Christmas and the MRI scan

Christmas - for that is what it is (not 'the holiday', not 'the season', not even 'yuletide') - was a happy event as ever. Watching Alex and his cousins rushing to the front of All Saints Church in Wokingham during the Christmas Eve family carol service reminded me just how important to a non-believer like me is the Christian festival of Christmas. The unaffected joy of children at hearing the familiar Christmas story being retold has a strong emotional power. John Betjeman's poem 'Christmas' (reproduced here and, of course, in Betjemen's Collected Poems) captures some of the strange feeling of wonder we non-Christians have at the significance and poetic beauty of the event being marked; that "No love that in a family dwells, No carolling in frosty air, Nor all the steeple-shaking bells Can with this single Truth compare -That God was man in Palestine And lives today in Bread and Wine".

We opened the 'tissued fripperies' as usual on Christmas Day morning and enjoyed ourselves hugely - albeit my being deaf proved a handicap to fully engaging in a family Christmas. Relatives took the time to speak slowly, asked about what was happening to me...and I have lost count of the number of thoughtful cards wishing me 'a better 2008' I have received. Whether or not Christmas is a Christian celebration hijacked by we near pagans for our own pleasure or not, it is undeniable that every year people seem, at this time of the year, to grow a little kinder, a little fonder and a little more sentimental.

I eventually got my MRI scan today, 29th December, at the Royal Free Hospital in Hampstead (US readers should note that all NHS hospitals are 'free', but the names of the nineteenth century foundations remain, so that the Royal Free Hospital founded in 1828 is a remaining monument to Victorian philanthropy made even better by state funding for free healthcare) . I sneaked a look at the scans, but made nothing of them, except that there seemed to be something very odd happening on one side of the head...more on that when I hear the diagnostic from the evidence.

Happy New Year for 2008.

KT and neuroprosthetics

Unlikely as it seems my daily role in the University (directing across the institution all our work in knowledge transfer, through all aspects of commercialisation to spin-out and by every other conceivable route) seems to be benefitting from a closer acquaintance with the world of neuroprosthetics. Not only is my University's own Biomodelling Group doing fantastic work on deep brain modelling, which now actually means something real to me, but in casting around for ideas on how such work can more readily reach a market I've been impressed with the work of other UK colleagues looking for innovative ways of quickly taking this kind of technology from the lab to healthcare. The Strathclyde Institute of Medical Devices, for instance, doesn't leave the business of commercialisation via technology partnering to people like me - generalist knowledge transfer people with a broad understanding of transfer protocols, but for whom the industry and context would be new. Their approach appears to be to ensure that throughout the unit, whether dealing with studentships, small business liason or licensing, scientists with a commercial and sector background lead the process.

There is a trend in knowledge transfer toward greater specialisation, and that is almost certainly a part of the story in the UK academic neuroprosthetic knowledge transfer arena. More than that, however, there is a need to cope with the inherent uncertainty in the technological trajectory. The reality is that the speed of development in these technologies now depends upon work in nanotechnology (possibly, one day, even bionano), microelectronics, brain imaging, brain chemistry, neuropathology, computer science and materials science, which progress without a thought to that particular technological trajectory. Specialist knowledge transfer people will know how far firms are able now to capitalise upon the coincident developments in each of these areas. For all of us awaiting the development of more effective neuroprosthetic devices, we need their skills to lead that transfer from the lab to the operating theatre and beyond.

How not to report on cochlear implants

Those of you who admire the ability of the press to miss the mark so comprehensively on occasion might be amused by the report in the (UK) Mirror newspaper about young Joshua Alexander at http://www.mirror.co.uk/news/topstories/2007/12/22/joshua-hears-after-bionic-op-89520-20262500/, in which I count five gloriously inaccurate statements about CIs. Have I missed any?

Friday came and went...

