EARS, NOSE & THROAT (ENT)

Topic: Ear Anatomy, Physiology, Embryology and Congenital Anomalies

Lecturer: Dr. Cruz, Melanie Grace

ANATOMY AND PHYSIOLOGY OF THE EAR Internal Auditory Canal:

Ear:  Also known as the internal
 Organ of hearing and balance acoustic meatus
 Related to the temporal bone  It lies on the temporal bone
The temporal bone is located at the lateral and exists between the inner
portion of the skull as depicted in the image ear and posterior cranial fossa
on the left in green.  CN VIII is composed of cochlear
and vestibular nerve
 CN VII and CN VIII enter and
Parts of the Temporal Bone: exit the internal auditory canal

ANATOMIC SUBDIVISIONS:

1. Squamous
o It forms the lateral wall of the skull and is the biggest part
2. Mastoid
o Located posteriorly and contains air cells
3. Tympanic
o Forms the bony ear canal
4. Styloid
o The elongated portion (based on the image above)
5. Petrous
o Not shown in the image above
o It contains many vital structures and is best seen in the
medial side

Development of the Mastoid Bone:

 The mastoid process is absent at birth and
the facial nerve is still superficial
o The facial nerve is therefore Lecture Discussion:
vulnerable to injuries during The ear is subdivided into the outer, middle and inner portion
delivery using obstetric forceps  Outer ear is from the auricle up to the tympanic membrane
 The mastoid tip elongates by the SCM  Middle ear is from the tympanic membrane up to the oval
(sternocleidomastoid) pull when the infant window
begins to hold up the head  Inner ear includes the labyrinth
 Pneumatization begins in infancy and up to
around 4-6 years old External Ear - Pinna
 The largest and most consistent air cell is Landmarks:
the Antrum  Helix and Anti Helix
 Tragus and Anti-tragus
3 Degrees of Temporal Bone Pneumatization:  Conchae Cavum and
Cymba
 Lobule

Lecture Discussion:
The most protuberant portion of the ear is the Pinna. It is comprised of
cartilage and skin EXCEPT for the lobule which is composed of skin and fat.
The structure of the pinna is intricate comprising of several elevations and
depressions.
 Helix – outermost rim of the auricle
Lecture Discussion:
 Conchae – deepest depression of the ear
A well pneumatized or well aerated mastoid will have numerous air cells as
represented here as the honeycomb-like appearance. Black representing air,
while the White septations as bone.
A diploic bone is partially pneumatized
A sclerotic bone is dense and has no air cells

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Topic: Ear Anatomy, Physiology, Embryology and Congenital Anomalies
Lecturer: Dr. Cruz, Melanie Grace

External Ear – Ear Canal Innervation of the External Ear and Tympanic
 EAC length: 2.5 cm Membrane:
 Lateral or Outer 1/3 is  VII – Sensory cutaneous branches
Cartilaginous  V – Auriculotemporal nerve
o Has cerumen  X – Arnold’s Nerve
glands and hair  C3 – GAN
follicles  C2, C3 – Lesser occipital nerve
 Medial 2/3 is Bony
o No cerumen glands or hair follicles Lecture Discussion:
 Isthmus – Junction It just shows that the External Ear and the Tympanic
Membrane have similar nerve innervation
o This is an anatomical constriction

External Ear – Tympanic Membrane

 8 mm (wide) x 9 mm (high) x 0.1  ALDERMAN’S NERVE/ARNOLD’S NERVE - Irritation of the Auricular
mm (thickness) branch of CN X may cause reflex coughing, vomiting or sneezing
 Pearly white in color
 Parts divided by malleolar folds: Middle Ear
1) Pars Flaccida  Wedge-shaped cavity with six walls
 Shrapnell’s  The space is very narrow from side-
membrane to-side
 Located superiorly
2) Pars Tensa Middle Ear Boundaries:
 Located inferior to the malleus 1. Roof – tegmental wall
 It has a great surface area 2. Floor – jugular wall
3. Posterior – mastoid wall
Tympanic Membrane Layers: 4. Anterior – carotid wall
1. Outer Ectoderm – continuous with the skin of the external ear 5. Lateral – membranous wall (represents the tympanic membrane)
2. Middle Mesoderm – fibrous (inner circular and outer radial) 6. Medial – labyrinthine wall
containing the handle of the malleus o This wall separates the middle ear from the inner ear
3. Inner Endoderm – continuous with the mucosal membrane of the (labyrinths)
middle ear
Notch of Rivinus

