
Width 55% of length

Helical rim protrudes 15 – 20mm from skull

Auriculocephalic angle 21 – 25


Conchalscaphal angle 75-105


Long axis tilted supero-posteriorly 20-30

from face

Angle between ear axis and bridge of nose is about 15 degrees

The ear is positioned 1 ear length (60-65mm) posterior to the lateral orbital rim between eyebrow and columella

Earlobe at nasal tip

Helix attaches to head at level of lateral canthus

Highest point helix level with arch of eyebrow

Ext aud meatus at midpoint between eyebrow and nasal tip

From Melnick’s study of 56000 pregnancies,

Any ear abnormality 1.1%

Severe abnormalities occur in 1 per 7000 births

Cultural variation - higher in Asians(1 in 4000), Navajo (1 in 1000)

Multifactorial inheritance

M:F 2:1

10% bilateral

50% had features of FAVS = Facioauricularvertebral sequence; aka first and second branchial artch syndrome. Includes 3 disorders: o Goldenhar syndrome/mildest
Most if not all signs of HFM but 10-33% bilateral +/- CLP, coloboma, dermoid cysts, cardiac and renal defects, vertebral fusion, N intelligence o Hemifacial microsomia/intermediate o Oculoauriculovertebral disorder/most severe

Most likely multifactorial

Theories:
1.
Vascular accident in utero
2.
Prenatal infections and teratogens including isotretinoin, thalidomide , and maternal rubella.

5% have immediate family history

30% associated with syndromes
1.
FAVS, oculoauriculo vertebral dysplasia, Goldenhar
2.
Treacher Collins syndrome (ie, mandibulofacial dysostosis, Franceschetti syndrome). a.
AD; TCF gene isolated to chromosome arm 5q; 1 in 50,000 b.
60% of cases of TCS arise de novo c.
abnormalities of the external ears, atresia of the external auditory canals and malformation of the middle ear ossicles, which result in bilateral conductive hearing loss d.
lateral downward sloping of the palpebral fissures with colobomas of the lower eyelids and a lack of eyelashes medial to the defect; e.
hypoplasia of the mandible and zygomatic complex f.
cleft palate
3.
Nager syndrome (acrofacial dysostosis) a.
atretic external auditory canals, down slanted palpebral fissures, a high nasal bridge, malar hypoplasia, and micrognathia

b.
Preaxial limb malformations include absent or hypoplastic thumbs, hypoplasia of the radius and shortened humeral bones
4.
Miller syndrome a.
AR b.
postaxial acrofacial dysostosis
5.
CHARGE syndrome: a.
Sporadic inheritance b.
C oloboma of eye (esp iris), H eart defects incl tetralogy of fallot,
VSD/ASK/PDA, A tresia of nasal choanae, R etardation of growth or development, G enital and urinary anomalies, E ar abnormality such as malformation of outer ears and bones of middle ears, improper function of eustachian tubes/obstruction of ear canals and hearing loss.
+/- Craniofacial abnormality, tracheoesophageal fistula and/or imperforate anus.
60-70% of microtia isolated. Associated defects:
1.
facial clefts and cardiac defects are the most common (30 percent)
2.
anophthalmia or microphthalmia (14 percent)
3.
limb reduction defects or severe renal malformations (11 percent)
4.
holoprosencephaly (7 percent).

Head and neck examination emphasizing:
1.
Facial asymmetry and craniofacial assessment
(configuration, symmetry, facial proportions, masticatory apparatus, occlusion, hair and skin condition, sensory function, speech, voice and deglutition, cleft palate)
2.
retrognathia or other airway concerns common to this group
3.
integrity of the facial nerve (Congenital CN7 palsy a/w ear anomalies and documented for medicolegal reasons after middle ear surgery)
4.
quality of non–hair-bearing skin in the vicinity of the auricle
5.
hairline
6.
position of the remnant auricle, and future lobule; preauricular tags/pits associated
7.
condition of the contralateral ear.
8.
Associated syndromes may be a/w internal organ dysfunction
( e.g. heart and kidneys), the nervous system and the skeleton (e.g. cervical spine) must be excluded by an interdisciplinary team (e.g. pediatrician, neurologist and orthopedist).

