CASE OF UNILATERAL CONGENITAL AURAL ATRESIA & MICROTIA WITH CHOLESTEATOMA

Abstract

We report a case of cholesteatoma associated with unilateral congenital aural atresia (CAA) and microtia, resulting in extracranial complication.

Management of Congenital Aural Atresia : A Case Report

“…In grade II, the helix is rudimentary and the lobule developed. In grade III, an amorphous skin tag is present [6]. In this case, we have a female patient with unilateral congenital aural atresia in the right ear and microtia grade III.…”

“…Microtia has an incidence at 1 in 7 000 to 8 000 births in the general population. The majority of the cases are unilateral and males are affected more commonly, and the reported ratio of right to left bilateral is approximately 5:3:1 with bilateral deformity occurring in only 10% of patients [5,6].…”

Unilateral aural atresia: a case report

“…The exact cause of congenital aural atresia is unknown and is probably multifactorial. It ultimately results from a disturbance of normal embryological development that begins as early as the sixth week of gestation, and this disorder affects the development of the first pharyngeal cleft, which is responsible for the development of the external auditory canal [ 3 ]. The most widely accepted theory is an early vascular insult of the stapedius artery, which is responsible for the development of the first and second pharyngeal arches [ 3 , 4 ].…”

“…It ultimately results from a disturbance of normal embryological development that begins as early as the sixth week of gestation, and this disorder affects the development of the first pharyngeal cleft, which is responsible for the development of the external auditory canal [ 3 ]. The most widely accepted theory is an early vascular insult of the stapedius artery, which is responsible for the development of the first and second pharyngeal arches [ 3 , 4 ]. Regarding the hearing, in the unaffected side, there is normal hearing in all the frequencies, and this is different in the affected ear with a mild to moderate level of hearing loss.…”

“…It is well known that the aural anatomic structures have a different embryologic origin; the auricle develops from the first and second branchial arches, external canal and tympanic membrane from the first branchial cleft [ 1 , 2 ]. The closely related development of the external acoustic canal and the middle ear led to the classification of the combined malformation as atresia aural congenital according to the literature [ 3 ].Three degrees of severity have been described: first-degree malformations present with mild deformities of the external auditory canal, a normal or hypoplastic tympanic cavity, malformed ossicles and a well-ventilated mastoid. Second-degree deformities are intermediate deformities.…”