Congenital Aural Atresia

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Congenital Aural Atresia

Otology/Audiology
Author Credentials 

J. Walter Kutz, Jr., MD, FACS
Associate Professor
Otolaryngology
University of Texas Southwestern Medical Center
Dallas, TX

Module Summary

Aural atresia usually presents with a conductive hearing loss that can be addressed with bone conduction hearing aids, or in select patients, atresiaplasty surgery. It is essential that children with bilateral congenital aural atresia be fitted with bone conducting hearing aids as soon as possible. The formation of cholesteatoma in partially atretic ears should be suspected. In children where cholesteatoma is not suspected, imaging should occur at 4-6 years. As bone conducting technology advances, the number of patients treated with atresiaplasty has and will continue to decline.

Module Learning Objectives 
  1. Describe the embryology of the external auditory canal and middle ear in relation to congenital aural atresia. 
  2. Perform a thorough and complete history and physical exam for a patient with congenital aural atresia.
  3. Recognize the importance of early auditory intervention in a child with bilateral congenital aural atresia.
  4. Interpret a CT temporal bone scan in the evaluation of a child with congenital aural atresia.
  5. Classify the candidacy for surgery of congenital aural atresia using the Jahrsdoerfer and de la Cruz grading scales.
  6. Discuss the treatment options in a child with congenital aural atresia and include differences in unilateral versus bilateral atresia.
  7. Discuss the expected surgical outcomes with atresiaplasty and list the possible complications of surgery.
  8. Recognize the bone conducting options available including the expected hearing outcomes.

Embryology

Learning Objectives 
  1. Discuss embryogenesis of the ear as it related to development of the pinna, external auditory canal and middle ear.
  2. Identify the branchial arch derivation of the middle ear and ossicles.
  • Malleus
  • Incus
  • Stapes
  • Stapes footplate
  • Tympanic membrane
  • Facial nerve
  1. Identify the first and second branchial arch abnormalities that may lead to congenital aural atresia.
  2. Recognize the severity of the microtia correlates with severity of the middle ear abnormalities.
References 
  1. Anthwal N, Thompson H. The development of the mammalian outer and middle ear. J Anat. 2016 Feb;228(2):217-32.
  2. Kountakis SE, Helidonis E, Jahrsdoerfer RA. Microtia Grade as an Indicator of Middle Ear Development in Aural Atresia. Arch Otolaryngol Head Neck Surg. 1995;121(8):885–886. 

Anatomy

Learning Objectives 
  1. Assess the anatomic abnormalities found in congenital aural atresia including:
  • Inner ear abnormalities
  • Ossicular abnormalities
  • Course of the facial nerve
  • Middle ear and mastoid pneumatization
  • Status of the round window
  1. Determine if the inner ear anatomy is normal, since atresiaplasty is usually contraindicated when inner ear abnormalities are present and a mixed or sensorineural hearing loss may be present.

 

References 
  1. 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.
  2. Yeakley JW, Jahrsdoerfer RA. CT evaluation of congenital aural atresia: what the radiologist and surgeon need to know. J Comput Assist Tomogr. 1996 Sep-Oct;20(5):724-31. 
  3. Dedhia K, Yellon RF, Branstetter BF, Egloff AM. Anatomic variants on computed tomography in congenital aural atresia. Otolaryngol Head Neck Surg. 2012 Aug;147(2):323-8. 
  4. Vrabec JT, Lin JW. Inner ear anomalies in congenital aural atresia. Otol Neurotol. 2010 Dec;31(9):1421-6. 

 

Incidence

Learning Objectives 
  1. Review the incidence and prevalence of microtia and congenital aural atresia.
References 
  1. Luquetti DV, Heike CL, Hing AV, Cunningham ML, Cox TC. Microtia: epidemiology and genetics. Am J Med Genet A. 2012 Jan;158A(1):124-39.

Genetics

Learning Objectives 
  1. Identify what genetic conditions are associated with microtia and congenital aural atresia.
  2. Discuss chromosomal abnormalities that have been associated with congenital aural atresia.
References 
  1. Gendron C, Schwentker A, van Aalst JA. Genetic Advances in the Understanding of Microtia. J Pediatr Genet. 2016;5(4):189-197.
  2. Veltman, J. A., Jonkers, Y., Nuijten, I., Janssen, I., van der Vliet, W., Huys, E., Vermeesch, J., Van Buggenhout, G., Fryns, J.-P., Admiraal, R., Terhal, P., Lacombe, D., Geurts van Kessel, A., Smeets, D., Schoenmakers, E. F. P. M., van Ravenswaaij-Arts, C. M. Definition of a critical region on chromosome 18 for congenital aural atresia by arrayCGH. Am. J. Hum. Genet. 72: 1578-1584, 2003. 
  3. Fatemeh Alasti, Guy Van Camp. Genetics of Microtia and Associated Syndromes. Journal of Medical Genetics, BMJ Publishing Group, 2009, 46 (6), pp.361.

