Can hearing impairment and/or sensitivity occur in EDS?

Hypermobility of the joints of the bones in the middle ear (malleus, incus and stapes) creates problems with effective sound conduction across the middle ear. This loss of energy in route to the inner ear leads to a mild to moderate conductive hearing loss. Because the inner ear is also responsible for balance, improper function leaves some affected with bouts of dizziness. Problems with speech can occur: articulation, swallowing, hoarseness/weak voice and fluency. [Amanda Jenner, Lynne Shields, PhD "Speech and Language Issues."]

The safe and effective way of treating mild to moderate hearing impairment which can occur in EDS is with hearing aid and necessary additional aids. There is a much more common inherited disorder of hearing known as otosclerosis which can be easily confused with the type of hearing impairment that is seen in EDS. It is vitally important for the ear surgeon to be able to differentiate between the two as the hearing loss in both is due to abnormality of the sound conduction across the middle ear. The standard treatment for otosclerosis is an operation called, a "stapedectomy." However, an attempt to undertake a stapedectomy on an EDS patient could easily end in disaster with total loss of hearing and a severe disruption of balance. [M. Hawthorne, FRCS-ENT Surgeon. "Hearing Impairment and EDS."]

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