The assessment panel yesterday wasn't an assessment panel so much as a funny old meeting. In effect the fact that I get the MRI scan (I hear by letter today) on 29th December has kind of screwed up the timetable. My consultant - a thorough and professional man - suggested he'd rather not give a concrete and definitive picture until we have that - but suffice it to say the rest of the messages given were all consistent with the view that widespread cochlear hair cell death via SNHL has happened, and the only option now is the CI - confirmation in detail, as it were, of what we knew. Details were that the bloods were all normal - although some sign of an autoimmunity factor, but not specific. I asked if AIED was an option and heard, quite properly, this is so rare we'd have to see a much higher and specific count for that to be likely at all: frankly, he thought not. The CT scan and the other medical tests all suggested nothing other than cochlear cell death is happening down there, but clearly we want to see that MRI. Other tests (speech discrimination etc) that are intended as threshold tests - i.e. are you deaf enough, and in the 'right way' for a CI? (i.e. would you benefit?). They were all 'positive' (i.e. the scores were lousy).

We talked about the recent 'supercharged' hearing in which I'd been able to hear things I'd not here since BEFORE July (like the beep of the door entry card reader at the office, so faint most people rarely notice it; or the key clicks made when I text Jane on my mobile phone....). I am pretty sure it is not me imagining it - I've been hard on myself on that - and I am sure it did happen (6-7 days of 'super hearing', albeit with only a modest improvement in speech discrimination). We talked about it, he said such variability was 'unusual' (I suspect doctor code for 'You're fooling yourself, son') but wanted to look at it. He immediately did the (only) test for patulous eustachian tube in case it had been missed earlier (which had been running through my mind too) but seemed to conclude not much sign of that. However he did want to get, he said, a better baseline of at all possible since the variability had cut across tests.

So he proposed, between now and getting the MRI done having a high dose steroid course intravenously, watch what happens and see if the improvement looks greater than one might expect (in that you'd normally expect very little change). This is thorough and considerate. He even said that he hoped the steroid course would have an effect, if only a marginal one, a benefit for Christmas if nothing else. Jane regards all of this as him just humouring me and being considerate, but I think he's right about trying to see if the steroids get us any improvement since, if they do a markedly good job (better than last week's 'super hearing spell') we may need to check that something else isn't going on.

For now, though I'm through most of the hurdles for the CI and would prefer that to nothing (or regular intratympanic injections even if that were possible!) barring the MRI. It appears to be only the timing of that in the sequence that is now holding us up...that and the small matter of NHS funding for the process.I'll hold over the blogging for the rest of Christmas and the New Year. A very Merry Christmas to you all, and a Happy New Year for 2008.

Strange things are happening...

I am trying to remember that medicine is a rational discipline, and that noting small changes is for the birds as far as diagnosis is concerned, but...

I can't quite understand why or how but over the last few days (4 or 5) I have begun to hear sounds again, admittedly with the hearing aid (but I needed that before too), that I haven't heard in a long long time.

Sounds like: the faint beep of the entry door to my office building as it accepts my entry card; the sound of my keys jingling in the door; the sound of the Tube train doors slowly closing, and that awful 'beeping' noise they make before they do...the point is that all of those sounds (and a whole lot more) were simply off limits since July and now, unbelievably, they are back. Moreover while I can't work out words from other voices I CAN hear people speaking again - and have even 'overheard' a conversation taking place several seats away on a rattling, noisy Tube train.

My own voice now sounds to me almost normal (the only difference from pre-July days is that it sounds like I have a couple of paper cups over the ear). The sounds I still can't hear fully in my own voice are now just 's' and 'sh', but I can hear enough even of them. Other voices remain a problem and the tinnitus is pretty insistent.

Now, here is the problem. Do I take these things seriously? Is there a different baseline here? Is there (I hardly dare mention the thought) the possibility that something else is going on here other than - or alongside - SNHL?

The team at the RNTNE Hospital have been wonderful at aiming first to determine absolutely the cause of the loss. I'm still pretty sure they will conclude hair cell loss from a viral infection of unknown origin, but if there is the POSSIBILITY that the hearing loss could come from anything else I want us to dig for the answer. After all, a CI is a big deal - time, expense, irreversibility. I'm not getting my hopes up, but things HAVE changed.

