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A speech pathologist who tried to save his hearing
Byron's story

December 1998: I knew for sure that I had hearing loss and tinnitus (ear noise or ringing). The alarm clock sounded very different, like low clicking, when my right ear was down in the pillow. I found myself having to switch to my right ear on the telephone. And I had some trouble communicating in noisy situations such as in a cafeteria, a gym, or an automobile, particularly when the speaker was on my left. I noticed that some of my friends and coworkers looked a little peeved at having to repeat themselves in noisy situations. In my work as a speech pathologist, I could hear the kids well in my quiet evaluation-therapy room as long as they took turns talking. I was not as effective in hallways or classrooms where there’s usually some competing noise. The tinnitus on the left was constant and sounded like crickets but I only really noticed it several minutes a day.

January 1999: My local (small town) ENT confirmed that I had hearing loss in the high frequencies in my left ear, and found that middle ear fluid was causing conductive hearing loss. The doctor and I agreed that I had a combination of conductive and sensory-neural hearing loss. However, my speech discrimination score was 100%. The prescriptions cleared up the conductive problems that were probably caused by allergies but I still had the rest.

February 1999: An audiologist verified the previous findings of severe/profound hearing loss in the high frequencies in my left ear. That’s 4,000 Hz, 6,000 Hz and 8,000 Hz. My right ear was within the normal range. Auditory Brainstem Response (ABR) testing was done. I was connected to a rather loud machine by headphones and electrodes on my ears and face. The results were said to be inconclusive. The reason for the trouble hearing in noisy environments was that noise often masks the normal speech frequencies ("upward masking"?); and my ability to use the high frequencies to compensate for that was limited. I thought the problem was probably in the cochlea and may have been due to dysfunction of a tiny blood vessel there (something very similar to a stroke but on an extremely small scale); but I thought I would never know for sure. I was told that the change of a growth on my auditory nerve was remote, and I chose not to have MRI (imaging) done. I planned to follow the advice of the audiologist: I would quit pretending and become more open and "proactive" about letting people know when I couldn’t hear them. I started learning the tactics to avoid and solve problems related to my hearing.

February 1999: I began to feel dizzy and lightheaded. Whenever I turned quickly, I lost my balance briefly, but never fell. I realized that this called for MRI (imaging).

March 1999: I had my first MRI done. I laid flat and still for about one hour, twenty minutes to have the pictures taken of the area of the internal auditory canals (IACs) which are passageways for a few cranial nerves. About halfway through, Prohance gadolinium was injected into my arm to highlight any abnormalities. I was a bit concerned going into the ENT’s office for the results because he had personally asked that I come earlier than scheduled. He showed me the images and I immediately saw a bright image of a tumor (medium-sized: 2.4-cm) against the pons and cerebellum. (I had studied some neurology in college.) It had grown from the IAC on the left and was pear shaped. He said it was an acoustic neuroma (AN) causing the symptoms by compressing the auditory and vestibular nerves, and he was referring me to a neurotologist and neurosurgeon in Atlanta.

I had heard about Gamma Knife for tumors on the radio so I asked about it when I saw the Atlanta doctors. They were nice enough and seemed very competent and they quickly and almost totally convinced me to forget the radiation options due to the danger of either not destroying the entire tumor or accidentally damaging the adjacent brainstem. Because of the size, shape and location of the tumor, microsurgery with the translabyrinthine approach was the only way. The idea was to play it safe with the facial nerve and avoid manipulation of the temporal lobe and cerebellum. They said they’d monitor the facial nerve electronically and otherwise. But there was no point in monitoring the other nerves with this approach, since I would lose my cochlea and semicircular canals on the left. They planned to drain fluid from my lower spinal column to reduce pressure during initial healing and use a little of my abdominal fat to fill the space left by removal of the semicircular canals. We scheduled the surgery in spite of my fears and I went away with confidence in those doctors.

April 1999: While making plans for missing time at work and arranging for support from family and friends, I read much more about the potential and real risks of surgery and other options and exchanged mail with some wonderful online AN patients. I decided to explore the options further. I cancelled the surgery and sent my MRI films to Dr. Jeffery Williams of Johns Hopkins Hospital in Baltimore, who judged from the images that I was a candidate for Fractionated Stereotactic Radiosurgery (FSR). Yeah!!!!  I made an appointment for a face-to-face meeting in May.