Tanzer (1977) - NB
I.
Anotia (very rare)
II.
Complete hypoplasia (microtia) a. with atresia of ext auditory canal b. without atresia of ext auditory canal
III.
Hypoplasia of middle third auricle
IV.
Hypoplasia of superior third a. constricted (cup and lop) b. cryptotia c. hypoplasia of entire third
V.
Prominent ear
Even with extremely small microtic remnants a lobular component is almost always present, but vertically orientated and superiorly displaced.
Nagata
Based on surgical technique required for correction
1.
Lobule type (remnant ear and ear lobule without meatus, concha, tragus)
2.
Concha type (remnant ear, lobule, meatus, tragus)
3.
Small Concha (remnant ear and lobule with small indentation for concha)
4.
Clinical Anotia
5.
Atypical
Staged reconstruction
Number of stages varies according to technique and amount of additional procedures.
Timing

Need to consider
1) ongoing growth of ears
2) contralateral ear characteristics
3) Costal cartilage development
4) Social ridicule
5)
Child’s cooperation
6) middle ear reconstruction, if planned
 should be done after auricular reconstruction as often leads to scarred, poorly vascularized tissue in the mastoid area and will compromise the quality of the soft-tissue cover for the framework.

At age 6 ribs area at satisfactory size, ear within 6 mm of full height, beginning of peer ridicule and child will probably cooperate


If the patient is small for age or the opposite ear is large may consider postponing surgery.

Tanzer showed comparable increases in height in both ears after reconstruction. i.e. the framework will grow concomitantly (he attributed this to intact perichondrium)
Preoperative

Preoperative photos

Xray film pattern is traced from the normal ear o Landmarks in relation to nose, lateral canthus can also be marked. o Patterns can be reversed and sterilized to use intraoperatively.

Position – height and vertical position of ear, vertical angulation in relation to nose, lateral canthus

In severe hemifacial microsomia, ear position is placed halfway between the vestige site and the markings from the contralateral ear. (Brent)

Size – Tanzer designs the ear 2-3mm larger than the contralateral side; Brent keeps in the same or even slightly smaller

Four-stage technique:
1) framework placement;
2) lobule transposition;
3) tragus construction and conchal excavation;
4) elevation of the ear framework with creation of the auriculocephalic sulcus.

Brent has modified this to include tragal reconstruction as part of Stage 1

Interval between stages 3 months (Tanzer = 4 months) (DOD = 6/12)

Contralateral rib cartilages harvested through a costal margin incision. o David Gillet takes split thickness contralateral ribs with periosteum o In bilateral cases, use shape of mother’s ears for template model

Synchondrotic region of the 6 th
and 7 th
ribs is used for the base plate.
 helical rim from the first floating rib (usually 8 th
)

Need 9-10cm rib cartilage on average to reconstruct helical rim (8 th
)

Avoid bony rib/osteochondral junction harvest as difficult to carve

Harvesting the anterior surface perichondrium in continuity with the cartilage helps in shaping the framework and in its vascularization.

Leaving the posterior perichondrium in situ minimizes the deformation as many children lay down some new cartilage in this bed.

A rim of cartilage can be left along the edge of the rib cage to decrease visual deformity at the donor site.


Maximum relief of the construct is essential for the highest quality of reconstruction

Brent – framework tip is made smaller to accommodate the lobule after transposition. If there is no usable lobule, the lower end is carved into a lobule shape

Nagata prefers to use the ipsilateral ribs o Sixth and seventh ribs for the base plate (use reversed/posterior side) o Eighth, and ninth costal cartilages to helical rim and antihelix o Rectangular block for tragal unit


May store some cartilage to bank for ear elevation

Need a high profile framework to achieve adequate projection of the reconstructed ear.

There is a loss of definition after skin cover and so exaggerate helical rim and antihelical complex initially.

Warping achieved in a favourable direction by sculpting and thinning.

Helical segment attached to the framework with sutures.