Patient Evaluation

Learning Objectives 
  1. Grade the degree of microtia 
  2. Identify the degree of aural atresia (complete vs. partial)
  3. Assess the status of the contralateral ear in patients with unilateral congenital aural atresia
  4. Demonstrate the use of the tuning fork exam to confirm the type and degree of hearing loss
References 
  1. Kelley PE, Scholes MA. Microtia and congenital aural atresia. Otolaryngol Clin North Am. 2007 Feb;40(1):61-80, vi.

Measurement of Functional Status

Learning Objectives 
  1. Assess hearing status using conventional audiometry including the type of testing used in young children.
  2. Describe how to measure inner ear function in a patient with congenital aural atresia using auditory brainstem response audiometry.

 

References 
  1. American Speech-Language-Hearing Association. (2004). Guidelines for the Audiologic Assessment of Children from Birth to 5 Years of Age [Guidelines]. Available from www.asha.org/policy.
  2. Vander Werff KR, Prieve BA, Georgantas LM. Infant air and bone conduction tone burst auditory brain stem responses for classification of hearing loss and the relationship to behavioral thresholds. Ear Hear. 2009 Jun;30(3):350-68.

 

Imaging

Learning Objectives 
  1. Determine the appropriate imaging modality and age to evaluate congenital aural atresia.
  2. Interpret a CT temporal bone in a patient with congenital aural atresia with an emphasis on using the Jahrsdoerfer criteria.
  3. Identify the following structures on CT in a patient with congenital aural atresia:
  • Malleus/uncus complex
  • Stapes
  • Incis-stapes connection
  • Stapes footplate
  • Round window
  • Course of the facial nerve
  • Middle ear and mastoid pneumatization
  • Inner ear structures
References 
  1. Yeakley JW, Jahrsdoerfer RA. CT evaluation of congenital aural atresia: what the radiologist and surgeon need to know. J Comput Assist Tomogr. 1996 Sep-Oct;20(5):724-31. 
  2. Tasar M, Yetiser S, Yildirim D, Bozlar U, Tasar MA, Saglam M, Ugurel MS,Battal B, Ucoz T. Preoperative evaluation of the congenital aural atresia on computed tomography; an analysis of the severity of the deformity of the middle ear and mastoid. Eur J Radiol. 2007 Apr;62(1):97-105.

 

Treatment

Learning Objectives 
  1. Recognize the importance of early intervention in children with bilateral congenital aural atresia and what type of hearing rehabilitation devices are available.
  2. Explain the treatment options for congenital aural atresia, including bone conducting hearing aids, bone anchored hearing aids, and atresiaplasty. 
  3. Compare the pros and cons of each treatment option.
  4. Compare the hearing outcomes with an intact ossicular chain compared to the requirement of using a prosthesis in atresiaplasty.

 

References 
  1. Casale G, Nicholas BD, Kesser BW. Acquired ear canal cholesteatoma in congenital aural atresia/stenosis. Otol Neurotol. 2014 Sep;35(8):1474-9.
  2. Dobratz EJ, Rastogi A, Jahrsdoerfer RA, Kesser BW. To POP or not: ossiculoplasty in congenital aural atresia surgery. Laryngoscope. 2008 Aug;118(8):1452-7.
  3. Jahrsdoerfer, R. A., Yeakley, J. W., Hall, J. W., Thomas Robbins, K., & Gray, L. C. (1985). High-Resolution CT Scanning and Auditory Brain Stem Response in Congenital Aural Atresia: Patient Selection and Surgical Correlation. Otolaryngology–Head and Neck Surgery, 93(3), 292–298.
  4. McKinnon BJ, Jahrsdoerfer RA. Congenital auricular atresia: update on options for intervention and timing of repair. Otolaryngol Clin North Am. 2002 Aug;35(4):877-90.

 

Surgical Therapies

Learning Objectives 
  1. Discuss the pros and cons of atresiaplasty surgery with the family and patient with congenital aural atresia.
  2. Predict the hearing outcomes of atresiaplasty based on the Jarhsdoerfer criteria.
  3. Summarize the basic steps in atresiaplasty.
  4. Identify pitfalls with atresiaplasty surgery and techniques to avoid these pitfalls.
  5. Discuss the possibility of cholesteatoma formation in children with partial aural atresia.
  6. Compare the advantages and disadvantages of a bone anchored hearing aid compared to atresiaplasty surgery.

 

References 
  1. Jahrsdoerfer RA, Yeakley JW, Aguilar EA, Cole RR, Gray LC. Grading system for the selection of patients with congenital aural atresia. Am J Otol. 1992 Jan;13(1):6-12.
  2. Kesser BW, Goddard JC. Otologic Surgery. Congenital malformations of the external auditory canal and middle ear. In: Brackmann D, Shelton C, Arriaga M. Otologic Surgery. 4th ed. Elsevier; 2015. p. 42-58. 
  3. Dobratz EJ, Rastogi A, Jahrsdoerfer RA, Kesser BW. To POP or not: ossiculoplasty in congenital aural atresia surgery. Laryngoscope. 2008 Aug;118(8):1452-7.
  4. Casale G, Nicholas BD, Kesser BW. Acquired ear canal cholesteatoma in congenital aural atresia/stenosis. Otol Neurotol. 2014 Sep;35(8):1474-9. 
  5. Cole RR, Jahrsdoerfer RA. The risk of cholesteatoma in congenital aural stenosis. Laryngoscope. 1990 Jun;100(6):576-8.
  6. Yellon RF. Atresiaplasty versus BAHA for congenital aural atresia. Laryngoscope. 2011 Jan;121(1):2-3.
  7. 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.

