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. The exact cause of congenital aural atresia is unknown and is probably multifactorial. According to the literature, congenital aural atresia refers to an anatomical malformation which mostly is presented with unilateral affection. The aim is to create a new anatomic pathway resembling a normal external acoustic canal by drilling the atretic bone and removing the present tissue ( Figs 5 and 6).Ĭongenital aural atresia may be found from the early stages of life, but the time of surgical repair depends on several factors. The incision was made behind the ear at the level of temporal bone, and the graft needed is removed from the temporal fascia and inserted as covering of the new canal. During surgery, a graft has been obtained in order to formate the covering of the new canal. Some weeks ago, canalplasty and tympanoplasty were performed by our surgery team. The patient decided to proceed to surgery many years after the first diagnosis. Diagnosis of this anatomical malformation was compatible with congenital atresia of the external auditory canal on the right temporal region. Left side of the external auditory canal was normal. There was a normal anatomy of the internal auditory canal on both sides. The computed tomography (CT) scan and mainly the magnetic resonance imaging (MRI) of the temporal bone revealed no mastoid air cells and no external auditory canal and auricle ( Figs 3 and 4). Blood test was normal, and there were no other medical issues from the personal history. There was no symptomatology in the left ear and the anatomic structures were completely normal. The clinical examination revealed that there was no external auditory canal in the right ear ( Fig. CASE REPORTĪ 56- year old male was presented in the Outpatient Department of Otolaryngology Clinic with main complaint of complete aural atresia of the right ear. In addition, pneumatization is inhibited. The external auditory canal is completely absent, the middle ear is hypoplastic and the ossicles are severely deformed. Third-degree malformations are severe deformities. These include a blind-ending or absent external auditory pathway, a narrow tympanic cavity, deformities and fixations of the ossicles and decreased pneumatization of the mastoid cells. Second-degree deformities are intermediate deformities. 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. 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. 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. The origins of this anatomic malformation are embryological. In some of them, bilateral congenital aural atresia may occur when the malformation is not an isolated condition but is in the context of a syndrome, such as CHARGE syndrome, in which newborns are affected by bilateral atresia in up to 60% of cases. In general, aural malformations can be present in a ratio of 1:3800 children. Mostly, it is unilateral approximately in 70–90% and it affects the right side because it can be more commonly affected by hypoperfusion than the left, where the heart is located and which generally has a pressure 10 mmHg higher than the right one.

In case of congenital aural atresia, the etiology is based on normal embryological pattern disruption. It can be congenital or acquired after trauma. Anatomical malformation of hypoplasia of the external auditory canal is quite rare and is present 1 in 10 000–20 000 births.

Aural atresia