Alloplastics

Silastic has been used in the past – very high extrusion rate (50%)

Porous polyethylene (medpor) - o Advantage: allows tissue ingrowth o Problems with erosion and exposure related to soft tissue cover o Avoided with the use of TPF flap o Dr John Reinisch California, good results reported but not reflected elsewhere.
DOD ‘spent more time taking out than putting in’ in US
Implantation

Need thin, pliable skin

An incision is made just anterior to the vestige, the skin dissected from the cartilage remnant.

Brent and Nagata describes discarding the remnant ear cartilage completely

Centrifugal relaxation technique.

Dissect pocket preserving subdermal vessels out 1-2cm peripherally.

Suction to the pocket (Brent)

Tanzer and Nagata use bolster sutures

Dressing that conforms to the auricle.
Soft tissue cover

Requirements: o skin must be thin, pliable, hairless o a good match in color and texture, o good vascularity o good elasticity to fit snugly over the underlying skeleton.

Often compromised by low hair line

If inadequate native skin, rely on local fascial flaps. o Temporoparietal fascial flap (superficial temporal artery) used quite commonly especially in conjunction with an alloplastic skeletal support o Deep temporal fascia has been used as a salvage when the TPF flap has become partially necrotic o DOD uses mastoid fascial flap when possible and preserves TPF as bailout/salvage flap
Tissue expansion

Problematic

Does not produce thin pliable, resilient skin. The expanded skin often contracts with time.

The invariable capsule created obscures detail of the implanted framework.
Postoperative

Close monitoring enables most complications to be treated simply.


Infection (antibiotic drainage/irrigation), pressure necrosis, skin necrosis.

Exposed cartilage – dressings, local flap/ fascial flap and skin graft.

Abnormal lobule is vertically orientated and positioned anteriorly

Z plasty transposition of a narrow inferiorly based flap.

Chondrocutaneous composite graft from the opposite ear anterolateral conchal bowl into a J shaped incision at the proposed tragal margin.
 also serves as a setback otoplasty for that ear

In bilateral microtia, the Kirkham method is used: anteriorly based conchal flap doubled on itself.

 framework is separated from the mastoid area taking care to preserve the connective tissue that overlies the cartilage framework.

The scalp is undermined and advanced as far anteriorly as possible (to the new sulcus).

A FTSG is applied to the remaining defect (which should be only the postero-medial aspect of the ear).

Extra projection can be achieve with cartilage grafts placed in the post-auricular sulcus
Other Techniques
Tanzer
1.
Rotation of the lobule into a transverse position
2.
Fabrication and placement of cartilage framework
3.
Elevation of the ear from the side of the head
4.
Construction of a tragus and conchal cavity.
Subsequently combined the first two to enable a three stage reconstruction.
Aguilar combines atresia repair into Brent’s technique
1.
framework construction and placement
2.
lobule creation
3.
atresia repair
4.
tragal creation
5.
auricular elevation
Nagata two stage repair
1.
framework and rotation of the lobule, and conchal excavation
2.
elevation, placement of cartilage graft in auriculocephalic sulcus covered with a temporoparietal fascial flap and skin graft.
DOD: Discuss Prosthetic/Alloplastic/Autologous reconstruction options first
1.
Framework from 6/7/8th costal cartilages (residual cartilage diced and returned to periosteal sleeve), inferior rotation of lobule, post skin flap elevated to cover cartilaginous framework with suction drain
2.
Elevation, crescentric cartilage graft to auriculocephalic sulcus (6/7/8 th
neoribs) and MASTOID fascial flap and skin graft (save TPF for salvage/bailout if reconstruction fails or breaks down)
3.
Scaphoid fossa incision, excavation of conchal bowl, triangular fossa and scaphoid fossa, helix and antihelix thinned and sculped, tragal prominence created with excess cartilage.
Timing: 6/12 apart, Not before 10 years or 9-10cm rib cartilage available, If bilateral prefers to do one side/a time. Firmin Unit Paris harvests cartilage in stage procedures but does second stage bilaterally, concern for nursing two recons at same time.

Problems
Hairline

Hair on the reconstructed rim.

Removed by
1.
Laser
2.
Electrolysis
3.
Lift flap and thin under dermis (risk vascularity of flaps)
4.
excision and grafting.

