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E. Kösemihal et al.
conduction thresholds in conductive hearing loss and the degree of hearing loss in cases where masking is required as well as for obtaining information about hearing levels in individuals with atretic ears. However, because the maximum intensity level at which the bone vibrator can be stimulated is usually 55–60dB nHL, this limits the estimation of bone conduction hearing thresholds beyond mild- to­moderate degrees [72, 73].
8.5.3.3 Auditory Steady-State Responses
Auditory steady-state responses (ASSRs) reect electrical potentials produced by stimulating the ear with a continuous and repetitive series of frequency (FM) and amplitude modulations (AM). ASSR software are typically integrated into a device with an ABR module. The electrode placement and earphones are the same as those of the ABR.The ASSR test has recently been added to audiological test batteries. This technique provides an objective testing option for threshold estimation with automated and statistically based response analysis. No waveform analysis or inter­pretation is required [74, 75]; however, recording quality should be monitored and results cross-checked with other tests to avoid artifactual results.
The ASSR is often evoked by pure-tone stimuli, which has the advantage of frequency-specic threshold estimation. A carrier frequency between 500 and 8000Hz is used with different modulation frequencies and depths. In clinical practice, 500–4000Hz is usually preferred due to time constraints. Frequency modulation (FM), amplitude modulation (AM), or mixed modulation (MM), which combines both, can be used to modulate the carrier frequency and enable response detection [75]. It is possible to assess multiple frequencies simultane­ously in both ears by modulating multiple stimuli at different frequencies at different rates. Using pure tones as stimuli in ASSR, it is possible to assess intensity levels of approximately 120dB HL.ABRs can measure responses up to levels of about 100dB nHL [74]. High intensity levels are needed for thresh­old determination for cochlear implant candidates, especially young children. As is the case for ABRs, ASSR measurements can be made with air and bone conduction transducers. Standard test and stimulus parameters are provided in Table8.8 [72].
Effect oftheStimulus Rate
Based on evidence for the response generators, ASSRs are typically divided into 40-Hz and 90-Hz ASSRs. The unit Hz used here refers to the approximate stimulus modulation/presentation rate. The 90-Hz ASSR is used for sleeping subjects of all ages (natural sleep, sedation, or general anesthesia), while the 40-Hz ASSR is used for assessing awake adults and older children. The 40-Hz ASSR can be considered similar to the middle latency response (MLR). The MLR is immature in neonates, so its use in neonates is not recommended. The 40-Hz ASSR is appropriate for older awake children (> 5years) and adults.
The 90-Hz ASSR is thought to originate from brainstem-level generators, similar to the ABR.It is identical to the ABR, in that it can be recorded from birth, is mainly unaffected by sleep, sedation, or general anesthesia, and is suitable for use at all
8 Behavioral andElectrophysiological Tests inAudiology
Table 8.8 Standard test and stimulus parameters for ASSR
Parameters Selections or options Transducers Insert earphones, supra-aural earphones, bone oscillators, and
loudspeakers for stimulation in the sound eld Carrier frequencies 250, 500, 1000, 2000, 4000, 8000Hz Modulation
frequencies Amplitude modulation
(AM) Frequency modulation 10% Advanced modulation
options Stimulus intensity
range Calibration reference dB HL
Different modulation rates from 70 to 110Hz
100%
Exposure modulation (AM2); phase-adjusted
0–125dB HL, depending on the frequency and the transducer
159
ages, including neonates. Since the ASSR stimulation rate is affected by the sleep state, it is essential to correctly select the child or adult as asleep or awake from the test protocols included in the software. Correction factors for the estimated HL to be obtained vary according to test protocol—these correction factors are included in the ASSR equipment [75, 76].
The ASSR also has potential disadvantages. Unlike the ABR, it is not a diagnos­tic test, and this test does not provide any information about the site of auditory dysfunction and cannot identify ANSDs. Therefore, the combined use of the ABR and ASSR is preferred for diagnostic evaluation and threshold estimation rather than the ASSR alone. A graph is generated from the result of the ASSR test, indicat­ing whether responses were obtained and at what intensity levels [75, 76].
8.5.3.4 Electrocochleography
This test records the electrical activity of the auditory nerve and cochlear hair cells in response to auditory stimuli. Electrocochleography (ECochG) provides information about the evaluation of peripheral auditory function, the diagnosis of ANSDs, the diagnosis of Meniere’s disease, and the localization of peripheral auditory dysfunction. Intraoperative neurophysiological monitoring of cochlear and CN VIII status during surgery that puts these structures at risk is another use of ECochG.
