4.

Ear Development1
In the adult, the ear forms one anatomic unit serving both hearing and
equilibrium. In the embryo, however, it develops from three distinctly different
parts: (1) the external ear, the sound-collecting organ; (2) the middle ear, a sound
conductor from the external to the internal ear; and (3) the internal ear, which
converts sound waves into nerve impulses and registers changes in equilibrium.
a. Internal Ear
The first indication of the developing ear can be found in embryos of
approximately 22 days as a thickening of the surface ectoderm on each side of
the rhombencephalon.

Figure 4.1 A. An embryo at the end of the fourth week of development showing the
otic and optic vesicles. B. Region of the rhombencephalon showing the otic placodes
in a 22-day embryo1

These thickenings, the otic placodes, invaginate rapidly and form the otic
or auditory vesicles (otocysts).

Figure 4.2 AC. Transverse sections through the region of the rhombencephalon
showing formation of the otic vesicles. A. 24 days. B. 27 days. C. 4.5 weeks. Note
the statoacoustic ganglia1

During later development, each vesicle divides into (1) a ventral

component that gives rise to the saccule and cochlear duct and (2) a dorsal
component that forms the utricle, semicircular canals, and endolymphatic
duct.

Figure 4.3 A,B. Development of the otocyst showing a dorsal utricular portion with
the endolymphatic duct and a ventral saccular portion. CE. Cochlear duct at 6, 7,
and 8 weeks, respectively. Note formation of the ductus reuniens and the
utriculosaccular duct1

On the development of the scala tympani and scala vestibuli the cochlear
duct is surrounded by a cartilaginous shell. During the 10th week, large
vacuoles appear in the cartilaginous shell. The cochlear duct (scala media) is
separated from the scala tympani and the scala vestibuli by the basilar and
vestibular membranes, respectively. Note the auditory nerve fi bers and the
spiral (cochlear) ganglion.

Figure 4.5 Development of the semicircular canals. A. 5 weeks. B. 6 weeks. C. 8

weeks. DF. Apposition, fusion, and disappearance, respectively, of the central
portions of the walls of the semicircular outpocketings. Note the ampullae in the
semicircular canals1

Together, these epithelial structures form the membranous labyrinth.

b. Middle Ear
The tympanic cavity, which originates in the endoderm, is derived from
the fi rst pharyngeal pouch. This pouch expands in a lateral direction and
comes in contact with the floor of the first pharyngeal cleft. The distal part of
the pouch, the tubotympanic recess, widens and gives rise to the primitive
tympanic cavity, and the proximal part remains narrow and forms the
auditory tube (eustachian tube, through which the tympanic cavity
communicates with the nasopharynx.

Figure 4.6 A. Transverse section of a 7-week embryo in the region of the

rhombencephalon, showing the tubotympanic recess, the first pharyngeal cleft, and
mesenchymal condensation, foreshadowing development of the ossicles. B. Middle
ear showing the cartilaginous precursors of the auditory ossicles. Thin yellow line in
mesenchyme indicates future expansion of the primitive tympanic cavity. Note the
meatal plug extending from the primitive auditory meatus to the tympanic cavity1

The malleus and incus are derived from cartilage of the fi rst pharyngeal
arch, and the stapes is derived from that of the second arch. Although the
ossicles appear during the first half of fetal life, they remain embedded in
mesenchyme until the eighth month, when the surrounding tissue dissolves.
The endodermal epithelial lining of the primitive tympanic cavity then
extends along the wall of the newly developing space. The tympanic
cavity is now at least twice as large as before. When the
ossicles are entirely free of surrounding mesenchyme, the
endodermal epithelium connects them in a mesentery-like
fashion to the wall of the cavity. The supporting ligaments of
the ossicles develop later within these mesenteries.

Figure 4.7 Ear showing the external auditory meatus, the middle
ear with its ossicles, and the inner ear 1

Because the malleus is derived from the fi rst pharyngeal

arch, its muscle, the tensor tympani, is innervated by the

mandibular

branch

of

the

trigeminal

nerve.

The

stapedius muscle, which is attached to the stapes, is

innervated by the facial nerve, the nerve to the second

pharyngeal arch.

