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Anatomy Physiology and

Disorders of the Hearing


Perry C. Hanavan, Au.D.
Audiologist

Major Divisions of the Ear


Peripheral Mechanism

VIII
Outer Middle Inner
Cranial
Ear
Ear
Ear
Nerve

Central Mechanism

Brain

Question
What is the purpose of the pinna?
A. Cosmetics
B. Sound collector
C. Same side localization
D. A and B
E. A, B and C

Outer Ear
Virtual Tour of the Ear
Pinna

Pinna

Preauricular Tags
Preauricular Pits
EAM

External
Auditory
Meatus

Cerumen
Q-tips

Function

Microtia

EAM resonance

Anotia
Atresia

Pinna

Question
Another name for pinna?
A. External auditory meatus
B. External auditory canal
C. Ear lobe
D. Auricle
E. None of the above

Function of Outer Ear

Collect sound
Localization
Resonator
Protection
Sensitive
(earlobe)
Other?

Pinna
The visible portion that is
commonly referred to as "the
ear"
Helps localize sound sources
Directs sound into the ear
Each individual's pinna
creates a distinctive imprint
on the acoustic wave
traveling into the auditory
canal

External Auditory Meatus


Extends from the pinna to the tympanic membrane
About 26 millimeters (mm) in length and 7 mm in diameter in
adult ear.
Size and shape vary among individuals.

Protects the eardrum


Resonator
Provides about 10 decibels (dB) of gain to the eardrum at around
3,300 Hertz (Hz).

The net effect of the head, pinna, and ear canal is that
sounds in the 2,000 to 4,000 Hz region are amplified by 10
to 15 dB.
Sensitivity to sounds greatest in this frequency region
Noises in this range are the most hazardous to hearing

Outer Ear Resonance


Influence of pinna (p)
Influence of ear canal
(m)
Combine influence (t)
At 3000 Hz, the final
amplification (t) is 20
dB

Question
Cerumen should be routinely removed from
the ear canal?
A. True
B. False

Cerumen
The purpose of wax:
Repel water
Trap dust, sand particles, microorganisms, and other debris
Moisturize epithelium in ear
canal
Odor discourages insects
Antibiotic, antiviral, antifungal
properties
Cleanse ear canal

Embryological Development
External Ear Development

Disorders of the Outer Ear


Accessory auricle

Additional pinna or auricular tissue

Anotia

Congenital absence of pinna

Cleft pinna

Congenital fissure of the pinna

Coloboma lobuli

Congenital fissure of the earlobe

Macrotia

Congenital excessive enlargement of the auricle

Microtia

Congenital small pinna

Melotia

Congenital displacement of the pinna

Low-set-ears

Congenitally displaced pinna

Polyotia

Additional pinna on one or both sides

Preauricular tags

Small appendage anterior to pinna

Scroll ear

Rim (helix) of pinna is rolled forward and inward

Outer Ear Hearing Disorders


Outer ear
CHARGE
Down Syndrome
Ears small and low set

Fetal Alcohol
Syndrome
Deformed ears

DiGeorge syndrome
Low set ears

Grow Ear?

External Ear Care


Hazardous to health:
Ear candling
Swabs
Foreign objects

Time of Onset
Congenital

Present at birth

Acquired

Occurring after birth

Adventitious

Occurring after birth

Acute

Sudden onset or short duration

Chronic

Long duration

Sudden

Rapid onset

Gradual

Occurring slowly

Temporary

Limited duration

Permanent

Irreversible

Progressive

Increasing in degree over time

Fluctuating

Increasing and decreasing in degree of loss

Middle Ear
The function of the middle ear is to?
A. Cause middle ear infections in young
children
B.Amplify sounds
C.Interpret sounds
D.Analyze sounds
E.None of the above

Middle Ear
The Eustachian tube is a part of the middle
ear?
A.No, it is a part of the inner ear
B.No, it isnt part of the hearing mechanism
C.Yes
D.I dont know

Middle Ear
Which is not true?
A.There are two middle ear muscles
B.There are three ossicles
C.There are three layers of tympanic
membrane tissue
D.The acoustic reflex occurs from
soft sounds
E.The stapes is the smallest bone in
the human body

