Congenital malformations of external ear are the most common malformations presented to otolaryngologists and facial plastic surgeons. Hence in the pursuit of in-depth knowledge, a seminar on the topic was presented by me in the month of May 2017 at ENT-HNS dept, Silchar Medical College.
• 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
• The distance between the lateral canthus of
the eye to the front of the ear is just over the
length of the 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
• The auricle develops from these arches as 6 small
buds of mesenchyme known as the six hillocks of
• 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
formation of which part of
ear from which hillock.
• The auricle begins to
develop in the anterior neck
region, then it is postulated
to migrate dorsally and
cephalad as the mandible
begins to develop during
the second and third
months of gestation. By the
5th month of gestation the
pinna lies in its adult
HILLOCKS PART OF PINNA
1 ANTERIOR PORTION
OF EAR LOBE
3 ASCENDING HELIX
PORTION OF LOBE.
• The external auditory canal begins to develop from
the first branchial cleft during the first two months of
• 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
• Very common
• Involves only skin but
usually contains a long
tail of cartilage
extending into the
• Liga clip – tag drops off
• Vertical elliptical
MIRROR EAR OR POLYOTIA
• Persistent pre-auricular
tissues lying on posterior
cheek resembling extra
• Skin is peeled off the
trimmed & packed into
anterior conchal hollow.
Skin redraped to give
cheek flatter shape
• Often bilateral &
• Track deeply to facial
• Excision with facial
ABNORMAL FOLDS OF PINNA
• Lop ear - Upper pole of the ear flops over
• ‘Mustarde’ type suture used to create U-shaped
cartilage prop at missing upper anti-helical fold. Ear
hitched to the mastoid fascia
• Other varieties are –
Kink of helical rim
Abnormal fusion of helical rim to the anti-helical fold
Whole ear appears collapsed vertically
• Surgical correction by scoring, tie-bar type
tethering sutures or direct wedge excision
PROMINENT (‘BAT’) EARS
• Prominence is due to an
absent anti-helical fold but in
some cases, the conchal bowl
is excessively deep
• Grading by Graham and Gault.
• Digital pressure on relevant
part of ear determines cause
and gauge strength of
• Recommended to do surgery
only after 5 years of age.
• Surgical techniques – anterior
scoring, reshaping of the
curves by the use of posterior
sutures and excision
techniques to set back the
• Very challenging surgery
• Scaphal hollow folded backwards to rest on conchal
• Surgical reconstruction by releasing the soft tissue
tethering between scaphal and conchal cartilages and
splinting these structures apart with a cartilage graft (
FOLDED OVER HELICAL RIM
– Helical rim sharply folded over providing double layered in
a single skin envelope, ear looks pinched.
– Folded over cartilage excised and then repositioned.
– Noonan Syndrome
– Bump on helical rim
– Hillocks 3 & 4 irregular fuison
– Groove or notch due to
poor fusion of hillocks 6 &
– In few, significant portion
of lobe mission due to
absence of hillock 6.
– Re-alignment of deep
tissues with deep suture.
– Rebuilt around cartilage
framework using conchal
• Excessively large ears.
• In normal ear, upper pole,
conchal hollow and lobe
take up an equal amount
of the height of ear,
splitting in to thirds.
• Big scaphal hollow or big
lobe are the reasons of
• Anterior crescent of skin
and cartilage be removed
from scaphal hollow.
• Wedge of tissue is
removed from oversized
• Helical rim is constricted to give prominent,
cone shaped ear.
• Difficult to correct.
• Several techniques used that include V-Y
plasty, recnstruction using carved costal
CRYPTOTIA ( ‘THE HIDDEN EAR’ )
• Lower two thirds of ear visible,
upper auricular sulcus seems
• When ear is pulled away from
the side of the head, the upper
pole cartilage becomes evident,
having hidden beneath scalp
• Upper pole is tethered and
lower lobe is prominent.
• Small ear buddies splint applied
at birth for non-surgical
correction. Later surgical
correction by releasing the
tethered portion of ear.
Defined as the abnormal development of the Pinna
resulting in a malformed auricle.
• Congenital aural atresia:
Defined as a failure of development of external
Congenital aural atresia is always associated with
a certain degree of microtia.
• 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
• 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.
• 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
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.
• 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
• Marx ‘s classification of external ear has proposed
four descriptive groups which was later amended
by Aguilar and Jahrsdoefer enabling the surgeon
to categorize auricular deformity easily and into
• Altman gave anatomical classification of
congenital aural atresia which was later modified
by Marquet and Cremers.
• De la Cruz classification of congenital aural atresia
based on HRCT scan is also used frequently by
GRADING OF MICROTIA ( MARX )
Slightly small or
Often there is no
GRADE I MICROTIA
An ear with
deficiencies such as
rim, missing helix
GRADE II MICROTIA
deformity with the
GRADE III MICROTIA
CLASSIFICATION OF AURAL ATRESIA
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 Marquet’s
modification, Course of facial nerve is
normal in sub-type ‘a’ and is abnormal in
sub-type ‘b’. In Cremer’s 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.
