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)