patients with hearing loss severity ranging from moderate to severe, amplification is typically a standard recommendation. For those with a minimal or mild hearing loss, however, management plans vary. Prior to the implementa­tion of universal newborn hearing screenings, a minimal or mild hearing loss may have easily been missed as its effects are often more subtle than that of a more severe hearing loss. Currently, minimal and mild hearing losses are identified more often and at earlier ages.
n
Research shows children with a minimal degree of hearing loss are at risk for negative
outcomes in areas including speech-language and education (McKay et al., 2008).
n
Some individuals from this population may benefit from amplification.
n
Current research supports using unaided SII when considering whether to recommend
amplification for a minimal/mild hearing loss. Calculations of SII should always include real ear to coupler difference measures, when possible. If the child has an unaided SII <0.8 in the better ear, they should be considered for amplification (McCreery et al., 2020).
n
All pediatric patients with minimal or mild hearing loss should be considered candidates for a
RM-HAT system per the American Academy of Audiology Pediatric Amplification Protocol (2013).
Auditory Neuropathy Spectrum Disorder (ANSD)
HA candidacy in the ANSD population is another instance of complex decision-making.
n
For children with ANSD, hearing sensitivity cannot be accurately determined using objective
test techniques in the same way as children with SNHL. The ABR can predict hearing thresholds for SNHL, but the ABR is significantly abnormal or absent with ANSD and therefore cannot be used for threshold estimation.
n
There is no current consensus on sufficient evidence for a hearing aid fitting protocol for
children with ANSD. According to the AAA 2004 Pediatric Amplification Guidelines,
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children with ANSD should begin a HA trial once behavioral audiologic testing indicates hearing sensitivity is not adequate for conversational speech audibility. If significant concerns exist regarding access to sounds in patients with ANSD, close behavioral observations of the child’s responses to sounds can be used to guide hearing aid fitting prior to obtaining true behavioral thresholds (Walker et al., 2016).
n
Unfortunately, even a complete audiologic evaluation is not always a reliable indicator for
amplification performance due to fluctuations in hearing sensitivity associated with ANSD.
n
The audiologist must determine if the patient’s speech understanding improves with the
addition of amplification.
n
Regardless of hearing thresholds, patients with ANSD should always be considered for a
RM-HAT system due to the poorer performance in noise than those with other types of sensorineural loss.
Unilateral Hearing Loss (UHL)
The management of patients with UHL is also considered a special case as there are several treatment options and the success of patients can vary significantly. An individual with unilateral hearing loss wherein the poorer ear presents with hearing loss from mild to severe and is therefore a candidate for traditional amplification may pursue the following options:
n
Unilateral, conventional HA fitting
n
No amplification
In this instance, environmental modifications may be discussed. Close audiologic monitoring is recommended even when the patient does not pursue
audiologic intervention.
For those cases of UHL in which the poorer-hearing ear is not aidable due to the severity of hearing loss and/or poor word recognition, the following options are available:
n
CROS
n
Bone-anchored hearing device for single-sided deafness (SSD)
n
Unilateral cochlear implant (CI)
n
No amplification
Conductive Hearing Loss
For patients with conductive or mixed hearing loss who have an intact ear canal, air-conduction HAs are an option. The managing audiologist should consider the following:
n
Power level: when an air-bone gap is present, more gain than for sensorineural losses (to
overcome the conductive component) with the same air-conduction thresholds is required.
