An Adult with Congenital Aural Atresia: Diagnostic and Treatment Implications

Congenital aural atresia is a deformity of the auricle present at birth. The development of the external ear, external acoustic meatus, ossicles, and middle ear space may be affected at varying degrees, potentially causing up to 60 dB of conductive hearing loss. This occurs in one in 10,000-20,000 births and is more often seen unilaterally than bilaterally. Congenital aural atresia affects functional hearing for children with unilateral atresia and there is an increased risk of speech-language delay and decreased academic performance.1 Providing a bone-anchored hearing aid (BAHA) for patients with unilateral congenital atresia can improve audiometric sensitivity and hearing handicap self-assessment scores.2 Additionally, for children and adults, reconstruction of the external ear can produce positive psychological outcomes with a post-operative increase in self-confidence and participation in social and leisure activities.3 CASE PRESENTATION A 65-year-old male patient with congenital atresia and Grade 3 microtia was surgically implanted with a first-of-its-kind auricular implant at age 5. From infancy through adulthood, even following the childhood placement of an auricular prosthesis, no auditory habilitation was provided for this individual or his family. His first audiological evaluation occurred nearly 60 years after the surgical procedure in childhood, following a workplace incident that resulted in an infected prosthesis. In this case, the initiation of an interdisciplinary care team that included infectiology, otolaryngology, radiology, plastic surgery, and audiology marked the beginning of a dramatic improvement in his overall wellness, communication, and quality of life. At 65 years of age, the patient's interdisciplinary care team identified left-sided congenital atresia and Grade 3 microtia. Imaging studies indicated the absence of an external acoustic meatus with malformed ossicles in a diminutive middle ear cavity. These malformities were assumed congenital. He was surgically implanted at age 5 with a first-of-its-kind soft silastic otoplasty prosthesis, a full-thickness inguinal skin graft.4 This cosmetic procedure did not involve creating an aperture for an external acoustic meatus. Less than a year after surgery, the patient was struck directly on his prosthesis while playing. As a result, it became infected, was removed, and was eventually replaced with a similar prosthesis. The replacement device remained intact on his left side without complication for nearly 60 years. Throughout his teenage and adult life, the patient received no specialty clinical care or multidisciplinary intervention, including audiological evaluation, rehabilitation, or preventive services. He described auditory, educational, and social development as areas of difficulty during childhood but developed strategies to improve communication in demanding situations. By early adulthood, the patient's employment had already involved several positions with exposures to hazardous occupational noise. For these jobs, he was enrolled in the hearing conservation program and received occupational audiometric monitoring, however, no comprehensive audiological evaluation or counseling was reported. The patient reported a workplace injury to his left auricular implant at around 63 years of age, where contact with a ladder caused a skin puncture resulting in a severe methicillin-susceptible Staphylococcus aureus infection (Fig. 1). Over five months, antibiotic therapy was delivered orally, intramuscularly, and intravenously through a peripherally inserted central catheter. These antibiotic treatments included Augmentin, Bactrim, clindamycin, cephalexin, doxycycline, ceftriaxone, daptomycin, and rifampin, which failed to resolve his infection (Fig. 2). The infected prosthesis was surgically removed, followed by plans for a new device. A hearing assessment was then administered for the first time in the patient's history.Figure 1: Left auricular prosthesis hours after being struck by a large object.Figure 2: Left auricular prosthesis after five months of antibiotic treatment; infection remains and soft silastic framework exposed on anterior superior edge, anterior inferior edge, and superomedial region.Figure 3: Initial comprehensive audiogram.Figure 4: Current state of the patient's left side: two osseointegrated screws for retention of new implant retained silicone prosthesis anterior to BAHA abutment.At age 64, an audiological evaluation revealed a moderately severe sloping high-frequency sensorineural hearing loss in the right ear, likely the composition of presbycusis and a longstanding history of noise exposure (Fig. 3). For his right ear, a dispensing auidiologist fitted him with a receiver-in-the-canal hearing aid. For his left ear, audiometric thresholds showed an asymmetric, severe-to-profound mixed hearing loss with large air-bone gaps. At a different clinical practice, an otolaryngologist surgically implanted his right ear with a BAHA. The surgeon implanted the osseointegrated hardware in preparation for his new implant-retained silicone ear prosthesis (Fig. 4).5 Due to the limitations of his health insurance, the right-ear hearing aid and left-ear BAHA were managed by clinicians at different practices. Ultimately, he reported that his hearing aid and BAHA improved sound awareness, localization, speech perception, socialization, and quality of life. DISCUSSION The 65-year-old male patient with congenital atresia and Grade 3 microtia was surgically implanted with a first-of-its-kind auricular implant at age 5. For most of his life, he has worked in the presence of occupational noise. Until age 63, the only member of his ear and hearing-related care team was his childhood plastic surgeon. Multidisciplinary teams comprised of an otolaryngologist, plastic surgeon, radiologist, audiologist, and speech-language pathologist may deliver early health outcomes more efficiently for individuals with congenital atresia.6 For conditions that impact social-emotional, speech-language, and educational development, early intervention and interprofessional care are crucial,7 and, if the aforementioned disciplines are collaborating, children with congenital atresia should have access to treatment options that include bone-conducting technologies. Requiring referrals to both a pediatric otolaryngologist and plastic surgeon is the best course of action for a child born today with congenital atresia and microtia. Failure to refer to each discipline may produce social-emotional concerns related to microtia and atresia, or social, educational, and speech-language concerns related to functional hearing.8 For cases of bilateral atresia, a BAHA is recommended by the International Microtia and Atresia Workgroup; however, for unilateral cases, it is less clear. The decision to pursue a BAHA should be made by the family and their care team, which should include audiology and radiology for candidacy considerations. A device trial should first be presented as an option. Second, if a BAHA is pursued, a collaboration between the otolaryngologist and plastic surgeon is needed to ensure that BAHA placement does not interfere with microtia repair. Surgical repair of atresia may be warranted and is dependent upon the anatomical state of the external acoustic meatus and middle ear space. If pursued, this surgery should take place after 6 years of age (with the best results after puberty, due to the potential for new bone growth) and before the surgical repair of microtia. Ideally, surgical repair of microtia should occur around 9 years of age but should be delayed if atresia repair is sought after puberty.9 Regardless of the provision of surgical repair or BAHA, the audiologist should consider the need for assistive listening devices and coordinate with educational personnel for the provision of classroom accommodations. Audiologists should provide consistent follow-up for preservation of hearing in the patient's contralateral ear.1 CONCLUSION Congenital aural atresia has been associated with conductive hearing loss, speech-language delay, and negative social, educational, and psychological consequences. Early identification and referral for specialty care should generate interprofessional collaborative services that include clinical and surgical intervention. This case demonstrates that providing comprehensive personalized care that extends beyond the initial evaluation, guiding patients and their families toward long-term hearing wellness, maximal communication, and improved quality of life should always be the primary goal of the hearing health care team. Finally, a care plan that includes continued follow-up is essential if the needs or hearing status of the person changes.