Figure 4.8 A. Derivatives of the fi rst three pharyngeal arches. Note the malleus and
incus at the dorsal tip of the first arch and the stapes at that of the second arch. B.
Middle ear showing the handle of the malleus in contact with the eardrum. The
stapes will establish contact with the membrane in the oval window. The wall of the
tympanic cavity is lined with endodermal epithelium1

During late fetal life, the tympanic cavity expands dorsally

by vacuolization of surrounding tissue to form the tympanic
antrum. After birth, the epithelium of the tympanic cavity
invades the bone of the developing mastoid process, and
epithelium-lined air sacs are formed (pneumatization).
Later, most of the mastoid air sacs come in contact with the
antrum and tympanic cavity. Expansion of infl ammations of
the middle ear into the antrum and mastoid air cells is a
common complication of middle ear infections.
c. External Ear
The external auditory meatus develops from the dorsal portion of the
first pharyngeal cleft. At the beginning of the third month, epithelial cells at
the bottom of the meatus proliferate, forming a solid epithelial plate, the
meatal plug . In the seventh month, this plug dissolves, and the epithelial
lining of the fl oor of the meatus participates in formation of the defi nitive
eardrum. Occasionally, the meatal plug persists until birth, resulting in
congenital deafness.

The eardrum is made up of (1) an ectodermal epithelial lining at the

bottom of the auditory meatus, (2) an endodermal epithelial lining of the
tympanic cavity, and (3) an intermediate layer of connective tissue that forms
the fibrous stratum. The major part of the eardrum is firmly attached to the
handle of the malleus , and the remaining portion forms the separation
between the external auditory meatus and the tympanic cavity.

Figure 4.9 A. Drawing of a 6-week-old embryo showing a lateral view of the head
and six auricular hillocks surrounding the dorsal end of the fi rst pharyngeal cleft. B.
Six-week-old human embryo showing a stage of external ear development similar to
that depicted in A. Note that hillocks 1, 2, and 3 are part of the mandibular portion of
the fi rst pharyngeal arch and that the ear lies horizontally at the side of the neck. At
this stage, the mandible is small. As the mandible grows anteriorly and posteriorly,
the ears, which are located immediately posterior to the mandible, will be

repositioned into their characteristic location at the side of the head. CE. Fusion
and progressive development of the hillocks into the adult auricle 1

The auricle develops from six mesenchymal proliferations at the dorsal

ends of the first and second pharyngeal arches, surrounding the first
pharyngeal cleft. These swellings (auricular hillocks), three on each side of
the external meatus, later fuse and form the defi nitive auricle . As fusion of
the auricular hillocks is complicated, developmental abnormalities of the
auricle are common. Initially, the external ears are in the lower neck region,
but with development of the mandible, they ascend to the side of the head at
the level of the eyes.
1. Sadier, TW. 2012. Langmans Medical Embryology 12th Edition. Lippincott
Williams and Wilkins, a Wolter Kluwer Business
7. Virus that can Cause Congenital Defect
a. Varicella virus: skin scaring, limb hypoplasio, musle atrophy, intellectual
disability
b. Rubella virus: cataracts, glaucoma, heart defects, hearing loss, tooth
abnormalities
c. Cytomegalovirus: microcephaly, visual impairement, intellectual disability
d. Herpes simplex virus: microcephthalmia, microcephaly, retina dysplasia
Sadier, TW. 2012. Langmans Medical Embryology 12th Edition. Lippincott
Williams and Wilkins, a Wolter Kluwer Business
12. Infant Cataract
Congenital cataract cause the lens to become opaque during intra uterine
life. Although this anomaly is usually genetical determined, many children born to
mothers who had Rubella (German measles) between the fourth and seventh
weeks of pregnancy cataracts.
If the mother is infected after the seventh week of pregnancy, the lens
escape damage, but the child may have hering loss as a result of cochleal

abnormalities. Because of the MMR vaccine, CRS has been nearly eradicted in
USA.
Sadier, TW. 2012. Langmans Medical Embryology 12th Edition. Lippincott
Williams and Wilkins, a Wolter Kluwer Business
16. e. Clinical Features of Rubella Disease

Cataracts & other ocular defect

Heart defects
Intra uterine growth retardation
Failure to thrive
Mortality within the first year
Microcephaly
Mental retardatin

Murray,

Patrick R. 2009. Medical Microbiology 6th Edition. Philadelphia :

