Hearing loss and previous hearing aid placement

• 
  

  Possible use of bone anchored hearing aid (BAHA)

Family history of ear abnormalities, facial clefts, congenital hand conditions, or other craniofacial syndromes

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  Visual impairment (e.g., oculoauricovertebral syndrome)

  
  
• 
  

  Renal disease (e.g., branchio-oto-renal syndrome)

  
  
• 
  

  Cardiac disease (e.g., CHARGE syndrome)

Classify anomaly

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  Unilateral or bilateral

  
  
• 
  

  Severity by grade

  
  
• 
  

  Presence or absence of external ear canal and/or hearing loss

Grades of ear hypoplasia (see Fig. 20-1)

1. 
   

   Mild hypoplasia: Smaller ear, all elements present

   
   
2. 
   

   Moderate hypoplasia: Some missing elements; “concha-type,” possibly canal stenosis

   
   
3. 
   

   Microtia: Small peanut-shaped remnant; “lobule-type,” usually with aural atresia

   
   
4. 
   

   Anotia: Complete absence of external ear

Characteristic associated conditions

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  Hemifacial microsomia: Facial asymmetry

  
  
  
  
  • 
    

    Goldenhar: Preauricular skin tags and epibulbar dermoids

  
  
• 
  

  Treacher Collins syndrome: Malar hypoplasia and micrognathia

  
  
• 
  

  Nager syndrome: High nasal bridge and thumb hypoplasia

  

Audiometric testing

Temporal bone and craniomaxillofacial computerized tomography protocols