Tables 9–5 and 9–6 review the target values for average (65 dB SPL) speech signal in the
Verifit2 values for a SNHL with bone-conduction thresholds equivalent to air-conduction thresholds and (2) prescribed values for a conductive hearing loss with bone-conduction
® for a flat 60 dB HL hearing loss with average RECD. This compares (1) prescribed
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TABLE 9–5. Comparison of Prescriptive Targets for NAL-NL2 With Sensorineural Versus Conductive Hearing Loss
FREQUENCY (Hz)
250 500 750 1K 1.5K 2K 3K 4K 6K 8K
NAL-NL2 target outputs for average
56 66 66 66 69 73 75 72 66 62 LTASS with a flat, 60 dB HL sensorineural hearing loss
NAL-NL2 target outputs for average
83 84 80 77 81 85 86 84 74 71 LTASS with a flat, 60 dB HL conductive hearing loss
Difference (conductive-SNHL) 27 18 14 11 12 12 11 12 8 9
TABLE 9–6. Comparison of Prescriptive Targets for DSL v5 Pediatric for a Sensorineural Versus Conductive Hearing Loss
FREQUENCY (Hz)
250 500 750 1K 1.5K 2K 3K 4K 6K 8K
DSL v5 pediatric target outputs for
81 82 80 80 79 83 83 83 78 75 average LTASS with a flat, 60 dB HL sensorineural hearing loss
DSL v5 pediatric target outputs for
85 86 84 84 82 87 87 87 81 77 average LTASS with a flat, 60 dB HL conductive hearing loss
Difference (conductive-SNHL) 4 4 4 4 3 4 4 4 3 2
thresholds at 10 dB HL. As shown for both NAL-NL2, in Table 9–5, and for DSL v5 pediatric, in Table 9–6, when there is an air-bone gap, additional gain is prescribed across the frequency range.
n
If gain requirements cannot be met with an air-conduction HA, a bone-conduction hearing
device (BCHD) should be considered.
Introduction to Cochlear Implants
Some individuals do not receive benefit from traditional amplification, for example, those with more severe degrees of hearing loss and/or poor word recognition abilities. To provide better access to the auditory signal for this population, a cochlear implant (CI) may be required. All CIs comprise two general components, external and internal.
n
The CI uses an external component to take an acoustic signal, digitally filter it into
components, and prepare it for electric stimulation. The external component is called the
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sound processor, which contains microphones and a digital signal processor, and an external coil and magnet with a transmitter
The external coil and magnet may be separate from the processor when the processor is at
ear level, or it may be contained within one unit and is all at the connection point on the head in an off-the-ear configuration.
n
The information is then transmitted to the internal component, which provides the electrical
stimulation to the cochlea via an electrode contact using biphasic electrical pulses (pulses containing both a positive and negative voltage). The internal component comprises a receiver-stimulator, which contains a communicating coil, a digital signal processor, a stimulatory device, and an electrode array.
n
The CI is intended to communicate directly via the auditory nerve, specifically with the spiral
ganglion cell bodies being the target of stimulation.
Candidacy
CIs undergo regulatory approval by the Food and Drug Administration (FDA) and are considered Class III medical devices that are subject to the most stringent approval and review process due to the risk level of the patient. The individual manufacturers carry the burden of presenting the quality and safety of their devices after extensive scientific testing. Because of the regulatory process, new CI components are typically only released every few years as compared to HAs, which are Class I devices and undergo less stringent review and therefore have more frequent releases.
n
Labeled indications are the guidelines developed by the manufacturer and approved by the
FDA that specify the patient profile for who is eligible for the device. The labeled indications specify the degree of hearing loss and word recognition score that makes a patient eligible for implantation with that device according to the FDA.
n
This further supports the insurance company’s approval or rejection of coverage of the device.
FDA criteria differ by manufacturer and may change, so they should be reviewed at the time of evaluation.
CI recipients may not meet the labeled indications on FDA criteria yet still undergo implantation. This is considered an off-label use of the device. Clinicians are typically granted the latitude to determine who is a CI candidate when the benefits are determined to be greater than the risks and their potential outcomes are greater than the best nonsurgical option. Evaluation of candidacy should address multiple questions.
CASE EXAMPLE
A patient with ANSD presents with behavioral hearing thresholds from 50 to 60 dB HL across frequencies in each ear of a sensorineural nature; however, this patient has a word recognition score of 36% at 95 dB HL. With binaural HAs, the patient has a binaural word recognition score of 40% at 55 dB HL. In noise, the patient achieves 12% at a +10 dB SNR. Although this patient would not meet FDA-labeled degree of hearing loss requirement, they may be recom­mended for cochlear implantation in an off-label capacity due to the poor speech recognition abilities in both quiet and noise.
