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Audiology Review: Preparing for the Praxis and Comprehensive Examinations
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APPENDIX 6–A. continued
ELECTROPHYSIOLOGIC MEASURE DIAGNOSTIC OUTCOME AUDITORY STATUS
LLR Abnormal P1/N1/P2 amplitudes Abnormal maturation or function of
the auditory cortex
Absent P300 response Abnormality in top-down (decision
making) or bottom-up (difference detection) processing; often seen with cortical lesions or delayed auditory development
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Vestibular Assessment and
Differential Diagnosis
Introduction
Chapter 7
Jamie M. Bogle
The vestibular system is a set of sensory end organs that detect angular and linear acceleration. This infor­mation is transmitted through various reflex pathways to maintain appropriate balance and visual stability. Vestibular assessments use these pathways to identify weaknesses and to suggest possible diagnoses.
Vestibular System Anatomy and Physiology
Vestibular system end organs are housed within the inner ear. The membranous labyrinth is located within the otic capsule and is filled with endolymph, a fluid with high potassium concentration (Smith et al., 1965). There are five vestibular end organs: three semicircular canals (SCCs) and two otolith organs (Figure 7–1). Each end organ contains its own sensory epithelium, the crista ampullaris for the semicircular canals and the maculae for the otolith organs. The hair cells within the sensory epithelium transmit mechanical energy into neural impulses encoding acceleration information. The superior ves­tibular nerve branch of cranial nerve VIII (CN VIII) innervates the horizontal and superior semicircular canals and utricle, while the inferior vestibular nerve branch innervates the posterior canal and saccule.
Semicircular Canals (SCCs)
SCCs detect angular acceleration, meaning that they encode rotational movements. They are labeled based on their orientation as the horizontal (or lateral), anterior (or superior), and posterior (or inferior) SCC. Each SCC detects angular acceleration in a specific plane to create a three-dimensional model of head movement.
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Each SCC has a sensory epithelium at one end with the other open to the common vestibule.
The ampulla houses the sensory epithelium, called the crista ampullaris, and the cupula. The cupula is a gelatinous structure that extends across the SCC to create a fluid-tight seal (Lysakowski et al., 1998). Think of the cupula as a sail, deflecting when the head turns to trigger the underlying sensory epithelium. The cupula only responds to angular acceleration
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Audiology Review: Preparing for the Praxis and Comprehensive Examinations
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FIGURE 7–1. Anatomy of the peripheral vestibular labyrinth. Source: From Lane JI, Witte RJ, Bolster B, Bernstein MA, Johnson K & Morris J. AJNR Am J Neuroradiol. 2008 Sep;29(8):1436–1440; used with permission of Mayo Foundation for Medical Education and Research, all rights reserved.
AUDIOLOGY NUGGET
The SCCs work together between ears as a pair — described as a co-planar or push-pull mechanism. The horizontal SCCs form a pair. For example, as you turn your head to the right, the right horizontal SCC experiences ampullopetal flow (excitatory), while the left experiences ampullofugal flow (inhibitory). Each anterior SCC is paired with the contralateral posterior SCC to describe the RALP (right anterior/left posterior) and LARP (left anterior/right posterior) planes. This co-planar relationship is vital to vestibular system performance because each head movement transmits both excitatory and inhibitory information to encode a range of accelerations.
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because it has the same specific gravity as the surrounding endolymph. This also means that it does not respond to linear acceleration.
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For the horizontal SCC, as the head turns, endolymph lags and pushes on the cupula
to depolarize (i.e., excite) the sensory hair cells. This type of endolymph flow is called ampullopetal. Conversely, a head turn in the opposite direction will hyperpolarize (i.e., inhibit) these same sensory hair cells. This type of endolymph flow is called ampullofugal. The anterior and posterior SCCs are oriented differently — ampullopetal flow inhibits, ampullofugal flow excites (Lysakowski et al., 1998).
Otolith Organs
The utricle and saccule are collectively known as the otolith organs. They are housed in the vestibular labyrinth (see Figure 7–1). These end organs are responsible for detecting linear acceleration, includ­ing gravity. The utricle generally encodes horizontal acceleration while the saccule encodes vertical acceleration.
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Each otolith organ contains a sensory epithelium called the otolithic membrane. This
structure is embedded with calcium carbonate crystals called otoconia or otoliths (Lundberg etal., 2006). The otoconia increase the specific weight of the otolithic membrane, causing it to pull toward gravity or to lag during linear acceleration, thus triggering the underlying sensory hair cells.
