Guidelines Summary

International Microtia and Atresia Workgroup

Consensus recommendations on the treatment of total congenital aural atresia (CAA), published in 2019 by the International Microtia and Atresia Workgroup (IMAW), include the following^[56]:

Bone-conduction technology for children with bilateral aural atresia is very strongly recommended to support speech and language development
Evaluation of the temporal bones using careful computed tomography (CT) scanning is recommended, as is precise assessment of the type of hearing loss and of preoperative motivation with the softband
To prevent the processor from interfering with microtia reconstruction, a discussion between the otolaryngologist/otologist/pediatric otolaryngologist and microtia surgeon regarding placement of the osseointegrated bone conduction hearing device is strongly recommended
It is recommended that children over age 6 years whose hearing and anatomy support surgical reconstruction (ie, those with normal inner ear function and a Jahrsdoerfer score of 7 or higher) undergo atresia surgery with the understanding that revision surgery may be required if the canal and hearing are compromised by new bone growth during puberty
It is strongly recommended that atresia repair be performed in combination with or subsequent to autologous rib graft microtia repair and prior to porous polyethylene microtia repair

Kountakis SE, Helidonis E, Jahrsdoerfer RA. Microtia grade as an indicator of middle ear development in aural atresia. Arch Otolaryngol Head Neck Surg. 1995 Aug. 121(8):885-6. [Medline].
Jahrsdoerfer RA, Kesser BW. Issues on aural atresia for the facial plastic surgeon. Facial Plast Surg. 1995 Oct. 11(4):274-7. [Medline].
Granstrom G, Bergstrom K, Tjellstrom A. The bone-anchored hearing aid and bone-anchored epithesis for congenital ear malformations. Otolaryngol Head Neck Surg. 1993. 109:46-53. [Medline].
Lo JF, Tsang WS, Yu JY, Ho OY, Ku PK, Tong MC. Contemporary hearing rehabilitation options in patients with aural atresia. Biomed Res Int. 2014. 2014:761579. [Medline]. [Full Text].
Nager GT. Congenital aural atresia: anatomy and surgical management. Birth Defects Orig Artic Ser. 1971 Mar. 07(4):33-51. [Medline].
Schuknecht HF. Reconstructive procedures for congenital aural atresia. Arch Otolaryngol. 1975 Mar. 101(3):170-2. [Medline].
Jahrsdoerfer RA. Congenital atresia of the ear. Laryngoscope. 1978 Sep. 88(9 Pt 3 Suppl 13):1-48. [Medline].
Dostal A, Nemeckova J, Gaillyova R, Vranova V, Zezulkova D, Lejska M. Identification of 2.3-Mb gene locus for congenital aural atresia in 18q22.3 deletion: a case report analyzed by comparative genomic hybridization. Otol Neurotol. 2006 Apr. 27(3):427-32. [Medline].
Nuijten I, Admiraal R, Van Buggenhout G, Cremers C, Frijns JP, Smeets D. Congenital aural atresia in 18q deletion or de Grouchy syndrome. Otol Neurotol. 2003 Nov. 24(6):900-6. [Medline].
Montino S, Agostinelli A, Trevisi P, Martini A, Ghiselli S. Check-list for the assessment of functional impairment in children with congenital aural atresia. Int J Pediatr Otorhinolaryngol. 2017 Nov. 102:174-9. [Medline].
Kiese-Himmel C, Kruse E. [Unilateral hearing loss in childhood. An empirical analysis comparing bilateral hearing loss]. Laryngorhinootologie. 2001 Jan. 80(1):18-22. [Medline].
Kesser BW, Krook K, Gray LC. Impact of unilateral conductive hearing loss due to aural atresia on academic performance in children. Laryngoscope. 2013 Sep. 123(9):2270-5. [Medline].
Reinisch JF, Lewin S. Ear reconstruction using a porous polyethylene framework and temporoparietal fascia flap. Facial Plast Surg. 2009 Aug. 25(3):181-9. [Medline].
Roberson JB Jr, Reinisch J, Colen TY, Lewin S. Atresia repair before microtia reconstruction: comparison of early with standard surgical timing. Otol Neurotol. 2009 Sep. 30(6):771-6. [Medline].
Cole RR, Jahrsdoerfer RA. The risk of cholesteatoma in congenital aural stenosis. Laryngoscope. 1990 Jun. 100(6):576-8. [Medline].
