MICROTIA

Average adult female ear is 59 mm tall and the

average male is 63mm tall.
85 % of height of ear is achieved by 3 years of
age and almost 100% by 10 years of age.
The size of ear remains the same size till the
age of 60 when it gradually enlarges
particularly the lobe.
The average adult ear protrudes 19 mm from
the mastoid skin
 The long axis of the ear slopes backwards in
line with the slope of the nose.
The ear lies between the level of the eyebrow
and a line of few millimetres beneath the
nasal columella.
The distance between the lateral canthus of
the eye to the front of the ear is just over the
length of the ear.
EMBRYOLOGY OF EAR
 During the sixty week of intrauterine life the
external ear begins to develop around the
dorsal end of the first branchial cleft. On either
side of this cleft lie the first (mandibular) and
second (hyoid) arches.
The auricle develops from these arches as 6
small buds of mesenchyme known as the six
hillocks of His.
The first arch gives rise to the hillocks 1 to 3 and
the second arch gives rise to hillocks 4 to 6.
 There has been always a
HILLOCKS PART OF PINNA
controversy regarding
formation of which part of 1 ANTERIOR PORTION
ear from which hillock. OF EAR LOBE

The auricle begins to 2 TRAGUS

develop in the anterior neck 3 ASCENDING HELIX
region, then it is postulated
to migrate dorsally and 4 ANTI-HELIX

cephalad as the mandible 5 HELIX

begins to develop during 6 POSTERIOR
the second and third PORTION OF LOBE.
months of gestation. By the
5th month of gestation the
pinna lies in its adult
location.
 The external auditory canal begins to develop from
the first branchial cleft during the first two months of
gestation.
During the first month a solid epithelial cell rest
forms in this area and is in contact with the
endoderm of the first pharyngeal pouch. There is an
intervening mesoderm preventing direct contact
between the ectoderm and endoderm
MICROTIA & CONGENITAL AURAL ATRESIA
 DEFINITION
Microtia:
Defined as the abnormal development of the
Pinna resulting in a malformed auricle.
Congenital aural atresia:
Defined as a failure of development of external
auditory canal.

Congenital aural atresia is always associated

with a certain degree of microtia.
 EPIDEMIOLOGY
Microtia and congential auditory atresia occur in
approximately 1 in every 6000 live births.
These deformities commonly occur unilaterally, more so
on the right side.
Men are affected thrice as common as women. The
degree of auricular deformity usually correlates with the
degree of middle ear deformity.
The incidence of inner ear deformities are very rare in
patients with congenital auditory atresia.
Microtia is associated with other anomalies of face 50%
of the time.
Women with four or more pregnancies are at increased
risk of bearing a child with microtia.
The incidence of microtia is higher in Japanese
population.
 ETIOLOGY
Exposure to teratogens like vitamin A, thalidomide, isotretinoin
Vascular insults and genetic aberrations.
Isolated microtia can occur with branchial arch anomalies.
Syndromic associations of with congenital aural atresia include
Goldenhar syndrome,
mandibular facial dysostosis,
brachio-otorenal syndrome
Hemifacial microsomia
Sticklers syndrome
Crouzons syndrome
Noonan syndrome
Foetal Alcohol Syndrome
CHARGE
 PATHOGENESIS
CONGENITAL AURAL ATRESIA
At 28 weeks, a core of ectoderm canalizes from medial to
lateral and eventually breaks through to communicate with
the conchal depression.
Failure of canalization or more rarely lack of ectodermal
migration can lead to atresia of the external auditory
meatus (EAM) and partial canalization leads to meatal
stenosis (an EAM with a diameter of less than 4 mm).
Disruption of normal canalization or ectodermal migration
can lead to arrested development of the tympanic ring
mesoderm with the formation of a dense atretic bony plate
in place of the tympanic membrane, an almost universal
finding in canal atresia.
MICROTIA
Malformation, such as anotia and microtia,
are likely to be caused by the disturbance of
development at seven to eight weeks
gestational age, whereas deformations (lop,
cup and prominent ears) are caused by a
problem later in the development or by
external compression.
GRADING OF MICROTIA
 CLASSIFICATION OF AURAL ATRESIA
GROUP DESCRIPTION
Grade I TM hypoplastic. Tympanic bone is normal
or hypoplastic. Ossicular malformation
present but stapes is usually mobile
Grade II Atretic plate present. Typanic bone is
normal or hypoplastic. Tympanic cavity
within normal limits. (In Marquets
modification, Course of facial nerve is
normal in sub-type a and is abnormal in
sub-type b. In Cremers modification,
there is partial bony atresia in sub-type a
and total bony atresia in subt-type b)
Grade III Above abnormalities may be found with a
severely hypoplastic tympanic cavity.
 MANAGEMENT
Investigations :
Age appropriate Hearing assessment
HRCT Temporal bones
X-ray Cervical spine
USG for congenital renal malformations
Panorex for malocclusion
 OPTIONS FOR MICROTIA MANAGEMENT
Type Details Advantages Disadvantages

Observation No Risk Appearance

Prosthetic Adhesive retained Appearance Less secure attachment

Ongoing prosthetic care
Daily maintenance Use
restrictions
Implant retained Appearance Multiple procedures
Secure retention Requires removal of remnant
and soft tissue Ongoing
prosthetic care Daily
maintenance Use restrictions