Electrocochleography response amplitudes vary with the type of electrode used. The best response amplitudes are obtained with a small needle electrode placed on the promontory through the ear canal via the transtympanic membrane approach. Other options include placement of electrodes on the tympanic membrane and in the ear canal [77, 78].
Cochlear microphonics (CMs), summation potential (SP), and action potential (AP) are the three components of ECochG.Cochlear microphonics originate from OHCs, whereas the summation potential mainly depends on inner hair cell activity [77, 78]. The action potential is generated by the simultaneous ring of the distal afferent eighth nerve bers and corresponds to ABR wave I [79].
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Electrocochleography Analysis
ECochG analysis involves conrming the presence of CMs, calculating amplitude and latency values for summation and action potentials, and calculating the SP/AP amplitude ratio. The SP/AP ratio is expressed as a percentage. SP/AP normative values vary depending on the electrode type. The normative SP/AP percentages for each electrode type are as follows: ear canal electrode type SP/AP >50%, tympanic membrane electrode SP/AP >40%, and transtympanic needle electrode SP/AP >30% [72, 80, 81].
In Meniere’s disease, an abnormally high SP/AP ratio is observed. The sensitiv­ity of the transtympanic membrane electrode technique was reported to be between 50% and 60% and its specicity was more than 90% in the diagnosis of endolym­phatic hydrops [81]. Another advantage of ECochG is that it is an important test in the diagnosis of ANSDs [82, 83]. Detection and analysis of ECochG wave morphol- ogy and wave components may allow for distinguishing whether dysfunction is in the presynaptic or postsynaptic region. The ECochG result contributes to cochlear implantation decisions in patients with ANSD.However, due to difculties in clini­cal practice, it is preferred to evaluate the presence or absence of CMs in ABRs for the diagnosis of ANSDs [82, 83]. ECochG recordings can be more invasive and time-consuming than ABR recordings, so it is more common for clinicians to check for CMs by examining the early part of the ABR waveform in response to separate polarity, high-level click stimuli.
8.5.3.5 Cortical Auditory Evoked Potentials
Cortical auditory evoked potentials (CAEPs) are brain responses generated in the auditory cortex and nearby areas in response to (typical) longer-duration auditory stimuli. They are the late latency members of the auditory evoked potentials family. The purposes of measuring these potentials are to assess the neural transmission of sound to the level of the auditory cortex and objectively evaluate auditory discrimi­nation skills, to evaluate individuals with hearing disorders, and to monitor auditory rehabilitation outcomes [8486].
Time-locked evoked cortical activity provides information about the timing and magnitude of sound processing as evidenced by CAEP latencies and amplitude. Some components occur within 50 ms after stimulus presentation in adults, but these are later in children. The late-latency responses have four components, referred to as P1, N1, P2, and N2. The rst of these components, P1, is the same as Pb, the last component of the MLR.The P1 wave occurs at approximately 50ms, the N1 wave at approximately 100ms, and the P2 wave at approximately 200ms. The nal wave, N2, occurs at around 300–350ms but is often ignored in clinical evaluations as it is less prominent that the P1–N1–P2 complex. More than one source is cited as the generators of these components. Although CAEPs mainly originate from the thalamus and primary auditory cortex in the supratemporal region, they are also affected by the auditory association areas and the frontal cortex [8486].
CAEP wave morphology shows signicant variability with age. The wave mor­phology we observe as the P1–N1–P2 complex in adults consists of a large and broad P1 component in the early years of life. As this wave formally differentiates
8 Behavioral andElectrophysiological Tests inAudiology
161
by age 7 or later, N1 and P2 components appear. CAEP waves, which can be obtained from birth, begin with a long latency (about 200–400ms). During adoles­cence, CAEPs reach adult latency and amplitude values between the ages of 14 and
16. This is considered an indicator of auditory cortical maturation [87].
Auditory pathways and centers in the brain become more efcient with increased myelination, neural synchronization, and axon density. As the auditory system matures, there is an increase in complex abilities such as increased sensitivity to and discrimination of auditory stimuli, automaticity in information processing, adaptive attention, learning, and memory [87].
Clinical applications of CAEP measures include:
– Evaluation of hearing aid and cochlear implant performance in infants and
children.
