According to a survey on the hearing loss in osteogenesis imperfecta, more than 50 percent of people who have osteogenesis imperfecta have hearing loss. Osteogenesis imperfecta(OI), most common heritable disorder of the bone associated with increased joint bone mobility, minor injury, and deafness.
Yes, osteogenesis imperfecta lead to hearing impairment but not every person who has OI develops loss of hearing. The incidence of OI is much higher than in the general population.
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In OI sometimes the cause is unknown. Sometimes is due to visible deformities in inner ear and ossicles may lead to deafness. It is important to how osteogenesis imperfecta lead to hearing loss.
Why hearing loss causes due to Osteogenesis Imperfecta?
The reason why the hearing loss causes due to osteogenesis imperfecta is not definitely known. Patients suffering from OI have a greater chance of skull fracture which is associated with the hearing loss. Footplate fixation is one of the reasons for the conductive hearing loss in the OI.
How Osteogenesis imperfecta lead to hearing loss
Osteogenesis imperfecta is due to mutation including several genes. A phenotype or genotype connected to hearing loss has not been established in OI. There are various environmental factors which contribute to the hearing problem in people with OI. These factors include noise, infection and head injury.
The loss of hearing generally starts at any age but with people who have osteogenesis imperfecta, hearing loss in those people starts at an early stage and increases with time.
Hearing impairment in osteogenesis imperfecta can be a conductive hearing loss, sensorineural hearing loss, mixed hearing loss depending on the symptoms.
Consult an audiologist for your hearing problem. He will recommend a hearing test. Your hearing test will decide the treatment of the hearing loss. The treatment can be hearing aid or cochlear implants depending on the type of hearing loss.
Symptoms
- Face difficulty in comprehending the words.
- Asked to repeat words.
- Difficulty in comprehending the conversations.
- Turn up volumes of media to a certain level that is too loud for others.
Diagnosis of OI
Infants and baby are diagnosed for hearing loss when they are born. An audiologist, physician of ENT perform the diagnostic test of hearing loss. Hearing diagnosing consist of different activities. Treatment is going to happen on the basis of the reports. A special test Evoked Response Audiometry is for the person who is not cooperating in the test process.
Management Of Hearing Loss in OI:
In osteogenesis imperfecta, the treatment of hearing loss can be divided into two categories based on the severity of the hearing loss and etiology. As we know that hearing loss can be sensorineural and conductive or a combination of both.
The conductive hearing loss in the person can be fully corrected theoretically. In the person, without OI the chance of getting well is not always possible, unfortunately. And the person suffering from OI have a higher risk of not getting well.
The people with or without is treated in the same manner for hearing loss. Hearing aids can help to reduce sensorineural, conductive, and mixed hearing loss.
The amount of loss is more important than the cause of loss because the amplified sound is going directly into the ear. Fitting a person with OI with a hearing aid is the same as fitting someone without OI.
Treatment for Osteogenesis Imperfecta
Till date, there is no known treatment for osteogenesis imperfecta, medicine, or surgery that will free you from osteogenesis imperfecta (OI). The aim of treatment is to prevent deformities and fractures and allow the child to function as independently as possible.
Treatments for preventing or correcting symptoms may include:
- Care of fractures
- Surgery
- Rodding
- Dental procedures
- Physical therapy
- Assistive devices, such as wheelchairs, braces, and other custom-made equipment
Recommendations
It is recommended to the children suffering from OI have a formal evaluation of hearing before they start going to school.
Testing should start at the age of 3-4 and be repeated every 3 years. A child who is suffering from OI demonstrates articulation problems, speech delays, ear infections, or parents suspect a hearing loss should have a formal audiology assessment regardless of age.
Adults with borderline hearing should go to test every year and follow up the appointments similar to the schedule for children. Adults who are having symptoms of hearing loss should also have an audiology assessment which includes a hearing test of air and bone conduction and speech reception threshold.
This kind of hearing tests help to determine whether the hearing loss is conductive or sensorineural.