Ngui LX, Tang IP. Bonebridge transcutaneous bone conduction implant in children with congenital aural atresia: surgical and audiological outcomes. J Laryngol Otol. 2018 Aug. 132 (8):693-7. [Medline].
Ren R, Zhao S, Wang D, et al. Audiological effectiveness of Bonebridge implantation for bilateral congenital malformation of the external and middle ear. Eur Arch Otorhinolaryngol. 2019 Oct. 276 (10):2755-62. [Medline].
Goycoolea M, Ribalta G, Tocornal F, et al. Clinical performance of the Osia™ system, a new active osseointegrated implant system. Results from a prospective clinical investigation. Acta Otolaryngol. 2020 Mar. 140 (3):212-9. [Medline].
Jahrsdoerfer RA, Yeakley JW, Aguilar EA, et al. Grading system for the selection of patients with congenital aural atresia. American Journal of Otology. 1992. 13:6-12. [Medline].
Trigg DJ, Applebaum EL. Indications for the surgical repair of unilateral aural atresia in children. Am J Otol. 1998 Sep. 19(5):679-84; discussion 684-6. [Medline].
De la Cruz A, Kesser BW. Management of the Unilateral Atretic Ear. Pensak M. Controversies in Otolaryngology-Head and Neck Surgery. New York: Thieme Medical Publishers; 1999. 381-5.
Imbery TE, Maldonado M, Mukherjee S, Kesser BW. Relationship Between Middle Ear Volume and Long-term Audiological Outcomes in Congenital Aural Atresia Repair. Otol Neurotol. 2019 Jul. 40 (6):782-8. [Medline].
Ju YH, Park E, Park S, Jung K, Lee K, Im GJ. The extent of inferior displacement of the mastoid tegmen is related to the severity of congenital aural atresia. Acta Otolaryngol. 2014 Mar. 134 (3):244-9. [Medline].
Halle TR, Soares BP, Todd NW. Inner ear anomalies in children with isolated unilateral congenital aural atresia. Int J Pediatr Otorhinolaryngol. 2017 Apr. 95:5-8. [Medline].
Jahrsdoerfer RA. Transposition of the facial nerve in congenital aural atresia. Am J Otol. 1995 May. 16(3):290-4. [Medline].
Dostal A, Nemeckova J, Gaillyova R, et al. Identification of 2.3-Mb gene locus for congenital aural atresia in 18q22.3 deletion: a case report analyzed by comparative genomic hybridization. Otol Neurotol. 2006 Apr. 27(3):427-32. [Medline].
Shonka DC, Jahrsdoerfer RA, Kesser BW. The Jahrsdoerfer Grading Scale in Surgery for Congenital Aural Atresia. Arch Otolaryngol Head Neck Surg. Aug. 2008. 134:873-7.
Neumann K, Thomas JP, Voelter C, Dazert S. A new adhesive bone conduction hearing system effectively treats conductive hearing loss in children. Int J Pediatr Otorhinolaryngol. 2019 Jul. 122:117-25. [Medline].
Wazen JJ, Caruso M, Tjellstrom A. Long-term results with the titanium bone-anchored hearing aid: the U.S. experience. Am J Otol. 1998 Nov. 19(6):737-41. [Medline].
Farnoosh S, Mitsinikos FT, Maceri D, Don DM. Bone-Anchored Hearing Aid vs. Reconstruction of the External Auditory Canal in Children and Adolescents with Congenital Aural Atresia: A Comparison Study of Outcomes. Front Pediatr. 2014. 2:5. [Medline].
van der Pouw KT, Snik AF, Cremers CW. Audiometric results of bilateral bone-anchored hearing aid application in patients with bilateral congenital aural atresia. Laryngoscope. 1998 Apr. 108(4 Pt 1):548-53. [Medline].
Attaway J, Stone CL, Sendor C, Rosario ER. Effect of Amplification on Speech and Language in Children With Aural Atresia. Am J Audiol. 2015 Sep 1. 24 (3):354-359. [Medline].
Brito R, Pozzobom Ventura LM, Jorge JC, Oliveira EB, Manzoni Lourencone LF. An Implantable Hearing System As Rehabilitation for Hearing Loss Due to Bilateral Aural Atresia: Surgical Technique and Audiological Results. J Int Adv Otol. 2016 Dec. 12 (3):241-6. [Medline].
Jahrsdoerfer RA, Lambert PR. Facial nerve injury in congenital aural atresia surgery. Am J Otol. 1998 May. 19(3):283-7. [Medline].
Oliver ER, Hughley BB, Shonka DC, Kesser BW. Revision aural atresia surgery: indications and outcomes. Otol Neurotol. 2011 Feb. 32(2):252-8. [Medline].
