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452 Disorders of the Auditory System
https://t.me/medicina_free
drome. American Journal of Medical Genetics,
57(4), 540–547.
Robin, N. H. (2008). Medical genetics: Its applica-
tion to speech, hearing, and craniofacial disorders. San Diego, CA: Plural Publishing.
Robin, N. H., Falk, M. J., & Haldeman-Englert,
C. R. (Updated 2011, June 7). FGFR-related
craniosynostosis syndromes. In M. P. Adam,
H. H. Ardinger, R. A. Pagon, S. E. Wallace,
L. J. H. Bean, K. Stephens, & A. Amemiya
(Eds.), GeneReviews
®
[Internet]. Seattle:
University of Washington. Retrieved from
https://www.ncbi.nlm.nih.gov/books/
NBK1455/
Robin, N. H., Moran, R. T., & Ala-Kokko, L.
(Updated 2017, March 16). Stickler syndrome. In M. P. Adam, H. H. Ardinger, R.
A. Pagon, S. E. Wallace, L. J. H. Bean, K. Stephens, & A. Amemiya (Eds.), GeneReviews
®
[Internet]. Seattle: University of Washington. Retrieved from https://www.ncbi.nlm
.nih.gov/books/NBK1302/
Saihan, Z., Webster, A. R., Luxon, L., & Bitner-
Glindzicz, M. (2009). Update on Usher syndrome. Current Opinion in Neurology, 22(1),
19–27.
Sennaroglu, L., & Saatci, I. (2002). A new clas-
sification for cochleovestibular malformations. Laryngoscope, 112(12), 2230–2241.
Shearer, A. E., & Hansen, M. R. (2019). Audi-
tory synaptopathy, auditory neuropathy,
and cochlear implantation. Laryngoscope
Investigative Otolaryngology, 4(4), 429–440.
Shearer, A. E., Hildebrand, M. S., & Smith, R.
J. H. (Updated 2017, July 27). Hereditary
hearing loss and deafness overview. In
M. P. Adam, H. H. Ardinger, R. A. Pagon,
S. E. Wallace, L. J. H. Bean, K. Stephens, &
A. Amemiya (Eds.), GeneReviews
®
[Internet]. Seattle: University of Washington.
Retrieved from https://www.ncbi.nlm.nih
.gov/books/NBK1434/
Smith, R. J. H. (Updated 2017, October 19). Pen-
dred syndrome/Nonsyndromic enlarged
vestibular aqueduct. In M. P. Adam, H. H.
Ardinger, R. A. Pagon, S. E. Wallace, L. J.
H. Bean, K. Stephens, & A. Amemiya (Eds),
GeneReviews
®
[Internet]. Seattle: University
of Washington. Retrieved from https://
www.ncbi.nlm.nih.gov/books/NBK1467/
Smith, R. J. H. (Updated 2018, September 6).
Branchiootorenal spectrum disorder. In
M. P. Adam, H. H. Ardinger, R. A. Pagon,
S. E. Wallace, L. J. H. Bean, K. Stephens, &
A. Amemiya (Eds.), GeneReviews
®
[Internet]. Seattle: University of Washington.
Retrieved from https://www.ncbi.nlm.nih
.gov/books/NBK1380/
Smith, R. J. H., & Jones, M.-K. N. (Updated
2016, August 18). Nonsyndromic hearing
loss and deafness, DFNB1. In M. P. Adam, H.
H. Ardinger, R. A. Pagon, S. E. Wallace, L. J.
H. Bean, K. Stephens, & A. Amemiya (Eds.),
GeneReviews
®
[Internet]. Seattle: University
of Washington. Retrieved from https://
www.ncbi.nlm.nih.gov/books/NBK1272/
Sotiriadis, A., & Makrydimas, G. (2012). Neu-
rodevelopment after prenatal diagnosis of
isolated agenesis of the corpus callosum:
An integrative review. American Journal of
Obstetrics and Gynecology, 206(4), 337.e1–5.
https://doi.org/10.1016/j.ajog.2011.12.024
Squier, W., & Jansen, A. (2014). Polymicrogyria:
Pathology, fetal origins and mechanisms.