Reconstruction Rib Cartilage Autogenous tissue Inconsistent appearance

(Autogenous) Minimal maintenance Donor sites Multiple
Becomes sensate surgeries Reconstruction
Atresia repair performed between 6 and
10 years of age

Medpor Less donor site Foreign body

morbidity Less More challenging to
variability in carving integrate with atresia repair
Reconstruction
performed at earlier
age
 MICROTIA RECONSTRUCTION WITH
AUTOGENOUS CARTILAGE
The technique was refined by Tanzer, father of Otoplasty.
Tanzer surgery: Performed in four stages. There is a three
month gap between these stages.
First stage: Rib cartilage is harvested and sculptured into
the shape of pinna and is placed under skin pocket of the
microtic ear.
Second stage: Formation of the lobule
Third stage: Elevation of the ear with insertion of a post
auricular skin graft
Fourth stage: Formation of the tragus with a skin/cartilage
composite graft from the contralateral ear and full-
thickness skin graft for the conchal area from the
contralateral ear
 Nagata technique: This is a two staged procedure
developed by Nagata. This involves constructing the
auricular framework form the sixth to ninth rib
cartilages. The framework is created using stainless
steel sutures. There is six months gap between the
two stages.
Stage 1: Cartilage Implantation and Lobule
Transposition
Stage 2: Elevation of the Ear and Creation of Post-
auricular Sulcus
COMPLICATIONS OF MICROTIA SURGERY

MAJOR
hematoma formation, skin flap necrosis, infection,
and pneumothorax.
MINOR
malpositioning, scar contracture or hypertrophy,
and poor contour.
MANAGEMENT OF CONGENITAL AURAL ATRESIA

When associated with microtia, congenital aural atresia

reconstruction may be undertaken before microtia
reconstruction, after completion of all stages, or in
combination with a stage of microtia repair.
Historically, most authors advocate CAA repair to follow
three stages of microtia reconstruction: cartilaginous
auricular reconstruction, lobule rotation, and postauricular
elevation and skin grafting.
Currently, the optimal age to begin auricular reconstruction
is generally considered age 7 years.
For the occasional case of CAA without microtia and for
CEACS, the atresiaplasty may be undertaken after the age
of 4 years.
 Jahrsdoefer et al. proposed a scoring system
for the selection of patients with congenital
aural atresia using a ten-point scale based on
CT-scan elements and the appearences of
external ear.
Score 8-10 achieved post-operative SRT of 5-
25 dB.
 SCORING SYSTEM FOR CANDIDACY FOR SURGERY FOR
CONGENITAL AURAL ATRESIA
SCORE
Stapes present 2
Oval window open 1
Middle ear space 1
Facial nerve 1
Malleus incus complex 1
Mastoid Pneumatization 1
Incus stapes connection 1
Round window 1
Appearance of external ear 1
Total availabl points 10
 Prognostic Rating Scale
10 Excellent
9 Very good
8 Good
7 Fair
6 Marginal
5 Poor

de Alarcon A, Jahrsdoerfer RA, Kesser BW. Congenital absence of the oval window: diagnosis, surgery, and audiometric outcomes. Otol
Neurotol. 2008;29:2328.
 SURGICAL APPROACHES OF
ATRESIAPLASTY
Three possible approaches can be followed for
congenital aural atresia repair. They are:

1. Anterior

2. Modified anterior

3. Mastoid
 ANTERIOR APPROACH
Is the most common approach used these days.
In this approach a post auricular incision is made and the
subcutaneous tissue and periosteum are raised anteriorly
up to the level of glenoid fossa.
If any remnant of tympanic bone is present drilling is
started at the cribriform area, and if no tympanic bone is
present the drilling begins at the temporal line just
posterior to the glenoid fossa.
Drilling is continued anteriorly and medially till
epitympanum is entered.
The most common anamoly encountered in the middle ear
of these patients is a fused malleal - incudal joint. Stapes is
usually normal in these patients.
 The atretic bone is carefully removed uncovering the
ossicles.
The facial nerve usually lie medial to the ossicular mass,
and must be protected at all costs.
Drilling is continued till the canal is about 10mm in size.
Ossicular chain reconstruction is performed and a neo
tympanum is fashioned using temporalis fascia graft.
Split thickness skin graft is used to line the external
auditory canal.
A wide meatoplasty is fashioned and a large wick is
inserted to stent the canal.
 MODIFIED ANTERIOR APPROACH
This approach is used in patients with a thick atretic plate
because of poor orientation during dissection.
This poor orientation may risk carotid artery, facial
nerve, and lateral semicirular canal to injury.
Orientation in these patients could be achieved by an
initial posterior dissection up to the level of sinodural
angle. This enables the surgeon to identify the level of
lateral canal and ossicular mass. From here on the
approach is similar to that described under anterior
approach
 MASTOID APPROACH
In this approach the external auditory canal is
created at the expense of mastoid cavity.
It involves drilling out the mastoid and
identifying the sino-dural angle.
This is a risky procedure because of distorted
anatomy of the facial nerve in these patients.
 COMPLICATIONS OF ATRESIAPLASTY
Facial Nerve Inury Transient or Permanent
SNHL
External auditory canal wall stenosis
Lateralized tympanic membrane causing CHL
Recurrent acute otitis, otitis media with effusion,
retraction pockets, cholesteatoma, mastoiditis,
chronic suppurative otitis media, tympanic
membrane perforation, or any other disease of
the ear