Vestibular Nerve
Cranial nerve VIII (CN VIII) innervates both the cochlea and vestibular system. The vestibular portion further divides into two branches. The superior vestibular nerve branch innervates the horizontal SCC, anterior SCC, and utricle. The inferior vestibular nerve innervates the posterior SCC and saccule (Naito et al., 1995).
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Both vestibular nerve branches travel from the peripheral vestibular system through the
internal auditory canal and cerebellar pontine angle to synapse at various vestibular nuclei in the brainstem.
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The vestibular nerve has a high spontaneous firing rate ranging from 70 to 100 spikes per
second (Goldberg & Férnandez, 1971; Lysakowski et al., 1995). When excited, the vestibular nerve can fire up to 400 spikes per second (Uchino et al., 1982). Because of the high resting neural firing rate, inhibition can be encoded, providing the mechanism for co-planar stimulation.
Blood Supply
The labyrinthine artery supplies blood to the vestibular system. This artery typically arises from the anterior inferior cerebellar artery (AICA) (Baloh & Honrubia, 2001). One branch (superior vestibular artery) supplies the horizontal SCC, anterior SCC, and utricle, while the other (common cochlear artery) supplies the posterior SCC, saccule, and cochlea. The blood supply to vestibular structures in the brainstem and cerebellum is provided from various branches of the vertebrobasilar artery.
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Central Vestibular System
Once information from the vestibular end organs enters the brainstem, it synapses within the vestibular nuclei (VN). The VN contains several substructures, including the superior VN, medial VN, lateral VN, and inferior VN. Each of these substructures transmits specific end-organ information to the appropriate reflex pathways.
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Cerebellum: the superior VN and lateral VN are highly connected to the cerebellum, with
predominant information transmitted from the otolith organs (Carpenter & Sutin, 1983; Pansky et al., 1988; Watson, 1991). The cerebellum transmits this information to the reticulospinal tract, lateral vestibulospinal tract, and reticular formation (Pansky et al., 1988). Information from the cerebellum is heavily used for motor control.
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Reticular formation: the reticular formation uses information primarily from the otolith
organs and descends the length of the spinal cord (Fetter & Dichgans, 1996; Honrubia & Hoffman, 1997). Vestibular system activation along this pathway may trigger sweating, nausea, and vomiting (Pansky et al., 1988).
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Thalamocortical projections: information from the VN ascends to the thalamus, further
projecting to the parietoinsular vestibular cortex (PIVC). The PIVC serves as a major integration site for vestibular, visual, and motor system information. Thalamocortical projections are also traced to the hippocampus, where they influence spatial perception and cognition (Bigelow et al., 2016; Brandt et al., 2005; Semenov et al., 2016) and descend to the VN/brainstem (Berthoz, 1996) to contribute to the efferent vestibular system.
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Autonomic system: vestibular system information also influences autonomic system function.
The autonomic system controls various sympathetic and parasympathetic responses. The otolith organs specifically contribute to blood pressure regulation during movement and postural change (Goldberg et al., 2012; Yates et al., 2014).
Vestibular Reflexes
Reflex pathways are used to evaluate vestibular system performance. There are several basic vestibular system reflexes.
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Vestibulo-ocular reflex (VOR): enables visual stability during motion. The VOR is a reflex
pathway from the SCCs to specific extra ocular muscles to produce equal and opposite eye movements in response to a head turn. If the VOR is not working properly, the patient may experience oscillopsia — the perception of oscillating movement in the environment during motion. Figure 7–2 provides the horizontal SCC VOR pathway. All SCCs provide information to the VOR, but the horizontal SCC is the most evaluated pathway (e.g., caloric testing, rotational chair testing, head impulse testing). The utricle also provides a translational VOR (e.g., ocular vestibular evoked myogenic potentials).
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Vestibulo-collic reflex (VCR): enables the head to remain still during movement (Hain &
Helminski, 2014). The proposed pathway for the VCR includes the saccule, inferior vestibular nerve branch of CN VIII, medial VN, CN XI (spinal accessory), and ending in the trapezius and sternocleidomastoid (SCM) muscles.