Acta Neuropathologica Communications, 2, 80.
https://doi.org/10.1186/s40478-014-0080-3
Stinckens, C., Standaert, L., Casselman, J. W.,
Huygen, P. L., Kumar, S., Van de Wallen, J.,
& Cremers, C. W. (2001). The presence of
widened vestibular aqueduct and progressive sensorineural hearing loss in the branchio-oto-renal syndrome. A family study.
International Journal of Pediatric Otorhinolaryngology, 59(3), 163–172.
Sutterd, C. A., & Leventer, R. J. (2014). Polymi-
crogyria: A common and heterogene
ous malformation of cortical development. American
Journal of Medical Genetics, 166C(2), 227–239.
Tatton-Brown, K., Loveday, C., Yost, S., Clarke,
M., Ramsay, E., Zachariou, A., . . . Rahman,
N. (2017). Mutations in epigenetic regulation genes are a major cause of overgrowth
with intellectual disability. American Journal
of Human Genetics, 100(5), 725–736.
Thelin, J. W. (2011). Hearing. In T. S. Harts-
horne, M. A. Hefner, S. L. H. Davenport, &
9. Hereditary and Congenital Hearing Loss 453
https://t.me/medicina_free
J. W. Thelin (Eds.), CHARGE syndrome (pp.
25–42). San Diego, CA: Plural Publishing.
Toriello, H. V., & Smith, S. D. (Eds.). (2013). Hered-
itary hearing loss and its syndromes (3rd
ed.).
New York, NY: Oxford University Press.
Trainor, P. A., Dixon, J., & Dixon, M. J. (2009).
Treacher Collins syndrome: Etiology, pathogenesis and prevention. European Journal of
Human Genetics, 17(3), 275–283.
Tranebjærg, L., Samson, R. A., & Green, G.
E. (Updated 2017, August 17). Jervell and
Lange-Nielsen syndrome. In M. P. Adam, H.
H. Ardinger, R. A. Pagon, S. E. Wallace, L. J.
H. Bean, K. Stephens, & A. Amemiya (Eds.),
GeneReviews
®
[Internet]. Seattle: University
of Washington. Retrieved from https://
www.ncbi.nlm.nih.gov/books/NBK1405/
U.S. National Library of Medicine. (2019a).
Genetics home reference. Biotinidase deficiency.
Retrieved from https://ghr.nlm.nih.gov/
condition/biotinidase-deficiency
U.S. National Library of Medicine. (2019b).
Genetics home reference. Branchiootorenal/branchiootic syndrome. Retrieved from https://
ghr.nlm.nih.gov/condition/branchiooto
renal-branchiootic-syndrome
U.S. National Library of Medicine. (2019c).
Genetics home reference. Down syndrome. Retrieved from https://ghr.nlm.nih.gov/con
dition/down-syndrome
U.S. National Library of Medicine. (2019d).
Genetics home reference. Norrie disease. Retrieved from https://ghr.nlm.nih.gov/con
dition/norrie-disease
U.S. National Library of Medicine. (2019e).
Genetics home reference. Pendred syndrome.
Retrieved from https://ghr.nlm.nih.gov/
condition/pendred-syndrome
U.S. National Library of Medicine. (2019f).
Genetics home reference. Periventricular heterotopia. Retrieved from https://ghr.nlm.nih
.gov/condition/periventricular-heterotopia
U.S. National Library of Medicine. (2019g).
Genetics home reference. Polymicrogyri. Retrieved from https://ghr.nlm.nih.gov/con
dition/polymicrogyria
U.S. National Library of Medicine. (2019h).
Genetics home reference. Refsum disease. Re-
trieved from https://ghr.nlm.nih.gov/con
dition/refsum-disease
U.S. National Library of Medicine. (2019i).
Genetics home reference. 22q11.2 deletion syndrome. Retrieved from https://ghr.nlm.nih
.gov/condition/22q112-deletion-syndrome
U.S. National Library of Medicine. (2019j).
Genetics home reference. Usher syndrome.
Retrieved from https://ghr.nlm.nih.gov/
condition/usher-syndrome
U.S. National Library of Medicine. (2019k).
