From Ski-Slope hearing loss to more uniform hearing loss? Cochlear hydrops?

Hi! I was born deaf – no idea what’s exactly behind it (nerve intact, no evident malformations, unknown genetic). For many reasons I got diagnosed with neurosensorial hearing loss only at 7yo and since then I use HAs. My “original” hearing loss resembles a sharp ski-slope hearing loss focused on high frequencies, with a massive drop at 1500 Hz. That has been my “hearing situation” for years (now I’m 24). Back in 2021, I realized I couldn’t follow video lessons as I used to and I’ve lost some sounds (microwave, traffic, and so on).
Some audiograms showed a consistent loss at “low-central frequencies”. Like, prior at 250Hz I had 35dB loss, now it’s 55/60dB loss over 2 years and a similar loss at 500 and 1000 Hz; high frequencies remain between 110 dB loss and no response at 4000Hz and 8000Hz. My ENT audiologist doctor suggested it all depends on cochlear hydrops. They were really confident I’d recover from my recent loss (prescribed me betahistine) and that this could be episodic - the same way Meniere’s disease does. In reality, 1) in my everyday life I don’t experience such a varibaility – to me, it seems like it’s only getting slowly worse 2) my last audiogram showed some uniform worsening and it’s almost impossible to follow speech from any minor distance and in noisy situations. My left ear has tons of tinnitus, while my right ear is more stable. So, my question is: what should I expect? Like, I’ve done the prescribed cure for months with no effect (the same doctor took me off that and suggested a follow-up visit in Autumn), I keep myself healthy and on a low salt diet and stuff. Can cochlear hydrops affect someone like me who already had some kind of hearing loss? It was all stable for years and now every 2-3 weeks I see more difficulties. Does anyone else experiencied something similar?

My understanding of Cochlear hydros is that’s it’s an atypical form of Ménière’s disease without the vertigo… It may or may not just disappear years after the initial onset. But rarely does the hearing return to normal… Now I could well be wrong.

IMO you have a tough loss for hearing aids to be of much assistance to you… Have you given any thought to getting Cochlear implants if this is possible with hydrops?

Thanks for the feedback! In the past (2006-2008) my doctor did not suggested cochlear implant in order to “save” the remaining natural hearing I had. In December 2021 they said I should look for new unpgraded hearing aids (mine have 6 years now, still I’m doubtful to change them). In my opinion, all this was influenced by the fact that 1) they truly believed I’d fully recover form an episodic drop (it did not happen, I wonder if I got even worse) 2) despite my audiogram being worsen, I did decently well at the speech recognition test – mainly because I was really trying to guess words I didn’t hear and I was full anxiety mode. In real life, as many people said up here, it’s a total different game. Speeched give me utter uncertainity. Now, I’m scheduling a follow up with the hospital for the next months and we’ll see what they suggest. In general, HAs it’s the only solution I’ve know for decades. They whistles a lot and my hearing loss “stress” them, expecially those high pitched loss I guess. Just saying, I know there is a specific cochlear implant that combine the CIs technolgy and Hearing Aids technology (EAS CIs) – deemed for atipycal hearing losses like mine.

With regard to the EAS, yes Cochlear do have an attachment that fits onto the processor. This is only used if your residual is useful after implanting. But preserving your residual is never guaranteed, I lost a chunk 15 months post CI… Then another smaller chunk rendering the EAS attachment useless.

The “hybrid” array hasn’t been used in Australia for a decade or more. This is a short array, and when the residual fails as it will eventually do, they have to remove it and insert a “full length array” (electrode). I was lucky my surgeon used a full length 522 array, so didn’t require further surgery.

Good luck to you on your hearing journey…