If there is a tight pocket and the framework is covered with hair bearing skin, may benefit from a temporal fascial flap and skin graft.

If there is a low hair line, Brent makes a smaller ear and does a scaphal crescent excision to reduce the normal contralateral ear – uses the cartilage for tragal reconstruction

Nagata avoids this by using temporoparietal fascia, and an ultra-delicate splitthickness scalp graft
Soft tissue cover
 skin must be supple, thin, and well vascularized to drape over the numerous convolutions of the framework to render an adequate definition.
 may be compromised by the presence of hair, previous trauma, or attempted microtia repair.
 temporoparietal fascial flap has become a workhorse flap
 anatomy and course of the superficial temporal system vary considerably in microtic patients

Complications:
1.
alopecia (28.6 percent)
2.
scalp numbness (17.4 percent)
3.
objection to the visible scar (25 percent in male patients)
 use of fascia combined with a skin graft may also result in less definition in the reconstructed ear compared with that using a supple skin flap

To overcome this shortcoming, the cartilage construct can be carved to a higher relief and the conchal vault expanded to accommodate for the additional thickness of the composite soft-tissue envelope and for the anticipated skin contraction.
 place the initial drain access sites remote to the superficial temporal system so as to avoid injury to a potential salvage resource, the temporal fascia.
Complications
Chest wall
1.
pneumothorax
2.
atelactasis
3.
Anterior chest wall deformity (>64%)

Asian surgeons prefer to delay ear reconstruction for this reason

4.
Chest wall scarring
Ear
1.
Exposure of the cartilage framework
2.
Skin flap necrosis a.
Pressure dressings should be avoided
3.
Infection (0.5%)
4.
Hematoma (0.3%)
5.
Significant resorption of the framework
6.
Suture/wire extrusion
Secondary Reconstruction

Tanzer – excise scarred skin and cartilage. Skin graft defect and wait until graft maturity then place new framework

Brent and Byrd – temporoparietal flap and skin graft over new cartilage framework after excision of failed reconstruction.

Nagata notes 2 nd
reconstruction is more difficult due to:
(1) all necrotic skin and scar tissue from the primary reconstruction must be removed, thus limiting the surface area of the skin for the secondary
(2) reconstruction; the presence of scar tissue and loss of tensility in the subdermal layer makes it difficult to construct a subcutaneous pocket for grafting of the three-dimensional costal cartilage framework;
(3) in patients with full-thickness skin grafts in the conchal and postauricular regions, contraction of the grafted skin was noted
Method:
(1)
(2) transfers a TPF flap to cover the costal cartilage framework during the first stage of the secondary ear reconstruction.
The second stage consists of elevating the ear and placing a cartilage graft in the auriculocephalic sulcus. The posterior auricle and sulcus are then covered with an innominate fascial flap(highly vascular loose connective tissue below superficial temporal fascia) and a skin graft.


Traditional ear prosthesis o Mechanical retention – with headbands, spectacle frames o Adhesives – problems with dermatitis, reduces the life of the prosthesis

Osseointegrated ear prosthesis first used in 1979 by Branemark

Advantages of autogenous ear reconstruction o Uses patient’s own tissues o Stable o No need maintenance, ongoing costs

Advantages of Osseointegrated ear o Shorter operation time, less stages

Disadvantages: o Regular daily aftercare o Need to remove prosthesis for diving, water skiing, contact sports o Embarassment of dislodgement o May result in compromise of future attempts at reconstruction o Need to replace after 2-3 years

Indications: o Major cancer resection o Radiotherapy o Absence of lower half of ear o Severely compromised local tissues o Patient preference o Failed autogenous reconstruction o Poor operative risk o Severe Craniofacial anomaly

Principles o Ensure stable healed wound o Adequate bone stock o Preferably over nonshearing surface - best over thin immobile SSG firmly attached to periosteum o Some find FTSG superior. o Low torque drill o Insertion of titanium fixture (usually 4mm) o An abutment is then screwed in on top of the fixture o Prosthesis attaches to the abutment


Complications include o Loss of SSG o Implant extrusion o Lack of bone stock o Bleed from sigmoid sinus o Pin site infection o Brain abscess o Growth of skin over abutment
Superiorly based SSG prepared over bone anchored hearing aid

Aural atresia – absent or incomplete EAM

Embryologically, the external ear is formed earlier than the middle ear, so that it is possible to encounter a normal auricle and a malformed middle ear

In the presence of microtia one should not expect to find a normal middle ear.
 most otologists relate the severity of the auricular defect to the status of the middle ear.