Genetics home reference. Waardenburg syndrome. Retrieved from https://ghr.nlm.nih
.gov/condition/waardenburg-syndrome
U.S. National Library of Medicine. (2019l).
Genetics home reference. Your guide to understanding genetic conditions. Retrieved from
https://ghr.nlm.nih.gov/
Van Camp, G., & Smith, R. J. H. (Updated 2019,
August 22). Hereditary hearing loss homepage.
Retrieved from https://hereditaryhearing
loss.org
Varga, R., Kelley, P. M., Keats, B. J., Starr, A.,
Leal, S. M., Cohn, E., & Kimberling, W. J.
(2003). Non-syndromic recessive auditory
neuropathy is the result of mutations in the
otoferlin (OTOF) gene. Journal of Medical
Genetics, 40(1), 45–50.
Wahid, F., Shehzad, A., Khan, T., & Kim, Y. Y.
(2010). MicroRNAs: Synthesis, mechanism,
function, and recent clinical trials. Bio-
chimica et Biophysica Acta, 1803(11), 1231–
1243.
Wallace, S. E., & Bean L. J. H. (Updated 2018,
February 12). Educational materials —
Genetic testing: Current approaches. In
M. P. Adam, H. H. Ardinger, R. A. Pagon,
S. E. Wallace, L. J. H. Bean, K. Stephens, &
A. Amemiya (Eds.), GeneReviews
®
[Internet]. Seattle: University of Washington.
Retrieved from https://www.ncbi.nlm.nih
.gov/books/NBK279899/
Wanders, R. J. A., Waterham, H. R., & Leroy, B.
P. (Updated 2015, June 11). Refsum disease.
In M. P. Adam, H. H. Ardinger, R. A. Pagon,
S. E. Wallace, L. J. H. Bean, K. Stephens, &
A. Amemiya (Eds.), GeneReviews
®
[Internet]. Seattle: University of Washington.
454 Disorders of the Auditory System
https://t.me/medicina_free
Retrieved from https://www.ncbi.nlm.nih
.gov/books/NBK1353/
Welch, K. O. (2006). Fundamentals of human
genetics. Seminars in Hearing, 27(3), 127–135.
Wingard, J. C., & Zhao, H. (2015). Cellular
and deafness mechanisms underlying connexin mutation-induced hearing loss —
A common hereditary deafness. Frontiers
in Cellular Neuroscience, 9, 202. https://doi
.org/10.3389/fncel.2015.00202
Wolf, B. (Updated 2016, June 9). Biotinidase
deficiency. In M. P. Adam, H. H. Ardinger,
R. A. Pagon, S. E. Wallace, L. J. H. Bean,
K. Stephens, & A. Amemiya (Eds.), Gene
Reviews
®
[Internet]. Seattle: University of
Washington. Retrieved from https://www
.ncbi.nlm.nih.gov/books/NBK1322/
Wolf, B., Spencer, R., & Gleason, A. (2002). Hear-
ing loss is a common feature in symptomatic
children with profound biotinidase deficiency. Journal of Pediatrics, 140(2), 242–246.
Zhu, Y., Chen, J., Liang, C., Zong, L., Chen, J.,
Jones, R. O., & Zhao, H. B. (2015). Connexin
26 (GJB2) deficiency reduces active cochlear
amplification leading to late-onset hearing
loss. Neuroscience, 284, 719–729.
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Glossary
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Acceptable Masking: The level at which
the patient can tolerate a masking sound
as a substitute for his or her tinnitus.
Acetylcholine: A common, usually excit-
atory neurotransmitter.
Acoustic Stria: Ascending output fiber
tracts from the cochlear nuclei, including the anterior, intermediate, and dorsal
striae.
Agenesis of the Corpus Callosum: The
failure of the corpus callosum to develop
during embryonic growth and development due to the absence of primordial
tissue.
Allele: A variant form of a DNA sequence.
For each gene, there are two alleles located
at a specific locus on a chromosome with
one allele inherited from each parent. The
combination of the alleles determines the
characteristics of the trait or condition
expressed in the offspring.
Aminoglycosides:
used to slow or destroy the growth of
bacteria.
Analgesics: A class of medicines used to
relieve pain.