Annulus Fibrosus
 Fibrocartilaginous ring supporting
the Tympanic Membrane
 Lies in the tympanic sulcus (groove
in the tympanic bone)
 Deficient superiorly at the NOTCH
OF RIVINUS
Annulus Fibrosus

Pars Flaccida
 Retracts readily if there is any absorption of air when Eustachian tube
is blocked and it bulges if there is fluid or inflammatory swelling Additional Information: Middle Ear Boundaries
 Superior - TEGMEN TYMPANI (epitympanum)
within the middle ear cavity
 Inferior - JUGULAR BULB (hypotympanum)
 When the ear drum retracts, the short process of the malleus
 Medial - PROMONTORY, OVAL WINDOW, ROUND WINDOW,
becomes prominent LATERAL SEMICIRCULAR CANAL
 When it bulges, the landmarks are obliterated  The footpiece of the stapes seals off the oval window.
 Lateral - EAR DRUM, Scutum
Retracted Pars Flaccida Bulged Pars Flaccida  Anterior - TENSOR TYMPANI, internal carotid artery, EUSTACHIAN
TUBE
 Posterior – FACIAL CANAL and aditus ad antrum

Middle Ear Contents:

 Ossicles – malleus, incus and stapes
 Branches of Facial Nerve – nerve to stapedius and chorda tympani
 Tympanic Plexus – sensory to middle ear over the promontory
 Middle Ear Muscles – tensor tympani and stapedius

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Topic: Ear Anatomy, Physiology, Embryology and Congenital Anomalies
Lecturer: Dr. Cruz, Melanie Grace

Ossicles Nerve Supply of the Middle Ear:

 Suspended in the middle ear cavity forming a chain for conduction of  Auriculotemporal branch of CN V
vibrations from the drum to the oval window  Auricular branch of CN X (Arnold’s nerve)
Maleus Head, neck and handle  Tympanic Branch of CN IX (Jacobsen’s nerve)
Body, short process, long process or lentiform
Incus
process Inner Ear
Head, anterior and posterior crus, foorplate; seals 2 Main Parts of the Inner Ear:
Stapes
off the oval window in a piston like fashion 1. Bony Labyrinth (Osseus Labyrinth) – the outer shell
2. Membranous Labyrinth – suspended within the bony labyrinth