Churcill Livingstone Elsevier

17. Red Flags
Red flag is an informal term that, in this context, simply implies that some
aspect of the childs development has been noticed as at risk for falling outside the
range deemed typical. A red flag may be discovered during a standardized
developmental screening or through the ongoing, daily interactions between the
infant/toddler caregiver and child. In essence, a red flag is a signal to pay
increased attention to the aspect of concern in a childs development, and to be
even more intentional in documenting observations and providing opportunities
for the child to acquire the skill.
Red flags guidliness (6-24 months):
1) 6 month of age
a. Fine motor: Infant is unable to hold or grasp an adult finger or a toy/object
for a short period of time, persistence of grasp reflex, and consistently
ignores or has difficulty using one side of body; or uses one hand
exclusively

b. Gross motor: Does not pull up to sit or does not roll over, baby is unable to
hold head in the middle to turn and look left and right and asymmetry (i.e.
a difference between two sides of body or body too stiff or too floppy)
c. Vision: Turning or tilting head to use only one eye to look at things,
Holding toys close to eyes, or no interest in small objects and constant
jiggling or moving of eyes side-to-side
d. Speech and language: Early babbling stops, Does not respond when called
and a lot of colds and ear infections
e. Cognitive : Unable to follow moving objects with his/her eyes and will not
reach out to explore/touch objects
f. Social : Unresponsive to a familiar voice, no eye contact, unresponsive to
social situations (i.e., flat affect), not smiling socially
g. Emotional: Unresponsive to familiar caregivers, Extreme irritability and
unresponsive to social situations
2) 12 month of age
a. Fine motor: Consistently ignores or has difficulty using one side of body
or uses one hand exclusively
b. Gross motor: Baby is unable to hold head in the middle to turn and look
left and right and asymmetry (i.e. a difference between two sides of body
or body too stiff or too floppy)
c. Vision: Unusually short attention span; will only look at you if he or she
hears you, turning or tilting head to use only one eye to look at things,
eyes that cross, turn in or out, move independently, no interest in small
objects and pictures, and constant jiggling or moving of eyes side-to-side
(roving)
d. Speech and language: Lost vocalization
e. Cognitive : Does not make sounds to get attention, does not search for
dropped or hidden objects and child does not respond to caregiver
interactions
f. Social : Will not show interest or participate in social situations,not
laughing in playful situations,hard to console, stiffens when approached
g. Emotional: Will not seek comfort when upset
3) 18 month of age
a. Fine motor: Infant is unable to hold or grasp an adult finger or a toy/object
for a short period of time, unable to use hands in a variety of ways,

turning, twisting, throwing, etc and unconsistently ignores or has difficulty

using one side of body, or uses one hand exclusively
a. Gross motor: Baby is unable to hold head in the middle to turn and look
left and right and asymmetry (i.e. a difference between two sides of body
or body too stiff or too floppy)
b. Vision: Eyes that itch or burn; sensitivity to bright light and sun, unusually
short attention span; will only look at you if he or she hears you,
avoidance of tasks with small objects, turning or tilting head to use only
one eye to look at things, eyes that cross, turn in or out, move
independently, and constant jiggling or moving of eyes side-to-side
c. Speech and language: Failure to do what they may to do
d. Cognitive : Does not imitate simple actions, does not show any
understanding of cause and effect. does not search for objects when moved
from within sight to out of sight
e. Social : Does not explore the environment, very passive responses, does
not show preferences and dislikes, child ignores, avoids or is hostile with
caregiver after separation, and shows little fear towards a new room or
stranger
f. Emotional: Facial expression shows little variation, child shows few
emotions, does not seek comfort in a person or object when distressed
4) 12 month of age
a. Fine motor: Unable to use hands in a variety of ways, turning, twisting,
throwing, etc
b. Gross motor: Unable to walk with heels down
c. Vision: Eyes that itch or burn; sensitive to bright light and sun, avoidance
of tasks with small objects, turning or tilting head to use only one eye to
look at things
d. Speech and language: Lack of face to face or eye contact and loss of
speech
e. Cognitive : Misses small objects when reaching for them and does not use
trial and error to solve problems
f. Social : Does not make face to face contact during play or any interactions,
does not show affection for familiar people or objects, child kicks, bites
and scream easily and without provocation, and rocks back and forth

g. Emotional: Failure to talk about feelings, show preferences of likes and

dislikes, show pride and pleasure at new accomplishments and xpress
negative feelings
Department of Maternal and Child Health. 2010. Infant/Toddler
Development, Screening, and Assessment. Chapel Hill : The University of
North Carolina