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462 Disorders of the Auditory System
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Posterior: At or toward the back of the
body or the head.
Poststimulus Time Histogram (PSTH): A
plot displaying the firing rate of a nerve
fiber to an eliciting stimulus over the
period of time that it fires.
Presbycusis: Hearing loss and decrease of
auditory function that occurs with aging;
also referred to as age-related hearing
loss.
Pressure Equalization (PE) Tube: Small
ventilation tube surgically placed in the
eardrum to compensate for a malfunctioning Eustachian tube; also referred to
as a tympanostomy tube.
Prevalence: The total number of cases of a
specific disease or disorder existing in a
given population at a certain time.
Proband: A person serving as the starting
point for the genetic study of a family.
Protympanum: The anterior-most space of
the middle ear that contains the opening
of the Eustachian tube.
Proximal: At or toward the trunk or point
of origin.
Prussak’s Space: Posterior epitympanic
space.
Putamen: A structure in the basal ganglia.
QT Interval: A defined part of the heart’s
electrical cycle.
Recruitment: The abnormal growth in loud-
ness perception as intensity increases.
Residual Inhibition:
which there is a temporary decrease or
elimination of the perception of tinnitus following the delivery of a masking
noise.
Retinitis Pigmentosa: A genetic eye disor-
der eventually leading to blindness.
Retrocochlear: The portion of the auditory
system that is “behind or beyond the
cochlea” (i.e., the auditory nerve and the
central auditory system).
Rhinorrhea: Discharge from the nose.
Rhinoscopy: Examination of the inside of
the nose.
Ribonucleic Acid (RNA): Similar to DNA,
RNA is composed of nucleotides. It con-
The phenomenon by
trols gene expression and the synthesis of
proteins.
Rinne Test: A tuning fork test that compares
a patient’s hearing by air conduction with
his or her hearing by bone conduction for
a single frequency.
Romberg Test: A test of vestibular func-
tion evaluating the maintenance of an
erect stance while standing with the feet
together and head straight, first with the
eyes open and then with eyes closed.
Rostral: Above another structure or toward
the head.
Sagittal: An anatomic plane that vertically
divides the head or the body into left and
right segments.
Schwartze’s Sign: A reddish hue at the pro-
montory that can be visualized on otoscopic
exam in many patients with otosclerosis.
Scutum: Sharp spur formed by the lateral
wall of the tympanic cavity.
Sensation Level: The intensity of a sound
above an individual’s threshold.
Sensorineural Hearing Loss: Type of hear-
ing loss resulting from impairment to the
inner ear, auditory nerve, or retrocochlear
auditory structures.
Sigmoid Sinus: A large vein posterior and
slightly inferior to posterior temporal
bone.
Stapedotomy: Surgical intervention for
otosclerosis that involves removing the
stapes’ superstructure, creating a hole in
the fixed footplate with a drill or laser,
and placing a prosthesis from the incus
to the stapedotomy site.
Stereocilia:
ing actin filaments that are arranged in an
orderly fashion on the tops of the inner
and outer hair cells of the cochlea; stereocilia are also found in the sensory cells of
the peripheral vestibular system.
Sternocleidomastoid: A large, superficial
cervical muscle located on the lateral
aspect of the neck which acts to rotate
the head.
Superficial: On or toward the surface of the
body or the head.
Hairlike protein rods contain-
Glossary 463
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Superior:
ward the head.
Syndromic Hearing Loss: An inherited
genetic condition that involves not only
the auditory system but also other systems, making it a multifaceted disorder.
Temporalis Fascia: Covering of the tempo-
ralis muscle.
Temporary Threshold Shift (TTS): A tem-
porary change in threshold hearing that
returns to preexposure levels over time.
Tensor Veli Palatini:
that tenses the soft palate.
Tinnitus: The internal perception of sound
in the absence of acoustic stimulus.
Torus Tubarius: The base of the cartilagi-
nous portion of the Eustachian tube.
Transcranial Magnetic Stimulation: Non-
invasive technique used to stimulate
various regions of the brain by applying
a magnetic coil to the scalp that generates
a current that passes through the scalp to
stimulate the brain tissue of interest.
Transient Ischemic Attacks: Temporary
decreases in blood supply to an area of
the brain that results in temporary (or
transient) symptoms.