Aneurysm: The ballooning of an artery,
usually secondary to weakness or damage along the vessel’s wall.
Ankylosis: Rigidity or stiffness at a joint
(can occur in the joints connecting the
bones of the ossicular chain resulting in
conductive hearing loss).
A class of medications
Anoxia: A severe decrease in the oxygen
levels reaching the tissues of the body.
Anterior: At or toward the front of the body
or the head.
Anticonvulsants: A class of medications
used to treat epileptic seizures that act to
suppress the rapid firing of neurons.
Antimalarials: A class of medications used
primarily to prevent or cure malaria.
Antioxidants: Molecules capable of inhibit-
ing the oxidation of other molecules, which
serves to protect the health of the cell.
Anxiolytics: A class of drugs used to treat
anxiety and its related disorders.
Aplasia: Defective development or congen-
ital absence of an organ or tissue.
Apoptosis: Programmed cell death which
occurs during growth or development.
Ataxia:
Atherosclerosis: A disease of the arteries
Atresia: A condition in which the external
Auricle: The outer-most structure of the ear
Motor disorder characterized by
the inability to coordinate muscle activity,
causing jerkiness, lack of coordination,
and inefficiency of voluntary movement.
characterized by the accumulation of
plaques of fatty material on the inner walls,
resulting in narrow or occluded arteries.
auditory canal fails to develop normally
resulting in an abnormally closed or
absent canal.
that is composed of elastic cartilage; also
known as the pinna.
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Autoinsufflation: The process by which
an individual equalizes the pressure
between the outer ear and middle ear
space by raising the pressure in the nose
to forcibly open the Eustachian tube.
This is accomplished by pinching one’s
nostrils while forcibly exhaling with the
mouth closed.
Autophony: Excessive loudness of one’s
own breathing and speech due to a patulous Eustachian tube.
Autosomal Dominant: A pattern of inheri-
tance whereby an individual requires
only one abnormal gene allele from a parent to inherit the trait or condition.
Autosomal Recessive: A pattern of inheri-
tance whereby two copies (alleles) of an
abnormal gene are needed in order for
the trait or condition to develop.
Autosome: A nonsex chromosome with an
equal number in males and females (22
pairs).
Avulsion: The tearing away of an ana-
tomic structure as a result of trauma or
surgery.
Axial: Situated around a central location or
plane.
Barotrauma: Injury to a body or organ that
is the result of rapid changes in barometric pressure; in the auditory system, damage to the middle ear and/or the cochlea
can occur.
Battle’s Sign:
also known as raccoon sign.
Bilateral Contralateral Routing of Sig-
nal (BiCROS) Hearing Aid: A hearing
aid arrangement developed for patients
with an asymmetric hearing loss with
the hearing in one ear being substantially
poorer than in the other ear; microphones
are provided for both ears, but the signal
from each microphone is directed to the
better ear.
Brachycephaly: Improper fusion of the
(cranial) coronal sutures resulting in a
“flat head” appearance.
Branchial Arches: External tissue bands
located under the early brain that lead to
the formation of the head and neck struc-
Bruising around the eyes;
tures, including the outer and middle
ears during embryonic development.
Brown’s Sign: The observation of a pul-
sation of a glomus tumor located in the
middle ear when positive pressure is
applied to the external auditory canal
during otoscopy.
Burst and Taper: A term used to describe
the process of treating patients with corticosteroids. Initially, a large dose is given
to control the inflammatory process, then
the dosage is reduced to minimize the
adverse effects of steroids.
Carhart Notch: An unusual audiometric
configuration with a notch observed in
the bone-conduction curve at 2000 Hz;
often noted in patients with otosclerosis.
Caudal: Below another structure or toward
the feet.
Central Gain: A compensatory increase in
central auditory activity within the brain
in response to the loss of sensory input
from the cochlea, which is hypothesized
to be a central nervous system mechanism that results in hyperacusis and/or
tinnitus.
Cerebellopontine Angle: An anatomic
recess in the auditory system where the
eighth nerve projects to the brainstem.
Cerebral Vasospasm: The contraction of a
blood vessel in the brain, narrowing its
lumen.
