eMedicine Specialties > Pediatrics: Surgery > Otolaryngology

External Auditory Canal Atresia: Differential Diagnoses & Workup

Author: Rahul K Shah, MD, FAAP, Assistant Professor of Otolaryngology and Pediatrics, George Washington University, Children's National Medical Center; Attending Physician, Department of Otolaryngologoy, Children's National Medical Center
Coauthor(s): Udayan K Shah, MD, Attending Surgeon, Division of Otolaryngology, Alfred I duPont Hospital for Children
Contributor Information and Disclosures

Updated: Jul 18, 2008

Differential Diagnoses

Cleft Lip and Palate
Crouzon Syndrome
Microtia
Osteogenesis Imperfecta

Other Problems to Be Considered

Goldenhar syndrome (hemifacial microsomia)
Acquired external auditory canal (EAC) stenosis
Assessment for other craniofacial abnormalities (important for implications in anesthetic management, particularly for perioperative airway obstruction)
Treacher-Collins syndrome

Workup

Laboratory Studies

  • Localization tests (eg, lacrimation, salivation, gustatory testing) for facial nerve function have a limited role.
  • Genetic testing for chromosomal or other genetic defects is not currently routine for nonsyndromic external auditory canal atresia (EACA). However, the likelihood of future work to identify genetic etiologies for craniofacial disorders makes the discussion of genetic etiologies and banking of blood important.

Imaging Studies

Radiologic imaging is critical for accurate counseling of the family and surgical decision making.

  • CT scanning (without contrast) of the temporal bones in axial and coronal direct sections (not reconstructions) obtained in thin 1-mm cuts is required (see Media files 4-5).
  • Radiologic assessment as close to the planned operation as possible, usually within 3-6 months of surgery, is ideal. Several scans may be obtained to determine the relationship to the temporomandibular joint and monitor development of critical structures, specifically mastoid tip and middle-ear structures. Some surgeons prefer to perform CT scanning soon after birth to ensure that no congenital cholesteatoma is present. This was demonstrated in a rare case report of a congenital cholesteatoma in a patient with congential aural atresia.2
  • CT scan review focuses on external-ear, middle-ear, and inner-ear development and on the boundaries of the EACA, specifically the middle cranial fossa superiorly, the carotid artery and jugular bulb, and the intratemporal fossa.
  • Relation of the pinna to the EACA and temporomandibular joint can be appreciated.
  • Development of the ossicles and presence of a cholesteatoma and possibility of middle ear fluid are evaluated. Mastoid development and facial nerve location are also assessed.

Other Tests

  • Literature based on children with OM has shown that parents and physicians are not able to reliably predict hearing loss due to ear infections. Objective audiologic assessment is therefore required.
    • Audiologic evaluation (see Media files 2-3) with age-appropriate audiometric evaluation by an audiologist with pediatric expertise is optimal. Periodic testing at regular intervals is best.
    • Behavioral testing is possible when children are able to sit upright and turn to stimuli, usually around 6 months of age.
    • Auditory brainstem response (ABR) audiometry may be used in children unable to undergo behavioral testing and for validation of abnormal behavioral results.
  • Formal vestibular testing may be required when symptoms of dizziness warrant. Sheykholeslami et al (2005) used vestibular-evoked myogenic potentials (VEMPs), a promising vestibular evaluation, to test children with bilateral EACA.3 The VEMP in neonates with audio-vestibular problems was successfully obtained and generated information about vestibular function. The advantage in testing vestibular function in this cohort of patients is that care and rehabilitative potential can be improved.
  • Facial nerve testing using electroneuronography (ENog) or electromyography (EMG) may be indicated for weak or nonfunctioning facial nerves, particularly before surgery is planned, and occasionally after surgery.
  • Note that audiologic function most closely correlates with formation and development of the oval/round windows and ossicular development.4

Staging

Many classification systems exist for the staging of the degree of atresia of the EAC. The more commonly used systems are noted below. All these categorize the development and relations of the external canal, the tympanic membrane, middle ear development, and ossicular structures.

