Just wondering if anyone can offer me their experience of aminoglycoside related hearing loss? I’m most specifically interested in loss attributed to tobramycin use if there’s anyone here.
Small person (now aged 11) has had over 40 courses of IVs using tobi since birth. She had a hearing aid for pretty bad glue ear between the ages of 18 months and 4, and although it’s never reoccurred, they subsequently identified her as having congenital, sensorineural loss over the low frequencies in both ears and so have kept her on their books for monitoring. She’s not currently aided as it’s not actually a big issue for her functionally, and in some instances is actually helpful with being autistic and having sensory difficulties (not being able to easily hear thunder for example), but I was given a print out at her last appointment that showed 4 audiograms from 2016 onwards so that I had a copy to hand to easily provide to the new services when we move in the summer.
Me being me, I had a look at them and saw that to a layman, her hearing across the high frequencies appeared to be dropping too when the audiologist has been routinely telling me her hearing is completely stable. We’ve been back today for a follow-up and I raised it for clarification: when the consultant has gone back through the digital audiograms, there is indeed a decrease on the right of 35 dB, which has occurred over the last 18 months over the 6 - 8000 hz range. She’s still a reasonable pass over the speech register, but her very high is now a clear fail with 6000 heading the same way. Mitochondrial testing for aminoglycoside sensitivity isn’t routinely carried out for cf patients in Wales, but the audiologist suggested that this progressive loss could be a result of the tobi use. My only issue with that is that everything I previously read when her hearing low frequency loss was diagnosed suggested that ototoxicity results in a quick loss rather than a gradual decline over time. At the last appointment she suggested that E would benefit from an MRI because she suspects the congenital loss may be related to the nerve and the specific condition can only be diagnosed by scan, but she also said it wasn’t clinically relevant, it would just be to see if we can identify the root cause: in the space of two appointments we’ve gone from ‘her hearing loss is completely stable, but an MRI might be a good idea in the future for the low frequency loss’ to ‘I think we should probably screen for sensitivity, and it’s a once in a career find and I’m over-cautious by nature, but she should have an MRI to rule out a really rare progressive nerve issue the next time you have her under anaesthetic’.
Anyone got any thoughts? If you’ve lost your hearing as a result of antibiotic use, was it slow or sudden? We discussed referring for testing via the national genetics service today, but by the time it goes through we’ll likely be in England, so I’m inclined to hold off until we get to the Brompton and get them on the case. But that said, she’s got one more planned course of IVs before then, and, only 10 days out of hospital where she isolated a multi-resistant pseudo (just for the lols), has gone wonderfully wet and chesty this morning; if I held my breath with regards to her managing to stick to her elective IVs I’d be long dead 🤦♂️
If she does have sensitivity, what are the implications for IV use going forward? I realise that continued use will result in worsening hearing loss, but I don’t see there being an effective alternative with her microbiology and disease profile. Any knowledge would be really gratefully received.
I don’t know, if it’s not one thing it’s a bloody nother!! 🙄😂
Sorry I have no helpful personal experience as I am intolerant in the main to the family of mycins.
Bless E’s heart, she has had to have so much treatment and medication. She is very lucky in one sense, that she has an amazing, proactive parent like you in her corner.
Your questions to the medics have contradictory replies - no wonder you must feel at sixes and sevens juggling everything.
I am pretty sure the treatments and care E will get at the RBH CF unit will be superior to that experienced so far. I expect they will start from scratch but do you think they will also want E to go through the national genetics service, if so I am wondering if it might be good to ‘get the ball rolling’.
It might be very helpful for you to talk things over with the BLF nurses. They are very knowledgeable about paediatric lungs as well as adult lungs. The number is on the home section but I am sure some kind person will pop the number here for you. I can really see your dilema with regard to a further round of IVs, especially as she is wet and chesty today.
Thank you, lovely lady. She’s just ridiculously complex. Her local consultant is amazing at taking darling sprog into consideration in her entirety as opposed to just focusing on the respiratory side, and he reckons that even if we never identify it, there’s probably an underlying genetic component to her health and odd presentations: we started with ‘just’ the cf confirmed at 5 days old and now have a running tally of 14 confirmed conditions and one significant ongoing query for mastocytosis/mast cell issues. Whilst some of them can be grouped together as established comorbidities, I find it really hard to believe that one child could be THAT unlucky without an underlying link. Particularly when family wise, the ex has a niece and nephew with autism and that’s it, my maternal grandmother had a pacemaker in her 60s as a result of heart disease, and years ago she reported a couple of childhood deaths from ‘lung problems’ in the family in the 1920s that I now realise could well have been cf, but otherwise not a single chronic or long-term medical problem in living memory. Not even hayfever, for goodness sake, and then along came Mush and here we now are on first name terms with half a dozen different specialities.
