Introduction
Congenital aural atresia (CAA) is diagnosed in 1 in 10,000 to 20,000 newborns 1. Among these cases, unilateral CAA (UCAA) is much more frequently observed than bilateral CAA 2. Additionally, the middle ear and ossicles are affected to varying degrees in patients with CAA, leading to conductive hearing loss2. Although patients with UCAA typically understand speech well in everyday listening situations in quiet, the growing interest in binaural hearing has led to an increased demand for hearing rehabilitation in patients with unilateral aural atresia3. Previously, middle ear and auditory canal reconstructive surgery to restore conductive hearing in patients with CAA was performed; however, that is generally considered one of the most difficult types of otologic surgery, and functional results are often unsatisfactory, leading to the need for an air conduction hearing aid3, 4. The alternative option is to consider a bone conduction (BC) hearing device or middle ear implant (the Vibrant Soundbridge [VSB], MED-EL Corporation, Austria)5. For patients with UCAA, the VSB can treat the impaired ear without affecting the contralateral ear, unlike the BC device4. The VSB, firstly implanted in children in 20096, is composed of two parts: 1) the implantable vibrating ossicular prosthesis (VORP) and floating mass transducer (FMT), and 2) the externally worn audio processor. It is able to bypass middle ear malformations and provide active auditory support directly to the cochlea. The VSB can be easily attached to a variety of middle ear structures, and it is well suited for implantation in the malformed middle ears of children with CAA6. Recently, it has been increasingly used to improve hearing without surgical complications in patients with UCAA 6.
CAA is often associated with microtia or other forms of craniofacial dysplasia. Therefore, children with atresia and microtia may face multiple surgeries to correct the malformation, indicating that they and their parents require the proper intervention of otologists and facial plastic surgeons 7. Most children with microtia require plastic reconstruction for cosmetic and functional benefits (i.e., to facilitate wearing glasses or a mask)7. Auriculoplasty using a rib graft is preferable to the use of a prosthesis in terms of skin infection and implant extrusion8. Correcting the microtia should be performed in patients older than 10 years as treatment at a younger age has unfavorable outcomes in terms of both the reconstructed ear and the donor-site thorax9.
For the early intervention in children with unilateral microtia-atresia, Frenzel et al. reported “simultaneous” VSB implantation with plastic reconstruction of the auricle without any negative effects in terms of the healing process or the cosmetic outcome of the repaired auricle6. However, in cases in which patients and their parents desire earlier hearing rehabilitation, the development of a new surgical approach is required. Here, we demonstrate a case of UCAA receiving VSB implantation prior to auricular reconstruction and highlight the importance of cooperation between otologists and plastic surgeons in the performance of the surgical technique presented herein.