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SUBJECT: EMBRYOLOGY

TOPIC: DEVELOPMENT OF THE EAR

LECTURER: DR. JOSE ANTONIO

AMISTAD

DATE: MARCH 2011

EAR: OVERVIEW

EYE EAR

Day 22 (but,
APPEARANCE Day 22 becomes apparent
at day 24)

PRIMORDIUM Optic Groove

Otic Vesicle
(ANLAGEN) (Vesicle)

DEVELOPMEN From Inside, From Outside,

T Outward Inward

At the level of
At the level of the
EMBRYONIC the
Rhombencephal
LOCATION Diencephalo
on
n
External Ear

• Collecting of sounds (sound waves)

• Pinna notching
o Cancelling noise
o Focusing on a specific sound
 E.g. as in, isolating the
voice of the person talking to you
in a noisy environment.

Middle Ear

• Amplification of Sound
• Conduction of vibration from the
tympanic membrane to the → cochlea
via the oval window.
General Function of the Ear
• Consist of ossicula (ossicle) – conducts
sound waves to form vibration
• Hearing (Audition) (mechanical)
• Perception of gravity through balance o Malleus (hammer)
(Equilibrium) o Incus (anvil)
o Stapes (stirrup)
The 3 Distinct Parts of the Ear (External,
Middle & Internal) Internal Ear

• Converts the sound waves (vibration) into

nerve impulses (electrical)  ramified
axons  CN VIII

*CN VIII: Vestibular part = Balance; Cochlear/Spiral =

Hearing
At 4 weeks age of gestation,
The Otic Pits are found dorsal/posterior to the
rhombencehalon.

EMBRYOLOGY: DEVELOPMENT OF THE EAR Page 1

From the surface ectoderm forms  the Otic
Placode
On day 24 the (A) otic placode invaginates to
form the (B) otic pit. The otic pit will continue to
invaginate, and the rims of the otic pit will fuse to
form the (C) otic vesicle.
Figure: A. 24 days. B. 27 days. C. 4.5 weeks
Otic Placode → Otic Pit → Otic Vesicle → Internal
Ear
Neural crest cells and some cells on the medial
side of the otic vesicle migrate medial to the otic
vesicle forming the statoacoustic ganglion.
Neural crest cells + medial otic vesicle = statoacoustic
ganglion
Development of the otocyst (otic/auditory
vesicle)
The dorsal portion will form the utricle and the
ventral portion will form the saccule. The
extension above the utricle forms the
endolymphatic duct which forms the
endolymphatic sac at the superior end. The
connection between the utricle and saccule is the
utricolosaccular duct or vestibule.
During embryonic period, the endolymphatic duct
is attached to the utricle. Upon birth until
adulthood, the endolymphatic is continuous with
the saccule.
Functions of the endolymphatics
• Secretes endolymph/absorb excess
endolymph
• Responsible for phagocytosis and
immunodefense
The inferior tip of the saccule will form a tubular
outpocketing (cochlear duct) and penetrate the
mesoderm (loose mesenchyme). It will extend
EMBRYOLOGY: DEVELOPMENT OF THE EAR
spirally forming two and a half (2 ½) turns.
(Wikipedia says: 2 ¾) The lower end of the saccule is
connected to the cochlear duct through a short
membranous tube called ductus reuniens.
The utricle, saccule and the endolymphatic
(duct/sac) form the membranous labyrinth,
and the bone that covers it forms the bony
labyrinth.
The innermost part of the vestibule is the macula
acusticae (sensory unit of the vestibule). It is
between the saccule and the utricle responsible
for perception of gravity (vestibular purposes). It
contains sensory cells called crista ampullaris
(also cupula)
In other references: The crista ampullaris is defined as
an elevation at the inner surface of the ampulla of
each semicircular canal.
Going back to the cochlear duct…
The cochlear duct becomes the main house for
endolymph. This endolymph is contained in the
scala media. Earlier, it was mentioned the
formation of cochlear duct will penetrate the
mesoderm; this mesoderm (around) will form a
cartilage completing the cochlea. It is shown in
the following diagram labelled ‘cartilaginous
shell’. This will form 2 cavities for the perilymph.
Located above is the scala vestibuli and below
is the scala tympani. The vestibular
membrane or the reissner’s membrane
separates the scala vestibuli and scala tympani.
Endolymph = ↑ K+, ↓ Na+
Perilymph = ↑ Na+, ↓ K+
**mneumonic: Magkano ang kendi? Piso
‘Kendi’ (‘K’ potassium; ‘Endi’ Endolymph), ‘Piso’ (‘P’
perilymph; ‘so’ sodium)
Development of the organ of Corti
The hair cells and the tectorial membrane found
at the floor of the cochlear duct overlying the
basilar membrane forms the sensory unit of the
cochlea called the organ of Corti.
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During 10 weeks there is a pseudostratified lining there is a membrane called the Hardesty
of the scala media, on the 28th day it is divided membrane.
into two ridges – the inner ridge (thinner) and the
outer ridge (thicker). The thinner inner ridge will
form the spiral limbus. The spiral limbus forms
the tectorial membrane (gelatinous protein-
polysaccharide) a.k.a. copula.