Gill believes the presence of a tragus in a microtic ear is an indication of a functional middle ear

The inner ear is rarely involved in microtia - Fukuda states that the presence of a small external auditory canal may indicate a severe mixed type (conductive and sensorineural) deafness, while atresia of the canal with common microtia is usually associated with deafness of a more simple conductive type.

Although the severity of the external deformity appears to correlate with the severity of the temporal bone abnormalities, no such association between the severity of the dysmorphic features and the degree of hearing loss has been noted
 predominant hearing deficit in microtia/aural atresia is conductive hearing loss (80 to
90 percent).

Middle ear deformity ranges from minor dysplasia of the ossicles to complete obliteration of the tympanic cavity.

Stapes is usually normal.
 important to conduct a complete radiographic and audiologic evaluation for every child with microtia, regardless of clinical presentation.

The facial nerve sometimes follows an anomalous course, a fact to be aware of during surgery for reconstruction.
Principles

Recognise differences in treatment in unilateral and bilateral atresia o May need to operate earlier on 1 ear in bilateral cases o Never operate until there is enough cartilage for framework

Grade the microtia

Examine for atresia

Evaluate hearing – can be done on neonates

Immediate hearing aid use with bilateral atresia, probably don’t need it if 1 side has normal hearing

Postpone surgery until age 5-6

Ensure correction of atresia (canloplasty- complication of restenosis/infections+) is not undertaken before commencement of microtia reconstruction o Scarring, reduced skin elasticity o Unreliable blood supply for flaps and cartilage framework o Malposition of ear to fit canal

Diagnostic Evaluation
 audiogram - in older children
 auditory brainstem response (ABR) testing - recommended to accurately determine the degree of sensorineural hearing loss and conductive loss in newborns and infants.

High-resolution CT - provide anatomic detail of the middle ear (age 4-6)
Hearing Aids

Following diagnosis of hearing impairment,
1.
Unilateral atresia - If patient has a unilateral atresia and normal hearing in the other ear there is generally no need for a bone conduction hearing aid. a.
Concern with reconstruction is the degree and predictability of hearing improvement that can be achieved, potential lifetime care of mastoid cavity, and risk to the facial nerve b.
Recent trend is to operate on properly selected patients at the age of 5 or 6 because of the importance of binaural hearing
2.
For most binaural hearing losses, 2 hearing aids are recommended: a.
mounting clinical evidence that indicates that failure to fit hearing aids on both ears of patients with binaural hearing loss can result in temporary and perhaps permanent decrease in the auditory function in the unaided ear. b.
other advantages of binaural amplification, including better sound localization, improvement of speech reception in the presence of noise, improved speech clarity, and more natural and less stressful listening.
3.
Conventional vs BAHA a.
Problems of conventional hearing aid - poor cosmetic result, inferior sound quality and discomfort resulting from the persistent pressure of the aid on the soft tissue over the mastoid. AKA ‘softband in paeds’ b.
Bone anchored hearing aids are better tolerated (90-95% success) i.
Generally placed age 2-10, earlier for cosmetic concerns – auricular prosthesis can be fixed to the anchor ii.
BAHA - implanted after age 2, usually age 5 iii.
Newer models such as BAHA 5/’connect system’ very thin subcutaneously implanted hearing aids ? superior to canaloplasty iv.
Problems
1.
skin irritation
2.
thin bone resulting in incomplete insertion of fixtures
3.
with temporal bone growth, fixtures may need to be revised
4.
NB ask ENT to place posteriorly on scalp to preserve mastoid fascial flap/TPF flap options
4.
Need frequent audiology follow up
5.
At age 4-6, consideration for ear reconstruction. At this time, consider indications for auricular reconstruction

Surgery for unilateral atresia is controversial.