Kesser BW, Cole ED, Gray LC. Emergence of Binaural Summation After Surgical Correction of Unilateral Congenital Aural Atresia. Otol Neurotol. 2016 Jun. 37 (5):499-503. [Medline].
Nadaraja GS, Gurgel RK, Kim J, Chang KW. Hearing outcomes of atresia surgery versus osseointegrated bone conduction device in patients with congenital aural atresia: a systematic review. Otol Neurotol. 2013 Oct. 34 (8):1394-9. [Medline].
Oliver ER, Lambert PR, Rumboldt Z, Lee FS, Agarwal A. Middle ear dimensions in congenital aural atresia and hearing outcomes after atresiaplasty. Otol Neurotol. 2010 Aug. 31(6):946-53. [Medline].
Edfeldt L, Stromback K. Surgical treatment of congenital aural atresia - is it still justified?. Acta Otolaryngol. 2015 Mar. 135 (3):226-32. [Medline].
Byun H, Moon IJ, Woo SY, et al. Objective and Subjective Improvement of Hearing in Noise After Surgical Correction of Unilateral Congenital Aural Atresia in Pediatric Patients: A Prospective Study Using the Hearing in Noise Test, the Sound-Spatial-Quality Questionnaire, and the Glasgow Benefit Inventory. Ear Hear. 2015 Jul-Aug. 36 (4):e183-9. [Medline].
Ahn J, Ryu G, Kang M, Cho YS. Long-term Hearing Outcome of Canaloplasty With Partial Ossicular Replacement in Congenital Aural Atresia. Otol Neurotol. 2018 Jun. 39 (5):602-8. [Medline].
Sakamoto T, Kikuta S, Kikkawa YS, et al. Prognostic factors for long-term hearing preservation after canal-tympanoplasty for congenital aural atresia. Eur Arch Otorhinolaryngol. 2015 Nov. 272 (11):3151-6. [Medline].
Dobratz E, Rastogi A, Jahrsdoerfer RA, and Kesser BW. To POP or not: Ossiculoplasty in congenital aural atresia surgery. Laryngoscope. Aug. 2008. 118:1452-7.
Digoy GP, Cueva RA. Congenital aural atresia: review of short- and long-term surgical results. Otol Neurotol. 2007 Jan. 28(1):54-60. [Medline].
Chang H, Song JJ, Choi BY, Lee JH, Oh SH, Chang SO. Partial ossicular replacement versus type II tympanoplasty in congenital aural atresia surgery: a matched group study. Otol Neurotol. 2009 Aug. 30(5):609-13. [Medline].
Lambert PR. Congenital aural atresia: stability of surgical results. Laryngoscope. 1998 Dec. 108(12):1801-5. [Medline].
De la Cruz A, Teufert KB. Congenital aural atresia surgery: long-term results. Otolaryngol Head Neck Surg. 2003 Jul. 129(1):121-7. [Medline].
Chang SO, Choi BY, Hur DG. Analysis of the long-term hearing results after the surgical repair of aural atresia. Laryngoscope. 2006 Oct. 116(10):1835-41. [Medline].
Imbery TE, Gray L, Champaloux E, Kesser BW. Long-term Audiometric Outcomes After Atresiaplasty for Congenital Aural Atresia. Otol Neurotol. 2020 Mar. 41 (3):371-8. [Medline].
Gray L, Kesser B, Cole E. Understanding speech in noise after correction of congenital unilateral aural atresia: effects of age in the emergence of binaural squelch but not in use of head-shadow. Int J Pediatr Otorhinolaryngol. 2009 Sep. 73(9):1281-7. [Medline].
Bouhabel S, Arcand P, Saliba I. Congenital aural atresia: bone-anchored hearing aid vs. external auditory canal reconstruction. Int J Pediatr Otorhinolaryngol. 2012 Feb. 76(2):272-7. [Medline].
Yellon RF. Atresiaplasty versus BAHA for congenital aural atresia. Laryngoscope. 2011 Jan. 121(1):2-3. [Medline].
Kesser BW, Cole ED, Gray LC. Emergence of Binaural Summation After Surgical Correction of Unilateral Congenital Aural Atresia. Otol Neurotol. 2016 Jun. 37 (5):499-503. [Medline].
Gray L, Kesser B, Cole E. Understanding speech in noise after correction of congenital unilateral aural atresia: effects of age in the emergence of binaural squelch but not in use of head-shadow. Int J Pediatr Otorhinolaryngol. 2009 Sep. 73 (9):1281-7. [Medline].
Wilmington D, Gray L, Jahrsdoerfer R. Binaural processing after corrected congenital unilateral conductive hearing loss. Hear Res. 1994 Apr. 74(1-2):99-114. [Medline].
[Guideline] Zhang TY, Bulstrode N, Chang KW, et al. International Consensus Recommendations on Microtia, Aural Atresia and Functional Ear Reconstruction. J Int Adv Otol. 2019 Aug. 15 (2):204-8. [Medline]. [Full Text].

