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.