Transverse: An anatomic plane that hori-
zontally divides the head or the body into
upper and lower segments.
Transverse Fracture: A fracture that runs
perpendicular to the long axis of the
petrous bone that occurs as a result of
blunt force trauma to the skull.
Trigeminal Neuralgia: Dysfunction of the
fifth cranial nerve (trigeminal), often
resulting in facial pain.
Tullio’s phenomenon: Vertigo elicited by a
loud sound.
Tuning Curve: A graph depicting the re-
sponse of a neuron, plotted as a function
of stimulus intensity versus frequency;
the lowest sound level to which the neuron responds is represented by the tip of
the tuning curve.
Tympanic Membrane: The membrane that
separates the outer and middle ears; also
commonly referred to as the eardrum.
Above another structure or to-
A ribbonlike muscle
Tympanometry:
measure the pressure, volume, and compliance of the middle ear system.
Tympanoplasty: Surgery in which disease
is removed from the middle ear and
a weakened or perforated eardrum is
repaired.
Vagus Nerve: The tenth cranial nerve re-
sponsible for many instinctive responses
from the body.
Valsalva Maneuver: Inflation of the middle
ear produced by closing the mouth and
pinching the nose and then exhaling (forcefully) air to open the Eustachian tube.
Valsalva Test:
expiration of air with a closed mouth
and an occluded nose while the tympanic
membrane is inspected.
Vascular Steel: A disorder in which one
area of the brain “robs” another area of
some of its blood supply.
Vasculitis: Inflammation of blood vessels.
Vasoconstriction: A narrowing of the lumen
of a blood vessel that results in a restriction of the flow of blood through the
involved vessel.
Vasodilators: Agents that increase the lu-
men of blood vessels and increase blood
supply.
Ventral: At or toward the front of the body
or the head.
Vertigo: A vestibular symptom in which the
patient either has a spinning sensation or
senses that the environment is spinning
while he or she remains stationary.
Videonystagmography (VNG): A test of
vestibular function in which nystagmus
is recorded using infrared goggles during stimulation of the vestibular system;
includes a battery of subtests divided
into three groups: ocular-motor, positional, and caloric.
Voxels: A volume element, representing a
value on a regular grid in a three-dimensional space. This is analogous to a pixel,
which represents two-dimensional image
data used in various types of imaging
(radiology, television).
Clinical measure used to
A test involving the forced
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Wallerian Degeneration: Degeneration of
the nerve fiber due to trauma. Specifically, the axon separates from the nerve
body.
Weber Test: A tuning fork test in which
bone-conduction signals are presented
to a midline site on the frontal bone; the
sound will lateralize to an ear with a conductive hearing loss if one is present.
X-linked: Mode of transmission of genes
on the sex-linked X-chromosome.
Zygosity: The similarity of a gene’s two
alleles.
We acknowledge the use of the following
resources to assist in the preparation of
this Glossary:
Mendel, L. L., Danhauer, J. L., & Singh, S.
(1999). Pocket dictionary of audiology. San
Diego, CA: Singular Publishing.
Musiek, F. E., & Baran, J. A. (2020). The audi-
tory system: Anatomy, physiology, and clinical
correlates (2nd ed.). San Diego, CA: Plural
Publishing.
Stach, B. A. (1998). Clinical audiology: An intro-
duction. San Diego, CA: Singular Publishing.
Wikipedia.com
Index
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Note: Page number in bold reference non-text material.