– Assessment of cortical maturation by examining P1 latency. – Estimation of the hearing thresholds of patients who have difculty cooperating
for behavioral testing or sleeping for ABR or in medico-legal cases.
– Evaluation of auditory processing disorders, including those with comorbid lan-
guage or learning difculties.
– Monitoring the progression of neurodegenerative or psychological diseases [87].
CAEP measurements are obtained by changing parameters and using a different protocol within the equipment used for ABR testing. It is a test that requires being awake and still. When performed during sleep, the response amplitudes decrease. The patient can watch videos during testing, or infants/children can be distracted with quiet toys or books. Electrode placement is similar to the ABR, but different placements can be used according to device guidelines. Typical parameters that may be recommended for cortical auditory measurements for clinical purposes are listed in Table8.9.
8.5.3.6 Event-Related Auditory Potentials
P300
The P300 response is a cortical response that occurs 250–350ms after stimulus presentation. It reects whether two different presented stimuli can be discriminated in the brain. Many anatomical regions in the brain may contribute to the formation of the P300. It is thought to originate in the hippocampus, which is responsible for conscious memory. The sensory cortex, centroparietal cortex, and frontal lobe are also thought to contribute [84, 86].
The P300 response, rst described in the 1960s, is also known as the P3 or P3b. It is presented with a stimulus train called the oddball paradigm. The P300 record­ing task involves actively detecting an infrequent “oddball” stimulus amongst pre­sentations of a frequent standard stimulus. The ratio of frequent-to-infrequent stimulus is typically 80–85% to 10–15%. P300 recording requires active participa­tion of the patient. It is necessary to be alert, attentive, and cooperative during the test and hence is not suitable for very young children. The patient may be asked to
162
Table 8.9 Typical parameters for cortical auditory evoked potential testing
Parameters Tone burst, speech (recorded or synthetic syllables or phonemes) Stimulus
duration Level of
intensity Polarity Alternate/rarefaction Stimulus
repetition Number of
averages Filter 1–30Hz Artifact
rejection Analysis time Electrode
montage
20–200ms
70–80dB (for supra-threshold measurement) can be the lowest level in threshold estimation
1.1/s
100–300
±100μV
100ms/+600ms Cz, Fpz, M1–M2, or A1–A2 (electrode placement can also be placed in the
supraorbital–lateral canthus to prevent artifacts caused by eye movements)
E. Kösemihal et al.
mentally count or press the button in their hand when they perceive the infrequent stimulus. Infrequent stimuli may vary in frequency, duration, or intensity compared to the standard (Fig.8.6a). The P300 potential elicited in response to the stimulus presented within the oddball paradigm, which includes stimuli that differ in fre­quency, is shown. In this measurement, at 80 dB nHL, the frequent stimulus is 1000Hz and the infrequent/rare stimulus is 4000Hz [84, 86].
The P300 response has two subcomponents: P3a and P3b. The P3a response indicates that new signals from the brain are being perceived. Its latency is shorter, and its amplitude is lower. The P3b component occurs in response to rare stimuli with activation of memory and attention. The highest amplitude responses are obtained from the parietal region [84, 86, 88].
The P300 is an endogenous response. As the difference between infrequent and frequent stimuli increases, it becomes easier to distinguish between them. Thus, the latency may be shorter and the amplitude more robust. The equipment, electrode placement, and parameters used to measure the P300 are similar to those used to measure the late latency responses. However, a separate protocol must be opened and preferences made for frequent and infrequent stimuli. The P300 response can be used to electrophysiologically assess higher-order processing in patients with hear­ing aids and/or cochlear implants. It is also used in the diagnosis and follow-up of central nervous system disorders such as dementia, brain injury, cerebrovascular events, depression, alcoholism, auditory processing disorders, epilepsy, Parkinson’s disease, or schizophrenia. In such cases, a decrease in P300 response amplitude and a prolonged latency have been reported [89, 90].
Mismatch Negativity
Mismatch negativity (MMN) is a neurophysiological response that reects the brain’s automatic ability to discriminate minor acoustic differences. It is a negative response that occurs approximately 200ms after stimulus presentation. It was rst
8 Behavioral andElectrophysiological Tests inAudiology
163
b
Fig. 8.6 (a) Examples of the P300 test result and the (b) MMN record
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described by Naatanen, Gaillard, and Mantsalo (1978). In addition to the auditory cortex, the frontal cortex and auditory subcortical areas have been reported to be sources of MMN.The MMN response is also elicited by the oddball paradigm but in a passive listening situation without the need for a patient response. Frequent and infrequent stimuli are presented 15–20% and 80–85% of the time, respectively. MMN latency and amplitude vary with the size of the difference between the stim­uli. A longer latency and lower amplitude response can be obtained when similar stimuli are presented [91].
