Congenital Stapes Fixation

What Is Congenital Stapes Fixation?

During the natural process of hearing sound waves travel through the air, down the ear canal funnel, and vibrate the eardrum. This sets the ossicles into motion. The ossicles are the three smallest bones in the human body. They are called the malleus, the incus, and the stapes. Their movements transmit sound into the inner ear for processing. Congenital stapes fixation results when the stapes is unable to move properly, or is fixed.

What Are the Symptoms of Congenital Stapes Fixation?

Conductive hearing loss results when the stapes is fixed. Conductive hearing loss is the type of hearing loss that occurs when sound waves are not smoothly transmitted into the inner ear. It is contrasted with sensorineural hearing loss which occurs when the processing of sound is impaired by loss of cochlea, auditory nerve, or central auditory function.

Some cases of congenital stapes fixation cause mixed hearing loss. This is a combination of both conductive and sensorineural hearing loss.

How Did I Get Congenital Stapes Fixation in the First Place?

Congenital stapes fixation occurs before you are born. During development, the stapes bone forms adjacent to the cochlea. If the window where the stapes sits in the cochlea fails to form, congenital stapes fixation occurs. A gene resulting in this malformation is located on the X chromosome. This means that the condition is carried by female that are unaffected. The male offspring of female carriers are at risk for congenital stapes fixation. Having congenital stapes fixation is a risk factor for poor development of other parts of the ear as well.

How Will My Doctor Test for Congenital Stapes Fixation?

A discussion of your symptoms combined with examination of your will lead your doctor to suspect congenital stapes fixation. A discussion of hearing loss in your family is also a helpful clue. A hearing test will be require and will show either a conductive or mixed hearing loss. A CAT scan of your ear is required to look for poor development in other parts of your ear.

Is Congenital Stapes Fixation Treatable?

The hearing loss of congenital stapes fixation is typically serviceable with a hearing aid. A conventional hearing aid is typically satisfactory. Bone conducting hearing aids are also options. Unlike stapes fixation due to other conditions, congenital stapes fixation is not treatable by stapes surgery. This surgery carries a high risk of hearing loss. Unfortunately, some cases mimic other conditions and are not diagnosed as congenital stapes fixation until surgery is performed and hearing loss occurs.