Media Gallery

Grade III microtia with complete absence (atresia) of the external auditory canal.
Appearance of surgically-corrected microtia with complete atresia of the external ear canal.
Axial CT scan in a patient with congenital aural atresia (CAA). Note the atretic plate just lateral to the ossicles.
Coronal CT scan of left temporal bone in a patient with congenital aural atresia (CAA). Note absence of the ear canal. The atretic plate may be seen just lateral to the ossicles.
Three-dimensional reconstructed CT scan of lateral skull in a child with aural atresia. Note the absence of a bony aperture (external ear canal) on the side of the left temporal bone.
Coronal CT scan of patient with ear canal cholesteatoma (right ear) in the setting of congenital aural stenosis. Note the rounded edges of ear canal bone filled with soft tissue density extending into the middle ear.
Very low tegmen (bone that separates brain from ear) and lack of middle ear aeration make this patient not a candidate for atresia surgery.
Intraoperative photograph of a newly drilled ear canal, middle ear space, and ossicular chain in a patient with congenital aural atresia (CAA).
Intraoperative image of the bony landmarks used to identify the site for drilling the bony canal in a right ear - zygomatic root, glenoid fossa, mastoid tip. Patient in the surgical position.
Intraoperative image of a left ear in the surgical position upon entering the middle ear in the epitympanum. The fused malleus-incus complex can be seen.
Intraoperative image of a right ear with a lateral facial nerve identified in the mastoid segment.
Intraoperative image of the right ear with the fascia graft draped over the ossicular chain
Intraoperative image of a right ear with the skin graft placed in position
Intraoperative image of a right ear meatoplasty.

of 14

Author

Bradley W Kesser, MD Robert W Cantrell Professor and Vice Chair, Department of Otolaryngology-Head and Neck Surgery, Department of Pediatrics, University of Virginia School of Medicine

Bradley W Kesser, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Neurotology Society, American Otological Society, Society of University Otolaryngologists-Head and Neck Surgeons, Triological Society, Virginia Society of Otolaryngology-Head and Neck Surgery

Disclosure: Received royalty from Nasco, Inc. as an inventor (< $250/year) for: Nasco, Inc.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Arlen D Meyers, MD, MBA Professor of Otolaryngology, Dentistry, and Engineering, University of Colorado School of Medicine

Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cerescan;Cliexa, eMedevents, Neosoma, MI10<br/>Received income in an amount equal to or greater than $250 from: , Cliexa;;Neosoma<br/> Received stock from RxRevu; Received ownership interest from Cerescan for consulting; for: Neosoma, eMedevents, MI10.

Additional Contributors

Cliff A Megerian, MD, FACS Medical Director of Adult and Pediatric Cochlear Implant Program, Director of Otology and Neurotology, University Hospitals of Cleveland; Chairman of Otolaryngology-Head and Neck Surgery, Professor of Otolaryngology-Head and Neck Surgery and Neurological Surgery, Case Western Reserve University School of Medicine

Cliff A Megerian, MD, FACS is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society, American Otological Society, Association for Research in Otolaryngology, Massachusetts Medical Society, Society for Neuroscience, Society of University Otolaryngologists-Head and Neck Surgeons, Triological Society

Disclosure: Nothing to disclose.

Matthew Ng, MD Associate Professor, Department of Otolaryngology-Head and Neck Surgery, University of Nevada, Las Vegas, School of Medicine

Matthew Ng, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Association for the Advancement of Science, American College of Surgeons, North American Skull Base Society, Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Nothing to disclose.

Drew M Horlbeck, MD Clinical Associate Professor, Department of Otolaryngology, Mayo Clinic; Director of Neurotology, Nemours Children's Clinic

Drew M Horlbeck, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, North American Skull Base Society

Disclosure: Nothing to disclose.

Acknowledgements

The author wishes to thank Dr. Robert Jahrsdoerfer for his advice and commentary.

Aural Atresia Guidelines

Guidelines Summary

International Microtia and Atresia Workgroup

References

Tables

Contributor Information and Disclosures

What would you like to print?