Friday beckons

Friday is the day of the panel meeting at which we will hear the clinical - at least - view on whether a CI is the best way forward, and advice on how we make the decision to commit to that.

We are still a long way from implantation of course. There is an outstanding MRI scan to complete, a meeting with a clinical psychologist, and other members of the team to see. There is the likelihood that I'll have some time to think about the decision and then, as faras NHS funding is concerned, there are funding decisions to be made.

However I can't speak highly enough of the Cochlear Implant Programme team at RNTNE Hospital. Knowing how long this has been left unattended by unnamed ENT Department and friends, they have done their very best to complete a full investigation as quickly as possible.

During last Thursday's appointment at the RNTNE Hospital we managed to catch up a good deal of the time. I had, in something of a rush (brilliantly coordinated by the team),

- an ABR test (to ensure nerve function is good enough and to confirm cochlea hair cell death)

- and autoimmune inner ear disease blood test and three other blood tests to identify (rule out) other causes

- a CT scan (with an MRI scan to come)

- four speech discrimination tests

- four online psychological profiles and 'expectations assessments'

- a face-to-face psychological profile (first of several I think)

All of which, together with three previous audiology tests (pure tone, pressure and one other I can't recall), a written psychological profile, a team/panel meeting and an information session, has covered just about all that needs to be covered before we begin the move into the 'informed decision' phase.

What happens now is that after another test (0930 on 21st December) and a trial of an Oticon Sprint 3 hearing aid - to rule out its utility for me [I think already that much is clear, but for the process we have to try it] I will have a meeting with the team and a panel decision will be made.

Off to Wiltshire

Off to Wiltshire tomorrow to see my parents for my mother's birthday. (They live in the beautiful town of Bradford-on-Avon, where I grew up). Naturally she's concerned about her 'little boy' - all of 46 years old - and his impending CI journey. But we won't let it dominate our thoughts. A friend emailed recently saying 'don't focus on the distant future, stay with the task in hand' and she is right, as is everyone else saying the same thing. There are moments when this doesn't seem such a big deal. Equally there are moments when it does feel like a choice between different forms of defeat...

Small steps.

How I got here...

By way of record, here is the text of the summary I sent along to the Cochlear Implant Assessment team ahead of my meeting with them on December 5th 2007. Names are omitted.

1. Patient history
I have been deaf in my left ear since birth and, since the late 1990s, I have been losing hearing function in my right. Except for occasional ‘bad days’, my hearing through the right ear has been good enough – until recently - to allow me to function fully as a husband, father and employee, and to engage in normal social interaction. Since 1993 I have held a series of senior positions in higher education, and sit on the boards of a number of companies and charities, requiring social interactions that would not be possible without tolerably good, if impaired, hearing. In short, I have until very recently managed to live with moderate SNHL successfully and required no further intervention beyond a hearing aid.

I was diagnosed with a mild to moderate idiopathic sensorineural hearing loss in the right ear by a Consultant, ENT, at the Oxford Radcliffe Hospital in about 2001 after an onward referral to him, at my request, from an ENT consultant at another hospital. Treatment consisted of the fitting of a hearing aid. No use was made of courses of steroid, carbogen or other interventions at the time at either hospital although (from memory) one non-steroidal relaxant was used once.

The SNHL remained bearable and a hearing aid[1] was sufficient until July of this year, when a more significant reduction in hearing took place while on holiday in Spain (see below, 5). A week after my return home, and after no positive improvement in hearing performance, I sought the advice of a GP, who suggested that barotrauma was a probable cause and recommended leaving things for 4-6 weeks to see if there was any improvement, and to use nasal inhalations and medicines in the meantime. After 7 weeks from onset I returned to him, and he referred me to an ENT consultant at a local hospital. I saw what I understood to be the consultant’s assistant on 12th September, who took an ear pressure test and conducted a pure tone audiometry test; he concluded that the problem was possibly sensorineural, that “it could still be barotrauma”, but also that there may be other causes. I was given a new hearing aid, and asked to return to see the consultant in December. No additional treatment was offered beyond 60mg betahistamine tablets, no blood or other tests were performed[2].

