Unusual Hearing Loss

Hello all,

My name is Nathan and I have an unusual circumstance. I have a normal hearing left ear and a right ear with mild-moderate hearing loss. I do not have as much of a problem hearing volume as much as I do recognizing speech. What is unusual is that no physician can tell me why I cannot hear. The last specialist I saw said something along the lines of having a problem with my cochlea sending messages to my brain. However they could not give me a definite reason. CT scans showed no tumors or anything as well. I was around 7-8 years old when all of this took place and I am now 24 years old with the same problem and no answers. I have a hearing aid and am going to demo an upgraded Oticon Intega 10 for a month. Has anyone else had a similar experience as mine? I have been frustrated over this for my entire life.

Do you have a fully masked audiogram for both ears?

Hello: I have had the same problem for over 20 years. I’ve spent over $60,000 on hearing aids, buying the newest technology every one to two years. I was never able to identify one time that I was hearing speech better. Finally, after much research, I found a group of parents discussing their babies diagnosis of Auditory Neuropathy Spectrum Disorder (ANSD), that was found from their newborn hearing tests. The most striking feature of this is that the tonal hearing can range from normal to severe, but word recognition or the ability to understand speech is severely affected. I immediately recognized the symptoms and proceeded to ask numerous audiologists if I could possible have ANSD. All of their responses were “no, you had to be born with it.” Undeterred, I went to a clinic at the University of North Carolina and sure enough, I was diagnosed with ANSD. My word recognition scores were at a terrible 10% in quiet and 0% in noise. I was shocked that I was relying totally on lip reading. ANSD is confirmed with an Auditory Brainstem Test and also testing of your acoustic reflexes. I had no response in either test. Despite the fact that I had moderate to mild tonal hearing loss, I was declared a candidate for a cochlear implant. Hearing aids do not help in ANSD and can make matters worse. No wonder I always yanked them out. Two weeks ago I underwent surgery to get a cochlear implant. An added bonus is that my surgeon is recognized in the field of retaining residual hearing during cochlear implant surgery. I am hearing out of my implanted ear so he was successful. I am scheduled for activation of my implant next wednesday! Please check out ANSD through the closest University affiliated Otolynragy Clinic and asked to be tested for ANSD. Sometimes audiologists do an acoustic reflex test as part of your audi workup. If you have a copy of your audiogram check and see if they are there. Also, do not go to an audiologist for this, they aren’t up to speed on this diagnosis. It’s a recent discovery that came as a result of the mandatory newborn screening testing. Good Luck!!

WOW I have to check that out because that sounds exactly like what is happening to me. During the audiogram tests I could hear words being said in my right ear but could not understand the words at all hardly. The audiologist tried to tell me that as a result of my audiogram I do not hear certain frequencies that determine the understanding of certain consonant sounds. However, the look on every audiologist face says it all. That they do not know for sure why I can not understand speech but a hearing aid should help somewhat. I will definitely check into this ANSD, however, I am a 24 year old professional football player (AFL) and do not want to give that up right now for a cochlear implant. Thank you so much for this information you have told me something that no Dr could for almost 20 years of my life. BTW I have not obtained a copy of my audiogram yet.

I know of a 20 something women in Australia that has ANSD that started when she was 8 years old. She also recently was implanted and is doing great. Mine didn’t kick in until age 30. At that time I noticed having a problem understanding speech in noise. My hearing tested normal in tones and speech in quiet. When they added background sound it dropped to 15% speech recognition. So, at first it was mild but each year got worse and worse until I couldn’t understand speech even in quiet. My brain sort of fooled me by converting other cues like lip reading, knowledge of topic, captioning,and familiar voices into speech sounds. Amazing really and quite confusing. Like you, I was told that I wasn’t hearing parts of words, in my case vowels and that’s why I couldn’t understand speech. But that made little sense to me. Often I would cycle through my hearing aid programs while listening to the radio in the car and 100% of the time, my favorite program was mute!! I’m wondering if it showed that perhaps filtering sounds would have helped me. My high frequencies are actually above normal for my age. ANSD is very easy to test for and they have also developed specific protocol for programming hearing aids if you have ANSD and can still be helped by hearing aids. It has to do with timing and most digital aids speed up the timing and that is BAD for ANSD. You might want to experiment with linear programming instead of the non-linear that current aids use. Ask your audie to switch the programming to linear and see if that helps. There is also something called attack and release that is bad for ANSD that can be adjusted on some hearing aids. There is a FB group of ANSD if your interested in joining. The moderator of it has a reverse slope hearing loss and is an electrical engineer and is a hearing aid dispenser and is really knowledgable about programming and ANSD. Search under groups and Auditory Neuropathy Spectrum Disorder. It’s a closed group so you’ll have to ask to join.

how is your CI working for ANSD ? did the implant increase your speech rec scores? i have ANSD and trying to decide if i want to do a CI… any input or advice would be much appreciated. thanks!