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Of note, insurance companies may also have their own criteria for cochlear implantation, which may be more or less strict than FDA-labeled indications. When a potential recipient is undergoing can­didacy evaluations, the clinician should take their insurance coverage into consideration and include that in information that is presented to the patient. If the patient is insured with Medicare, the CI team should follow the Centers for Medicare & Medicaid Services (CMS) indicated guidelines in addition to FDA indications. In 2022, the CMS guidelines were expanded and indicate a candidate is someone who meets the following criteria:
n
Bilateral moderate-to-profound SNHL
n
Limited benefit from HAs as indicated by less than or equal to 60% correct on aided, open-set
tests of sentence recognition
n
No medical contraindications to undergoing surgery
n
Appropriate motivation and cognition to undergo rehabilitation
Cochlear Implant Team
CI candidacy is determined by a team approach. In adult candidacy evaluations, the CI team at a minimum should include the audiologist, the otolaryngologist or otologist, and the patient.
n
Audiologist: responsible for determining audiologic components of candidacy and testing for
FDA indications, setting appropriate expectations, and assisting in manufacturer and device selection based on the patient’s motivation and lifestyle factors
n
Otolaryngologist/otologist: responsible for medical evaluation and medical determinants
of candidacy, which may include imaging studies and assessment of health regarding anesthesia
n
Patient: the patient is a key team member. It is important for the patient to understand
potential risks and benefits of undergoing cochlear implantation. They must also have significant motivation to commit to habilitation with the device.
In pediatric candidacy evaluations, the CI team should include the audiologist, otologist/neu­rotologist, patient and their family, and the speech-language pathologist. Other team members may include a neuropsychologist or social worker.
n
Audiologist and otologist: serve similar roles for pediatric candidacy as for adult candidacy.
Medical evaluation may be slightly different for the pediatric team as they may require
different imaging, medical prevention of meningitis, and other medical evaluations based on the child’s clinical presentation.
n
Patient/guardian: depending on age, the patient may be a member of the team; however,
the parent or guardian often assumes that key role. Guardian buy-in of the process and understanding of realistic expectations and workload for success with a CI are vital.
n
Speech-language pathologist (SLP): SLPs can be certified as a Listening and Spoken
Language Specialist (LSLS) and/or an Auditory Verbal Therapist (AVT). This is ideal for the CI team as the overwhelming majority (95%) of children born with hearing loss have parents with normal hearing. The SLP contributes important information regarding the child’s speech and language progress that is utilized for FDA indications. They will evaluate
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the child’s speech and language skills compared to normative data for children their age and determine delays. They should also be involved for auditory verbal therapy post implantation for ideal outcomes.
n
Social worker: can address disparities in access to services and transportation and improve
overall outcomes.
n
Psychologist/neuropsychologist: can evaluate the nonverbal IQ and help set realistic
expectations as well as help the parents in the acceptance of the hearing loss.
Adult Audiologic Candidacy Evaluations
Adult audiologic CI candidacy measures should also be used to assess CI recipients postoperatively. Cochlear implantation is historically underrecommended in the field of audiology (Buchman et al.,
2020). Expanding indications have made improved access to sound a possibility for many patients. Updated guidelines support the 60/60 rule, which states that patients should be referred for a CI candidacy evaluation if their unaided pure-tone average is poorer than 60 dB HL in the better-hearing ear and if their single-word score in at least one ear is poorer than 60% (Zwolan et al., 2020).
n
Unaided evaluations: standard audiologic assessment with air- and bone-conduction
assessment and immittance measures. An ideal assessment includes frequencies from 125 to 8000 Hz.
CI candidates should not have an air-bone gap and ideally are free from fluctuating middle
ear issues.
Speech recognition may be included in the unaided evaluation but is not required for
candidacy.
n
Subjective measures: a complete candidacy evaluation will include subjective questionnaires.
Some examples include:
Nijmegen Cochlear Implant Questionnaire (NCIQ): 60 questions, serves as a measure to
assess quality of life. Comprehensive look at patient from the perspective of CI candidacy; however, may be unfamiliar to insurance companies and is not as commonly used with limited normative data and therefore interpretation can be more challenging.
Cochlear Implant Function Index (CIFI): evaluates performance with hearing in the
candidate’s day-to-day life. Looks at things such as need for visual cues and hearing in a variety of listening situations. The questionnaire has been found to correlate well to speech perception measures and patient performance after cochlear implantation. It is not widely used and therefore not as well known.
Other questionnaires may be used, for example, the Hearing Handicap Questionnaire,
APHAB, COSI, and Speech, Spatial, and Quality of Hearing Scale (SSQ).
n
Aided evaluations
All candidacy evaluations should be completed in the best-aided condition. It is essential
that for candidacy evaluations, the recipient is in appropriately fitted HAs. All HAs should be evaluated using real ear (probe microphone) measures to determine if HAs match prescriptive settings for each individual’s hearing loss. Refer to the real ear measures section of this chapter for guidance on programming HAs to prescriptive target.