It can be difficult to correct the conductive problem – 50% of patients will have >30 dB of improvement from atresia repair.

Development of binaural hearing requires functional difference between the two ears to be within 15 – 20db .

Most with significant differences are born adjusted to monaural hearing.
Contraindications
1.
Generally no surgery for unilateral atresia if they have a normal contralateral ear i.e. middle ear reconstruction is contraindicated in unilateral microtia. a.
Some would operate on the middle ear if a final air-bone gap of 30 dB or better is anticipated
2.
Predominant deficit is sensorineural
3.
Lack of pneumatization of the mastoid air cells by age 4 denotes inadequate development
Indications
The Jahrsdoerfer rating scale
– grading system out of 10 based on using high resolution CT scan of the temporal bone in conjunction with physical examinatio n:
1.
2 points if the stapes is present
1 point each if there is
2.
an open oval window
3.
adequate middle ear space
4.
normal course of the facial nerve
5.
a malleus-incus complex
6.
good mastoid pneumatization
7.
incus-stapes connection
8.
good external ear appearance
9.
ear canal stenosis with malleus bar

A score of 8 or higher indicates that the patient is a good candidate for atresia surgery.

A score of 5 or less contraindicates surgery, as does a predominately sensorineural hearing loss, complete lack of pneumatization of the mastoid, or obstruction of the mandibular condyle and/or glenoid fossa

Constricted ear encompasses lop and cup types. (N2K Tanzer classification NB)
Lop ear
– downward folding or deficiency in the helix and scapha at level of Darwin’s tubercle. The deformity is associated with a malformed antihelix, usually at the superior crus
Cup ear – prominent ear and lop ear characteristics . Deep concha, deficient superior helical margin and antihelical crura and small vertical height.

Constricted ears have varying degrees of helical and scaphal hooding, and flattening of the antihelix.

Reconstruction is either by reshaping existing tissues or supplementing with skin/ cartilage.

For moderate height discrepancies the ipsilateral cartilage can be modified or augmented with contralateral grafts.
 If there is a greater than 1.5cm height difference skin and cartilage should be added as for a formal microtia repair.

Cosman concludes that
1.
it is seldom necessary to detach the helical crus from the face
2.
there is often more tissue available for the reconstruction in the constricted ear than initially apparent
3.
whenever possible one should avoid procedures that add tissue, as they are more complex and therefore prone to complications and skin color mismatch.

Double banner flap – 2 interdigitating flaps rotated 180

. More elevation can be obtained by using a conchal cartilage graft to fill in the window under the flaps.

Bartel-Friedrich et al.: Classification and diagnosis of ear malformations
Table 3: Various dysplasia grades of the pinna with subgroups according to Weerda [1]
Figure 3: Various cup ear deformities: a = type I, slight deformity; b = type IIA, deformity at slight to moderate level; c = type
IIB, deformity at moderate to strong level; d = type III, severe deformity (from Weerda [1])
of the frequently concomitant middle ear malformations with conductive hearing loss.
The classification of malformations of the EAC according to Weerda [1] describes three types (A-C; Figure 4).
• EAC stenosis type A is a marked narrowing of the EAC along with an intact skin layer.
• EAC stenosis type B shows partial development of the
EAC with an atresia plate at the medial part.
• Type C involves complete bony EAC atresia. Often it is advisable to implement hearing-aid treatment in view
The closely interrelated development of the EAC and the middle ear led to the classification of the combined malformation termed atresia auris congenita according to
Altmann [7]. Three degrees of severity are described:
GMS Current Topics in Otorhinolaryngology - Head and Neck Surgery 2007, Vol. 6, ISSN 1865-1011 10/21

Musgrave radial incisions – a conchal cartilage strut is sutured to the tips to hold position.

Tumbling concha-cartilage flap (Park PRS 2000). flap is bent back on itself and sutured to the lidded helix or scapha. The recoiling force of the flap on the conchal side holds the lidded portion of the helix erect

Congenital deformity in which the upper part of the ear cartilage is hidden beneath the scalp.