5-fluorouracil, ototoxicity of, 202
a
AAO-HNS (American Academy of
Otolaryngology-Head and Neck
Surgery), 394
Clinical Practice Guidelines on otitis
media, 130
Ménière’s disease diagnosis and,
209–210
Abnormal auditory cortex activation
model, 406
ABR (Auditory brainstem response), 31,
85–86
acoustic neuroma and, 267, 268–270
AD and, 333
cutoff criteria, 87
Down syndrome and, 433
HB and, 347
mass/vascular lesions and, 320–322
MS and, 331–332
noise-induced hearing loss, 195
ototoxicity and, 204
tests, 118
vascular loops, 298
surgery and, 299
Acoustic neuroma, 263–284
auditory, 267–271
acoustic reflexes, 268
brainstem response, 268–270
management of, 272
otoacoustic emissions, 268
pure-tone threshold and, 267–268
speech recognition tests, 267–268
vestibular evaluation, 270–271
case study, 275–284
described, 263–264
etiology/pathology of, 266–267
incidence/prevalence of, 265–266
lesion site, 267
medical
examination, 271–272
management of, 272–274
MRI and, 86
surgery for, 272–274
risks of, 273–274
symptoms, 264–265
vascular loops and, 298
Acoustic reflexes (AR), 20
acoustic neuroma and, 268
audiologic assessment, 75–77
decay, 76
HB and, 347
stapedial, 75
testing, otosclerosis and, 154
Acoustic stapedial reflex, 75
Acquired, cholesteatoma, 137
465
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Action potentials (AP), 212
of electrocochleographic recording, 38
AD (Alzheimer’s disease), 325
dementia and, 328
diagnosis of, 329–330
etiology of, 329
incidence/prevalence of, 328
medical, management of, 330
Adaptation, defined, 41
Adenine
in DNA, 414
in RNA, 414
Africans, MS and, 328
Age, AD and, 329
Agenesis of corpus callosum, 442–443
Age-related hearing loss (ARHL), 221–228
audiological, management of, 225–226
audiology and, 225
etiology/pathology of, 224
incidence/prevalence of, 224
lesion site, 224–225
medical, examination, 226
symptoms, 224
AICA (Anterior inferior cerebellar artery),
55, 56
vascular loops and, 296
vestibular system and, 63
AIED (Autoimmune inner ear disease),
219–221
Air
conduction
hearing thresholds, aural atresia and,
118
test, temporal bone injuries and, 161
pressure, barotrauma and, 246
Alcohol, occlusive disease and, 316
Alleles, 414, 415
mutated, 418
Alpert syndrome, 429
Alport syndrome, 427
Alzheimer’s disease (AD), 325
dementia and, 328
diagnosis of, 329–330
etiology of, 329
incidence/prevalence of, 328
medical, management of, 330
American Academy of Otolaryngology-
Head and Neck Surgery (AAO-
HNS), 394
Clinical Practice Guidelines on otitis
media, 130
Committee of Hearing and Equilibrium
Ménière’s disease diagnosis and,
209–210
American College of Medical Genetics and
Genomics Guideline for the Clinical
Evaluation and Etiologic Diagnosis
of Hearing Loss, 444
Amikacin, ototoxicity of, 201
Aminoglycosides
ototoxicity of, 201–203
blood serum levels, 205
Amplification
hearing loss and, 213
sensorineural hearing loss and, 205
SSHL and, 238
AN (Auditory nerve)
abnormalities, 439
AR and, 20
cochlea
brainstem and, 37
function of, 38–41
and, structure of, 37–38
coding, 40–41
described, 263
disorders
acoustic neuroma, 263–284
auditory neuropathy spectrum
disorder, 284–296
vascular loop syndrome, 296–303
fibers, 38
vascular loops and, 298
Analgesics, ototoxicity of, 199
Anatomic terminology, 12
Aneurysms, 315
brain, 316
Annular ligament, 18
ANSD (Auditory neuropathy spectrum
disorder), 435–436
Anterior, defined, 12
Anterior inferior cerebellar artery (AICA),
55, 56
vascular loops and, 296
ventral cochlear nucleus, 42–44
vestibular system and, 63
Antiarrhythmics, tinnitus and, 395
Antibiotics
immune system and, 219
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ototoxicity of, 199
Anticonvulsants
tinnitus and, 395
vascular loops and, 298
Antidepressants, tinnitus and, 395