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Aided evaluations must include sentence recognition measures as addressed in FDA
guidelines. Individual word scores are not currently included in some manufacturer’s FDA-labeled indications; however, studies are trending in support of using word scores for candidacy. Word scores also serve as an important baseline from which to compare implant performance. Both words and sentences should be used for pre- and postoperative evaluation of CI candidates.
The Minimum Speech Test Battery (MSTB) is often utilized for both pre- and
postoperative assessment. The MSTB utilizes the four following tasks:
●
AZBio sentences in quiet (full 20-word list)
●
AZBio sentences in noise (full 20-word list)
Presented at +10 or –5 dB SNR
●
CNC words in quiet (full 50-word list)
●
BKB-SIN (16-sentence pair pre or 20-sentence pair post)
The clinician should use the same assessment measures for pre- and postimplant testing.
Postoperative testing should be completed for adults at 3 months postactivation and then at 6 and 12 months. Patients should then be evaluated every year thereafter.
Test conditions
●
60 dBA presentation level
●
Sound-treated room
●
Soundfield speaker at 0 degrees azimuth 1 meter from the recipient
●
Calibrated recorded materials
AUDIOLOGY NUGGET
HA recipients may not tolerate devices at their prescribed gain levels and there­fore their day-to-day programming of the devices may be based on comfort rather than appropriate audibility. During candidacy evaluations, the patient’s HAs may need to be reprogrammed to meet prescribed targets. If the potential recipient is not a CI candidate with appropriately fit HAs, this is an excellent counseling opportunity.
n
Candidacy evaluations should address the following:
The patient’s communication and listening goals Expected performance based on predictive measures Patient performance with their best nonsurgical treatment Patient’s motivation and lifestyle
Pediatric Audiologic Candidacy Evaluations
Children undergoing cochlear implantation typically should strive to wear appropriately programmed HAs during all waking hours. This includes HAs fit using real ear probe microphone measures to
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prescriptive targets. If the child has been wearing appropriately fit amplification and are still unable to access soft speech sounds, they should undergo a candidacy evaluation for CIs. Due to the complexity of each child’s speech/language skills and developmental differences, there is no common consensus of pediatric testing evaluations. As such, many of the evaluations are dictated by the child’s abilities. Some general guidelines are provided here.
n
Children require more access to sounds for development of speech and language and therefore
off-label considerations are particularly important in the pediatric environment (Park et al.,
2021). Additionally, greater outcomes are achieved with earlier implantation and implantation for children with better hearing than the most lenient current criteria.
n
Unaided evaluations: standard audiologic assessment with air- and bone-conduction
assessment and immittance measures.
For pediatric patients, testing may include otoacoustic emissions and ABR evaluation.
Other behavioral electrophysiologic measures may be useful, for example, auditory steady-state response (ASSR) or cortical auditory evoked responses (CAER). For detailed information regarding these assessment measures, refer to Chapters 5 and 6.
Regardless of reliable objective information, an attempt at behavioral threshold assessment
and speech perception should always be made in a potential pediatric CI recipient.
Speech recognition may be included in the unaided evaluation but is not required for
candidacy.
n
Subjective measures
Although these measures should be used with all children, these are required to be used
when children cannot participate in behavioral testing and serve as a substitute for word recognition measures.
Subjective questionnaires may include the auditory skills checklist, LittlEars, and/or
IT-MAIS. Certain questionnaires may be recommended by the insurance company for candidacy.
n
Aided evaluations
Auditory access to speech sounds is of critical importance to pediatric patients. As soon as
the child is developmentally appropriate for speech recognition testing, the clinician should obtain a baseline assessment. Frequent reevaluation of the child’s speech recognition should
occur during early childhood. All candidacy evaluations should be completed in the best-aided condition. The Pediatric Minimum Speech Test Battery (PMSTB) is a useful resource for developing
a preoperative and postoperative protocol for audiologic assessment. According to the
PMSTB, there are many options for evaluating speech perception when assessing implant
candidacy. Descriptions of these tests and tips for success in pediatric CI candidates and
recipients can be found in Table 9–7.