Filaments of the vestibular nerve pass through the

crista( of the crista ampullaris) to reach hair cells on its
surface; the hair cells are capped by the copula, a
gelatinous protein-polysaccharide mass

Components of the tectorial membrane:

• α-tectorin
• β-tectorin
• otogelin (which makes it thicker)
• collagen type 11 (which comes from the spiral
The Hensen’s stripe is the attachment of the
limbus) outer ridge to the spiral ligament that holds the
scala media.

In other references: inner part of the tectorial

membrane is thin and overlies the auditory teeth
of Huschke; its outer part is thick, and along its
lower surface, opposite the inner hair cells, is a
clear band, named Hensen’s stripe, due to the
intercrossing of its fibers.

The bony axis of the cochlea which holds the

scala media is formed by the modiulus. This also
holds the ramifying axons of the auditory nerve.

Development of the semicircular canals

At the end of the utricle forms flattened

The inner ridge it connected to the outer ridge outpocketing which later form the semicircular
through teeth like structure called the auditory canals. This will form a continuous space inside.
teeth of Huschke. Medial to these semicircular formations, the
apposing walls will start to fuse and become
invaded by caspases leading to its
disappearance.

3 semicircular canals
1. Transverse/Lateral/Horizontal
2. Posterior (nothing else, NOT inferior)
3. Anterior/Superior

In other references, the acoustic teeth is defined as,

tooth-shaped formations or ridges occurring on the
vestibular lip of the limbus lamina spiralis of the
cochlear duct.

The outer ridge will form 3 rows of outer hair cells

and 1 row of inner hair cell. The superior of the
inner hair cells is composed of stereocilia in
progressively increasing heights. The tallest
cilium is called the kinocilium which is
connected superiorly to the tectorial plate. This is
important the formation of signals to the brain.
Saccule forms the bony cochlea the utricle forms
The outer hair cells are shorter than the inner the semicircular canal
hair cells. To reach the tectorial membrane the
outer hair cells are supported by the Deiters The semicircular canal has a
cells (not Deiter’s). Between the outer hair cells, dilated and a non-dilated end. The
Deiters cell and the inner haircell is a space dilated end is the crus ampullare
called the subtectorial space. To maintain the and the non-dilated end is the crus
distance between the inner and outer hair cells, non-ampullare. The Ampulla
contains the crista ampullaris

EMBRYOLOGY: DEVELOPMENT OF THE EAR Page 3

 (which is composed of cupula The innermost part of the cleft between PA 1 and
and ,kinocilium) 2 (Pharyngeal groove) which is ectodermal
undergoes meatal plugging or solid epithelial
The close proximity of the endolymphatic duct proliferation. This leads to the elongation of the
and perilymphatic duct with the cranial vault may canal. During 7th month age of gestation, this
cause the CSF to flow out from the ear during meatal plug dissolves. The floor of this meatal
injuries at this location – as in, vehicular plug forms the myringa (transparent vibrating
accidents. membrane; lower portion of tympanic membrane)

MIDDLE EAR
• Incus (Pharyngeal arch 1)
• Maleus (Pharyngeal arch 1)
• Stapes (pharyngeal arch 2)

**Mneumonic: IM#1; Second

**Modern alternative theory: some parts of

Maleus come from PA2; for the purpose of
studying we stick with the above.

The dorsal end of pharyngeal arch 1 and 2 will

form the pinna.
In Myringotomy or Incision of the tympanic
membrane is done at the area of the myringa
The pharyngeal pouch (endodermal) it will extend using a knife for paracentesis called
laterally to the condensed mesenchyme, this myringotome. This paracentesis allows
condensed mesenchyme will form the tympanic accumulated fluid to move out of the middle ear
cavity that will house the ossicles. It also forms through the tympanic membrane.
the area of otic tension of the tympanic
membrane.

The 3 ossicles are the earliest bones that will

ossify. During 12 weeks age of gestation they
ossify within 2 days. Of the 3, the stapes is the
oldest bone. The rapid ossification deprives these
bones to grow bigger causing them to be very
small.

