Some people with Charcot Marie Tooth can experience hearing difficulties as part of their CMT, particularly in the presence of background noise. The type of hearing loss seen is called auditory neuropathy (also referred to as auditory neuropathy spectrum disorder).
Auditory neuropathy is uniquely different to the typical type of hearing loss that occurs with age, and hearing aids are not always as helpful as we might expect. This means we need to “think outside the box” and research different ways of testing and managing this type of loss to try to improve outcomes.
Dr Kirsty Gardner-Berry and Jordan Claude are audiologists currently conducting research in the area of hearing loss in adults and children due to auditory neuropathy at the National Acoustic Laboratories (NAL) in Sydney.
What will this study focus on?
The main aim of this research is to try to better understand the mechanisms underlying auditory neuropathy in individuals with this condition. The results from the study may help to guide future research into new testing techniques audiologists can use to assess patients in the clinic, and hearing aid program settings that are more likely to help.
Who is eligible for the study?
If you have CMT and are experiencing difficulty hearing what people are saying in both quiet and noisy places you may be eligible. Some people describe this as being able to hear people speaking, but it not being ‘clear’ what they are saying, and they need to face people in order to understand them.
It would be helpful if you have already had the auditory brainstem response (ABR) test to confirm you have auditory neuropathy, but we can consider arranging this additional test for you if needed.
- The study involves two, 2 hour sessions at the National Acoustic Laboratories, which is based at the Australian Hearing Hub at Macquarie University in Sydney. Individuals who participate are given $20 at each visit to assist with any travel expenses.
- There are several tests involved which look at different parts of the hearing system which are listed below. These tests are spread over 2 test sessions.
- We will perform a standard hearing test. This involves presenting sound through earphones and asking you to respond to indicate when a sound is heard.
- We will check to make sure your eardrums are moving normally. This involves putting a small plug into the ear which produces a small puff of air into the ear canal.
- We will test your otoacoustic emissions (OAEs). This involves putting a plug into the ear which makes soft clicking and beeping noises and records automatic responses from the inner ear (cochlea) to sound.
- We will be measuring automatic responses from the brain to sounds presented through small earphones at different levels. A cleansing paste will be used to clean a small area on the surface of the skin in four areas (forehead, top of the head, and behind each ear). Small recording sensors will be placed on the same four areas and these sensors will be connected to a computer via thin wires.
- We will test your speech discrimination ability. This involves playing sentences to you and asking you to repeat them back.
- We will test your ability to detect small changes in the loudness of sound. This involves presenting a set of three sounds through earphones and asking you to indicate which one was changing in loudness by tapping the computer screen.
- We will ask you to complete a questionnaire about how well you think you hear in different listening environments.
What is in it for participants?
There is no direct benefit to participants at the time of the study. It is hoped that through gaining a greater understanding of the underlying mechanisms of this type of hearing loss we will be in a better position to understand what type of assistive hearing devices are more likely to help.
Who do I contact if I would like more information or would like to take part in the study?
If you would like more information you can contact either Jordan Claude or Dr Kirsty Gardner-Berry via the contact email addresses below.
Jordan Claude: Jordan.firstname.lastname@example.org
Masters of Audiology candidate
Dr Kirsty Gardner-Berry: Kirsty.email@example.com
BSc. M.Aud. MASA (ccp) PhD – Research Audiologist
Ph. (02) 9412-6885
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