External Auditory Canal Atresia Clinical Presentation

  • Author: Rahul K Shah; Chief Editor: Glenn C Isaacson, MD, FACS, FAAP   more...
 
Updated: Oct 11, 2011
 

History

Diagnosis of a congenital ear malformation is usually made at birth, when a malformed pinna or atretic canal is noticed during the secondary survey of the newborn. Some cases may not be noticed at birth, such as those with a normal pinna and a blunted or partially patent canal. Some of these patients are detected during a screening examination performed in school.

  • When soliciting information regarding the history of the present illness, determine how and when the problem was discovered and ask about the child's language and social development and associated problems.
  • Survey the family for history of hearing loss and syndromic conditions and for genetic information.
  • Inquire into maternal and neonatal health.
  • Identify the state of patient's social and language development.
    • The child's interactions with other children, caregivers, and family are important.
    • The patient's language development, with age of babbling and first words or number of words, is a useful measure.
    • Children with unilateral external auditory canal atresia (EACA) usually have normal speech and language development. Hearing and learning may be impeded in noisy environments, such as group discussions or large lectures, and sound localization may be a problem at play and when outdoors.
  • Rarely, an adult presents with a report of a hearing loss, and the physical examination reveals EACA.
  • Reports of vertigo may require a formal assessment of the vestibular system.
  • Pain, fever, or drainage from the atretic ear may indicate cholesteatoma or infection of the ear canal.
  • History of facial nerve problems must be identified, particularly before surgical repair. Problems with eye closure may manifest as dry, itchy, or sandy eyes and rarely as exposure keratitis. Problems drinking liquids or drinking with a straw may also indicate facial nerve dysfunction.
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Physical

The global level of functioning of the child should be addressed first. Complete head and neck examination then follows with attention to craniofacial anomalies. Then, otologic structures are assessed. For complete care of patients with EACA, physical examination must be combined with radiographic evaluation.

Examine the remainder of the body so as not to overlook other abnormalities, especially cardiac, renal, or ophthalmologic malformations.

  • Pinna: Assess for microtia.
  • Concha: A thumbprint concha is a classic sign for EACA (see the image below). The meatus of the EAC may simply be a pinpoint, indicating severe canal stenosis rather than complete atresia. Care of these patients is similar to that of patients with EACA. Photograph of the right ear in a patient with unilPhotograph of the right ear in a patient with unilateral external auditory canal atresia is shown. Note the relatively normal pinna and thumb print concha.
  • Congential ear malformations
    • These can be divided into major and minor forms.
    • Major ear malformations consist of an absence of the external canal and tympanic membrane, a small middle-ear cavity, and malformations of the malleus and incus. Microtia and a hypoplastic mandible are concomitant findings in patients with major ear malformations.
    • Minor ear malformations consist of abnormal ossicles with a patent EAC and tympanic membrane, although the latter 2 structures may be smaller than normal.
  • Lateral skull
    • The relationship of the temporomandibular joint in relation to the mastoid tip and middle ear is documented.
    • Mastoid tip development should be assessed and especially compared to the contralateral side. Poor mastoid tip development portends abnormal facial nerve anatomy.
  • Facial nerve
    • Evaluation should be performed using scoring or grading according to established systems, such as those by Fisch or House and Brackmann.
    • An objective assessment may be indicated in cases of palsy (see Lab Studies).
  • Documentation
    • Photographic records of anomalies aid referrals, teaching, and objective analysis of surgical results.
    • Images should be standardized with respect to lighting and dimensions.
    • Appropriate consents from the family and patient should be obtained for teaching and publication purposes, ideally at the time of such documentation.
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Causes

Precise etiology of the failure of EAC canalization is not known. Associations have been postulated between EACA and low birth weight, intrauterine trauma, toxins, or infection. Genetic defects are being identified for several craniofacial anomalies. Because of the importance of future genetic studies, serum needs to be obtained and banked for eventual genetic testing.

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Contributor Information and Disclosures
Author

Rahul K Shah  MD, FACS, FAAP, Associate Professor of Otolaryngology and Pediatrics, Medical Director, Peri-operative Services, Children's National Medical Center, George Washington University School of Medicine and Health Sciences; Attending Physician, Department of Otolaryngology, Children's National Medical Center

Rahul K Shah is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American College of Medical Quality, American College of Physician Executives, American College of Surgeons, Massachusetts Medical Society, Phi Beta Kappa, and Triological Society

Disclosure: Nothing to disclose.

Coauthor(s)

Udayan K Shah, MD  Associate Professor of Otolaryngology-Head and Neck Surgery and Pediatrics, Jefferson Medical College, Thomas Jefferson University; Director, Fellow and Resident Education in Pediatric Otolaryngology, Attending Surgeon, Division of Otolaryngology, Nemours-AI 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 of Pediatric Otolaryngology, International Society for Optical Engineering, Pennsylvania Medical Society, Phi Beta Kappa, and Society for Ear, Nose and Throat Advances in Children

Disclosure: Gyrus-ACMI Royalty Device development; Reiseman, Rosenberg LLC Consulting fee medico-legal case review

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John E McClay, MD  Associate Professor of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, Children's Hospital of Dallas, 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.

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

Glenn C Isaacson, MD, FACS, FAAP  Professor of Otolaryngology-Head and Neck Surgery and Pediatrics, Temple University School of Medicine

Glenn C Isaacson, MD, FACS, FAAP 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 Laryngological Rhinological and Otological Society, American Society of Pediatric Otolaryngology, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Covidien Honoraria Consulting

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Photograph of the right ear in a patient with unilateral external auditory canal atresia is shown. Note the relatively normal pinna and thumb print concha.
Audiogram of a 5-year-old girl with right unilateral external auditory canal atresia (EACA) is shown. The audiogram demonstrates a right conductive hearing loss seen by the difference in hearing levels between the red triangles and the black brackets. The left ear hearing levels shown by the blue line are normal. This child demonstrated good function at home and in preschool and did not require intervention at this point.
Audiogram 1 year later of the same child as in the image above. Her functioning in kindergarten continues to be excellent, and she has noticed hearing improvement at home. Her physical examination shows a pinpoint opening at the external auditory canal (EAC) now, and this audiogram confirms improved hearing in the right ear compared to the prior audiogram (see image above). Her right conductive hearing loss is shown by the distance between the black brackets and the red triangles.
CT scan in the axial plane of right unilateral external auditory canal atresia (EACA) is shown. The right external auditory canal (EAC), to the left of this photo, is not developed compared to the normally developed left EAC. The image is slightly tilted in the horizontal plane.
CT scan showing unilateral auditory canal atresia in coronal section of the right ear. No ear canal is seen to the left of this picture (patient's right).
 
 
 
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