  • Altmann classification
    • Type I
      • Small external canal
      • Hypoplastic temporal bone/tympanic membrane
      • Normal/contracted middle ear
      • Normal/malformed ossicles
    • Type II
      • Absent external canal with atretic plate
      • Small middle ear
      • Fixed/malformed malleus and incus
    • Type III
      • Absent external canal
      • Contracted/absent middle ear
      • Present/absent ossicles
  • De la Cruz classification
    • Minor
      • Mastoid pneumatization normal
      • Normal oval window
      • Normal inner ear
      • Facial nerve/oval window relationship acceptable
    • Major
      • Mastoid poorly pneumatized
      • Oval window absent or abnormal
      • Inner ear malformations
      • Facial nerve aberration
  • Ombredanne classification
    • Minor
      • Normal/small external canal
      • Normal middle ear
      • Fixed/deformed ossicles
      • Microtia (possibly)
    • Major
      • External canal and tympanic membrane absent
      • Aberrant/dehiscent facial nerve
      • Microtia

More on External Auditory Canal Atresia

Overview: External Auditory Canal Atresia
Differential Diagnoses & Workup: External Auditory Canal Atresia
Treatment & Medication: External Auditory Canal Atresia
Follow-up: External Auditory Canal Atresia
Multimedia: External Auditory Canal Atresia
References
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References

  1. Jahrsdoerfer, RA. Congenital Atresia of the Ear. Laryngoscope. 1978;88(9 pt. 3 Suppl 13):1-48. [Medline]. [Full Text].

  2. Caughey RJ, Jahrsdoerfer RA, Kesser BW. Congenital cholesteatoma in a case of congenital aural atresia. Otol Neurotol. Oct 2006;27(7):934-6. [Medline].

  3. Sheykholeslami K, Sheykholesami K, Megerian CA, Kaga K. Vestibular-evoked myogenic potentials in infancy and early childhood. Laryngoscope. Aug 2005;115(8):1440-4. [Medline].

  4. Ishimoto S, Ito K, Karino S, Takegoshi H, Kaga K, Yamasoba T. Hearing levels in patients with microtia: correlation with temporal bone malformation. Laryngoscope. Mar 2007;117(3):461-5. [Medline].

  5. Sheykholeslami K, Mohammad HK, Sebastien S, Kaga K. Binaural interaction of bone-conducted auditory brainstem responses in children with congenital atresia of the external auditory canal. Int J Pediatr Otorhinolaryngol. 2003;67(10):1083-90. [Medline].

  6. Siegert R, Mattheis S, Kasic J. Fully implantable hearing aids in patients with congenital auricular atresia. Laryngoscope. Feb 2007;117(2):336-40. [Medline].

  7. Lambert PR. Major congenital ear malformations: surgical management and results. Ann Otol Rhinol Laryngol. Nov-Dec 1988;97(6 Pt 1):641-9. [Medline].

  8. Jahrsdoerfer RA, Yeakley JW, Aguilar EA, et al. Grading system for the selection of patients with congenital aural atresia. Am J Otol. Jan 1992;13(1):6-12. [Medline].

  9. Glasscock ME 3rd, Schwaber MK, Nissen AJ, Jackson CG. Management of congenital ear malformations. Ann Otol Rhinol Laryngol. Sep-Oct 1983;92(5 Pt 1):504-9. [Medline].

  10. Battelino S, Hocevar-Boltezar I, Zargi M. Intraoperative use of mitomycin C in fibrous atresia of the external auditory canal. Ear Nose Throat J. Dec 2005;84(12):776-9. [Medline].

  11. Yamane H, Sunami K, Takayama M, et al. Application of modified transmastoid approach methods to congenital atresia of the external ear canal with middle ear infection. Acta Otolaryngol Suppl. Oct 2004;38-44. [Medline].

  12. Teufert KB, De la Cruz A. Advances in congenital aural atresia surgery: effects on outcome. Otolaryngol Head Neck Surg. 2004;131(3):263-70. [Medline].

  13. Satar B, Yetiser S, Ozkaptan Y. Acoustic characteristics of reconstructed ear canal after atresia surgery. Br J Audiol. 2000;34(6):379-81. [Medline].

  14. De la Cruz A, Linthicum FH Jr, Luxford WM. Congenital atresia of the external auditory canal. Laryngoscope. Apr 1985;95(4):421-7. [Medline].