The tympanic membrane is formed by the fusion

of 3 germ layers – Meatal plug ectodermal,
tympanic cavity (endodermal), and the
intermediate layer of the tympanic cavity called
fibrous stratum (mesodermal).

FGF-ERK2 – responsible for type and shape of

ossicles;( ERK=extracellular signal regulated
kinase 2)

NOX2 – (NADPH Oxidase Homeobox 2)

DUSP6 – (Dual Sensitivity Phosphate 6) Shape of

the pinna

Development of the Pinna

Clockwise numerical
Hillocks (1-6)

1 –tragus
6 – anti-tragus

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2-5 – helix Amblyopia – Poor vision caused by abnormal
development of visual areas of the brain in
Formation of the other parts such as anti-helix is response to abnormal visual stimulation during
secondary to the development of 2-5 early development.

 Say-Barber-(Miller) syndrome
o Macrotia
o Bulbus nose
o Hypertelorism
o Telecanthus
o Hirsutism
o Skin folding of the neck
o Macrostomia
o Absence of mammary glands

 Eerler Landos

o Hypersensitivity of ear’s elastic cartilage

o Lysyl oxidase deficiency

 Börjeson-Forssman-Lehmann
syndrome

o Selhurst handle (ear)

o Shortened toe
o Gynecomastia (male)
 Zaheer’s ear – Absence of hillock 6
o Mental retardation
(earphone syndrome)
 Selhurst’s handle– enlarged 2-5 (cup o Trunkal obesity
handle ear)
 Stahl’s ear – formation of a third crura at  SHORT syndrome
the superior crus
 Cryptotia – Absence of Auricular fossa o Tooth decay
 Cryptophthalmia – Fusion of eyelids o Short stature
 Darwin’s tubercle – a small inconstant o Corectopia
projection from the upper end of the posterior
portion of the incurved free margin of the
helix of the auricle

 Microtia
CLINICAL CORRELATION
Grade

I – miniature ear

II – cleft, “hemi-ear”

III – absence of ear canal, peanut vestige

IV – dorsal end or total absence of ear

*Grade III & IV have absence of ossicles and

tympanic membrane, although surgically
repairable

*BAHA – Bone anchor hearing aid

* Grade I & II – stop in ear development

A and B = Preauricular pits  Strabismus

C and D = Preauricular tag or appendages o Entotropia – type of strabismus in which

the visual axes converge
These are hints of internal ear problem or o Exotropia – type of strabismus in which
indicate early presbycusis. the visual axes diverge

EMBRYOLOGY: DEVELOPMENT OF THE EAR Page 5

 Lamellar cataract - a cataract in which the
opacity is limited to the cortex

Globular cataract

leukocoria, leukokoria - Reflection from a white

mass within the eye giving the appearance of a
white pupil.

Red eye reflex – secondary to mirroring of light

on the tapetum lucidum

Ishihara test (3 seconds flashing of image)

Thompson wool test

 Monochromacy

cone monochromacy

o Grayscale vision
*Entropion - infolding of the margin of an eyelid

*Ectropion - A rolling outward of the margin of a rod monochromacy

part
o Blindness rely to other wavelengths such
*Corectopia - Eccentric location of the pupil so as sound
that it is not in the center of the iris. o Loud talking

o Embryotoxon Dichromacy

o Congenital limbal(limbus) opacity Trichromacy

Common cause of congenital cataract – German Red 564-580 nm

measles
Green 532-540 nm
Adult cataract – commonly caused by Diabetes
mellitus type 2 Blue 400-440 nm

Cyclopia – single eye Protanopia (red)

Synophthalmia – fused eye Red = dark brown/gray

Proboscsis – A long flexible snout Green = yellow

Coloboma – found in CHARGE syndrome Blue = Blue

CHARGE syndrome (association) Deuteronopia

coloboma of the eye, heart defects (typically Red = yellow

tetralogy of Fallot, patent ductus arteriosus,
or ventricular or atrial septal defect), atresia Green = yellow
of the choanae, renal anomalies and
retardation of growth and/or development, Blue = blue
genital anomalies in males such as small
penis or cryptorchidism, and ear
Tritanopia
abnormalities or deafness
Red = red
4 types of cataract
Green = green / blue-green
Sutural/Structural Cataract (inverted ‘Y’) - a
congenital type of cataract with opacities along
the Y sutures of the fetal lens nucleus; usually Blue = light blue / pink
does not affect vision

EMBRYOLOGY: DEVELOPMENT OF THE EAR Page 6

Lea’s Test

Landolt chart (C-like letters)

Snelen’s chart

-letters discovered by sloan

END OF
TRANSCRIPTION
SUMMER!!!

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