Middle Ear Joints

Diarthrodial Joint Syndesmotic Joint
 Malleoincudal  Stapes
 Incudostapedial  Oval window Osseus Labyrinth
 One of hardest bone in the body
Branches of the Facial Nerve (Mastoid Segment)  Contains the organ for hearing and balance
 Nerve to the stapedius which is attached to the stapes  Specifically it contains the:
 Chorda tympani which is responsible for taste sensation to the 1) Bony cochlea
anterior 2/3 of the tongue 2) Bony vestibule
3) Bony semicircular canals
Middle Ear Muscles  3 canals lie at right angle to one another
Muscle TENSOR TYMPANI STAPEDIUS
ORIGIN Cochleariform Process Pyramidal Eminence  Contains perilymph – it is the fluid within the labyrinth
ATTACHMENT Malleus Stapes
Nerve to the Stapedius 3 Types of Semicircular Canals:
NERVE SUPPLY CN V-C3 which is a branch of  Superior or anterior semicircular canal
CN VII  Lateral or horizontal semicircular canal
Pulls the handle of the Pulls the stapes away  Posterior semicircular canal
malleus medially from the oval window
ACTION
3 Openings of the Cochlear Canal:
Pulls the tympanic Pulls the stapes
 Round window – fenestra cochlea
membrane inward outward
 Oval window – fenestra vestibule
Function of Middle Ear Muscles:  Cochlear Aqueduct - opens into a tiny canal from the basal turn of
 Contract in response to high intensity sound or anticipation to the cochlea through the temporal bone to the subarachnoid cavity
vocalization by stiffening the ossicular chain to protect the inner ear
against overstimulation Membranous Labyrinth
3 Parts of Membranous Labyrinth:
 Dampening effect
1. Membranous Semicircular Canals
o 2 openings from the Lateral
Eustachain Tube
SCC
 The eustachain tube is lined by pseudostratified columnar ciliated
o 3 opening from the Superior
respiratory epithelium found in the nose, nasopharynx, and middle
SCC and Posterior SCC (joining
ear
at the crus communis)
 Main function: Equalize the pressure between the ear and the
o Ampulla – dilated ends
atmosphere
 2 Muscles that dilate the Eustachian Tube:
2. Membranous Vestibule
o Tensor Veli Palatini – innervated by the mandibular
o Utricle and saccule
branch of CN V from the Otic Ganglion
o Utricular duct (UD) & Saccular duct (SD)
o Levator Veli Palatini – innervated by pharyngeal branch
o Endolymphatic duct – fusion of the UD and SC
from CN X
3. Membranous Cochlea
Blood Supply of the Middle Ear:
o Scala media/cochlear duct
 Deeper auricular branches of the internal maxillary artery
o Ductus reuniens – connecting the cochlear duct and the
saccule

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Topic: Ear Anatomy, Physiology, Embryology and Congenital Anomalies
Lecturer: Dr. Cruz, Melanie Grace

Tectorial Membrane
 The membranous labyrinth contains endolymph  Gelatinous structure which lies on top of OHC and IHC

Cochlea Pillars of Corti

 Lies horizontal in front of the vestibule  Structure in between OHC and IHC
 Resembles a snail shell of 2 ½ (2 ¾) turns
 Apex – lower frequency Perilymph vs. Endolymph
 Base – higher frequency Perilymph Endolymph
 Spiral lamina – attachment of the Basilar membrane Volume 78.3 cu.mm 2.76 cu.mm
 Spiral ganglion - average of 31,000 ganglion cells Sodium Content 310 mg/100 mL 15 mg/100 mL
 Cochlear Nerve Potassium Content 30 mg/100 mL <600 mg/100 mL
Protein Content 70-100 mg/100 mL 20-30 mg/100 mL
pH 7.2 7.5
Specific Gravity Lower Higher

Lecture Discussion: Perilymph vs. Endolymph

The most important to remember here is:
 Perilymph  Sodium content is higher compared to endolymph
 Endolymph  Potassium content is higher compared to
perilymph

AUDITORY SYSTEM
 The pinna collects
sound waves.
 The sound waves are
transferred to the ear
canal and into the ear
drum.
 The ear drum vibration
will cause ossicular
Different Parts of the Cochlea: vibration or coupling.
 SCALA VESTIBULI – above the  The ossicular vibration will have what is called impedance matching.
Cochlear Duct (Perilymph)  Impedance matching – efficient sound transmission
 SCALA TYMPANI – below the o Without this, sound will just bounce off the oval window
Cochlear duct (Perilymph)
 Vibration of ossicles ( malleus - incus - stapes)
 SCALA MEDIA – endolymph
 Footplate of stapes strikes the oval window
 Perilymphatic displacement leads to basilar membrane displacement
 Modiolus
which will cause endolyphatic compartment displacement
o Central Conical Axis
allows passage of
the auditory nerve fibers Area Ratio Lever Ratio
TM: footplate Malleus to Incus
 Helicotrema
17-20:1 1.31:1
o Apex of the modiolus
 Stria vascularis
Hair Cell Activity
o Blood supply of the cochlea
 Stereocilia moves towards kinocilium.
 There will be depolarization (180 spikes/sec).
Organ of Corti
 There will be opening of gated channels.
 Principal sensory
 There will be influx of potassium from the endolymph.
structure in cochlea
 The membrane potential will be positive once there is potassium
epithelial structure on
influx.
top of the basilar
membrane  The calcium channels will be activated.
 Calcium channel activation will cause calcium influx.
Hair Cells  Glutamate will be released into the nerve endings.
 Functions as receptor
cells that transduce mechanical movement into electrochemical Von Bekesy Travelling Wave Theory
signal  Wave from cochlear base transfers to apex
o Inner Hair Cells (IHC)  The motion of the basilar membrane takes the form of a traveling
 Single row wave, like the one that occurs when you flick a rope
 Most important sensory cells of hearing  The wave oscillates at the frequency of stimulation
o Outer Hair Cells (OHC)  For the Organ of Corti to be given maximal displacement, creating
 3 rows COCHLEAR MICROPHONICS which is a local electrical potential
 Acts as amplifiers

