Background: Developmental disorders of the ear can impair hearing and cause cosmetic deformities. In recent years, new surgical treatments have become established, above all in audiological rehabilitation.
Method: We selectively searched the PubMed database up to May 2013 for publications in English and Germanabout the therapeutic options.
Results: No randomized trials have been performed, for both ethical and practical reasons (inadmissibility of placebo surgery, specialization of surgeons for individual techniques). To correct prominent ears, cartilage-sparing suture techniques are preferred, as they lead less often than scoring and incisional techniques to the formation of persistent, incompletely correctable ridges and scaffolding defects. The successful esthetic rehabilitation of severe deformities of the external ear is achievable through pinna reconstruction with costal cartilage (main risks: tissue defect at donor site, scaffolding resorption) or porous polyethylene (main risk: implant extrusion). The functional rehabilitation of conductive or mixed hearing impairment due to ear-canal atresia and major middle-ear deformities is preferably achieved with active middle-ear implants or bone-conduction hearing aids. Functional rehabilitation should be provided even when the hearing impairment is unilateral, in order to improve directional hearing and hearing with ambient noise. In cases of purely cochlear, unilateral, severe hearing impairment or deafness, a boneconduction hearing aid can be tried, and the individual indication for a cochlear implant can be considered.
Conclusion: The treatment options described here enable the affected children to benefit from complete functional and esthetic rehabilitation before they start school.