What is the key to hearing problems in congenital ear deformities?

The main concern when parents are consulted about hearing problems is hearing. They either think that there is no hearing at all on the affected side, or they think that hearing will be restored with a cavity, or even that there will be an ear canal inside if the skin is cut open, etc. In fact, they all make a conceptual mistake, which has to be explained in terms of embryonic development. The ear consists of three parts: the outer ear, the middle ear, and the inner ear. The outer ear consists of the auricle and the external auditory canal; the inner ear is a snail-like cochlea, which plays an electronic system-like role in transmitting information to the brain via the auditory nerve to form the sense of hearing; the middle ear is an air-containing cavity in between the two, which contains three small bones of the hammer bone, anvil bone and stirrup bone to form a movable chain of auditory bones. During embryonic development, the outer and middle ears originate from the same tissue mass, primarily the first and second gill arches; however, the inner ear is a distinct tissue origin. Therefore, patients with microtia also have a simultaneous malformation of the middle ear, while the inner ear is normal, so the affected side of the patient with microtia also has partial hearing (bone conduction hearing). The key to the problem is air conduction, as the patient has a hearing threshold of 40-60 dB (0-20 dB normal) on the affected side due to external atresia and middle ear malformation. Fortunately most microtia patients have a healthy normal ear, plus the affected ear itself has partial hearing, so it is essentially unaffected in daily life and learning, except for a slight impact on auditory localization. Surgical correction of hearing problems requires avoiding the facial nerve to make holes in the bone and tissue grafts to form the tympanic membrane, but the deformed, fused auditory tuberosity may not be repairable. There are also many problems with implants in the bone tunnel, with the result that chronic fluid flow and subsequent infection producing an unpleasant odor often occur, and the open bone cavity (external auditory canal) is often narrowed. Even in the absence of complications and recent hearing improvement, the long-term results are hardly lasting. In addition, patients should avoid water in the ear canal and swimming after surgery. Given the above-mentioned surgical limitations and healing issues, most physicians internationally currently believe that the results of middle ear surgery do not offset the postoperative risks and complications, so it is only used for patients with small bilateral ears. Some physicians even believe that if they cannot handle a unilateral microtia patient, they should not do it bilaterally. For those who want to perform hearing surgery for bilateral microtia, it is generally believed that ear reconstruction should be done first, because hearing surgery will destroy the skin there and form a scar, making later outer ear reconstruction difficult. As for the surgery of outer ear reconstruction and hearing reconstruction at the same time, however, the results of performing both surgeries at the same time are affected by each other and are not very satisfactory, and people now have difficulty in accepting such not-so-good results.