Charcot-Marie-Tooth Disease:
eral degenerative nerve disorder that
often affects the auditory nerve.
Chelation Therapy: Administration of
therapeutic agents, usually to remove
heavy metals from the blood.
Cholesteatoma (External Auditory Canal):
A collection of epithelial skin cells in the
external auditory canal which can result in
inflammation, pain, and/or aural discharge.
Cholesteatoma (Middle Ear): Collection
of keratinizing squamous epithelial cells
resulting in a benign, yet potentially destructive, mass within the middle ear space.
Chromosome: A threadlike structure that is
made up of DNA located in the nucleus
of a cell.
A periph-
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Cisterns (Outer Hair Cell):
tures along the walls of the outer hair
cells that aid in the expansion and contraction of these cells.
Cleft Palate: A craniofacial anomaly in
which the two sides of the developing palate fail to fuse together during
development.
Click Stimulus: Traditional stimulus (a
short broadband noise) frequently used
to elicit an evoked auditory response.
Comminuted Fracture:
bone is broken, splintered, or crushed
into a number of pieces. This is different
than a compound fracture in which the
bone protrudes through the skin.
Commissure: A bundle of nerve fibers that
passes from a structure on one side of the
brain to a similar structure on the opposite side.
Conductive Hearing Loss: Type of hearing
loss resulting in impairment of sound
transmission from the outer and/or middle ear to the inner ear.
Consanguinity: Parents related by descent.
Contralateral: Relating to the opposite side.
Contralateral Routing of Signal (CROS)
Hearing Aid: A hearing aid arrangement
originally developed for patients with
unilateral hearing loss where a microphone is located on or in the poorer ear
and the signal is routed to the better ear.
Coronal: An anatomic plane that vertically
divides the head or the body into front
and back segments.
Crisis of Tumarkin: A sudden fall that occurs
without provocation or warning and with
no associated loss of consciousness.
Cytomegalovirus: A common viral infec-
tion that affects many individuals and
is often asymptomatic in healthy adults;
however, it is a cause for concern particularly in pregnant women as the viral
infection can be passed to the fetus and
result in congenital hearing loss.
Deep: Away from the surface of the body
or the head.
Demyelination: The loss of myelin along
the axon of a neuron.
Tubular struc-
A fracture in which
Deoxyribonucleic Acid (DNA):
acid that contains the genetic blueprint
for all living organisms.
Depolarization: Process by which the
electrical potential within a neural cell
changes from a large negative value to a
less negative value through an increase in
sodium flow into the cell.
Digenic Inheritance: An inheritance pat-
tern involving or controlled by the interaction of two nonallelic genes.
Diplopia:
Disequilibrium: Loss of balance; inability
to maintain an upright posture.
Distal: Away from the trunk or point of
origin.
Diuretics: A class of medications used to
increase the excretion of excess fluid from
the body.
Dorsal: At or toward the back of the body
or the head.
Dysarthria: Slurred or slowed speech.
Ectopic (Areas of the Brain): Brain tis-
sue occurring in an abnormal position
and lacking connection with other parts
of the brain, presumably the result of
a cell migrational problem; the term
is often used interchangeably with
heterotropia.
Effective Masking: Refers to the ability to
successfully cover the perception of tinnitus so that the patient can no longer hear
it; also refers to the ability to effectively
mask a sound at a given level during
audiometric testing if the possibility of
crossover of the signal from the test ear
to the nontest ear by bone-conduction
mechanisms is possible.
Effusion: Fluid collection within the mid-
dle ear.
Electronystagmography (ENG): A test of
vestibular function in which nystagmus
is recorded electrophysiologically during stimulation of the vestibular system;
includes a battery of subtests divided
into three groups: ocular-motor, positional, and caloric.
Embolism: A type of clot that can travel from
the heart to the brain where it arrests the
Double vision.
Nucleic
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blood supply to the specific brain structure(s)
supplied by the artery affected.
Endocochlear Potential: A direct current
(DC) potential in the scala media of the
inner ear that is essential for normal hair
cell function.
Ependymoma: A central nervous system
tumor originating from ependyma cells
that line the ventricles in the brain and/
or the center of the spinal cord through
which cerebrospinal fluid flows.