Have kids, they said. It’ll be fun, they said. 👌😂
The ‘national genetics service’ is only national within Wales - anything even possibly genetic requires a referral to the service for counselling before any tests. From prior experience, in addition to them not always knowing their stuff to begin with, the setup also means the wait to be seen can be pretty long and why I’m on the fence as to whether to request screening here or just let the RBH start from scratch, as you say. I hope they do start from scratch across the respiratory board to some extent.
The only way I can see it making any difference now is if it was positive (and I could well be wrong, but I have a strong gut feeling ototoxicity is not the issue here) and would change her inpatient treatment, but whilst RBH limit use to alternate admissions if they can, I can’t see how they could possibly avoid aminoglycoside use completely: at the risk of sounding awful, I somewhat facetiously explained it to my resident teenage sister as being a case of ‘well, going deaf doesn’t particularly matter if you’re already dead from lung disease’. We know she’s definitely got one, potentially two, chronic pseudo infections, and whilst at her best she’s still got remarkably good fev1, she needs a significant amount of intervention 🤷♂️
I might see if I can get hold of her consultant here tomorrow and speak to him about it - he doesn’t work Mondays, unfortunately. Or at least talk it through with the community nursing ladies, if only to say it out loud: sometimes I just need a verbal sounding board to clarify my thoughts. I am annoyed that I was the one to spot it on the audiograms, though - they were *only* given to me because we’re moving. A consistent 5 or even 10dB drop is one thing, but a 35dB drop is a pretty substantial decline for a professional to miss over multiple appointments, regardless of whether they’d actually issue a hearing aid as a result!
Bless your little one - she definintely is very complex. It is great her consultant treats her holistically and not just from the respiratory point of view. I wish more would do that. It is very good practice.
I didn’t realise the ‘national genetics service’ is only in Wales. I would have thought RBH would have access to a genetics service, if not that one. It’s very perplexing isn’t it! It would be interesting, though obviously sad, to be able to know much more about the 2 children who had died in childhood from ‘lung disease’. The problem is of course it was the 1920s and could well indeed have been bronchiectasis, be that cf or non cf. The aeteology would be unknown as it could have been a virus or bacterial infection, which given the date would have been untreated, which then caused severe lung damage. It’s speculative but obviously could well be of interest to them working out any genetic component.
No wonder you are very unhappy and concerned with regard to ittle E’s hearing loss not being routinely picked up by the professionals and in the end was down to you because of the move. This shoud definitely not happen, especially when it’s their ‘speciality’ and dealing with a child whose other medications interact so very much. I can see how this happens - though inexcusable. I have IBS and over a couple of years lost about a stone and a half. As I have to attend appointments frequently (respiratory and gI) they look at the weight from the previous appointment and there might have been a few pounds lost. Nobody pick up, until I pointed it out to them that if they looked at the bigger picture over a longer period of time they would see I had and continue to lose weight unintentionally and can’t afford to keep doing so. It’s a good job, by the sound of it you are moving, as your eagle eye spotted, what should have been the obvious, straight away.
Limiting IVs seems to be the present day thinking in the respiratory world. Our bronchiectasis service and I ‘think’ it applies to the CF service too no longer give routine say twice yearly IVs as a given. I would have thought possible resistance is the way the resp community are thinking.
I am wondering if you managed to speak to E’s consultant or nursing staff. I know what you mean - sometimes whilst talking through a problem with someone it seems to help to work stuff out, often for yourself - very helpful.
Her local doc is an actual saint, I’m not kidding; hugely knowledgeable, incredibly compassionate and conscientious, extremely thoughtful both clinically and as a human being, and incredibly calm in a crisis. He’s also just the nicest man to boot and E adores him. We’re both really going to miss him when we go, but there is a standing job offer for him as E’s personal physician complete with relocation for him and his family subject to winning the lottery 😂
From what I understand, the situation is very different in England with genetics. Here we have the All Wales Genetics Service run out of Cardiff, and if you want any genetic testing done patients are referred to the team where a consultant acts as a ‘gatekeeper’. The vast majority of the time, even a request with a clear clinical basis results in the patient/parent attending a genetic counselling appointment to discuss the request, even if another member of the family has a confirmed diagnosis of a genetic illness. And that includes the likes of cardiac ones where an undiagnosed child could collapse without warning and a sibling has already been diagnosed. There’s a lot of insanity in NHS Wales, but that actually blows my mind. In contrast, I’ve been led to believe that genetic testing in England is undertaken on request as long as a consultant gives a clinical justification for doing it, or it certainly was like that in the not too distant past according to someone who would know.