Dissatisfied with this state of affairs, I referred myself to Professor Tony Wright at his private clinic (150 Harley Street) on 5th October, where he diagnosed a progression in my SNHL; the pure tone audiometry test suggested the loss was now profound. Professor Wright administered an intratympanic steroid injection, which helped to ease hearing a little for 48 hours. Professor Wright’s diagnosis is yet to be confirmed by any further testing (e.g. ABR) but was persuasive.
Throughout the episode since July, there has been no attempt to identify any viral or other cause, or to administer anti-virals or steroid courses to slow the progression or manage symptoms of any ongoing inner ear hair cell damage. There has been no other investigation than pure tone tests and a pressure test. No other conventional treatment has been offered at any time other than the betahistamine tablets.

2. Speed of onset
The onset is dated to a cable car ascent in mid-July 2007 (see 5 below), but symptoms of the most recent severe loss have emerged over the period July to October 2007, with an increasing rate of loss since the end of September/beginning of October.

3. Symptoms
There has been a progressive, staged, loss of hearing, with the following features:
(a) a normal ‘steady state’ in which hearing is impossible for all sounds without a hearing aid (see below);
(b) in the normal ‘steady state’ with a hearing aid used, it is possible to hear as if through a pillow or two (and in a rather ‘tinny’ form) percussive sounds and my own voice but little else. Other voices and many other sounds are damped, tinny and sound interrupted (like a radio offering poor reception with static) to the point of being indistinguishable as sounds and voices require lip-reading (which may or may not be successful) to make sense of the sound. Both for my own voice and that of others certain sounds within the speech banana are wholly lost (‘s’, ‘th’, ‘v’…). Tinnitus is a constant presence, but with variable intensity;
(c) there is an ‘agitated state’ in the ear in which rushing/vascular sounds are intermittent (usually with rising intensity, until the ‘steady state’ reappears). Hearing becomes slightly more acute, albeit through the rushing sound, during this ‘agitated state’. Tinnitus becomes more distant and usually takes the form of ringing. During periods of the ‘agitated state’, blood pressure readings are slightly higher;
(d) occasionally, in the ‘agitated state’ (and with increasing frequency of late), there is a sound very like the sticker at the end of a child’s toy arrow being pulled from a door, or like the sound of two small sticks being hit together lightly. This may be the expelling of gas or liquid (nitrogen bubbles under pressure? perilymph?), vascular pressure in the inner ear or middle ear or, more likely, associated with observed muscle ‘jerks’ in the roof of the mouth recognised as (mild and occasional) palatal myoclonus.
Both (c) and (d) are more frequent now than was the case in August. In summary:

Deafness
Without hearing aid, pure tone audiometry shows loss across all frequencies, most with hearing at no less than 90+Db. With Danalogic 6 hearing aid, there has been no calibrated test to date but, effectively, there is only ‘hearing’ of low frequencies, no higher frequencies, no speech discrimination (although lip reading helps to some extent). Raising the ‘gain’ in the hearing aid only makes vibrations more acute (leading to a sensation of dizziness that is instantly corrected by turning down the hearing aid) and does not improve sound discrimination. With the fitting of a Senso hearing aid, a recent audiogram shows some movement across the hearing range.

Tinnitus
This is now much more severe, continuous and (in tone) variable than was formerly the case, with a normal or background version and an ‘agitated’ version. ‘Musical hallucinations’ are also present.

Palatal myoclonus
Observed, and with ear ‘clicks’ consistent with this condition. Strangely, this is not always present. This is a new phenomenon, beginning in October.

Hypertension (mild)
Consistent home testing shows readings for blood pressure consistent with mild hypertension, in both ‘agitated’ and ‘non-agitated’ states of tinnitus and hearing, but with slightly higher blood pressure readings during ‘agitated’ states.

