Category Archives: Auditory Neuropathy

Going Up North….

July 24, 2012 By in ANSD, Auditory Neuropathy, auditory neuropathy spectrum disorder, Cochlear Implant 1 Comment

When Ollie was diagnosed, after the initial shock, we emailed everyone that we thought we’d need in the journey, therapists, surgeons and audiologists were immediately put on notice, that their help would be demanded.  We then calmed down and took time to look at our gorgeous little boy and smile a bit.  But, in the back of our mind has always been the need to somehow give something back.  For every family like us, who are sure where they are going, is a family like us 4 years ago, that is frightened and confused and needs a friend.  We are determined to give something back and help anyone that doesn’t know what to do.  Chris and I answer every email, every comment and will always do, so as everyone needs a hand in the crazy world of ANSD.

Each child with ANSD is different and beautiful, but more importantly our children have a multitude of causes and outcomes.  We’ve found ourselves in the genetic, no possibility of getting better, CI early camp, but friends have found themselves in other parts of the umbrella (maturation, jaundice, no nerves…..).  We’re all part of the same spectrum but clearly our journeys and outcomes will vary.  Whilst this is all very interesting, for parents, particularly at first diagnosis, this means that the clinician’s plan can seem vague or parents are asked to wait and see for ages.  There are good reasons for this, but with limited information it is incredibly frustrating.

Help is at hand though!  Clever people globally are all working on improving the understanding of ANSD and more specifically wonderful, clever and practical people (I met two of these amazing women today) are working on gaining more useful information to aid parents and clinicians in choosing a pathway for their particular ANSD.

What does all of this mean?  Today Ollie had CAEPs done.  These are shown to give more information to clinicians than an ABR can with ANSD.  From our perspective it was no more intrusive than an ABR, although the smallest Campbell decided that he didn’t particularly want to play,  but a smile from such a cute young man instantly melts the heart.

We are waiting for the team to analyse the results but when Alice had her CAEPs done, it gave us confidence that a CI might work.  For Ollie this wasn’t our reason for the test, but frankly I’d go a lot further than Manchester (particularly as we get a lovely guest suite and gourmet dining at Chez Delaney) if it meant that in time, someone will benefit from this research.

We have made some new friends in Francesca and Kai and we hope to be involved in further studies.  As a call to arms to our many readers, if they ask, would you?

Oliver…

May 23, 2012 By in an/ad, ANSD, Auditory Neuropathy, auditory neuropathy spectrum disorder, Cochlear Implant 2 Comments

So today is the hottest day of the year. 28 Degrees in South West London I am told. This is all jolly nice and, don’t get me wrong, makes something of a change from the incessant wind and rain of the past two months. On such days it is customary to take lunch on the grass, take a stroll by the river, swim in the pool and generally to celebrate the coming of summer.

But that would all be too easy.

Today was Oliver’s first appointment under Audiology / CI programme at St. George’s. A familiar trip, the irony being that Alice and Oliver’s poor Mummy have to be back there at 4pm with Alice as her new ear is playing up….again.

For those of you not familiar with the process of ABR’s, it essentially consists of playing a variety of different frequency sounds directly into the ear, at different volumes, and then measuring the activity in the brain that is stimulated by these sounds. This is Oliver’s 3rd set of ABRs, and the poor mite is only 1 month old. At least at this age they can sleep through the procedure rather than have to be sedated (well, only if they’re deaf as even I, who can sleep through a thunderstorm, would be woken up by 120db straight into my ear). So this is how the day goes:

  1. Take one, small, one-month-old child.
  2. Make sure he is asleep
  3. Cuddle him to make sure he stays asleep
  4. Place in an air-tight, soundproofed room with no air conditioning on the hottest day of the year.
  5. Stick electrodes to his head and stick wires in his ears.
  6. Cuddle him for two hours while an array of tests are done.
  7. Sweat profusely.
  8. Tell us the results are “what we expected”.

I am, of course, being flippant. Dhaval the audiologist is so very good with parents and children alike. This is all part of the process and will need to be repeated in six weeks time to make sure there has been no spontaneous improvement (children with the Otoferlin mutation do not mature out of this). In the mean time, Oliver had his moulds taken and will collect his first set of hearing aids in 10 days time. They won’t work of course, but needs must.

Poor little lamb.