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FIGURE 7–2. The vestibulo-ocular reflex (VOR) pathway includes the vestibular nuclei (VN), cranial nerve VI (CN VI), cranial nerve III (CN III), the medial longitudinal fasciculus (MLF), lateral rectus muscle (LR), and medial rectus muscle (MR). Source: From Balance Function Assessment and Management, Third Edition (pp. 1–717) by Jacobson, G. P., Shepard, N. T., Barin, K., Burkard, R. F., Janky, K., & McCaslin, D. L. Copyright © 2021 Plural Publishing, Inc. All rights reserved.
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Vestibulo-spinal reflex (VSR): triggers upper and lower limb responses to changes in balance
(Schubert & Shepard, 2016). Patients with damage to the vestibular system may demonstrate imbalance acutely but should recover as vestibular compensation occurs. The VSR can be evaluated using a range of bedside (e.g., Romberg test) and forceplate measures (e.g., computerized dynamic posturography).
Vestibular Nystagmus
Most vestibular diagnostic testing measures nystagmus — rapid, involuntary eye movements. Nystag­mus allows the clinician to (1) evaluate a possible site of lesion in those with spontaneous nystagmus and (2) define peripheral vestibular system performance as measured in induced nystagmus. Nystag­mus represents the VOR (Figure 7–3). The slow phase component corresponds to vestibular system information, while the fast phase is a reset saccade initiated to bring the eyes back to midline. Nys­tagmus is named by the fast phase for most analyses and is measured by the velocity (°/second) of the slow phase.
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FIGURE 7–3. Vestibular nystagmus. In this example, the eyes drift slowly to the left (slow phase; downward on the graph) and quickly reset to the right (fast phase; upward on the graph). This tracing describes right-beating nystagmus.
KNOWLEDGE CHECKPOINT
Peripheral vestibular nystagmus follows Ewald’s laws (Ewald, 1882). These laws describe how endolymph flow reliably influences the VOR to create predictable nystagmus.
1. The axis of nystagmus parallels the axis of the SCC that generated it.
2. Ampullopetal endolymph flow produces a stronger response than ampullofugal flow in the horizontal SCCs.
3. Ampullofugal endolymph flow produces a stronger response than ampullopetal flow in the anterior and posterior SCCs.
Vestibular Case History and Questionnaires
The case history is one of the most important components of the vestibular assessment. A thorough case history should include:
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Temporal features of dizziness symptoms: timing, triggers
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Dizziness characteristics: spinning, rocking, imbalance, lightheadedness, drunk feeling, spacey,
and so on
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Additional symptoms: hearing loss, tinnitus, headache, nausea/vomiting, unsteadiness, other
neurological concerns
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Vascular risk factors: for example, cardiac syncope, atherosclerosis, heart disease,
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vertebrobasilar artery insufficiency
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Neurological risk factors: for example, migraine, stroke, transient ischemic attack (TIA),
concussion, multiple sclerosis
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Medications (Table 7–1)
Some medications may cause vestibulotoxicity and many others cause dizziness/imbalance. Many patients use multiple medications to manage various conditions. Polypharmacy — the
simultaneous use of >4 medications (Masnoon et al., 2017)
— is a common cause of dizziness. Nearly 70% of patients presenting with dizziness and polypharmacy most often have diagnoses including diabetes, hypertension, other cardiovascular abnormalities, and depression (Jeong et al., 2022).
Questionnaires are useful tools to identify the patient’s self-perceived symptoms as well as how symptoms impact quality of life. There are many questionnaires available, with commonly used options provided in Table 7–2.
TABLE 7–1. Medications Associated With Vestibulotoxicity, Dizziness, and Imbalance
Classes of Vestibulotoxic Medications
Classes of Medications Associated With Dizziness/ Imbalance
Antibiotics (e.g., gentamicin) Platinum-based chemotherapeutics Loop diuretics Antimalarial drugs Nonsteroidal anti-inflammatory drugs (NSAIDs) Acetylsalicylic acid
Antispasmodics used for intestinal mobility disorders Diuretics Beta-blockers Hypertension medications Antihistamines Hypnotic/anxiolytic medications First-generation antipsychotics Tricyclic antidepressants Analgesics Migraine medications Antiepileptic medications Parkinson medications Antibiotics HIV medications Corticosteroids Hormone therapies
Source: Altissimi et al. (2020).
