Abstract
"Boomerang" malleus-incus fusion deformity is identified on axial high-resolution CT in a subset of patients with congenital aural atresia, and it is associated with an absent incudostapedial joint and stapes capitulum and attachment of the incus to the tympanic segment of the facial nerve canal. Twelve patients with this deformity were identified on a retrospective review of imaging from a cohort of 673 patients with congenital aural atresia, with surgical confirmation in 9 of these patients. Eight of 9 patients underwent partial ossicular replacement prosthesis reconstruction with improvement in hearing outcome. We hypothesize that the boomerang anomaly represents a more severe ossicular anomaly than is normally seen in congenital aural atresia, arising from an arrest earlier in the embryonic development of the first and second branchial arch. This has potentially important implications for surgical planning because hearing outcomes with placement of prosthesis may not be as good as with conventional atresia surgery, in which reconstruction is performed with the patient's native ossicular chain.
Highlights
SUMMARY: “Boomerang” malleus-incus fusion deformity is identified on axial high-resolution CT in a subset of patients with congenital aural atresia, and it is associated with an absent incudostapedial joint and stapes capitulum and attachment of the incus to the tympanic segment of the facial nerve canal
While patients with Congenital aural atresia (CAA) have a wide variety of ossicular malformations and configurations seen on high-resolution CT (HRCT), we present a series of 12 patients with a characteristic, consistent “boomerang”-shaped fusion abnormality of the malleus and incus on axial sections through the epitympanum
Because the incus is not attached to the stapes, this deformity will most often require a partial ossicular replacement prosthesis (PORP) reconstruction; this finding should be communicated to the referring surgeon for optimal clinical decision-making, surgical planning, and patient counseling
Summary
SUMMARY: “Boomerang” malleus-incus fusion deformity is identified on axial high-resolution CT in a subset of patients with congenital aural atresia, and it is associated with an absent incudostapedial joint and stapes capitulum and attachment of the incus to the tympanic segment of the facial nerve canal. While patients with CAA have a wide variety of ossicular malformations and configurations seen on high-resolution CT (HRCT), we present a series of 12 patients with a characteristic, consistent “boomerang”-shaped fusion abnormality of the malleus and incus on axial sections through the epitympanum. In this anomaly, the malleus is hypoplastic, with an absent neck, manu-. Because the incus is not attached to the stapes, this deformity will most often require a partial ossicular replacement prosthesis (PORP) reconstruction; this finding should be communicated to the referring surgeon for optimal clinical decision-making, surgical planning, and patient counseling
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