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Topic: Ear Anatomy, Physiology, Embryology and Congenital Anomalies
Lecturer: Dr. Cruz, Melanie Grace

 It is important to note that the basilar membrane is narrow near the Blood Supply:
base of the cochlea and wide at the apex. It is also stiffer at the base  INTERNAL AUDITORY ARTERY (branch of anterior inferior cerebellar
than the apex  this is the reason why at the base, we can hear artery) – blood supply to the cochlea that enters into the internal
higher frequency sounds while at the apex, we can hear lower auditory canal
frequency sounds
EMBRYOLOGY AND CONGENITAL ANOMALIES
Auditory System: External Ear:
E Eight Nerve  The ear starts to develop at the 3rd-7th week of gestation
Lecture Discussion:
C Cochlear Nucleus This is the auditory pathway of  Modification of the surface ectoderm by which the skin is brought to
O Superior Olivary Nucleus how we are able to hear. From the functional relationships with the ossicles at the drum and the
L Lateral Lemniscus periphery, impulses will travel external canal
I Inferior Colliculus from the cochlear nerve which will
M Medial Geniculate Body go to the Eight Nerve all the way to
the Auditory Cortex
A Auditory Cortex

 BA 41 – Primary Auditory Cortex

 BA 42 – Secondary Auditory Cortex

Balance Apparatus
 3 Semicircular Canals – angular acceleration
 Vestibule – linear acceleration
o Utricle – horizontal Hillocks of His
o Saccule – vertical  6 Hillocks of His
 1st Branchial Arch
Semicircular Canals o 1 – Tragus
 Dilated ends at the vestibule called ampulla containing crista o 2 – Helical Root
ampullaris o 3 – Helix
 Cristae ampullaris contains hair cells (sensory)  2nd Branchial Arch
 On top of the hair cells is a gel- like structure called cupula where it o 4 – Anti helix
is suspended to the wall of the opposite ampulla o 5 – Anti tragus
 Coplanar o 6 - Lobule
o If one side is excitatory the other side is inhibitory  Pinna – 1st and 2nd BA
 Angular acceleration of the body causes movement of the endolymph
with the semicircular canal parallel to the rotation. ANOMALIES OF THE EXTERNAL EAR
 Due to inertia, movement of endolymph causes displacement of the  Maldevelopment of the 1st and 2nd arches
hair cells opposite to the direction of the acceleration stimulus.  Auricular deformities are the most prominent
 Torsional pendulum model  Most common is a LOP EAR deformity.
 MACROTIA – abnormally large ear
Utricle and Saccule  MICROTIA – abnormally small ear
 Linear acceleration in relation to  ANOTIA – absence of ear
gravity
 Ear Canal Atresia/Stenosis - Partial or complete stenosis of the ear
 Contains macula canal.
 Hair cells suspended in a  Rudimentary ear appendages
gelatinous matrix
 Failure to fuse of the Hillock of His leads to
 Otoliths (CaCO3) crystals are formation of pre-auricular sinus cyst
suspended on top of the otolithic
membrane

Vestibular Function:
 Vestibule-ocular reflex - stabilize eye gaze
 Vestibulocolic reflex – posture and gait
 Vestibulospinal reflex - posture and gait, extension of limbs
 ANS – adjust hemodynamic reflex maintaining cerebral perfusion
 Cerebellum – coordination and adaptation of vestibular reflex
 Cortex – perception of movement and orientation

