Hearing Loss

Simplified Explanation

Nearly 50% of people born with Pfeiffer syndrome will have some degree of hearing loss. The types of hearing loss are conductive, sensorineural, and mixed.

  • Conductive hearing loss: Sound is not conducted efficiently through the outer ear canal to the eardrum and the tiny bones of the middle ear. This is common in people with Pfeiffer Syndrome due to the early fusion of bones in the area of the ear.
  • Sensorineural hearing loss (SNHL): There is damage to the inner ear (cochlea), or to the nerve pathways from the inner ear to the brain.
  • Mixed hearing loss: A combination of conductive and sensorineural hearing loss.


An Auditory Brainstem Response (ABR) test is an objective test that determines hearing loss. It tests hearing sensitivity and identifies neurological abnormalities of the auditory nerve and the auditory pathway up through the brainstem. Young children are often sedated for the test. Common treatments for hearing loss include hearing aids or cochlear implants. Speech therapy may also be required; a speech pathologist can evaluate and recommend treatment.

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