Epileptic Focus: The locus of seizure activ-
ity in the brain, determined by EEG
methods.
Epitympanum: Portion of the middle ear
space above the tympanic membrane that
contains the head of the malleus and the
short process of the incus.
Eustachian Tube: The canal or channel
that connects the middle ear space to the
nasopharynx.
Eustachian Tube Dysfunction: A condition
that occurs when the Eustachian tube
does not open normally to equalize the
air pressure between the outer ear and
the middle ear space; it also can refer to
a Eustachian tube that is patulous (i.e.,
always open).
Exostosis: A bony growth in the medial
(bony) portion of the ear canal.
Extra-axial: External to but typically im-
pinging on the brainstem, usually referring to the locus of a lesion.
Facial Nerve:
primary function is to control the muscles
of the face. A branch of this nerve courses
through the middle ear and innervates
the stapedius muscle.
Fasciculation: Small, involuntary muscle
contractions in a particular area.
Fenestration: A surgical procedure that
involves creating an opening in the bony
labyrinth between the inner ear and
the tympanum to replace bone openings
(oval or round windows) that are not
functional.
Fissula Ante Fenestrum: Area just anterior
to the stapes footplate/oval window,
Seventh cranial nerve whose
which is a common location for otosclerosis to occur.
Fistula: An abnormal hole within an ana-
tomic structure such as the cochlea.
Fistula of the Labyrinth: A hole in the co-
chlear labyrinth, often involving the oval
or round window.
FM System: An assistive listening device
often used with school-aged children in
which the sound is transmitted directly
from the sound source to a receiver via
frequency-modulated (FM) waves.
Free Radical: A molecule that has a single,
unpaired electron that can be damaged
by oxidative stress.
Friedrich’s Ataxia: An inherited disorder
that results in progressive degeneration
of the nervous system, in particular the
sensory nerves.
Frontal: An anatomic plane that vertically
divides the head or the body into front
and back segments.
Fundus: Part of a structure that is located
opposite of its opening; for example, the
fundus of the internal auditory meatus
would be the end opposite of its opening
into the posterior fossa.
Gadolinium: Contrast agent often used
during MRI to change the magnetic property of the tissue, resulting in an enhancement of the anatomic structures for visual
inspection.
Gamma Knife Surgery:
procedure often utilized in place of traditional surgery; also referred to as stereotactic radiologic surgery.
Genotype: The genetic constitution of an
individual.
Glasgow Coma Scale: A measure used
to determine mild, moderate, or severe
degrees of head or brain injury.
Globus Pallidus: One of the three nuclei
making up the basal ganglion.
Glomus Tumor: Term often used inter-
changeably with paraganglioma tumor to
refer to a class of benign, slow-growing,
highly vascularized tumors at the base of
the skull.
A noninvasive
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Glossopharyngeal Nerve:
nerve whose primary function is to
receive sensory input from the tonsils,
pharynx, middle ear, and tongue.
Glutamate: A key neurotransmitter in the
cochlea as well as other regions of the
ascending auditory system.
Head Shadow Effect: Decrease in signal
intensity presented to one side of the
head when it is measured at the opposite
side of the head.
Hematoma:
secondary to bleeding, such as subdural
hematoma.
Hemiparesis: Weakness of one side of the
body.
Hemorrhagic Stroke: A medical condition
that occurs from damage to blood vessels
and results in loss of blood flow from the
associated brain tissue, as well as a mass
effect in the brain.
Hemotympanum: The presence of blood
behind the eardrum.
Heterotopias: Abnormal migration pat-
terns of neurons in the brain during fetal
development that results in morphologic
abnormalities in the cortex, which can
result in a central auditory processing
disorder if the auditory regions of the
brain are affected.
Heterozygote: A person possessing two dif-
ferent forms (alleles) of a particular gene.
Hidden Hearing Loss: A hearing loss
that is not detectable on the traditional
audiogram.
Histopathology: The assessment of
changes in tissue or cells related to a disease process.
Homozygote: A person possessing two
similar alleles of a particular gene.
Hyperacusis: Consistently exaggerated or
inappropriate responses to sounds that
are neither threatening nor uncomfortably loud to a typical person.