Stimulation of the vestibular organs result in neural excitation that travels via
the vestibular nerve to the 4 vestibular nuclei in the brain

Nystagmus
 A Reflex Eye Movement elicited upon stimulation of the semicircular
canals
 Jerk Nystagmus: Slow and Fast Phases

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Topic: Ear Anatomy, Physiology, Embryology and Congenital Anomalies
Lecturer: Dr. Cruz, Melanie Grace

1st Branchial Cleft/Groove ANOMALIES OF THE MIDDLE EAR

 Ectoderm  Involve the ossicles
 If forms the following:  Congenitally deformed, disrupted, or fixed.
o Meatal plug which  Common deformities include a missing part of the incus and fixation
hollows out of the stapes.
o External auditory  Isolated ossicular malformations may be correctible through ear
canal, glands and surgery.
hair  If the stapes is fixed, stapedectomy and prosthesis replacement will
o External layer of re-establish hearing.
the TM (tympanic
membrane) Development of the Inner Ear
 Develops as an ectodermal thickening on the lateral surface of the
1st Branchial Pouch head at the level of the 4th ventricle in embryos of 4 mm in size (9 –
 Endoderm 10days)
 It forms the following:  Otic placode (ectoderm) – forms the otic pit - pinches off to form otic
o Tubotympanic recess cyst/otic vesicle
o Middle ear space  Neural crest migrate to otic cyst - acousticofacial ganglion - geniculate
o Eustachian tube ganglion moves away from otic cyst leaving the - vestibulocochlear
o Mastoid air cells ganglion hair cell formation

Development of the External and Middle Ear Otic Vesicle

 The meatal plug and tubo-  Medial becomes endolymphatic diverticulum
tympanic recess meet to  Lateral becomes utriculosaccular chamber that becomes later the
form the tympanic ring utricle and saccule
 At birth - the ring is present o (DORSAL) Utricular chamber becomes the utricle and
but has not yet developed semicircular canals
into bony meatus. o (VENTRAL) Saccular chamber becomes the saccule and
 After birth - The tympanic cochlea
ring extends by  The endolymphatic duct is shifted down to form a connection
intramembranous between the utricle and saccule.
ossification, adding a new bone laterally forming the bony meatus  14 – 15 days (6 mm size) – endolymphatic ducts
 MIDDLE EAR – developmentally an air sinus and develops as an  37 days (15 mm size) – semicircular canal
outpouching of the pharynx  Macula (statoreceptors) reaches adult form by 14-16 weeks
 The ossicular chain – develops from the upper end of the first  Crista reaches adult form by 23 weeks
(mandibular) and second (hyoid) cartilages  Organ of corti reaches adult form by 25 weeks
 The ossicles develop outside the middle ear cavity and are  Cochlea – evolves parallel with the middle ear and ossicles
extramucosal and are secondarily incorporated to the M.E. by  The cartilagenous otic capsule conceals the inner ear and later
formation of attic recesses. becomes petrous bone.

1st and 2nd Branchial Arches: Development of the Otic Vesicle

1st Branchial Arch  A region of ectoderm overlying the Rhombencephalon thickens to
 Meckel’s cartilage form the otic placode which invaginates to form otic pits which will
 Mesoderm will develop into tensor veli palatine and tensor tympani eventually become the otic vesicle
 Neural crest will develop into maxilla, malleus and body of the incus
Development of the Inner, Middle, and External Ear
2nd Branchial Arch  ach otic vesicle will subdivide into dorsal and ventral compartments
 Reichert’s cartilage and then become the organs of the inner ear
 Styloid  The ossicles will emerge from the mesenchyme of the first and
 Stapedius second pharyngeal arches
 Lenticular process of the incus  Dorsal Compartment
 Stapes o Endolymphatic duct
o Utricle
o Semicircular canal
 Ventral Compartment
o Saccule
o Cochlear duct
 The auditory tubes surrounds the ossicles creating the tympanic
cavity of the middle ear
 The point of contact between the tympanic cavity and the external
ear becomes the tympanic membrane or ear drum
 The pharyngotympanic tube will dissociate to form the Eustachian
tube and the tympanic cavity