●
The test is chosen based on the patient developmental age and language abilities. Choose
a test that corresponds with response ability of the child.
●
Closed-set versus open-set tasks are available.
●
Although the tests do have recommended age ranges in many cases, children with severe
to profound hearing loss may not present with language abilities composite with their chronological age.
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TABLE 9 –7. Pediatric Speech Assessment Measures That Are Recommended for Cochlear Implant Candidacy Evaluations
OPEN/
TEST
CLOSED STIMULI CONDITION ADVANTAGES DISADVANTAGES
ESP Closed Words Quiet ESP Pattern: assess speech
abilities with low verbal component
Complex to administer; toys may be distracting; multiple trials needed
ESP spondee and ESP monosyllable: assess higher verbal skills with large closed-set field
PSI Closed Words,
sentences
MLNT/
Open Words Quiet Varying levels of
LNT
Quiet or noise
Different stimuli available Few lists; may encounter
learned practice effect
No normative data; difficulty; word and phoneme scoring
may encounter learning
effects
CNC Open Words Quiet Carrier phrase, adult test Full 50-word list
required, may be limited
by child’s attention
BKB Open Sentences Quiet Carrier phrase, single
male talker
Not designed to be
completed in quiet;
norms for children
>5years
BKB-SIN Open Sentences Noise Carrier phrase, single
male talker, variable SNR
Norms for children
>5years
Pediatric AzBio (BabyBio)
Note. Table is arranged in order of task difficulty with easier tasks at the top. Source: Adapted from Uhler et al. (2017).
Open Sentences Quiet or
noise
Normative data; equivalent lists; low context clues (more difficult)
It is critically important to choose a developmentally appropriate test to get a best
measure of the patient’s performance.
If the child scores <25% on the task you attempt, consider floor effects and administer
a less difficult test.
If the child scores from 25% to 79%, that test is currently appropriate and should be
reassessed at follow-up.
If the child scores >80%, consider ceiling effects and administer a more difficult test.
●
Test conditions vary based on test difficulty and patient ability. At a minimum, test
conditions are as follows:
Sound-treated room Soundfield speaker at 0 degrees azimuth 1 meter from the recipient
Female talkers only
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Ensure calibration of CD materials
n
Candidacy evaluations should address the following:
Family’s communication and listening goals Expected performance based on predictive measures Patient performance with their best nonsurgical treatment Family motivation and lifestyle Family resources for access to services
●
If the family does not have adequate access to services or resources to travel to
appointments, other team members should be included to help the family access adequate services during the rehabilitation process.
Predicting Outcomes
Patient predictive factors are reviewed and assessed to help predict outcomes after cochlear implantation. An important thing to note about this section is that it refers to the relative overall word recognition score (i.e., sentence score) and performance in noise in comparison to other CI users. In general, even if the patient is likely to have poorer outcomes based on these predictive factors, candidates are still likely to have improved performance compared to their score with their best-fit nonsurgical amplifica­tion. Word and sentence recognition and quality of life can be improved with CIs, but audiologists can use these predictive factors to set expectations related to how the individual patient’s performance will be compared to all CI recipients. Predictive factors for adult and pediatric patients are shown in Tables9–8 and 9–9, respectively.
TABLE 9–8. Predictive Factors for Cochlear Implant Performance in Adults
PREDICTIVE FACTOR
Duration of hearing loss
Age at implantation
Preoperative hearing status
Etiology Sudden idiopathic
Cognition Better performance with
BETTER OUTCOMES (RELATIVE)
Shorter duration Longer duration Full-time amplification use offsets
Younger age Older age Duration of hearing loss linked to
Better residual hearing Poorer residual hearing Preoperative amplification offsets
SNHL, Meniere’s disease, genetic
higher cognitive status
POORER OUTCOMES (RELATIVE) SPECIAL CONSIDERATIONS
duration
age; quality of life improvements with CI irrespective of age
status
Temporal bone fracture, acoustic neuroma, acquired ANSD
Potentially poorer performance with lower cognitive status
Bacterial meningitis: average performance; consider site of lesion for all patients
Dementia exacerbated with hearing loss, improving sound access prevents further cognitive decline; cognition may be improved with improved auditory signal access
Source: Key factors identified via Wolfe (2020).