Aural Atresia Workup

  • Author: Bradley W Kesser, MD; Chief Editor: Arlen D Meyers, MD, MBA   more...
 
Updated: May 9, 2012
 

Laboratory Studies

In a healthy patient with no other abnormalities, laboratory investigation is generally not necessary. A small subset of patients with aural atresia will have 18q chromosome deletion syndrome. These children typically have normal auricles with complete atresia of the ear canal. Genetic testing can identify this rare condition.[7, 15]

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Imaging Studies

Thin section (1 mm sections), high-resolution, axial and coronal computed tomography (CT) of the temporal bone is the study of choice in the workup and evaluation of patients with congenital aural atresia (CAA).

  • Perform the study by obtaining contiguous images with a thickness of 1 mm or less (submillimeter thickness) in both axial and coronal planes (coronal reconstruction is adequate with the appropriate software).
  • Imaging provides information on whether surgical repair is feasible based on the anatomy present.
  • Obtain imaging when surgical correction is contemplated or just before surgery.
  • As long as audiologic evidence reveals that the inner ear is functional, imaging is not necessary during early infancy and childhood.

In 1992, Jahrsdoerfer et al developed a 10-point grading system to determine surgical candidacy based on key features from the CT scan and the appearance of the external ear.[11] This scheme is also used to indirectly estimate the likelihood of success if surgical correction is performed.[16]

Jahrsdoerfer operates on unilateral patients with scores of 7 or higher; bilateral patients with a grade 5-6 or higher. This system bases surgical success on a postoperative speech-reception threshold (SRT) of less than or equal to 30 dB HL, which, depending on the anatomy, is attainable in over 80% of patients.[16] As a reflection of the conservative nature of this grading system, a score of 10 is never given.

The following is a summary of this 10-point grading system:

  • Stapes present = 2 points
  • Oval window open = 1 point
  • Middle ear space = 1 point
  • Facial nerve = 1 point
  • Malleus-incus complex = 1 point
  • Mastoid pneumatization = 1 point
  • Incus-stapes connection = 1 point
  • Round window = 1 point
  • External ear appearance = 1 point

Each ear is given a grade based on the CT anatomy and this scale. The anatomy predicts the candidacy of that ear for atresia surgery.

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Other Tests

Audiometry is equally as important as imaging in the evaluation of the patient with CAA. Air and bone conduction pure tone thresholds at 250 Hz, 500 Hz, 1000 Hz, 2000 Hz, 4000 Hz, 6000 Hz, and 8000 Hz, speech reception thresholds, and speech discrimination scores comprise the routine audiological assessment. Air conduction thresholds in the atretic ear tend to run in the 60-70 dB HL range with similar speech reception thresholds. Bone conduction thresholds in the atretic ear are typically in the normal range (and if they are not, the patient may not be a candidate for surgery), with excellent speech discrimination scores.

The masking dilemma arises when testing the patient with bilateral CAA. How does the audiologist establish bone conduction thresholds for each ear in bilateral CAA? The sensorineural acuity level test (SAL) allows the establishment of individual bone conduction thresholds in bilateral CAA, as follows:

  • Determine patient's thresholds through earphones to 500, 1000, 2000, and 4000 Hz.
  • Place a bone oscillator on the forehead and present masking through it at the following intensities:
    • 500 Hz - 50 db HL
    • 1000 Hz - 50 db HL
    • 2000 Hz - 60 db HL
    • 4000 Hz - 60 db HL
  • With masking present, reestablish air conduction thresholds.
  • At each frequency, determine the difference between the masked air conduction threshold and the unmasked air conduction threshold
  • Norms of threshold shift with bone conduction masking are as follows:
    • 500 Hz - 40 db HL
    • 1000 Hz - 50 db HL
    • 2000 Hz - 45 db HL
    • 4000 Hz - 50 db HL

If the masked threshold shifts more than the norm, the bone conduction threshold is 0 dB HL. If the masked threshold shifts less than the norm, then the bone conduction threshold is the difference between the norm and the amount of shift (eg, if the norm is 50 and the amount of shift is 30, the masked bone conduction is 20).

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Contributor Information and Disclosures
Author

Bradley W Kesser, MD  Associate Professor, Department of Otolaryngology-Head and Neck Surgery, University of Virginia

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

Disclosure: Nasco, Inc. Royalty Inventor

Coauthor(s)

Matthew Ng, MD  Clinical Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Keck School of Medicine of the University of Southern California; Clinical Assistant Professor, Department of Surgery, Division of Otolaryngology, University of Nevada 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, American Laryngological Rhinological and Otological Society, Association for Research in Otolaryngology, Johns Hopkins Medical and Surgical Association, North American Skull Base Society, Society of University Otolaryngologists-Head and Neck Surgeons, and Vestibular Disorders Association

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 and North American Skull Base Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Cliff A Megerian, MD, FACS  Medical Director of Adult and Pediatric Cochlear Implant Program, Vice-Chairman and Director of Otology and Neurotology, University Hospitals of Cleveland; Professor, Department 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, and Triological Society

Disclosure: cochlear americas Consulting fee Board membership; Grace Corporation Consulting fee Board membership

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

Disclosure: Medscape Salary Employment

Gregory C Allen, MD  Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine

Gregory C Allen, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Cleft Palate/Craniofacial Association, American College of Surgeons, American Laryngological Rhinological and Otological Society, American Medical Association, Christian Medical & Dental Society, and Colorado Medical Society

Disclosure: Nothing to disclose.

Christopher L Slack, MD  Private Practice in Otolaryngology and Facial Plastic Surgery, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders

Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association

Disclosure: Nothing to disclose.

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, and American Head and Neck Society

Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation Unrestricted gift Unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Medvoy Ownership interest Management position; Cerescan Imaging Consulting; Headwatersmb Consulting fee Consulting; Venturequest Royalty Consulting

Additional Contributors

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

References

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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).
 
 
 
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