Hyperbilirubinemia: A condition that is a
result of high bilirubin levels in the blood
that can affect the health of nuclei within
the central nervous system.
Localized collection of blood,
Ninth cranial
Hyperpolarization:
larization; the process by which the electrical potential of a cell’s membrane becomes more negative. This inhibits the
action potential and increases the amount
of stimulus required to elicit a response.
Hypnagogic: A transitional state between
wakefulness and sleep that occurs immediately before falling asleep.
Hypnopompic: A transitional state between
sleep and wakefulness that occurs immediately before waking up.
Hypoplasia:
complete development of a tissue or organ.
Hypopneumatic Atresia: Atresia in which
there is total stenosis along with a poorly
pneumatized mastoid and a facial nerve
with an aberrant course.
Hypotympanum: The portion of the mid-
dle ear space that is the location of the
bony covering of the jugular bulb.
Hypoxia: A decrease in the oxygen levels
reaching the tissues of the body.
Immittance: A term representing energy
flow through the middle ear.
Incidence: The frequency of occurrence of a
condition or a disease or the rate at which
it occurs.
Inferior: Below another structure or toward
the feet.
Interaural Intensity Differences (IID): The
difference between the intensity information arriving at the right and left ears.
Interaural Time Differences (ITD): The
difference between the time information
arriving at the right and left ears.
Intra-axial: Within the brainstem, usually
referring to the locus of a lesion.
Iodinated Contrast Agents: These com-
pounds work by altering the magnetic
properties of nearby hydrogen nuclei,
thereby enhancing the visual resolution
of the image.
Ipsilateral: Relating to the same side.
Ischemia: Lack of blood supply that can
lead to damaged tissue.
Ischemic Stroke: A medical condition that
occurs when cerebral arteries are occluded
The underdevelopment or in-
The opposite of depo-
460 Disorders of the Auditory System
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as a result of vessel narrowing due to atherosclerosis or the lodging of a blood clot
in a vessel.
Kernicterus: A medical condition that
occurs when high levels of bilirubin in
the blood are left untreated; can result in
irreversible brain damage and both neurosensory and neuromotor disability.
Kinocilium: The tallest cilium on the sen-
sory hair cells located in the cochlear and
vestibular end organs.
Landau-Kleffner Syndrome: A rare genetic
disorder seen in children that results in a
seizure disorder with a focus at or near
the auditory cortex; typical manifestations of the disorder include language
delay or regression, as well as auditory
symptoms.
Lateral: At or toward the side of the body
or the head.
Levator Veli Palatini: A muscle that serves
to elevate the soft palate.
Longitudinal Fracture: The most common
type of temporal bone fracture that occurs
parallel to the long axis of the petrous
temporal bone as a result of blunt force
trauma to the skull.
Loudness Discomfort Level: Level at which
sound is perceived to be uncomfortable; a
level of loudness that a patient would not
want to listen to for an extended period
of time.
Magnetic Resonance Angiogram (MRA):
An MRI procedure that serves to facilitate the observations of blood vessels
during radiologic imaging.
Magnetic Resonance Imaging (MRI):
radiologic imaging procedure used to
visualize detailed internal structures.
Mastoid Antrum: Cavity in the middle
ear space that communicates with the
mastoid air cells and the epitympanic
recess.
Mastoidectomy: Surgical procedure that in-
volves removal of infected mastoid cells.
Medial: At or toward the midline of the
body or the head.
Megalencephaly: An abnormally large brain.
A
Mendelian Inheritance: A set of laws re-
lated to the transmission of hereditary
characteristics from parent to offspring.
Meningioma: A relatively common, usu-
ally benign brain tumor arising from the
meninges.
Mesotympanum: A portion of the middle
ear space that contains the handle of the
malleus, the long process of the incus, the
stapes, the oval and round windows, and
a portion of the facial nerve.
Microcephaly: Smaller than normal head
circumference.
Microtia: Malformation or absence of the
pinna.
Misophonia: Dislike of a particular sound.
Mitochondria: Energy-producing organ-
elles, usually found in large numbers
within a cell.