TMJ
Since 2004, when a TMJ distortion was first noticed and (in 2005) I consulted a dentist with this as a specialist area with a view to modifying alignment. Since 2005, the TMJ has continued to be problematic, though not as painful, but may affect structures around and below the right ear (this, on dental ex-rays, showed the most dental distortion).

Other things that may/may not be useful to know

Frequent gastric reflux (long diagnosed: not a problem, but more frequent of late)
Only other illnesses/conditions: mild asthma, very occasional allergies

4. Duration of the SNHL
The latest SNHL hearing depletion episode has lasted since 17th July 2007.

5. Associated symptoms such as vertigo, tinnitus, aural fullness, headaches, vision changes.
There has been some fullness in the ear, more marked (but variable) tinnitus, and some not inconsiderable auditory hallucination (whole concerts!) at times. There has been no vertigo, significant spells of dizziness, vision changes, or headaches.

6. Potential precipitating events
The moderate SNHL loss remained variable (with some good spells, some less good), with some slight reduction overall, from 2001 until July of this year. At that stage, a trip in a cable car whilst on holiday in northern Spain to a mountain top (some 2500 ft (800m) climbed in 3 minutes) seemed to cause a severe hearing loss that did not resolve itself for the rest of the holiday (a further 10 days). A descent on foot to a lower elevation that afternoon had no effect and a return from the mountainous region (in which we spent our first week of holiday) to sea level three days later similarly had no effect.

7. Past medical history
Tonsillectomy and adenoid removal age 6; cancerous tumour removed aged 32. Mildly asthmatic – otherwise in good health. Treatments for routine infection in the past have included antibiotics with possible ototoxic consequences. Occasional upper respiratory tract infections.

8. Family history of hearing loss and other diseases.
No hearing loss in any part of my close family or extended family. There is a family history of circulatory and heart problems (angina, heart attack) and arrhythmia.

9. Audiometry.
Regular pure tone tests have been performed since 1999. The latest test showed profound loss.

10. Auditory-evoked brainstem response (ABR)
None carried out to date.

11. Electronystagmography
None carried out to date.

12. Magnetic resonance imaging (MRI) scan
None carried out to date.

13. Complete blood cell count with differential (CBC)
None carried out to date.

14. Erythrocyte sedimentation rate (ESR)
None carried out to date.

15. Coagulation profile (including prothrombin time, partial thromboplastin time, and clotting time)
None carried out to date

16. Electrolytes with fasting blood glucose
None carried out to date.

17. Cholesterol and triglycerides
No recent test

18. Thyroid function tests
None carried out to date

19. ACTH plasma cortisol stimulation test
None carried out to date.

20. Serologic tests for syphilis
None carried out to date.

21. Autoimmunologic tests such as antinuclear antibody (ANA) and rheumatoid factor (RF).
None carried out to date.

[1] I moved from a low power in-the-ear model suitable for mild loss to a BTE model with earmould at some point after 2001.

[2] “Recently, a survey was sent to 100 consultant otolaryngologists in the United Kingdom inquiring about their assessment and management of patients presenting with SHL. Results showed that 78% would perform routine blood tests including CBC, ESR, and syphilis serology. 38% would order an MRI at the first presentation. 98.5% of the consultants would treat with steroids alone or as part of a combination. 41% treat with Carbogen, 31% with acyclovir and 35% with betahistine”. (http://www.utmb.edu/otoref/Grnds/SuddenHearingLoss-010613/SSNHL.htm)

The way ahead

First things first. I needed to have a fair degree of audiological assessement. Notwithstanding the fact that a consultant at one hospital had told me to 'come back in December', at least that hospital [whose ENT Department shall be nameless] did take the hearing loss seriously enough to look to step up through the hearing aids.

So on November 1st I moved to using a Sprint 2 hearing aid, and then - on reassessment on 23rd November - up to a Senso aid, both magnificent hearing aids but increasingly not powerful enough to cope with the hearing loss.

We had at that stage completed NO - note, NO - tests other than one lonely pressure test in September and a series of pure tone audiometry tests at 'nameless ENT Department'. There had been no ABR test, no stapedial impedance test...nothing. Zip.