I managed to speak to the local consultant last night. The long and the short is that regardless of whether we try and get it done here or wait for the RBH, if she has a high frequency loss then we have to assume that it could be tobi ototoxicity until we have evidence otherwise, particularly as we don’t actually know over what period it occurred beyond saying in the last 2 years. He did agree though that it doesn’t necessarily fit the normal clinical presentation for loss due to ototoxicity. He’s going to speak to tertiary about what the options are, but he’s saying that they will have to modify her treatment in the interim and suggested that half-dosing could be a possibility just as they would in someone with renal complications. All a bit of a nightmare though, tbh, in part because of the timing! If this had been noticed earlier we could have referred and know what’s what instead of being in limbo knowing full well that she’s cultured pseudo 3 times in the last 6 months and one of them was multi-resistant. I was expecting a call with sputum culture results today that hasn’t materialised, too; for once I’m actually hoping the lab is running behind, because a clear culture is normally back within 48 hours Monday to Friday and it went in on Monday morning...👀
I understand the principle behind limiting IVs and clearly have very real concerns about the rising levels of antibiotic resistance because of how much people with chronic respiratory diseases rely on them, but there are some who really do benefit from regular, planned use. E did 24 weeks on unplanned IVs before she reached her first birthday and every course was necessary. We consequently put her onto routines at her first annual review (four times a year) and she did 6 courses in her second year but then stabilised massively: between the ages of 2 and 6 she only did her 4 courses annually, her pseudo was kept very well under control and she had fairly limited courses of orals inbetween bar cover for colds and coughs, perhaps 3 or 4 a year. At her sixth review we were told that elective IVs were no longer best practice and they were to be immediately stopped and, contrary to my expectations, she actually did surprisingly well for about 12 months although with significantly higher oral antibiotic need.
After that? All hell broke loose. She cultured pseudo for the first time in 2.5 years, nose-dived spectacularly on orals and ended up in for 3 weeks. Cultured pseudo again just 10 days post discharge, but even ignoring the culture she still was far from right on getting out and she just didn’t improve. A month later she had her first experience of mild haemoptysis, a month after that and her fev1 was down to just 46% and that Christmas we literally got out after another three week stint at 4pm on the Christmas Eve: from the September to the following August, she spent almost 5 months in hospital *properly* unwell, including a full month with a pseudomonal pneumonia that wouldn’t respond to mero, ate her body weight in steroids and orals, and I was told by tertiary I had to ‘accept’ that this was her new clinical normal and that her best baseline fev1 would be around 60%. Two years on from that, having spent 15 months directly under tertiary (because they didn’t accept she could actually be so much of a challenge and thought we must be over-reacting locally so requested we have direct care) and having persuaded them to reinstate elective IVs (albeit three times a year rather than the four), her best fev1 in the last year was 82% and we’ve more than halved her oral use again. We’ve got annual review on the 19th and I’ll be able to report 6 weeks of IVs and 12 weeks of orals (most for confirmed cultures) in the last 52 versus 12 weeks of IVs and almost 26 weeks of orals in 2017. I think what I’m trying to say is the idea of blanket limiting IV use makes me very wary and just a touch annoyed because if identical twins with cf can have two very different disease progressions, then how can they justify applying the same restrictions to everyone? There will always be exceptions to a rule and someone shouldn’t have to get really ill before they explore doing something differently!! Aaaaaand end rant, partly because I know I’m likely preaching to the converted 😂
Will keep you posted as things develop, but I’m still not sure what to do for the best with regards to requesting testing here. x
I don’t know about the drug your talking about but I do know azithromycin although effective in treating my bronchiectasis, long term usage of it (a year st 3 times a week) caused me to have hearing loss, I now wear hearing aids x
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Loss of a dear friend Carole 
Can you help us better understand the experiences of those with a lung condition?
A sobering experience- and an update to an earlier post
We are all so poorer for the loss of our dear friend Skischool
Made it home ok thanks all. Another day another saga. 