Antiemetics, vertigo and, 213
Antihelix, 15
Antimalarial medication, ototoxicity of,
199, 203
Antineoplastics, ototoxicity of, 199, 202
Antioxidants,
ARHL and, 226
noise-induced hearing loss, 196
Antitragus, 15
Anxiety, tinnitus and, 393
Anxiolytics, tinnitus and, 395
AP (Action potentials), 212
of electrocochleographic recording, 38
Apert syndrome, 428
Aplasias
inner ear, 437,438
AR (Acoustic reflexes), 20, 154
acoustic neuroma and, 268
audiologic assessment, 75–77
HB and, 347
stapedial, 75
Arachnoid cysts, treatment of, 275
ARHL (Age-related hearing loss),
221–228
audiological management of, 225–226
audiology and, 225
etiology/pathology of, 224
incidence/prevalence of, 224
lesion site, 224–225
medical, examination, 226
symptoms, 224
Aricept, AD and, 330
Arnold’s nerve, 148
Artery
anterior inferior cerebellar, 56
ascending pharyngeal, 55
basilar, 56
internal auditory, 55
labyrinthine, 55
middle meningeal, 55
posterior auricular, 55
pterygoid, 55
superficial temporal, 55
Arthritis, Stickler syndrome and, 432
Aspirin
noise-induced hearing loss, 196
ototoxicity of, 202, 203
Astrocytomas, 312
Asymmetric sensorineural hearing loss
acoustic neuroma and, 264
MRI false positives and, 269
AT (Auditory training), learning difficulties
and, 371
Ataxia
acoustic neuroma and, 265
heavy metals and, 338
MS AND, 328
Usher syndrome and, 426
Atherosclerosis, 315–316
Atresias, inner ear, 437
Atretic ear canal, surgical correction of, 119
Audiogram, 71
described, 70
Audiologic
evaluation, Ménière’s disease and, 211
examination, aural atresia and, 118
processing tests, 80–83
Audiologic assessment
auditory processing tests, 80–83
electroacoustic measures, 76–80
electrophysiologic assessment, 83–91
hearing sensitivity, 70–73
immittance audiometry, 73–80
acoustic reflexes, 75–77
tympanometry, 73–75
speech recognition ability, 70–73
Audiology
Eustachian tube dysfunction and, 124
neurotoxins and, 342–347
Audiology and neurotoxins
CANS and, 342–347
case study, 342–344
described, 342
heavy metals, 342–344
solvents, 342–344
Audiometer, described, 70
Auditory
abnormalities, Stickler syndrome and,
432
cortex, 48–52
cortices
auditory hallucinations and, 404
lesions of, 321
468 Disorders of the Auditory System
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Auditory (continued)
neuropathy, 270
spectrum disorder (ANSD), 435–436
problems, MS and, 329
processing
central, 80
tests, audiologic assessment, 80–83
system
detection of sounds, 11
electrophysiologic assessment of,
83–91
peripheral, temporal bone, 13–14
vascular supply for, 55–56
vestibular system and, 11
training (AT), learning difficulties and,
371
Auditory brainstem response (ABR), 31,
85–86
acoustic neuroma and, 267, 268–270
AD and, 333
cutoff criteria, 87
Down syndrome and, 433
HB and, 347
mass/vascular lesions and, 320–322
MS and, 331
ototoxicity and, 204
tests, 118
vascular loops and, 298
surgery and, 299
Auditory canal
external, 115
aural atresia and, 116
internal, MRI of, 103
Auditory hallucinations, 386, 401–408
causes of, 404
etiology/pathology of, 403
evaluation of
audiologic, 405–406
medical, 405–406
incidence/prevalence of, 402–403
lesion site, 403, 405–406
models for generation of, 406
symptoms, 402
treatment of
audiologic, 406–407
medical, 406–407
Auditory nerve (AN)
abnormalities, 439
AR and, 20
cochlea
brainstem and, 37
function of, 38–41
structure of, 37–38
coding, 40–41
described, 263
disorders
acoustic neuroma, 263–284
auditory neuropathy spectrum
disorder, 284–296
vascular loop syndrome, 296–303
fibers, 38
vascular loops and, 298
Aural atresia, 116–122
audiologic management of, 118–119
audiology and, 118
case study, 120–122
classification system, 117
described, 116
etiology/pathology of, 117
incidence/prevalence of, 117