DE LA CRUZ CLASSIFICATION
• Mastoid Pneumatization
• Inner ear normality
• Facial nerve
DE LA CRUZ CLASSIFICATION
• Normal mastoid
• Normal oval
• Good facial nerve–
• Normal inner ear
DE LA CRUZ CLASSIFICATION
• Abnormal or absent
• Abnormal course of
• Abnormalities of
Age appropriate Hearing assessment
HRCT Temporal bones
X-ray Cervical spine
USG for congenital renal malformations
Panorex for malocclusion
Type Details Advantages Disadvantages
Observation No Risk Appearance
Prosthetic Adhesive retained Appearance Less secure attachment
Ongoing prosthetic care
Daily maintenance Use
Implant retained Appearance
Requires removal of remnant
and soft tissue Ongoing
prosthetic care Daily
maintenance Use restrictions
Reconstruction Rib Cartilage
Donor sites Multiple
performed between 6 and
10 years of age
Medpor Less donor site
variability in carving
performed at earlier
More challenging to
integrate with atresia repair
OPTIONS FOR MICROTIA MANAGEMENT
MICROTIA RECONSTRUCTION WITH
• 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
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
• 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
– Stage 1: Cartilage Implantation and Lobule
– Stage 2: Elevation of the Ear and Creation of Post-
COMPLICATIONS OF MICROTIA SURGERY
– hematoma formation, skin flap necrosis, infection,
– 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
• Score 8-10 achieved post-operative SRT of 5-
SCORING SYSTEM FOR CANDIDACY FOR SURGERY FOR
CONGENITAL AURAL ATRESIA
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
SURGICAL APPROACHES OF
• Three possible approaches can be followed for
congenital aural atresia repair. They are:
2. Modified anterior
• 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
• 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
• 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
• 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
• 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
• 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
CONGENITAL EXTERNAL AUDITORY
• Congenital external auditory canal stenosis (CEACS) is
defined by Jahrsdoerfer et al. as an EAC with a
diameter of less than 4 mm.
• A four level classification of CEACS was developed by
• Meatal stenosis occurs as a result of partial lack of
embryologic canalization of the EAC from the usual
medial to lateral direction.
• Isolated meatal stenosis may be repaired, if severely
narrow, via canaloplasty with drilling of the bony ear
canal and thinning of the soft tissues with skin flap
ALTERNATIVES FOR CONGENITAL AURAL ATRESIA:
IMPLANTABLE HEARING AIDS IN CHILDREN
• Several types of implantable hearing aids may
be used for hearing rehabilitation of CAA in
children over 5 years of age, as an alternative
– percutaneous (crosses skin) bone-conduction
devices (BAHA, Ponto),
– transcutaneous (closed-skin) bone-conduction
devices (Sophono), and
– active middle ear implants (Vibrant Soundbridge).
• Was first described by Tjellström and colleagues.
• BAHA is considered the gold standard in terms of closure of
the air-bone gap and the air-conduction PTA gain in
children with CAA.
• The BAHA/Ponto may be useful for children with CAA who
have unfavorable anatomy for reconstruction, those who
are poor candidates for a lengthy atresiaplasty procedure
for medical reasons, or for families that desire good hearing
with less surgical risk.
• The standard surgical procedure includes the placement of
a titanium fixture in the temporal bone with the reduction
of the subcutaneous tissue and creation of a zone of
alopecia around the abutment
• The standard surgical procedure includes the
placement of a titanium fixture in the temporal
bone with the reduction of the subcutaneous
tissue and creation of a zone of alopecia around
• The planned site for BAHA placement is 5.5 mm
posterosuperior to the EAC or planned site of the
• It is 6.5 to 7.0 cm from the planned site of the
EAC in ears with planned future microtia
reconstruction, rather than the usual 5.5-cm
• The image of the BAHA shows how a button (or stud
if you prefer) is inserted into the bone of the skull
above the ear.
COMPLICATIONS OF BAHA SYSTEMS
– Failure of osseointegration
– Chronic infection
• Soft tissue
– Irritation of the skin surrounding the implant
– Skin flap necrosis (death of the skin flap, e.g. due to
interrupted blood supply)
– Overgrowth of skin over the device
– Wound dehiscence (splitting apart of the wound)
– Bleeding or hematoma formation
– Persistent pain
The BAHA device is attached to the
button within the skull. It is unobtrusive
(once the hair is put back into place).
(image is from Island Hearing. )
The PONTO device is attached to an
abutment (i.e. stud) behind the ear
(image is from Ponto web site. )
The SOPHONO device is attached using a
magnet (image is from Sophono web