The superior auriculo-cephalic sulcus is absent (but can be demonstrated by gentle digital pressure).

The scapha tends to be underdeveloped and the antihelical crus is sharply curved.

Common in Asians 1 in 400

Bilateral in 40%; R>L

Possibly secondary to abnormalities of
1.
the intrinsic transverse and intrinsic oblique ear muscles.
2.
extrinsic superior auricularis

Treatment
Non operative
Splinting is effective if used < 3 mths of age – splint for 6 weeks
Ear is mouldable in first 3 months due to circulating maternal oestrogens
Operative
1.
Requires the addition of skin to deficient retroauricular sulcus a.
skin grafts, z-plasties, V-Y advancements, rotation flaps, TE, preauricular flap
2.
correction of the malformed cartilage
3.
division of abnormal insertion of the intrinsic muscles.

A helical rim deformity.
1.
3 rd
crus (going posterio-superiorly )

2.
Flat helix
3.
Malformed scaphoid fossa
4.
The superior crus may be malformed or totally absen t

Rare in Westerners. More common in Orientals.

Cause unknown.

? in utero compression – unlikely due to frequency of bilateralarity

Skoog reported that third crus deformity was the result of abnormal growth of the perichondrium.

Most believe that Stahl's ear is caused by a developmental error occurring around the third embryonic month, when the helix and scaphoid fossa are developing

? abnormal intrinsic muscle (Yotsuyanagi PRS 1999)
Treatment in neonates is by splintage as cartilage is soft and malleable
Later presentations require operative treatment, but, because of the wide variability of the condition, this needs to be tailored to the patient.
Options:

Z-plasty of the cartilage

Remove, reverse and replace as graft

Remove, rotate and replace as a graft

Cartilage scoring and advancement
 wedge excision of the third crus with helical advancement.

Periosteal tether/string

Cartilage turnover and rotation
i.
9 yo unilateral microtia with CHL ii.
19/12 old unilateral microtia with lobular remnant microtia (T3) and EAC atresia iii.
5 yo bilateral microtia, tetralogy and accessory auricles iv.
3 yo bilateral constricted ear deformity (R TIIa, L TIIb), Dad with similar ears, prominence and imperforate anus at birth with ASD/PFO now closed, normal cranial and renal uss, normal spinal xray and normal genetics. DOD advises anterior approach for correction of this with bolster cartilage graft +/- SG as post approach disappointing results and scar heals well anyway v.
2 yo unilateral microtia with softband hearing aid, planned for BAHA/Connect system- parents asking what caused it, did they do anything to cause, will hearing be improved (no CT yet as unable to lie still for same, canaloplasty not likely given BAHA now low profile and more reliable hearing improvement than surgical correction with normal contralateral ear despite repeat contralateral ear infections.) vi.
12 yo Treacher Collins, bilateral severe lobular T3 microtia, unilateral BAHA,
HFM, Mandibular distraction, downslanting upper eyelids, coloboma, cleft, low hairline- planning to visit firmin unit in Paris for opinion about surgery abroad vii.
3 yo unilateral constricted ear deformity and prominence post op otoplasty 1 st
then lop ear correction 2 nd
stage viii.
14 yo post 3 rd stage reconstruction, seeking 4 th to improve projection (common problem due to cartilage resportion and STSG scar contracture- treated with further cartilage block and FTSG to post auricular sulcus) and increase excavation of concha- planned for 5 th
stage as only safe to open ant OR post in one operation in terms of skin vascularity ix.
Note: Big overlap in classification of congenital ear deformities - Tanzer classification of congenital ear deformity and of contricted ear is original best and
Nagata ‘lobular type remnant, conchal type remnant’ etc.