lesion site, 117
medical
examination, 118
management of, 119–120
symptoms, 116–117
syndromes associated with, 117
Aural fullness
barotrauma and, 246
fluid pressure and, 210
Ménière’s disease and, 209
Auricle, 14, 115
Auricular perichondrium, tympanic
membrane (TM) and, 141
Autoimmune inner ear disease (AIED),
219–221
Autoinsufflation, Eustachian tube and,
123
Automobile accidents, temporal bone
injuries and, 161
Autonomic nervous system, secondary
reactions, 59–60
Autosomal recessive inheritance pattern,
415
inheritance pattern conditions, 417
pedigree showing, 417
Autosomes, 413
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AVCN (Anterior ventral cochlear nucleus),
42–44
Azathioprine, MS and, 330
Azithromycin, ototoxicity of, 203
B
Baclofen, vascular loops and, 298
BAER (Brainstem mauditory evoked
potential), 85
Balance
acoustic neuroma and, 264, 265
heavy metals and, 338
MS AND, 328
ototoxicity and, 202
Barotitis media, 246
Barotrauma, 160, 246–248
Basilar
artery, 56
membrane (BM), 20, 23
composition of, 24
spiral ligament and, 28
Battle’s sign, 162
Beating eye movement, balance system
and, 271
Behavioral tests, 80–83
Benign paroxysmal positional vertigo
(BPPV), 94
Beta-amyloid, 329
Bi-CROS hearing aid, SSHL and, 238
Bilateral
acoustic reflexes, AR and, 20
sensorineural hearing loss, EVA and, 245
tumors, NF2 and, 435
Bilirubin, as neurotoxic agent, 338
Binaural
function test, 83
interaction tests
mass/vascular lesions and, 320
MS and, 331
Biofeedback, tinnitus and, 393
Biotinidase deficiency, 427, 433
Blasts
barotrauma and, 246
noise-induced hearing loss and, 191
tympanic membrane perforation and,
172
Bleomycin, ototoxicity of, 202
Blindness, Norrie disease and, 433
Bloody otorrhea, temporal bone injuries
and, 161
Blunt trauma, 160
Blurred vision, MS AND, 328
BM (Basilar membrane), 20, 23
composition of, 24
spiral ligament and, 28
Bone-conduction testing
aural atresia and, 118
temporal bone injuries and, 162
Bony
capsule, of cochlea, 18
cochlea, function of, 22–23
ear canal, 13
Border cells, 29
BORSD (Branchiootorenal spectrum
syndrome), 428, 431
Bottcher’s cells, 29, 30
Boyle’s law, barotrauma and, 246
BPPV (Benign paroxysmal positional
vertigo), 94
Brain
aneurysms, 316
ectopic areas of, 370
lesions, auditory hallucinations and,
403
MS and, 329
plasticity, 311
Brainstem
acoustic neuroma and, 264
auditory nerve and, 37
intra-axial tumors of, 312
mauditory evoked potential (BAER), 85
Branchial arches, 117
Branchiootorenal spectrum syndrome
(BORSD), 428, 430–431
Bruising patterns, temporal bone injuries
and, 161
Brun’s nystagmus, 271
Burnout, Ménière’s disease and, 209
c
CA (Cochlear aqueducts), 23
Caloric testing, 95
Canal wall up (CWU) mastoidectomy,
cholesteatoma and, 140–141
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CANS (Central auditory nervous system)
disorders of
audiology and neurotoxins, 342–347
auditory lesions, 317–325
degenerative disorders, 325–338
described, 309–311
head trauma, 347–358
learning difficulties and, 368–372
mass lesions, 311–314
neurotoxicity, 338–342
surgical interventions, 364–368
temporal lobe epilepsy, 358–364
traumatic brain injury, 347–358
vascular disorders, 314–317
malformations, 439–443
CAPD (Central auditory processing
disorder)
evaluation of, 80
learning difficulties and, 368–372
Carbamazepine
epilepsy, 360
vascular loops and, 298
Carbon monoxide, noise-induced hearing
loss, 192
Cardiovascular
disease, noise-induced hearing loss,
192
system, CHARGE syndrome and, 435
Carhart notch, 155
Carotid body tumors, 147
Cataracts
Stickler syndrome and, 432
Usher syndrome and, 429
Caucasians
cholesteatoma and, 137
glomus tumors and, 147
Ménière’s disease and, 210
otosclerosis and, 154
Caudal, defined, 12
CBT (Cognitive behavioral therapy),