May 1999: After traveling to Baltimore and spending a comfortable night at a hotel in the Mount Vernon neighborhood, I awoke to a beautiful sunrise highlighting the Washington Monument just outside my eighth floor window and proceeded to breakfast, the hospital travel agent and to meet Dr. Jeffery Williams and some of his staff at Johns Hopkins Hospital. They were all very nice and seemed quite competent. They corrected some clerical errors in my file, added to it and asked me about my symptoms. Dr. Song did a brief neurological evaluation including touching my face with a sharp toothpick and a dull swab and asking me to tell him whether it felt sharp or dull in various locations with eyes closed.

By this point, my facial (tongue) nerve was affected by the AN. About 1.5 cm back from the tip of my tongue, in an area the size of a smallish paper clip, the sensation in my taste buds was very noticeably different. Most foods were at least a little strange; some breads had a metallic taste; even water and cool air felt odd and a little raw.

Dr. Williams answered all my questions patiently and told me openly that the chances of re-growth with FSR wouldn’t really be known for many years. The six years or so, since he started, was not very long on the AN scale of time. But judging solely by the appearance of the tumors in follow up MRI films, the future looked very good. He told me that he has done the "traditional surgery" and still does for some patients who "want it out". But he much prefers FSR. We scheduled the radiosurgery for June, made a re-locatable mask to hold my head in place, and made some more images for planning and programming.

June 1999: I had the second solo trip to Baltimore for my FSR treatment; going solo was not a problem. Afterwards, I wrote the following passages in a letter to family and friends:

The treatments, Fractionated Stereotactic Radiosurgery (FSR), went as planned. Five days in a row of short (40 min. max), painless outpatient visits to Johns Hopkins Hospital during which I wore the re-locatable thermoplastic mask-helmet (that we had made on the first visit) connected to a metal head frame. They x-rayed my head from two angles each day to line me up. Based on the CAT scan images, a huge robotic machine was programmed to shoot gamma radiation into the tumor from many different angles using the locked-on head frame as a reference. Dr. Jeffery Williams was in charge and was present and involved much of the time. He and his technicians said the x-ray pictures looked good and everything was working well. The doctor answered all my questions and seemed unhurried and willing to talk as long as I wanted. We discussed worst-case scenarios so that I would know what to watch for and what to do; but the problems aren't likely. So I can easily be optimistic as we do the follow up MRI's. I'll be having MRI films made locally and sent to Baltimore every 3 months, then every 6 months, and annually after that. We'll be watching for swelling in the treated area and changes in the size of the tumor and its absorption of the contrasting agent they shoot into me. If the neuroma appears dark in the interior then we assume that it is no longer receiving as much, or any, blood supply. We'll see...

When I first found out about this tumor, I had very little understanding of what I might be "in for". The traditional surgery has major scary risks. But now, thanks to the doc and his people with their wonder-machines, all I've had to deal with was a little nausea from the x-rays (I think) (I didn't vomit but felt a queasiness in my stomach at times) and fatigue. And I'm not sure the fatigue wasn't from the traveling, touring and the unusual foods.

I stayed in the Mount Vernon neighborhood surrounded by restaurants of all ethnic varieties and with a view of a park and their Washington Monument, which is older than the one in DC. I really enjoyed the nearby art museum, The Walters, with its wide range of exhibits from Sumerian stone writing tablets from 3000 BC, Egyptian statues from 2465 BC, a mummy, Greek and Roman figures, statues and helmets, Medieval art, Islamic art, Renaissance paintings (my favorites), and decorative arts of Faberge and Tiffany among all sorts of jewels. There were also Asian exhibits...

My favorite meal was the lumpy crab cake with boiled potatoes and rapini (of the broccoli family) with a fancy view of the harbor. The Egyptian pizza with lamb strips at Al Pacino's Cafe was really good, too. The soft crab sandwich at the Women's Industrial Exchange, among their ancient customers, wasn't so good; but I should have ordered something more familiar that nauseous day. Their dessert was good though and they offered to sell me some of the local women's handiwork...

The combination of traveling, touring, radiation treatments and eating strange foods caused fatigue by the third and fourth days. Earlier in the week, when I was feeling normal, I spent several great hours, unrushed by my absent companions, in the National Aquarium. It was by far the best I've seen. Another day I went cruising on various types of water taxi boats up and down the harbor and out to Fort McHenry, the defense of which inspired our National anthem in the war of 1812, and which was interestingly designed in a star shape. They built furnaces below some of their cannon to fire red-hot cannon balls at the British enemy ships to set them afire as they attempted to enter the harbor to take Baltimore.

