# Congenital anomaly of the ear

- Category: chronic
- Review status: reviewed (every claim checked against a document named on this page)
- Sources: GeneReviews - Branchiootorenal Spectrum Disorders (NCBI Bookshelf NBK1380) - https://www.ncbi.nlm.nih.gov/books/NBK1380/ · ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class HD55 - condition scope only, no dose · No dose - referral pathway, no medicine given in primary care
- Verified date: 2026-08

## Verified against

- No dose - referral pathway, no medicine given in primary care
- Congenital anomaly of the ear - disease-level clinical article (congenital-ear-anomaly-clinical.txt)

## Treatment metadata

- No drug therapy in primary care (Referral & Advice)

## Complete treatment card

```text
CONGENITAL ANOMALY OF THE EAR
Sources: GeneReviews - Branchiootorenal Spectrum Disorders (NCBI Bookshelf NBK1380) -
         https://www.ncbi.nlm.nih.gov/books/NBK1380/ · ICPC-3 (WONCA International Classification of
         Primary Care, 3rd edition) class HD55 - condition scope only, no dose · No dose - referral
         pathway, no medicine given in primary care
Review status: REVIEWED against No dose - referral pathway, no medicine given in primary care,
               Congenital anomaly of the ear - disease-level clinical article
               (congenital-ear-anomaly-clinical.txt)  (2026-08)

IS IT THIS? - reference only, to read alongside your own examination
  SYMPTOMS - what the patient reports (3)
    - Microtia is usually noticed at birth or during infancy, sometimes on a routine newborn exam
    - Microtia can occur alone or together with hearing loss, facial asymmetry, inner ear problems,
      heart defects, or kidney malformations  [hearing loss]
    - Patients may describe difficulties with body image, self-confidence, and acceptance by peers
  SIGNS - what you find (5)
    - A fully formed adult ear is roughly 6 cm tall, tilted about 20 degrees back, and set 2 to 2.5
      cm from the mastoid bone
    - In more severe cases, only parts of the earlobe and helix may be present
    - Associated findings can include an underdeveloped half of the face, pits in front of the ear,
      extra skin tags, or absence of the ear canal
    - Conductive hearing loss is common, and a tuning fork test can help confirm it  [hearing loss]
    - In the most severe grade, the whole outer ear and earlobe are absent, called anotia
  TESTS (6)
    - The Marx grading system is widely used to classify how severe the ear underdevelopment is
    - Auditory brainstem response testing should be done early, especially in young children
    - One-sided absence of the ear canal typically causes moderate to severe conductive hearing loss
      of 50 to 65 dB, and 10 to 15% also have some sensorineural loss
    - The unaffected ear should also be tested rather than assumed to hear normally
    - CT of the temporal bone grades the severity of ear canal absence and assesses surgical
      candidacy, but should wait until age 6 to limit radiation exposure
    - A 10-point surgical-candidacy score is used, with a score of 7 or higher predicting a better
      hearing outcome after repair
  IF NOT THIS - what else fits (3)
    - Goldenhar, Treacher-Collins, and Melnick-Fraser syndromes should be considered when an ear
      anomaly is found
    - Prominent ears, cryptotia, cup or lop ear, Stahl's ear, and lobule deformities can be mistaken
      for microtia but are treatable with ear molding within 3 weeks of birth because newborn
      cartilage is still flexible
    - Absence of the ear canal is linked to the more severe grades of microtia and should be
      evaluated for
  Source  StatPearls "Ear Microtia" - disease-level clinical article
  Status  traced to the source above

1. NO DRUG THERAPY IN PRIMARY CARE (REFERRAL & ADVICE)    [1st line]
   Adult    Structural ear anomalies present at birth (skin tags, prominent ears, and rarer
            anomalies of the pinna or canal); most need only reassurance, with referral to ENT or
            plastic surgery for cosmetic correction or when hearing may be affected. - Refer, with
            advice
   Peds     Children follow the same pathway: recognise and refer. No primary-care medicine is
            implied.
   Source   No dose - referral pathway, no medicine given in primary care
   Why      Structural ear anomalies present at birth (skin tags, prominent ears, and rarer
            anomalies of the pinna or canal); most need only reassurance, with referral to ENT or
            plastic surgery for cosmetic correction or when hearing may be affected.
   Caution  Associated canal atresia or other malformation affecting hearing must be assessed with
            audiology; syndromic features (renal, craniofacial) warrant broader work-up.
            No medicine is prescribed for this in primary care - this entry is for recognition and
            referral. Anything given is decided by the service it is referred to.
            RED FLAG - Infants with microtia or aural atresia require early objective hearing
            assessment (auditory brainstem response testing), including testing of the non-atretic
            ear.

Prices are indicative (dataset snapshot 2026-06); verify with the pharmacy.
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