Osteogenesis imperfecta

Others

• OI from the cellular and molecular perspective
• The bone in OI
• Other orthopedic disorders caused by OI
• Oro-cranial disorders in OI
• Hearing in OI
  • 1. Ear and hearing
  • 2. Hearing assessment
  • 3. Types of hypoacusia
  • 4. Hearing loss in OI
  • 5. Management of hearing loss
  • Summary chapter 5
• Vision and OI
• Other clinical features of OI
• Results of the study about osteogenesis imperfecta (Rare Commons)

Summary chapter 5

 

• The ear is the organ responsible for hearing and balance. It is made up of three parts: the outer ear (pinna and external auditory canal), the middle ear (eardrum, auditory ossicles, Eustachian tube) and the inner ear (cochlea, labyrinth, auditory nerve).
• Sound waves travel through the external auditory canal, strike the eardrum and produce a vibration that is transmitted through the 3 ossicles of the ear. The stimulus reaches the inner ear where the sound pressure is converted into electrical impulses that travel through the auditory nerve to the brain.
• Depending on where the hearing impairment is located, we may have conductive, sensorineural or mixed hearing loss.
• The risk of hearing loss in OI is higher than in the general population. It is usually detected from the second or third decade of life and is progressive.
• The management of hearing loss in OI must be individualised for each patient according to the type and degree of hearing loss. In mild to moderate cases, the use of hearing aids may be sufficient, while in some cases middle ear or inner ear surgery may be necessary.

Last modified

30 August 2023