I wandered the city streets some. I accidentally found Edgar Allen Poe's grave in a would-have-been-spooky-at-night cemetery; I turned a partially deaf ear to panhandlers who looked healthy to me; I watched a guy get busted on the street and heard him saying he just got out of jail two days before, until a cop told me and the others to "keep moving, no loitering"; there were apparently homeless people arguing with imaginary companions; I lingered and bought bananas and a tasty 'Mystic' drink at a classic market with inner-city blacks and Asians selling their raw sea foods, poultry, fruits and vegetables to each other.

Now I'm home...

September 1999: The disequilibrium increased for only a few weeks.

December 1999: I start having earaches and headaches, which are attributed by my local ENT to TMJ problems (involving the joint of the lower jaw and temporal bone near the left ear).

January 2000: Dr. Williams sent this report regarding my January MRI films:

I have compared this study to the 9/99 and the simulation images from 6/99.  There is clearly a marked decrease in the enhancement and a small decrease in the size on the current scan when compared to the prior images.  There is no evidence for toxicity or edema on the scan as well. In summary, the MRI looks very good! I recommend repeating in 3 months.

January 2000: I’m very happy with the shrinkage and apparent necrosis of the tumor. My hearing and tinnitus same as before, the other symptoms are gone. I am patiently working on the allergy and TMJ problems. The headaches have been scattered around the TMJ site and are not very severe. I’ve not mentioned this to Dr. Williams but I will if it continues. I’m trying to put less tension and strain on the joint.

Byron Jones <beedj AT atnex.net>
Georgia, USA.
February 2000


Updates

(1/01/07) I had translab surgery in May 2006 by House Clinic doctors in Los Angeles, following failed FSR at Johns Hopkins Hospital in 1999. The most recent MRI images showed significant tumor growth, which was confirmed by a few physicians, including Dr. Rigamonte at JHH. And facial nerve damage was becoming evident; there was muscle twitching, and the affected eye was abnormal in its response to threat, it didn't blink like the other eye. So there was no doubt that further treatment was needed, and no one recommended more radiation. And no one thought they could save my hearing.

The surgery went well. I recovered quickly and was able to see some of the LA area with my sister and daughter. If you ever need this surgery, I'd say go prepared with plenty of eye drops and with a protective contact lens to prevent cornea drying/damage. The nurses did not do enough eye dropping. I should have done more myself. And I was late in acquiring the lens. That was uncomfortable. Maybe I should have had that lens immediately after the surgery.

I have been waiting for my facial nerve function to return to normal, and I'm now happy to report that the recovery is indeed happening. My face has steadily improved. There is certainly improvement in cheek and lip muscle movement. My mouth looks pretty much normal now with some obvious movement on the affected side when I talk and smile. But the smile is still a little asymmetrical. My work as a speech therapist is back to near normal. I still can't puff out my cheeks, or blow up an air mattress (or a victim's lungs in CPR), or swish mouthwash without using a finger to seal the weak spot. I can't whistle with my lips, yet.

The eye is taking care of itself much better now. I recently stopped the eye drops (after about 6 months) and more recently stopped the night time ointment and taping of the eye (after about 7 months) and the eye feels good. I have to rub it to facilitate the blinking to work up some tears, now and then. But the eye strangely produces abundant tears when I'm eating. The tears flow down the cheek when I'm really enjoying a meal. I get some looks.

The tinnitus is still with me, no change there. My balance was only slightly affected. The total hearing loss on my left gives me some trouble, as with anyone with single sided deafness; the sound localization problem causes some weirdness and can be a little hazardous. I'm to follow up with an MRI in May 2007 (one year). We'll see if the sliver of tumor along the facial nerve shows up. They left it, in order to avoid permanent facial nerve damage.

(3/06) Almost seven years after FSR I'm facing surgical removal of the tumor. It has grown significantly. The Johns Hopkins radiation oncologist reported neuroma growth from 6.8 cc in 1999 to 7.5 cc in 2006. He will not repeat FSR . An Atlanta radiation oncologist agreed that further irradiation would be too risky for the facial nerve. A local neurotologist measured the linear growth from 2.4 cm to 3.3 cm (medium to large, he said). He strongly recommended the translab approach. Mild vestibular symptoms have come and gone but hearing and facial areas have not changed. People are still saying that FSR is successful 95% of the time. I hope that's true. I say if you choose any radiation treatment, you should repeat MRI every year and have a third party neuroradiologist or neurotologist do the measurements. 