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TABLE 7–2. Commonly Used Questionnaires to Evaluate Patients With Dizziness/Imbalance
QUESTIONNAIRE PURPOSE
General Scales
Hospital Anxiety and Depression Scale (HADS) (Zigmond & Snaith, 1983)
Generalized Anxiety Disorder–7 (GAD-7) (Spitzer et al., 2006)
Patient Health Questionnaire–9 (PHQ-9) (Kroenke et al., 2001)
Dizziness Scales
Dizziness Handicap Inventory (DHI) (Jacobson & Newman, 1990)
Activities-Specific Balance Confidence Scale (ABC) (Powell & Myers, 1994)
Visual Vertigo Analog Scale (VVAS) (Dannenbaum et al., 2011)
Dizziness Symptom Profile (DSP) (Jacobson et al., 2019)
Bedside Evaluation
Determine levels of anxiety and depression in an outpatient setting
Screen for generalized anxiety disorder
Screen for depression
Evaluate self-perceived handicap due to dizziness
Assess balance confidence and determine fall risk
Quantify dizziness symptoms to visually provoking situations
Assist in identifying common vestibular disorders
Patients presenting for audiometric testing may also report dizziness/imbalance. Bedside testing provides clues to identify patients who may be experiencing vestibular system dysfunction (Table 7–3).
Electronystagmography/Videonystagmography (ENG/VNG)
The most common vestibular diagnostic battery is the ENG/VNG. The name of the test describes the recording method; however, the subtests are the same for both.
ENG
ENG recording takes advantage of the corneoretinal potential (CP). Electrodes are placed on the inner canthi, outer canthi, and above and below the eyes to record electrical activity. Measuring the changes in electrical activity identifies nystagmus and other oculomotor movements. Most clinicians use bitemporal electrode placement, with the horizontal electrodes placed on the outer canthus of each eye (Figure 7–4). With this montage, the electrical activity between the eyes is averaged and the tracing cannot identify disconjugate eye movements. Monocular (i.e., recording each eye individually) can also be completed. ENG is rarely completed today due to advances in video recording technology.
TABLE 7–3. Bedside Evaluation Options Summary
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TEST PURPOSE TECHNIQUE INTERPRETATION
Spontaneous vestibular nystagmus
Identify a static imbalance between right and left peripheral vestibular systems
Head impulse test Identify VOR
dysfunction
Head shake nystagmus
Identify asymmetrical neural integration of VOR; measure of compensation
Dynamic visual acuity test
Identify oscillopsia with head movement
Observe the patient’s eyes for nystagmus in vision and vision-denied conditions.
Examiner moves the patient’s head in low-amplitude, high­velocity head turns; observe the patient’s eyes for loss of visual fixation.
Patient oscillates the head for 25 cycles at 2 Hz; vision denied.
Patient reads to the lowest level on an eye chart with the head stable and with the head oscillating at 2 Hz.
Normal:
no nystagmus
Abnormal: nystagmus; determine if it follows Alexander’s law
Normal:
no loss of fixation
Abnormal: loss of fixation; patient demonstrates a “catch-up” saccade to regain fixation
Normal: <3 beats of post­headshake nystagmus
Abnormal:
> 3 beats of post-headshake nystagmus; fast phase typically beats toward the better ear
Normal:
≤2 lines of change
on the eye chart Abnormal:
> 2 lines of change on the eye chart
Valsalva-induced nystagmus
Identify the effect of intracranial pressure change on nystagmus
Modified Clinical Test of Sensory Interaction
Measure postural control under various
conditions on Balance (mCTSIB)
Romberg Test Measure postural
control under various
conditions
Patient increases intracranial pressure by performing Valsalva maneuver for 10–15 seconds.
Patient maintains balance for 30 seconds in four conditions: (1) eyes open, firm surface; (2) eyes closed, firm surface; (3) eyes open, foam surface; (4) eyes closed, foam surface.
Patient maintains balance for 30 seconds in two conditions: (1) eyes open, firm surface and (2) eyes closed, firm surface.
Normal:
no dizziness or
nystagmus Abnormal:
conjugate eye movement toward the contralesional ear; nystagmus, dizziness
Normal: ability to maintain balance in all conditions for 30 seconds
Abnormal: inability to maintain balance in all conditions for 30 seconds
Results may also be compared to age-specific normative values using forceplate analyses.
Normal: ability to maintain balance in all conditions for 30 seconds
Abnormal: inability to maintain balance in all conditions for 30 seconds
continues
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