So seeing Tony Wright at 150 Harley Street on 5th October, in which he readily posited a significant progression of my SNHL (and kindly gave me an intratympanic steroid injection to help...the first act of intervention other than a few betahistamine tablets from 'nameless ENT Department') was both a shock and a relief.

I left the consultation reeling. It was as bad as I suspected, but what were the options? Tony has shown me a photograph of a section through the cochlear, pointing out with a move of the hand that many hair cells were 'gone', and that that in all probability - to be confirmed - was the cause of the hearing loss. He had suggested that I needed, quickly, to get referred to the Cochlear Implant programme at the RNTNE Hospital.

I knew about cochlear implants of course, but not enough. My usual mode when faced with anything new is to search the literature (a good academic impulse!) but I also made my way to the bookshops. I bought Michael Chorost's book 'Rebuilt: How Becoming Part Computer Made Me More Human' (Houghton Mifflin, 2005) [Michael's website at http://www.michaelchorost.com/] is helpful for the uninitiated].

That book completely shocked and disoriented me. At once amazed at the technology, fearful of the implications (a CI is a one-way bet; if it doesn't work, you can't just take it out and return to your deaf-but-still-getting-a-tiny-bit state...) and surprised by Chorost's evident love of the 'cyborg' state that implantation produces, I was genuinely thrown by it. I sat up, night after night, hoping that having a CI did not demand this kind of celebratory acceptance, even love, of the technology. I couldn't find it in my heart to feel warmth and gratitude to the bioengineers and software developers...it seemed inhuman, even a denial of the human.

I remember sitting at the kitchen table, at half two in the morning, sobbing quietly at the thought that this was likely to be the one option. That it was not going to produce a return to 'hearing'. That it would mark me as profoundly deaf so totally I'd wear a processor and hook-up on the outside of my head like a sherriff's badge in a western movie...A CI may be a wonderful thing technologically, but it is not a perfect solution.

That, however, is breakthrough thinking. Once you accept that even this brilliance - or the technological brilliance to come as CIs improve - is not going to make you whole again, you can begin to face it, even embrace it, then (possibly) yearn for it with realism.

I will be honest here. I cannot love the prospect of a CI yet. I need it, it is my only option, but I cannot (yet) love the thought of the thing.

Base Camp

The beginning...

My journey to a cochlear implant started one fine July day in 2007 - although the trek to 'base camp' was a long one.

On that July morning, while on holiday in the Picos de Europa in Cantabria (Spain) I took a cable car ride - with my wife Jane and eight year old son Alex - at Funte De. As we reached the top, my right ear began to feel different, then sound began to disappear. I hoped that a barotrauma had temporarily left me deaf, but on the wonderful walking descent to Espinama, nothing changed. I was deafened. For the rest of the holiday, even after returning from the mountains to sea level, there was no return to 'normal' hearing.

Before the onset of full hearing loss I'd had a mild to moderate loss in my right ear, which a hearing aid enabled me to cope with, and total hearing loss in my left from more or less birth. At both Oxford University (1997-2005) and Middlesex University (2005-) I'd held senior jobs that required a fair degree of interaction and usually - except on 'bad hearing days' - I coped well. The transition to more or less profound hearing loss was totally different.

Immediately on return from holiday I saw a GP who said, 'It's probably barotrauma....come back in 6 weeks', then after that six week delay I saw a consultant who said, 'It's possibly barotrauma still, although the readings don't suggest it...come back in December'. I was not impressed.

So I went to see Professor Tony Wright privately at 150 Harley Street, who immediately insisted on a referral to his colleagues comprising the utterly brilliant team at the Royal National Throat Nose and Ear Hospital in London.

That's base camp...now read on as we prepare to tackle the summit. As I write this I don't know if I'll meet the criteria for one, if I'll go ahead and have one...anything, other than that I know this is probably my only option if I want to retain a semblance of membership of the hearing world. (And to those Deaf readers, I DO know that I want to continue as far as possible to remain in a 'hearing' world, albeit as a deafened person).