394–395
CC (Corpus callosum)
agenesis of corpus callosum, 442–443
function of, 53–54
structure of, 52–53
Centers for Disease Control and
Prevention, 250
CMV and, 248
Central auditory
behavioral tests, 322
dysfunction, 423
function of, surgery for, 364–368
syndromic hearing loss, 436
Central auditory nervous system (CANS)
disorders of
audiology and neurotoxins, 342–347
auditory lesions, 317–325
degenerative disorders, 325–338
described, 309–311
head trauma, 347–358
learning difficulties and, 368–372
mass lesions, 311–314
neurotoxicity, 338–342
surgical interventions, 364–368
temporal lobe epilepsy, 358–364
traumatic brain injury, 347–358
vascular disorders, 314–317
malformations, 439–443
agenesis of corpus callosum, 442–443
heterotopias, 440–441
megalencephaly, 439–440
microcephaly, 439–440
polymicrogyria, 441–442
Central auditory processing
assessment of, 80
evaluation form, 82
tests of, 81
processing disorder (CAPD)
evaluation of, 80
learning difficulties and, 368–372
Central auditory system, 41–54
auditory
cortex, 48–52
subcortex, 48–52
cochlear nucleus, 42–44
corpus callosum, 52–54
inferior colliculus, 47
lateral lemniscus, 46–47
medial geniculate body, 47–48
superior olivary complex, 44–46
vascular supply to, 56
Central vestibular abnormalities, eye
tracking tests and, 271
Cerebellopontine angle (CPA), 38, 263
acoustic neuroma and, 265, 267, 272–274,
296–298, 303
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auditory nerves and, 38
vascular loops and, 296
Cerebellum, vestibular nuclei and, 59
Cerebrospinal fluid (CSF)
acoustic neuroma surgery and, surgery
for, 274
AD and, 329
leak
stapedotomy and, 157
temporal bone injuries and, 161, 163,
164
Cerebrum, AD and, 329
CF (Characteristic frequency), 40
Characteristic frequency (CF), 40
Charcot-Marie-Tooth disease, 435
CHARGE syndrome, 428, 434–435
CHARGE VATER syndrome, 117
Chemotherapy, malignant tumors and, 314
Children, genetic evaluation of, 445
Cholesteatoma, 75, 134–146
acquired, 137
audiology and, 139
congenital, 137
described, 134, 137
etiology/pathology of, 137–138
of external canal, 117
incidence/prevalence of, 137
lesion site, 138–139
medical, management of, 139–140
symptoms, 137
Chorda tympani injury, stapedotomy and,
157
Chordomas, CPA tumors and, 274
Chromosomes, 413–414, 419–420
Chronic otitis media, 134
Chronic pain, tinnitus and, 393
Cisplatinum
cochlea and, 205
ototoxicity of, 202, 203
Cisterns, 28
Classroom, fM system, 119
Claudius cells, 29, 30
Claustrophobia, MRI and, 102
Cleft lip, Waardenburg syndrome and, 430
Cleft palate
aural atresia and, 117
Pierre Robin syndrome and, 118
Stickler syndrome and, 432
Waardenburg syndrome and, 430
Clinical Practice Guidelines on otitis
media, American Academy of
Otolaryngology-Head and Neck
Surgery (AAO-HNS), 130
CMV (Cytomegalovirus), 248–250
CN (Cochlear nucleus), nucleus of, 42–44
Cochlea
AR and, 20
ARHL and, 225
auditory nerve and
function of, 38–41
structure of, 37–38
blood supply to, 55
bony capsule of, 18
brainstem and, 37
CHARGE syndrome and, 434
described, 20, 103
electrolyte imbalance and, 211
electrophysiologic tests of, 91
electrophysiology of, 35–37
fluid wave in, 193
function of, 31
mechanics of, 31
medial wall of, 18
membranous, function of, 30–37
noise-induced hearing loss and, 192
structure of
bony, 22–23
membranous, 23–30
Cochlear
aplasias, 438
aqueducts (CA), 23
malformations, 438–439
hearing losses, reflexes and, 76
hypoplasias, 438
implants, ARHL and, 226
malformations, 438
Cochlear nucleus (CN), 42–44
AR and, 20
Cochleotoxic, 201
Cochleovestibular artery (CVA), 55
Coding, AN and, 40–41
Cogan’s syndrome, 219
Cognitive
behavioral therapy (CBT), tinnitus and,
393, 394–395
deficits, heavy metals and, 338
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