MMN recording is performed using similar parameters and electrode placement to the P300. Unlike the P300, it is a test that does not require patient participation. For this reason, it can be assessed in infants and toddlers. Maturation differences are observed into adulthood. While there may be no signicant age differences in the MMN response obtained with simple stimuli, as the task becomes more difcult (with complex stimuli such as speech), a reduction in latency is observed into adulthood (Fig.8.6b). The recording in Fig.8b was obtained by changing both the stimulus and intensity of the infrequent stimulus. The frequent stimulus was presented at 1000Hz and 80dB nHL, and the infrequent stimulus was presented at 4000Hz and 70dB nHL [9193].
Because MMN does not require attention and participation in the test, it can be assessed in all age groups. It evaluates the effects of auditory experience, matura­tion, treatment, training, and learning on the central auditory system. It is an adjunc­tive test for evaluating hearing aid and cochlear implant performance and for diagnosing and monitoring neurological and psychological conditions [9193].
Acoustic Change Complex
The acoustic change complex (ACC) assesses neural processing ability in response to acoustic changes, similar to MMN.It was rst described by Ostroff (1998) [94]. The ACC consists of two separate P1–N1–P2 complexes within the same waveform, as a result of presenting two different tonal or speech stimuli sequentially. The ACC represents discrimination ability at the level of the auditory cortex. It has been used to assess speech perception and has the ability to detect auditory differences [95] and to evaluate auditory skills, especially in hearing aid and cochlear implant users, as well as in psychiatric cases [85, 9597]. It has been reported that differences of up to 3dB in intensity between two stimuli can be detected using the ACC [85, 96, 97].
The electrode placement used in the ACC is similar to other cortical potential measures, but the stimulus presentation is different. Two different stimuli are pre­sented an equal number of times. Each stimulus can last up to 500ms. For this reason, it is preferred that the analysis window be at least 1000ms. When long-term sustained stimulation is used, the time for neural ring to recover increases and waves with larger amplitudes are obtained. Stimulus characteristics vary depending on the purpose of the assessment.
The ACC obtains two separate waves in a single trace. It does not require eval­uating the difference between the separate waveforms of two stimuli as in MMN or P300 evaluation. Because of this ease of use, it is preferred to MMN in some clinics.
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If we summarize CAEPs overall, these responses are obtained from auditory cortical areas and associated areas during the presentation of auditory stimuli. Sleep or sedation is not required. The P1–N1–P2 complex indicates that the sound has reached the cortex and that neural processing has begun. When more complex recording techniques are used for the P300, MMN, and ACC, the variables affecting response increase. Therefore, a single set of normative data has not been developed. It is recommended that each clinic obtains normative data according to their meth­ods [85, 96, 97].
Failure to obtain a CAEP response or an abnormal response may not directly reect a cortical problem. Failure to obtain a response despite reliable testing may reect a problem at any stage in the auditory pathway from the outer ear to the audi­tory cortex. Because the thresholds obtained correlate with behavioral thresholds in adults, CAEP estimation of thresholds is preferred in medicolegal cases. ACC and MMN tests do not require attention and provide information about sound discrimi­nation. The P300 response requires attention to the stimuli and is hence inuenced by cognition. For this reason, it is used in children over the age of 7 and in coopera­tive adults.
CAEPs are a tool that allows us to electrophysiologically image neural process­ing and evaluate the impact of auditory rehabilitation processes in the brain. Technological innovations will make these tests even more powerful in the future [85, 96, 97].

8.6 Conclusion

This section provides an overview of audiological assessments for neurotologists. It is recommended that audiology textbooks be consulted for detailed information on audiological testing. Best practices in diagnostic audiology require a test battery with appropriate behavioral and electrophysiological tests. In audiology, each test is of particular importance in evaluating the entire auditory function. The audiologist is responsible for selecting these tests according to the patient’s needs. With an appropriate test battery, diagnostic audiological tests can provide valuable informa­tion to guide decisions in the diagnosis and management of auditory and neuroto­logical disorders.

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