In cases of severe malformation, the meatus and canal are usually absent. However, their presence raises several issues. First, if the auricle is not severely malformed and if a canal exists, the potential for hearing preservation or reconstruction is enhanced (38). Second, the presence of a canal, especially if narrow, makes the patient susceptible to secondary cholesteatoma. Third, auricular reconstruction will need to accommodate to this fixed canal position yet be symmetrically located in relation to the contralateral ear. Fourth, for hearing habilitation in bilateral microtia, an air- rather than bone-conduction hearing aid may be preferred. Fifth, the otologic surgeon must not be tempted to reconstruct a canal stenosis until after the external ear has been repaired.

The presence of a tympanic membrane raises the possibility that hearing levels may be acceptable or a conductive loss more easily corrected. Additionally, the tympanic membrane allows otoscopic monitoring because the microtic ear is at risk for otitis media and extended complications (39).

Even with severe microtia, a hairless cutaneous rim persists in most cases. With anotia, on the other hand, the hairline can be extremely low. The significance of a hairless zone is apparent during the first stage of rib reconstruction because the superior portion of the cartilaginous framework of necessity will reside under hair-bearing skin. In the patient who has an adequate zone of hairless skin, fewer hair removal techniques, such as skin grafting or hair lasering, will be needed later.

The cartilaginous appendage varies in appearance and orientation. Importantly, in less severe malformations, the superior cartilaginous portion of the auricle may have only a moderate deformity and may be reconstructed using less morbid techniques adapted from otoplasty (40). In severe microtia, the volume of cartilaginous remnant determines the degree of overlying soft tissue cover. If rib reconstruction is elected, skin coverage is critical. The greater the size of the cartilaginous remnant, the greater will be the amount of pliable soft tissue cover with which the surgeon can work. Another important point is whether the auricle remnant has a smooth covering or whether the skin convolutes to fit the contours of the auricular remnant. Should the convolutions be very deep and pitted, preservation of intact skin will be more difficult. Also, at times a rudimentary conchal bowl and tragus will exist. Auricular features that are well formed can be incorporated into the reconstruction. Conversely, an abnormally located conchal bowl and tragus may add to the challenge of removing the cartilaginous remnants during the reconstruction phase, because the location of the cartilaginous framework as it relates to the contralateral auricle is a priority in the repair of microtia.

A lobular remnant is present in the typical case, even with severe malformation. During reconstruction, this tissue is rotated posteriorly and inferiorly to abut the costal framework at its inferior margin. The presence of lobular tissue provides for some flexibility in establishing the overall vertical height of the new auricle as it is normally transferred at a secondary stage and allows for accurate design at this point. Additionally, this lobule-like tissue hopefully will be adequate to place an earring, which some patients desire and which assists with eventual aesthetic appeal.

On lateral view, the microtic ear is smaller in vertical height than the contralateral, normal ear. The most cephalad portion of the normal ear lies approximately at the level of the
lateral brow, whereas the microtic ear is caudad to this level. Microtic ears also exhibit a more vertical axis of orientation than a normal auricle. In the normally oriented auricle, an imaginary line drawn from the most superior portion of the helix through the lobule is roughly parallel with the dorsum of nose. This relationship is very helpful in properly placing the cartilage framework during surgery. Another common finding is that in the frontal view, the lobule tends to be 3 to 5 mm higher on the microtic side than on the contralateral normal side and is coupled with a lack of tragal prominence.

Skin tags are often present. Their removal should be delayed until after placement of the reconstructed cartilaginous framework to avoid vascular compromise of the overlying skin. Occasionally, a skin tag can be used in the reconstructive effort.

Especially in Goldenhar’s syndrome (oculoauriculovertebral syndrome or hemifacial microsomia), the mandible may be smaller on the microtic side. This finding carries implications for the aesthetics of ear reconstruction because the mandible may be reconstructed at another time. Depending on the reconstruction technique, rib grafting may be used for mandibular augmentation, as well as for auricular repair. Should distraction osteogenesis be planned, the mandible will expand quickly and the position of the ear will appear to change rapidly. If mandibular reconstruction is not planned, the presence of an asymmetric mandible makes it necessary for the surgeon to rely upon aesthetic judgment rather than strict measurement in determining the proper location of the newly created auricle. I see patients in conjunction with an oral surgeon so that simultaneous ear and jaw reconstruction can be planned.

It is very important to evaluate the contralateral ear prior to the reconstruction. Good hearing is more likely in a normal-appearing ear; however, the hearing of both ears must be tested. Further, minor otologic anomalies are often present in the contralateral ear. In addition to these concerns, the normal contralateral ear serves as the model for reconstruction and associated otoplasty techniques are often necessary to enhance the overall symmetry of the repair.

Because auricular malformation is less common and less familiar than other congenital malformations such as cleft lip or club foot, the parents will require extensive counseling. Compounding the situation, the malformed auricle is a visible condition during the first several months or even years of life because of the lack of hair to camouflage the condition. After the initial concern about the auricular appearance, parental attention then focuses on hearing. A thorough brainstem evoked response audiometric test, not merely an auditory brainstem response (ABR) screening, should be performed as soon after birth as possible. During the first few weeks of life, brainstem evoked response audiometry can be performed under natural sleep conditions rather than under sedation. Even in cases with unilateral microtia, the hearing must be evaluated. Although the contralateral normal-appearing ear has normal hearing in most cases, this must be proven. The abnormal ear usually has a maximum conductive hearing loss of about 60 dB. However, a lesser conductive loss or associated sensorineural loss may be present (41).