  15. Wetmore RF, Muntz HR, McGill TJ. Congenital malformations of the ear. In: Pediatric Otolaryngology Principles and Practice Pathways. 2000:235-52.

  16. Evans AK, Kazahaya K. Canal atresia: "surgery or implantable hearing devices? The expert's question is revisited". Int J Pediatr Otorhinolaryngol. Mar 2007;71(3):367-74. [Medline].

  17. Blevins NH, Byahatti SV, Karmody CS. External auditory canal duplication anomalies associated with congenital aural atresia. J Laryngol Otol. Jan 2003;117(1):32-8. [Medline].

  18. Caversaccio M, Romualdez J, Baechler R, et al. Valuable use of computer-aided surgery in congenital bony aural atresia. J Laryngol Otol. Apr 2003;117(4):241-8. [Medline].

  19. Hol MK, Cremers CW, Coppens-Schellekens W, Snik AF. The BAHA Softband. A new treatment for young children with bilateral congenital aural atresia. Int J Pediatr Otorhinolaryngol. Jul 2005;69(7):973-80. [Medline].

  20. Jacobsen N, Mills R. Management of stenosis and acquired atresia of the external auditory meatus. J Laryngol Otol. Apr 2006;120(4):266-71. [Medline].

  21. Parrish KL, Amedee RG. Atresia of the external auditory canal. J La State Med Soc. Sep 1990;142(9):9-12. [Medline].

  22. Swartz JD, Faerber EN. Congenital malformations of the external and middle ear: high-resolution CT findings of surgical import. AJR Am J Roentgenol. Mar 1985;144(3):501-6. [Medline].

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Further Reading

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Keywords

external auditory canal atresia, congenital aural atresia, congenital atresia of the external auditory canal, EAC, EACA, EAC atresia, conductive hearing loss, ossicular malformations, craniofacial syndromes, atretic external canal, microtia, otitis media, OM, mastoiditis, facial nerve palsy, cholesteatoma

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Contributor Information and Disclosures

Author

Rahul K Shah, MD, FAAP, Assistant Professor of Otolaryngology and Pediatrics, George Washington University, Children's National Medical Center; Attending Physician, Department of Otolaryngologoy, Children's National Medical Center
Rahul K Shah, MD, FAAP is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, Massachusetts Medical Society, Phi Beta Kappa, Society of University Otolaryngologists-Head and Neck Surgeons, and Triological Society
Disclosure: Nothing to disclose.

Coauthor(s)

Udayan K Shah, MD, Attending Surgeon, Division of Otolaryngology, Alfred I duPont Hospital for Children
Udayan K Shah, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Society for Laser Medicine and Surgery, American Society of Pediatric Otolaryngology, International Society for Optical Engineering, Massachusetts Medical Society, Pennsylvania Medical Society, Phi Beta Kappa, and Society for Ear, Nose and Throat Advances in Children
Disclosure: Nothing to disclose.

Medical Editor

Orval Brown, MD, Director of Otolaryngology Clinic, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas
Orval Brown, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Society of University Otolaryngologists-Head and Neck Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

John E McClay, MD, Assistant Professor, Department of Otolaryngology, Division of Pediatric Otolaryngology, Children's Medical Center, University of Texas Southwestern Medical School
John E McClay, MD is a member of the following medical societies: American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, and American Medical Association
Disclosure: Nothing to disclose.

CME Editor

Paul D Petry, DO, FACOP, FAAP, Consulting Staff, Freeman Pediatric Care, Freeman Health System
Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
Disclosure: Nothing to disclose.

Chief Editor

Maureen Strafford, MD, Arnold P Gold Foundation Associate Professor, Departments of Anesthesiology and Pediatrics, Tufts University and Tufts-New England Medical Center
Maureen Strafford, MD is a member of the following medical societies: American Medical Women's Association, American Pain Society, American Society of Anesthesiologists, International Anesthesia Research Society, Society for Education in Anesthesia, Society for Pediatric Anesthesia, and Society of Cardiovascular Anesthesiologists
Disclosure: Nothing to disclose.

 
 
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