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Topic: Ear Anatomy, Physiology, Embryology and Congenital Anomalies
Lecturer: Dr. Cruz, Melanie Grace

ANOMALIES OF THE INNER EAR C. NON-GENETIC

 Pregnancy exposure to rubella virus
A. CONGENITAL
especially vulnerable during the 1st
 No cochlear and labyrinthine development trimester
Michel’s Aplasia
 Complete aplasia  SNHL
 Partial aplasia Congenital Rubella
 Pathology shows aplasia of the saccule and
 1.5 cochlear turn (bony and membranous the Organ of Corti.
labyrinth affected)  Cardiac anomalies, blindness, and mental
Mondini’s Deformity  Flattened cochlea with development of retardation.
only the basal turn  Erythroblastosis faetalis results from RH
 Abnormally dilated vestibule and vestibular blood incompatibility of the parents
duct characterized by bilirubin deposition in the
 Autosomal recessive Kernicterus brain
 Cochleosaccular dysplasia (membranous  There’s jaundice, mental retardation,
Scheibe’s Deafness
labyrinth only) cerebral palsy, and deafness may be
 The bony labyrinth is fully developed present shortly after birth
 Aplasia of the Cochlear duct  Thyroid disease associated with deafness
 Organ of Corti and adjacent ganglion cells as in this condition is referred to as
Alexander’s Deafness
 The bony and membranous labyrinth are Cretinism endemic cretinism. Presenting with mixed
normal hearing loss having both conductive and
 Lateral displacement of the lacrimal points sensorineural component
and medial canthus
 Hyperplasia of eyebrows D. OTHER ABNORMALITIES
 Partial albinism in the form of a white  This disease begins in early childhood
Waardenburg’s Disease
forelock  Skeletal deformity, dwarfism, mental
 Flat nasal root Hurler’s Syndrome
retardation, blindness
 Mild to severe SNHL (sensorineural hearing  Profound SNHL
loss)
 Skeletal defects such as fusion of the
 Maybe autosomal dominant, recessive or cervical vertebra, spina bifida, scoliosis,
Albinism sex-linked, characterized by bilateral Klippel-feil Syndrome torticollis
severe to profound SNHL  Vestibular dysfunction and profound SNHL.
 Congenital male dystrophy  Autosomal recessive
Dominant Deafness  Congenital SNHL – high frequency  Obesity, DM, retinitis pigmentosa, and
Onychodystrophy (DDOD)  Small, short fingernails, toenails progressive deafness.
 Rare Alstrom’s Disease  Hearing loss starts at age 10 and slowly
 Retinitis pigmentosa progresses.
Usher’s Syndrome
 Moderate to severe SNHL  Inherited as an autosomal recessive trait
 Craniofacial dysostosis
 Premature closure of the cranial sutures,
B. CHROMOSOMAL short upper lip, protruding lower lip,
Trisomy 13-15 D underdevelopment of the midface,
Crouzon’s Disease
“Patau’s Trisomy” exophthalmos, hook nose
 Atresia of the external meatus and mixed
deafness.
 Autosomal dominant trait
 Low set ears, undifferentiated pinna,  Autosomal recessive
absence of external and middle ears  Mental deficiency
Richards-Rundle
 Cleft lip and palate, microphthalmia,  Ataxia
Syndrome
coloboma iridis, and aplasia of the optic  Hypogonadism
nerve  Severe SNHL at childhood
 Infants die within a short period of time  Autosomal recessive, X-linked
 Childhood progressive glomerulonephritis
Alport’s Disease  Progressive SNHL particularly on higher
frequencies.
 Type IV collagen defect

Trisomy 18 E
“Edward’s Syndrome”

 Malformed pinna, low set ears

 Flexion of the index finger over the 3rd
finger
 Micrognathia, a prominent occiput.
 Patients with this syndrome fail to
thrive and dies in infancy

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