 | Before | After |
Severe deformity of the external ear (microtia) with absence of an ear canal and tympanic membrane (aural atresia) occurs in 1 in 10,000 to 20,000 births. Aural atresia/microtia is usually an isolated condition, but can be associated with a number of other craniofacial anomalies such as Treacher Collins syndrome or hemifacial microsomia. Although auditory nerve function is typically normal, there is a severe loss of hearing secondary to the absence of a sound-conduction pathway to the cochlea. In addition to the hearing loss, the psychological implications of a severe cosmetic ear deformity in a young child can be significant. 
Paul Lambert, MD |
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The Department has assembled an experienced team of individuals to manage this challenging problem. Pediatric otolaryngologist
David R. White, M.D., has particular interest in auricular reconstruction using rib cartilage, and he has been able to achieve consistently excellent cosmetic results. Our team discusses the option of a prosthetic ear with patients and families as an alternative to the multi-staged surgical reconstruction.
Betsy Davis, D.M.D., is recognized as a national expert in fashioning various head and neck prostheses, including the outer ear. Her approach utilizes 3D imaging to optimize form and contour match with the contralateral side in unilateral cases. Paul R. Lambert, M.D., and Ted A. Meyer, M.D., perform the surgery to reconstruct the ear canal, tympanic membrane, and ossicular chain. Dr. Lambert’s interest in this surgery spans 25 years, and his results have been detailed in numerous publications and book chapters. Critical to the success of treating pediatric patients with various ear deformities and diseases is an accurate assessment of hearing and, when appropriate, hearing aid fitting as early as several months of age. Kimberly A. Orr, M.A., has been our Director of Pediatric Audiology for more than 15 years and is extremely skilled in the audiological assessment of young patients. In addition to conventional bone-conduction hearing aids, the use of the BAHA device has provided a new avenue of rehabilitation. At our Center, the highly predictable results with the BAHA has also enabled us to be highly selective in choosing patients for hearing reconstruction. Of the more than 500 patients with microtia and atresia evaluated over the years, approximately 40 to 50 percent have undergone surgery. Finally, it is important to provide genetic counseling to parents of patients with microtia and atresia. G. Shashidhar Pai, M.D., Director of the Division of Genetics and Developmental Pediatrics, provides families with such counseling and genetic evaluation as needed. Pediatric patients with microtia and/or atresia are typically seen by otolaryngologists within the first few weeks of life. Proper use of hearing aids in a timely fashion is especially important when bilateral atresia or underlying sensorineural hearing loss is present. Surgical treatment of microtia/atresia is typically begun between five and seven years of age. As in many areas of our specialty, a multidisciplinary approach is necessary to optimize patient care. We are proud of this group of individuals who are dedicated to the comprehensive management of aural atresia/microtia. In addition to being a major referral center for microtia/atresia, various members of the team are involved in mission trips to the Dominican Republic with Project Ear to treat children with this problem. Key Faculty  David R. White, M.D. – Pediatric Otolaryngologist
Betsy Davis, D.M.D. – Prostheses Expert
Paul R. Lambert, M.D. – Surgeon
Ted A. Meyer, M.D. – Surgeon
Kimberly A. Orr, M.A. – Pediatric Audiologist
G. Shashidhar Pai, M.D. – Genetic Counseling
Research Lambert PR. Major congenital ear malformations: Surgical management and results. Ann Otol Rhinol Laryngol 97:641-649, 1988. Lambert PR. Congenital absence of the oval window. Laryngoscope 100:37-40, 1990. Lambert PR. Complications of Surgery for Congenital Atresia. Complications in Head and Neck Surgery, ed D. W. Eisele, Mosby, pp 660-665, 1993. Lambert PR. Congenital Atresia of the Ear. Head and Neck Surgery-Otolaryngology, ed. B.J. Bailey, J.B. Lippincott, pp 1579-1591, 1993. Revised for second edition, 1998. Revised for third edition, 2001. Hashisaki GT and Lambert PR. Congenital Aural Atresia. Current Therapy in Otolaryngology-HNS, ed G Gates, St. Louis, C.V. Mosby, pp 4-8, 1998. Lambert PR. Repair of Congenital Aural Atresia. Atlas of Head and Neck Surgery, Ed. B.J. Bailey, J.B. Lippincott, pp 422-425, 1996. Lambert PR. Congenital aural atresia: Stability of hearing results. Laryngoscope 108:1801-1805, 1998. Maceri DR, Lambert PR. Aural Atresia. The Ear: Comprehensive Otology Lippincott, Williams & Wilkins, pp 359-370, 2000. Witte MC, Lambert PR. Revision Surgery for Congenital Aural Atresia. Revision Surgery in Otolaryngology ed S Selesnick, 2001. Lambert PR. Management of the Unilateral Atretic Ear. Controversies in Otolaryngology, ed. M. Pensak, Thieme, pp 376-380, 2001. Lambert PR and Eavey R. Microtia and Atretic Ear Surgery. Complications in Pediatric Otolaryngology, ed. D. Wohl and G. Josephson, Singular Publishing. Pp 513-526, 2005. Lambert, PR. Congenital Aural Atresia. Head and Neck Surgery – Otolaryngology, ed. B. Bailey, J. Johnson, S. Newlands. Lippincott, pp 2027 – 2040, 2005. |