 

Rehabilitation

Learning Objectives 
  1. Differentiate the expected hearing outcomes between a bone anchored hearing aid and atresiaplasty surgery.
  2. Explain the effect of untreated unilateral atresia on school performance, grade retention, and academic success.
References 
  1. Moss WJ, Lin HW, Cueva RA. Surgical and Audiometric Outcomes for Repair of Congenital Aural Atresia and Hypoplasia. JAMA Otolaryngol Head Neck Surg. 2016 Jan;142(1):52-7.
  2. Jensen DR, Grames LM, Lieu JEC. Effects of Aural Atresia on Speech Development and Learning: Retrospective Analysis From a Multidisciplinary Craniofacial Clinic. JAMA Otolaryngol Head Neck Surg. 2013;139(8):797–802. 
  3. van Hövell Tot Westerflier CVA, van Heteren JAA, Breugem CC, Smit AL, Stegeman I. Impact of unilateral congenital aural atresia on academic Performance: A systematic review. Int J Pediatr Otorhinolaryngol. 2018 Nov;114:175-179.
  4. 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.
  5. De la Cruz, A. D., & Teufert, K. B. (2003). Congenital aural atresia surgery: Long-term results. Otolaryngology–Head and Neck Surgery, 129(1), 121–127. 

 

Staging

Learning Objectives 
  1. Classify the candidacy for surgery of congenital aural atresia using the Jahrsdoerfer grading scale.
  2. Classify the candidacy for surgery of congenital aural atresia De la Cruz grading scale.
References 
  1. Jahrsdoerfer RA, Yeakley JW, Aguilar EA, Cole RR, Gray LC. Grading system for the selection of patients with congenital aural atresia. Am J Otol. 1992 Jan;13(1):6-12.
  2. De la Cruz A, Linthicum FH Jr, Luxford WM. Congenital atresia of the external auditory canal. Laryngoscope. 1985 Apr;95(4):421-7.

 

Case Studies

  1. A 3-month-old male presents to your clinic with bilateral microtia and congenital aural atresia. His perinatal history of normal, and he has no other medical conditions. Exam shows bilateral grade 2 microtia and complete bilateral aural atresia. What audiologic test would you order? Would you order imaging at this point? If so, what imaging would you order? If not, at what age would you order imaging? Would you consider rehabilitation options at this point? If so, what rehabilitation option would you recommend?
  2. A 5-year-old female presents with left microtia and congenital aural atresia. She has a history of Goldenhar’s syndrome and has a left hypoplastic mandible and facial asymmetry. Exam shows a grade 3 microtia and complete aural atresia in her left ear. What audiologic testing would you order? Do expect a conductive, sensorineural, or mixed hearing loss? What imaging would you order? How would you counsel the family on treatment options? Is atresiaplasty surgery advisable in this patient?
  3. A 4-year-old male present with a right grade 1 microtia and partial congenital aural atresia. His parents report drainage from the right ear for the past three months. Exam shows a grade 1 microtia and a narrow external auditory canal that prevents examination of the tympanic membrane. The external auditory canal is filled with purulence. What imaging would you order? What condition are you most concerned about? What treatment option for congenital aural atresia would you initially recommend?

Complications

Learning Objectives 
  1. Discuss the potentials complications of atresiaplasty surgery, both perioperative as well as delayed.
  2. Discuss the potentials complications of bone anchored hearing aid surgery, both perioperative as well as delayed.

 

References 
  1. Shapiro S, Ramadan J, Cassis A. BAHA Skin Complications in the Pediatric Population: Systematic Review With Meta-analysis. Otol Neurotol. 2018 Aug;39(7):865-873.
  2. Van der Gucht K, Vanderveken O, Hamans E, Claes J, Van Rompaey V, Van de Heyning P. Adverse skin reactions following percutaneous bone conduction implant surgery using the linear incision technique with and without subcutaneous tissue reduction. Acta Otolaryngol. 2017 Feb;137(2):149-153.
  3. Li CL, Dai PD, Yang L, Zhang TY. A meta-analysis of the long-term hearing outcomes and complications associated with atresiaplasty. Int J Pediatr Otorhinolaryngol. 2015 Jun;79(6):793-7.
  4. House JW, Kutz JW Jr. Bone-anchored hearing aids: incidence and management of postoperative complications. Otol Neurotol. 2007 Feb;28(2):213-7.

 

Review

References 
  1. What are the 9 factors in the Jahrsdoerfer grading scale? What factor counts as 2 points?
  2. What are the four factors of the de la Cruz grading scale?
  3. What is the first step in managing a child with bilateral congenital aural atresia?
  4. What serious condition may occur in a child with partial aural atresia that requires imaging for evaluation?
  5. Does an atresiaplasty or a bone anchored hearing aid provide better a audiologic result?
Resources 

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