Mixed Hearing Loss: A type of hearing loss
resulting from both conductive and sensorineural involvement.
Monogenic Inheritance: An inheritance
pattern involving or controlled by a single gene.
Multiplanar Reconstruction (MPR): A
procedure that permits CT images to be
developed from an original axial plane
in either the coronal, sagittal, or oblique
plane.
Myelin: An insulating covering (com-
posed of a mixture of proteins and phospholipids) that surrounds the axons of
some, but not all, nerves; the presence of
which speeds the transmission of nerve
impulses.
Myringotomy:
which a small incision is made in the
tympanic membrane in order to allow
fluid to drain and/or to be suctioned
from the middle ear.
Nasopharyngoscope: A specialized scope
used to examine the nose and throat.
Neoplasm: An abnormal growth of benign
or malignant tissue.
Neural Crest: Paired strips of cells arising
from the ectoderm that migrate to various
locations during development and even-
Surgical procedure by
Glossary 461
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tually differentiate into many cell types
within the embryo. They are responsible for development of the 12 cranial
nerves.
Neuroepithelium:
of a sensory organ (visual, auditory, etc.).
Neurofibroma: A benign tumor involv-
ing the sheath (covering) of a peripheral
nerve.
Neuropathy: Damage to nerves of the
peripheral nervous system.
Neurotoxin:
function or the anatomy of a central nervous system structure or structures.
Nonsyndromic Hearing Loss: An inher-
ited genetic trait that is not part of a syndrome and involves primarily the auditory system.
Oblique (Images): An angle of view, usu-
ally between vertical and horizontal
planes in imaging or anatomy.
Odd-Ball Paradigm: A manner of present-
ing a signal that varies in relation to other
signals in the paradigm; a common procedure used for P300 (P3) evoked potential assessments.
Ossicles: Three small bones located within
the middle ear space that collectively are
responsible for transmitting sound vibrations from the outer to the inner ear.
Ossiculoplasty: Surgical reconstruction of
the ossicular chain.
Osteoma (Ear Canal): A slow-growing,
benign tumor that develops in the bony
portion of the external auditory canal.
Otalgia: Ear pain.
Otic Capsule: The bony shell that houses
the membranous labyrinth of the inner
ear.
Otitis Media: Inflammation of the middle
ear.
Otomicroscopy: Otoscopy using a binocu-
lar microscope.
Otorrhea: Discharge from the ear.
Otosclerosis: A metabolic bone-remodeling
disease of the temporal bone that primarily affects the otic capsule and the
ossicles.
Highly specialized cells
A substance that alters the
Ototopical:
peutic substance to the ear.
Ototoxicity: The effects of toxic substances
on the structures of the ear, particularly
the hair cells in the cochlea and vestibular
organs.
P50 Response: An evoked potential occur-
ring at approximately 50 msec after the
onset of the stimulus.
Paraganglioma: Term often used inter-
changeably with glomus tumor to refer to
a class of benign, slow-growing, highly
vascularized tumors at the base of the skull.
Patulous Eustachian Tube:
tube that fails to close and remains open.
Perilymph Fistula: A perforation resulting
in abnormal communication between the
middle and inner ear spaces.
Permanent Threshold Shift (PTS): A per-
manent change or loss of hearing sensitivity caused by exposure to excessive
noise levels.
Phenotype: The observable characteristics
or properties of an organism that is determined by the organism’s genotype.
Phonophobia: Fear of sounds.
Pinna: Outer-most structure of the ear that
is composed of elastic cartilage; also
referred to as the auricle.
Plasticity: Alteration of nerve cell pathways
to better conform to immediate environmental influences; often associated with
behavioral change.
Pneumatic Insufflation: Process by which
mobility of the tympanic membrane is
evaluated by delivering air manually to
the external auditory canal.
Politzer Test: Inspection of the tympanic
membrane while air is injected into the
nasopharynx as the patient swallows.
Polymicrotia: Malformation of cortical
development that results in overfolding
and the overproduction of small gyri in
the brain’s surface.
Polypoid: Resembling a polyp.
Pontomedullary Junction: An anatomic
de-marcation between the medulla and
pons.
Surface application of a thera-
A Eustachian
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