Atresiaplasty versus BAHA for congenital aural atresia

For congenital aural atresia, surgical hearing rehabilitation may be accomplished with atresiaplasty or Bone Anchored Hearing Aid (BAHA, Cochlear Americas, Centennial, CO). The Jahrsdoerfer CT grading system has been used to determine which patients are candidates for atresiaplasty.1 Patients with a Jahrsdoerfer score of 6 or higher are considered to be candidates for atresiaplasty. The higher the grading score, the better the chance for a favorable hearing outcome for atresiaplasty.1 Hearing results for atresiaplasty range from excellent to fair.1, 2 BAHA is an alternative to atresiaplasty. Hearing results for BAHA are generally excellent.3 However, cosmesis is not very good with the BAHA with a visible titanium abutment and snap-on hearing aid, and frequent wound care is required. A decision for BAHA versus atresiaplasty needs to be individualized for each patient because both have advantages, disadvantages, and potential complications. In the best of hands, the mean postoperative long-term speech reception threshold (SRT) was ≤25 dB HL in 75% of atresiaplasties.1 However, other excellent surgeons report more modest hearing results such as Teufert and De la Cruz,2 who reported a mean long-term SRT of 35 dB HL. Atresiaplasty requires excellent judgment and advanced skills. Atresiaplasty is relatively lengthy and carries a very small risk of facial nerve injury and sensorineural hearing loss. Meatal stenosis and recurrent infection are fairly common.2 Failure of complete closure of the air–bone gap following atresiaplasty may be corrected with a conventional hearing aid or BAHA. Cosmesis is generally good for atresiaplasty. An excellent hearing result for atresiaplasty is the best possible outcome because the hearing improvement is continuous, spontaneous learning is always possible, and no external electronic device is needed. BAHA fixture placement is relatively rapid and simple, but two surgical stages are required for young children with thin skulls. Cosmesis is not ideal with a visible metal abutment and a snap-on hearing aid. Hearing results are usually excellent for BAHA with an expected air–bone gap of only 16–18 dB HL.3 BAHA complications include occasional loss of the fixture from infection, and fairly common surgical site infections with flap thickening that may require revision surgery.4 The BAHA requires regular wound care, which may be difficult in a young child (Table I). The child must also be careful to avoid trauma to the BAHA site, which may result in loss. The BAHA may be offered to all patients with a Jahrsdoerfer score of 5 or less who are not considered to be atresiaplasty candidates and are not satisfied with their conventional hearing aid. BAHA is approved for children at least 5 years of age and is particularly important for children with bilateral microtia and aural atresia to overcome bilateral maximal conductive hearing loss. Microtia reconstruction and atresiaplasty may be performed at a later date. Atresiaplasty may be performed at 4–5 years of age for the occasional patient with congenital aural atresia without microtia. However, because most children with aural atresia also have microtia, atresiaplasty should follow microtia reconstruction, which avoids potential compromise of the temporal blood supply and gives the best chance for successful microtia reconstruction. Microtia reconstruction is best delayed until at least 7 years of age when more rib cartilage is present and the children are more cooperative than younger children. As an alternative, multiple unilateral BAHA fixtures may be placed for the hearing aid and also as anchors for a prosthetic auricle. Family preference for BAHA versus atresiaplasty must be considered, as well as nonsurgical options such as BAHA Softband,5 conventional hearing aids, and glue-on prosthetic auricles. BAHA Softband is an excellent noninvasive device for hearing rehabilitation in very young children with aural atresia5 and may be used as a trial prior to the implantable BAHA. For patients with Jahrsdoerfer grade 5 or less, who are poor candidates for atresiaplasty, BAHA is a good option for auditory rehabilitation. It is safe, reliable, and has low surgical risk, but is cosmetically less appealing, requires regular wound care, and trauma to the site must be avoided. In favorable surgical candidates for atresiaplasty (Jahrsdoerfer grade 6 or higher), either atresiaplasty or BAHA may be considered. Successful atresiaplasty offers the best opportunity for life-long, continuous, amplification-independent improvement in hearing. Conventional hearing aids and BAHA may also be useful for rehabilitation of nonoptimal atresiaplasty hearing results. Nonsurgical options such as BAHA Softband,5 conventional hearing aids, and family preference must also be considered. Overall, the level of evidence concerning BAHA and atresiaplasty is low (level 4) for these five retrospective studies. There are no prospective studies of atresiaplasty or BAHA, and none that directly compare outcomes. The authors of reference 3 disclose financial support from the manufacturer of the BAHA that raises the possibility of study bias. More prospective independent studies are needed.