Middle Ear
Virtual Tour of the Ear
Middle Ear Cavity

Tympanic Cavity
Tympanic Membrane
Ossicles
Middle Ear Muscles
Eustachian Tube
Mastoid

Ossicles
Middle Ear Muscles
Mastoid
Eustachian Tube
Function

Amplifier

Cholesteatoma
Temporal bone fractures
Otitis Media
PE tubes

Otosclerosis

Function of Middle Ear


Conduction
Conduct sound from the outer ear to the inner ear

Protection
Creates a barrier that protects the middle and inner areas from
foreign objects
Middle ear muscles may provide protection from loud sounds

Transducer
Converts acoustic energy to mechanical energy
Converts mechanical energy to hydraulic energy

Amplifier
Transformer action of the middle ear
only about 1/1000 of the acoustic energy in air would be
transmitted to the inner-ear fluids (about 30 dB hearing loss)

The middle ear:


A. Converts acoustic energy to hydraulic
B. Converts hydraulic energy to mechanical
C. Converts acoustic energy to mechanical
D. Converts acoustic energy to electrical
E. Converts mechanical to electrical

Middle Ear
Tympanum:
Timpani, or kettledrums, are musical
instruments in the percussion
family.
A type of drum, they consist of a
skin called a head stretched over a
large bowl commonly made of
copper.
They are played by striking the head
with a special drum stick called a
timpani stick.
Timpani evolved from military
drums to become a staple of the
classical orchestra in the 17th
century.
Today, they are used in many types
of musical ensembles including
classical orchestra

Tympanic Membrane
The eardrum separates the outer
ear from the middle ear
Creates a barrier that protects the
middle and inner areas from
foreign objects
Cone-shaped in appearance
about 17.5 mm in diameter

The eardrum vibrates in response


to sound pressure waves.
The membrane movement is
incredibly small
as little as one-billionth of a
centimeter

The pars tensa portion of the TM:


A. Consists of 2 layers of tissue
B. Consists of 4 layers of tissue
C. Consists of 1 layer of tissue
D. Consists of 3 layers of tissue
E. Consists of 5 layers of tissue

Eustachian Tube
The eustachian tube connects the front wall of the
middle ear with the nasopharynx
The eustachian tube also operates like a valve,
which opens during swallowing and yawning
This equalizes the pressure on either side of the
eardrum, which is necessary for optimal hearing.
Without this function, a difference between the static
pressure in the middle ear and the outside pressure may
develop, causing the eardrum to displace inward or
outward
This reduces the efficiency of the middle ear and less acoustic
energy will be transmitted to the inner ear.

The Eustachian tube:


A. Opens when one yawns
B. Opens when one smiles
C. Opens when one blinks
D. It is always open
E. Never opens

Ossicles
Malleus
(hammer)
Incus (anvil)
Stapes
(stirrup)
smallest
bone of the
body

The middle ear amplifies sound:


A. About 15 dB
B. About 25 dB
C. About 35 dB
D. About 20 dB
E. About 40 dB

Transformer/Amplifier
Transform the vibrating motion of the eardrum into motion of
the stapes.
The middle ear enhances the transfer of acoustical energy in
two ways:
The area of the eardrum is about 17 times larger than the oval window
The effective pressure (force per unit area) is increased by this amount.

The ossicles produce a lever action that further amplifies the pressure

Without the transformer action of middle ear, about 1/1000 of


acoustic energy in air transmitted to inner-ear fluids (about 30
dB loss).
Malleus and incus vibrate together, transmitting the sound
waves from the eardrum to the footplate of the stapes (this
pushes the oval window in and out)(mechanical energy)

Transformer/Amplifier
Area ratio
Thumbtack

Lever
crowbar

Which provides the most


amplification in the middle ear?
A. The lever hypothesis
B. The area/ratio hypothesis
C. The Hanavan principle
D. I dont know
E. None of the above

Middle Ear Muscles


Tensor tympani
Attached to malleus
Innervated by V, trigeminal nerve

Stapedius
Attached to stapes
Innervated by VII, facial nerve

Middle Ear Muscle Function:


Help maintain ossicles in proper position
Protect inner ear from excessive sound
levels
When ear exposed to sound levels above
70 dB, the muscles contract, decreasing
amount of energy transferred to inner ear

