Anotia is a rare hereditary malformation that causes complete absence of the auricle, the outer part is referred as 'Pinna' and lack of external auditory meatus. 'Auditory Meatus' has two passages one is ear drum and other for blood vessels and nerves. This disease may cause conductive hearing loss, deafness.

The Human Ear and Processing Sound

The human ear divided into three specialized functional regions.

  • The outer ear acts like a tube and takes in the sound.
  • The middle part of ear contains ear drum section and several bones which are moved accordingly when sound waves enter the ear through the canal. 
  • The inner region of ear is called as cochlea which holds small hair like cells that respond to noise and transmit the sound waves to brain via nerve impulses.

Defects of the ear: Anotia/Microtia

This is only disease not connected with syndrome, of anotia is not known, although it is supposed to be of hereditary basis. Anotia/Microtia disease causes the ear when the certain tissues connected with the auricle do not develop. This kind of defect takes place due to a part of syndrome or as an isolated abnormality. This rare defect may occur as part of a syndrome.

Type I: External portion of the ear is small and the auricle keeps possession of most of its usual structure.

Type II: The external ear is quite abnormal and can be hook, ‘S’ or ‘?’ shaped in appearance.

Type III: The external is a basic soft tissue structure with no cartilage; the earlobe does not have a usual form.

Type IV: Anotia is that type of disease where in all structures of external ear are not present.


  • Acutane embryopathy
  • Goldenhar disease
  • Pallister-Hall Syndrome
  • Verloes Gillerot Fryns syndrome
  • Wisconsin syndrome

Causes of Anotia

  • Treacher Collins-Franceschetti syndrome
  • Hurst's microtia-absent patellae-micrognathia syndrome
  • Ablepharon-macrostomia syndrome
  • Goldenhar syndrome


The treatment varies from patient to patient, but the most common method used to treat anotia is surgical repair. It reconstructs the ear's normal shape and repairs the canal of the ear. Bone Anchored Hearing Aid (BAHA) surgery is used to restore the hearing.

  • Home Testing and Anotia
  • Home Ear Infection Testing
  • Home Ear Tests
  • Home Hearing Testing
  • Anotia Research Doctors and Specialists
  • Ear, Nose & Throat Specialists:
  • Otolaryngology (Ear, Nose & Throat)
  • Pediatric Otolaryngology (Child Ear, Nose & Throat Specialist)
  • Otology / Neurotology (Ear/Hearing Specialists)
  • Plastic Surgery within the Head & Neck