Christopher J. Hartnick and Daniel I. Choo

Unilateral congenital aural atresia represents one of the most complex and challenging problems that an otolaryngologist may have to face. The surgical procedure itself is fraught with potential pitfalls such as facial nerve paralysis and sensorineural hearing loss. There are differing schools of thought concerning the repair of congenital unilateral aural atresia; one school of thought is represented by Crabtree,1 who states that “one does not usually operate in cases of unilateral atresia until the patient is an adult and expresses the desire for surgery.” The other school of thought is represented by Jahrsdoerfer et al.2 who writes that “whether the ear malformation is unilateral or bilateral plays no part in our selection process. Because we believe that hearing results are predictable, we operate on unilateral atresia patients routinely.” The underpinnings of and the differences between these two surgical beliefs are in part philosophical, but another part rests in technological advances that have allowed for greater facility in predicting the results of surgery. This chapter, discusses the controversy over managing patients with unilateral congenital aural atresia by describing the preoperative evaluation that goes into formulating a treatment algorithm and our rationale for offering unilateral atresia repair to pediatric patients and their families.


Embryology and Epidemiology


The incidence of congenital aural atresia is 1:10,000 to 20,000.3 Unilateral atresia is five to six times more common.1 The embryologic basis of aural atresia is the failure of the external auditory canal to recanalize during the sixth and seventh months of gestation. The external auditory canal is derived from the first branchial groove. In the developing embryo, an epithelial core of cells arises and fills the external auditory canal. It is the failure of these cells to canalize that gives rise to aural atresia. Aural atresia occurs either together with, or in isolation from, external or middle ear malformations. It is important to note that middle ear malformations are much more common in the atretic ear where there is also microtia present; they are also more common in the normal ear where the other ear canal is atretic than if there are two normal ears.1


Surgical Considerations


As it is not the purpose of this chapter to present a technical manual of aural atresia surgery, the extensive details of the surgical procedure are not discussed in this chapter. However, an alternative approach to the anterior atresiaplasty that is now commonly advocated is presented. As described by several surgeons,35

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Jun 5, 2016 | Posted by in OTOLARYNGOLOGY | Comments Off on Christopher J. Hartnick and Daniel I. Choo

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