This protective reflex termed "acoustic


reflex"

The VII cranial nerve innervates:


A. Tensor tympani muscle
B. Incus
C. Stapedial muscle
D. Malleus
E. Stapes

The tensor tympani:


A. Innervated by the facial nerve
B. Innervated by the trigeminal nerve
C. Innervated by the VII cranial nerve
D. Innervated by the VIII cranial nerve
E. Innervated by the VI cranial nerve

Ligaments of Middle Ear


Function
restrict and confine
the effect of ossicles
to act as a lever
restrict movements to
reduce the chance of
damage to the inner
ear
prevents distortion to
sound

Mastoid

Mastitis
A. Inflammation of the mastoid
B. Inflammation of the breast
C. Cancer of the mast cells
D. A and B
E. B and C

Question
The correct order of the ossicles from the TM
to inner ear is?
A.Anvil, hammer, stapes
B.Hammer, incus, anvil
C.Hammer, anvil, stapes
D.Stapes, anvil, hammer
E.Malleus, stapes, incus

Development of Middle Ear


Middle Ear Development

Middle Ear Disorders


Middle Ear disorders

Acute otitis media


Otosclerosis
Disarticulation
Mastoiditis
Tympanosclerosis
OME
TM Perforation
TM Retraction
Cholesteatoma

Down Syndrome
Treacher Collins Syndrome
BOR Syndrome

OM/OME

Cholesteatoma

Otosclerosis
Develops most frequently between ages
of 10 and 30.
About 1015% of patients have unilateral
loss.
Affects women more frequently than men
by a ratio of 2:1.
Pregnancy once thought to be a risk factor
for the development and / or worsening of
otosclerosisrecent studies have disputed
this.
May progress to nerve deafness called
cochlear otosclerosis.

Question
The Amy Tan syndrome is:
A.Down syndrome
B.BOR syndrome
C.Treacher Collins syndrome
D.Measles syndrome
E.Waardenburg syndrome

Question
The function of the inner ear:
A.Balance
B.Hearing
C.Touch
D.All the above
E.A and B

Inner Ear
Virtual Tour of the Ear
Vestibular

Auditory

semicircular canals
utricle and saccule

Cochlear
traveling wave

Vestibular

traveling wave
traveling wave
pathologies

Function of Inner Ear


Convert mechanical sound
waves to neural impulses
that can be recognized by
the brain for:
Hearing
Balance

Balance
Linear motion
Rotary motion

Question
Which is true about the inner ear
and balance?
A.The semicircular canals detect
linear motion
B. The utricle and saccule detect
linear motion
C. The cochlea detects linear motion
D.The utricle and saccule detect
rotary motion

Cochlea
The cochlea resembles a
snail shell and spirals for
about 2 3/4 turns around
a bony column
Within the cochlea are
three canals:
Scala Vestibuli
Scala Tympani
Scala Media

Question
The channel that houses the organ of Corti:
A.Scala tympani
B.Scala media
C.Scala vestibuli
D.Semicircular canals
E.B and D

Anatomy of the adult mammalian cochlea

Barald, K. F. et al. Development 2004;131:4119-4130

Organ of Corti

Organ of Corti

Organ of Corti

OHC vs. IHC Function

OHC Motility Models

Afferent Neural Innervations

Hair Cells
Outer Hair
Cells
Inner Hair
Cells

OHC movi
e

OCH Cilia Theory: Tip-links

<<<IHC
OHC >>>

OHC Cilia: Tip-links

Tip Link Protiens

Stereocilia

Stereocilia move sideways and lengthen (Oct 2, 2012)

Flexoelectric Effect
Quiet sounds are magnified by bundles of tiny, hair-like tubes
atop "hair cells" in the ear (stereocilia: when the tubes dance
back and forth, they act as "flexoelectric motors" that amplify
sound mechanically.
"It's like a car's power steering system. "
" You turn the wheel and mechanical power is added. Here, the
incoming sound is like your hand turning the wheel, but to drive,
you need to add power to it. These hair bundles add power to the
sound. If you did not have this mechanism, you would need a
powerful hearing aid.
http://www.medicalnewstoday.com/articles/147081.php

OHC Somatic Motility

Question
Which cells are motile?
A.Brain cells
B.Inner hair cells
C.Outer hair cells
D.B and C
E.None of the above

OHCWith and Without Prestin

No other cells
except OHC
Prestin is the
motor protein
of mammalian
OHCs.
It is highly
expressed in
OHC, and is
not expressed
in nonmotile
IHC.

