6. •Week 6: Hillocks begin to form around
1st and 2nd branchial arch and a process
of fusion produce a primitive ear in 50
days old embryo.
•Starts low and anteriorly then migrates
dorsally and cephalad during months
2-3.
•In final position at month 5.
7.
8.
9.
10.
11.
12.
13.
14. The external canal starts to hollow out
(recanalize) during the 6th month and progresses
from medial to lateral.
Arrest of recanalization process leads to the
various deformities seen in atresia.
17. Malformation of External Ear may be
related to a
Size of auricle
Microtia
Macrotia
Anotia
Shape of auricle
Cup shaped
Lop ear
Dysplastic
Elfin ear(Pointed ear)
Lob malformation
18. Position of the ear
Melotia (ear located on cheek due to
lack of aural ascent)
Synotia (low set ear very close to each
other in midline)
Aurical fistula/sinus
Aurical appendages
External acoustic meatus
Atresia
Duplication
Septation
19. Microtia
An under development of external ear
resulting in deformed auricle
–Ranges from mild deformity to complete
agenesis of ear.
–1:10000-20000 births
–Most often unilateral
–Right side predominance (twice left)
–2.5:1 Male to female ratio
–Associated with congenital aural atresia
20. Etiology
In most cases, causes is unknown.
Congenital microtia can occur sporadically as an
isolated congenital anomaly, associated with other
anomalies or as a part of recognized syndrome.
In 15% cases, genetic & enviremental causes like foetal
alcohal syndrome, maternal diabetic embryopathy,
thalidomide and isotretinoin exposure.
21. Microtia classification
system
•Type I: Mild deformity, major
structures present to some degree,
no tissue needed
•Type II: major structures present, but
tissue deficit to degree additional
tissue needed
•Type III: Few recognizable landmarks,
lobule usually present to some
degree
•Type IV: anotia
25. Prosthetic ear
Osseo-integrated anchoring device
Indication:
–Failed autogenous reconstruction
–Severe soft-tissue/skeletal hypoplasia
–Low or unfavorable hairline
–Acquired total or subtotal auricular defect,
usually in adults –cartilage may be unfavorable
prosthesis changes every 2 to 3 years
–$2000-4000
Meticulous hygiene at skin/implant interface
Preclude future autogenous reconstruction
27. Surgery
Timing of repair
Minimum of 6 years old
–Ear is 85-90% of adult size at this age
–Need time for sufficient rib cartilage to grow
•Ribs Not85% adult size at this age
•Rib cartilage retains growth potential of source
–Psychologic issues start around this age
Any surgical correction of the external ear
needs to consider the effect of surgery on
possible EAC or middle ear reconstruction
29. Brent Reconstruction Technique
Modification of Tanzer technique
Four stage technique
–1: Rib harvest of construction of framework
–2: Lobule interposition
–3: Elevation of ear
–4: Tragus construction
Minimum of two months between first and
second and then three months between
subsequent stages
30. Nagata Reconstruction
Multiple variations of Brent technique,
Nagata being most popular
Two-stage technique
–Stage I: fabrication of auricular framework
from ipsilateral costal cartilage, tragus
reconstruction, and lobule transposition
–Stage II: framework elevation
31. Medpor reconstruction
No rib harvest
Earlier age of repair
Excellent contour and shape
Shorter learning curve?
Does not grow with patient
Implant extrusion
Need for TP flap
33. Macrotia
Auricle is very large but well shaped
Most exaggerated part is scaphoid fossa
Causing psychological disturbances
Associated with
Marfan syndrome
Cerebro-oculo-facial-skeletal syndrome
Fragile X-syndrome
Variant of De Lange type 2 syndrome
Anophthalmia Plus sundrome
41. Lop ear
External ear stands away
from head at agreater
angle
Autosomal Dominant
Inheritance
Resemble fetal stage of
pinna development
Associated with Ehler –
Danlos syndrome
42. Lobe formation
Adherent lobe – male ›
female
Cleft lobe (coloboma)
Abscent lobe- Seckel
syndrome
Hypertropic thickened
lobe
Lobe malformation may be
part of part of
Wolf-Hirschhorn
syndrome
Beckwith-Wiedmann
syndrome
43.
44.
45. Darwinian tubercle
A small projection
from the decending
part of helix.
Darwin regarded this
variation as a remnant
of the pointed ears of
some arthropods.
46. Melotia
Ear located on cheek
Due to lack of aural ascent
Due to underdevelopment of the
auricle
47. Low set ear
Below an arbitary line
drawn between the
lateral canthus of eye &
occipital protruberance
Multifactorial etiology
Associated anomalies
are Noonan syndrome,
Pena –shokeir
Phenotype, Trisomy -18
48. Synotia
Agnanthia-Synotia-
Microstomia
Ears are very close to
each other due to
absence or hypoplasia
of mandible . The
external ears assume a
horizantal position
with the lobule located
near the midline.
49. Auricular appendages
Tag of skin with or without a cartilaginous base
Frequently located in the line of junction of the
mandibular and hyoid arches.
May be sessile or pedunculated.
Location
in front of auricle
with in the ear
behind the ear
on the lobule
May associated with Macrotia, Melotia or oblique facial
features
53. PAS
Blind ending narrow tubes or pits.
Location- Antrerior margin of ascending limb of helix,
center of lobule, calloaural.
Most are harmless but get infected, form retention
cysts & causes chronic discharge from sinuses.
Autosomal Dominant Inheritance with variable
expressivity and incomplete penetrance.
Treatment- Excision under local/ General anaesthesia.
54. Congenital anomalies of External
Acoustic Meatus
Atresia – It may be of osseous or membranous portion.
Seen in severe cases of Mandibulo-facial
dysostosis.
Duplication- Blind ended accessary canal above or
below the cnal leads to the drum.
Septa- Dividing the external auditary meatus .
Change in curvature of canal.
55. Atresia
Embryology – 7th Month
Canalization complete
Mastoid separation from mandible
Normal posterior-
inferior growth
No mastoid
growth
Normal Atresia
56. Epidemiology
1 in 10,000 to 15,000 births.
Up to 50% of the time associated with some
craniofacial syndrome.
Unilateral : Bilateral, about 3:1
30% are bilateral
Atresia : Microtia, 7:1
Slightly more common on the right
Male : Female, 2:1
57.
58.
59.
60. Syndromic association
Microtia with aural atresia associated with various syndromic conditions such
as
Treacher Collins (Mandibulofacial Dysostosis)
Nager Syndrome (Acrofacial Dysostosis)
Cruzoun’s Craniofacial Dysostosis
Goldenhar’s Syndrome
Sticklers
Foetal alchohal syndrome
CHARGE
Hemifacial Microsomia
Noonan syndrome
61.
62.
63. Goldenhar
Oculo-auricular-vertebral
syndrome.
Characterized by incomplete
development of the ear, nose,soft
palate, lip, and mandible.
It is associated with anomalous
development of the first branchial
arch and second branchial arch.
Common clinical manifestations
include limbal dermoids,
preauricular skin tags, and
strabismus.
64. Hemifacial microsomia
congenital disorder
that affects the
development of the
lower half of
theface, most
commonly the ears,
the mouth and the
mandible
65. Stickler syndrome
Autosomal dominant condition.
Presenting with cleft palate, micrognathia, severe
myopia and retinal detachment associated with
conductive hearing loss.
Some cases have a progressive high sensorineural
hearing loss.