(8/03) I had very good news from my audiologist, supporting my perception that I'm functioning better. My pure tone hearing is stable and my speech discrimination score was much improved - 100% in each ear tested separately, with masking. I still have some problems with some noisy situations but I am happy to see/hear improvement. 

I'm still doing very well with very good recent reports and am very glad that I chose FSR with Dr. Williams at JHH. My hearing loss and tinnitus are still with me but the other symptoms have gradually faded away.  My speech discrimination score, done with masking as part of the recent audiological evaluation, has risen remarkably even though the pure tone results have remained the same. My audiologist and I were pleased but not able to explain except that the brain is so complex and adaptable... perhaps now after the tumor damaged the acoustic nerve, I have learned to comprehend speech better using the acoustic/auditory signals that I have. In other words, maybe I'm just learning to use what I have left.

Summary:

1. All symptoms of the AN reduced since treatment.
2. Hearing: slightly improved.
3. Tinnitus: still with me, but reduced.
4. Balance: back to normal.
5. No headaches.
6. MRIs show considerable shrinkage and necrosis of the tumor.
7. Fear of the long term effects of FSR: very little.

Dr.Rigamonti at JHH wrote about my July 2003 MRI: there has been no significant change... no evidence for toxicity or adverse effect in the surrounding normal brain. He also wrote: after four years, you only need MRI and audiograms every two years.

(3/02) I'm still doing well and am very glad that I chose FSR with Dr. Williams. My pre-FSR hearing loss appears to be stable and the tinnitus is still with me. But the most recent MRI in June 2001 showed no growth of the tumor and my other symptoms have faded away. My speech therapy work with children is going well, in my quiet therapy room, but there are some problems in noisy situations. I want patients to feel free to contact me with questions. Good luck to you, whether you choose the FSR or some other route.

(8/02) My latest MRI follow up was done in July, 2002 (just over three years post-FSR). I sent the recent MRI films and an email to Dr. Williams, not knowing of his death. Tammy Cuda called me to give me the terrible news.  I was very much saddened by the news that Dr. Williams of Johns Hopkins Hospital had died. He was a great man (and my doctor).

Soon after, I received a letter from Johns Hopkins signed by Danielle Rigamonti, M.D., Professor of Neurosurgery and Radiology. The message was very similar to the previous letters, in content and style. The news was good: no significant change in the size or enhancement of the tumor.

I'm doing fine. My hearing and tinnitus seem unchanged since before the FSR. I haven't noticed any problems in balance, taste, etc. I'm very pleased with the results, so far.  

(12/00) My latest MRI follow up was done in December, 2000 (18 months post-FSR). Dr. Williams of Johns Hopkins Hospital wrote of this MRI study: "When compared to the prior scan dated June 12, 2000, the size is approximately the same. However, the enhancement (brightness) has decreased, consistent with killing of the acoustic neuroma cells."

I'm very pleased with the results, so far.  I'm doing fine. Lately, I haven't noticed any imbalance except in total darkness, a welcomed improvement. The hearing and tinnitus seem unchanged since before the FSR. I no longer have the occasional eyelid twitching and strange lip sensations that I once had when tired or immediately following strenuous exercise. So far, so good.

(5/00) I just received the reports on my latest (one year post-FSR) MRI. I'm very pleased with the results, so far. Dr. Williams wrote that the AN is smaller, "the change is moderate but definite, indicating that the procedure is continuing to work and result in decreased size." The local radiologist also measured the tumor and found that it is somewhat smaller than before.

I'm doing fine. I haven't noticed any imbalance or taste problems in several months. The TMJ earaches and headaches ended soon after I became aware of how I was causing them. The hearing and tinnitus seem unchanged since before the FSR. I plan to have an audiological evaluation in July. I've had some occasional eyelid twitching and strange lip sensations when tired or immediately following strenuous exercise. When I asked the doctor about that he responded: "based on the imaging study I can't correlate the eye twitching and lip sensations to the scan. I am optimistic these sensations will decrease over time." I'll be optimistic, too.

Byron Jones <beedj AT atnex.net>
Georgia, USA