The often bilateral or multiple ear pits and cysts (Figure 2) correspond to epithelial retentions whose localization resembles that of ear tags. The pits and cysts, lined by squamous or respiratory epithelium, are mostly found in preauricular location and around the crus helicis. Clinic- ally, these preauricular cysts and pits are often first de- tected in conjunction with inflammations.
Additionally, upper neck pits or ear pits have been repor- ted that correspond to EAC doublings resulting from alter- ations of the first branchial groove. These have been di- vided into two types:
 Type I is a “duplication” of the EAC normally lined by skin. The pits and cysts are more frequently postauri- cular than preauricular, run parallel to the EAC and usually end blindly lateral or superior to the facial nerve.
 TypeIIcystsandpitsarethetrueEACdoublings,lined by skin and also normally containing cartilage. Often they end blindly in the transitional region between the cartilaginous and bony EAC and open in front of the sternocleidomastoid muscle. Close attention must be paid to the individual anatomy in patients treated with surgical excision, because the cysts and
Figure 2: Position of ear pits and ear cysts: a = type I, b = type II, c = topography of the facial nerve to the type II pits (from
Weerda [1])
Various deformities resulting from involvement of single or multiple hillocks can occur. Table 2 represents several typical hillock malformations following Weerda [1].
According to Weerda [1] the classification of the pinna malformations shows increasing grades with increasing severity of the malformations (Table 3).
• In dysplasia grade I (slight malformation) most structures of the normal pinna are recognizable. The reconstruction only occasionally requires the use of additional skin or cartilage.
• Malformations at moderate level amount for dysplasia grade II , also termed microtia grade II. Some structures of a normal auricle are recognizable. Partial reconstruction of the pinna requires the use of some additional skin and cartilage.
• In dysplasia grade III there are severe malformations, also described as microtia grade III with anotia. None of the normal structures of the pinna are recognizable.
Total reconstruction requires the use of skin and large amounts of cartilage.
Besides a wide range of findings within each grade of dysplasia, additional transitional forms have been noted, especially between grade II and grade III.
The cup-ear deformities described by Weerda [1] following
Tanzer [51] are classified as follows (Figure 3):
• Type I (slight deformity, corresponding to dysplasia grade I in Table 3) affects only the helix. A slight capshaped projection of the helix hangs over the scapha; the crus inferius anthelicis is normally present. The longitudinal axis of the pinna is slightly shortened. Often there is concomitant prominence of the ear.
• In type II deformity, the helix, the anthelix with its crura and the scapha are affected.
• Type IIa (slight to moderate deformity, dysplasia grade I in Table 3) shows a hood-like overhang of the helix accompanied by flattening or absence of
• the crus superius anthelicis and a pronounced crus inferius anthelicis. The shortening of the longitudinal axis of the pinna is greater. The ear is often prominent. Straightening of the pinna rarely reveals skin defects.
In type IIb (moderate to strong deformity, dysplasia grade I in Table 3), the hood-shaped helix overhang and the shortening of the longitudinal axis are more marked. The ear is decreased in width, particularly at the upper part. The crura of the anthelix and the anthelix itself are flattened or absent. The pinna is prominent, and straightening usually reveals insufficient skin.
• Type III (severe deformity, dysplasia grade II in Table
3) shows marked underdevelopment of the upper pinna, extreme overhanging of the superior auricular components and considerable deficits in height and width of the ear. There is often dystopia, showing low and anterior positioning, and EAC stenosis is frequently present, occasionally EAC atresia.
GMS Current Topics in Otorhinolaryngology - Head and Neck Surgery 2007, Vol. 6, ISSN 1865-1011 6/21

Bartel-Friedrich et al.: Classification and diagnosis of ear malformations
(Continued)
Table 2: Typical hillock malformations with illustrations and clinical findings. The arrows identify the pinna structures normally developing from these hillocks. Illustrations and some photographs modified from Weerda [1]
GMS Current Topics in Otorhinolaryngology - Head and Neck Surgery 2007, Vol. 6, ISSN 1865-1011 8/21

Bartel-Friedrich et al.: Classification and diagnosis of ear malformations
(Continued)
Table 2: Typical hillock malformations with illustrations and clinical findings. The arrows identify the pinna structures normally developing from these hillocks. Illustrations and some photographs modified from Weerda [1]
GMS Current Topics in Otorhinolaryngology - Head and Neck Surgery 2007, Vol. 6, ISSN 1865-1011 9/21