OHCWith and Without Prestin


About 55 dB
difference
OHC in Prestin
Knockout
Mouse

Traveling Waves

Traveling wave
Basilar membrane
Traveling Wave info
Cochlear Traveling Wave

Question
High frequencies stimulate this part of inner
ear:
A.Apical portion of inner ear
B.Basilar portion of inner ear
C.Utricle
D.Saccule
E.Semicircular canals

Inner Ear Etiologies


Genetic
Connexin 26

Excessive Noise
Head Trauma
Metabolic
Diabetes, kidney disfunction, thyroid dysfunction

Ototoxic
Gentamiacin, cisplatin, etc.

Disease

Connexin 26

Noise Trauma

Question
Persons with diabetes are at greater risk for
hearing lossthis would be?
A. Metabolic
B. Noise related
C. Ototoxic
D. Genetic
E. Acoustic trauma related

Question
Persons with Connexin 26 are at greater risk
for hearing lossthis would be:
A. Metabolic
B. Noise related
C. Ototoxic
D. Genetic
E. Acoustic trauma related

Developmental
Inner Ear Embryological Development

Question
The hearing nerve is located in:
A.Eustachian tube
B.External auditory meatus
C.Internal auditory meatus
D.Organ of Corti
E.Tympani

Question
The auditory nerve is which cranial nerve:
A.VI
B.VII
C.VIII
D.V
E.X

VIII Cranial Nerve

Virtual Tour of the Ear


Auditory Branch

Auditory Branch
Vestibular Branch

Vestibular Branch
Spiral ganglion
Acoustic Tumors

Standard ABR
Cross-section of Internal Auditory Canal
Sup. Vest Nerve

Facial Nerve

HIGHFREQUENCY
FIBERS

Acoustic Nerve
Large Tumor
Abnormal Standard ABR

TUMOR

The wave V latency used in the


standard ABR IT5 and I-V delay
measures is dominated by neural
activity from the high-frequency
regions of the cochlea. Thus, unless
the tumor affects these high-frequency
fibers sufficiently, standard ABR
latencies will be normal.

Inf. Vest Nerve


Small Tumor
Abnormal Standard ABR

Small Tumor
Normal Standard ABR

IAM

VIII Cranial Nerve

Spiral Ganglion

Question
The Auditory Nerve is:
A.V Cranial Nerve
B.VI Cranial Nerve
C.VII Cranial Nerve
D.VIII Cranial Nerve
E.IX Cranial Nerve

Acoustic Neuroma
Tumor

Acoustic Neuroma

Question
Another term for acoustic tumor:
A.VII cranial nerve tumor
B.Vestibular schwannoma
C.Facial nerve tumor
D.Ear schwannoma

Stem Cells Restore Hearing

Human stem cells regenerate auditory nerve cells in gerbils

Central Auditory Mechanism


Virtual Tour of the Ear
Auditory Path

Auditory Path

Central Auditory Path

Auditory Pathway - CNS

Tonotopic

Etiologies
Central auditory processing disorders
Brainstem
Cerebrum
Corpus callosum

Learning disorders
Vascular
Stoke

Head trauma
Tumors

Nonorganic Hearing Loss


Sometimes referred to as functional,
feigning, etc.
No physical evidence of hearing loss
Conscious and unconscious
Adults: medical/legal reasons
Children: attention, psychological, reward,
etc.

Development of the otocyst

Barald, K. F. et al. Development 2004;131:4119-4130

Inductive interactions that regulate otocyst induction and ventral patterning

Barald, K. F. et al. Development 2004;131:4119-4130

Development of the cochlear duct and organ of Corti

Barald, K. F. et al. Development 2004;131:4119-4130

Effects of modulating gene expression on development of the organ of Corti (OC)

Barald, K. F. et al. Development 2004;131:4119-4130

Generation of stereociliary bundle orientation

Barald, K. F. et al. Development 2004;131:4119-4130

Six1 expression during inner ear development

Zheng, W. et al. Development 2003;130:3989-4000