Case study

Title: “Doctor, why am I so tired and dizzy?”

----------------------------------------------------------------Step 1---------------------------------------------------------------

Alexis, an active and physically fit psychology professor, was regularly dropping items. She thought she
was just clumsy and had a bad case of the “butterfingers.” Her husband started calling her “the dropper”
making fun of what he also perceived as her clumsiness. She enjoyed running half marathons (Fig. 1), but,
unfortunately, during one of her last training runs she took two very hard falls – one during the first mile
and another in the 9th mile. She joked with friends about her stubbornness because she kept running,
despite being bloodied up enough that a “Good Samaritan” driver stopped to ask if she was OK. Both falls
happened from her toe getting caught on a rise in the pavement. Alexis chalked it up to being clumsy and
not lifting her leg high enough. During her next runs, she was careful to watch the ground closely, so that
she could lift her leg enough to avoid further falls. She also had a problem with drooling. Seeing food
(sometimes even thinking about food) caused her to involuntarily drool (an appropriate Pavlovian
response for an experimental psychologist!). Neither she nor her husband thought the dropping or
drooling indicated a medical problem; they just thought it was mildly amusing (though she perceived
both as inconvenient).

Within a month, more symptoms materialized. In addition to drooling and tripping while running, Alexis
developed constant dizziness and extreme fatigue. Despite sleeping 11 hours per night, she still had to
shorten her 2-4 pm class to 2-3 pm because of her need for even more sleep, which now included a 2-
hour nap. Not only did she have dizziness, fatigue, tripping, and dropping symptoms, but her condition
escalated within a short period of time to include the following symptoms:

 deteriorated handwriting (which led to her joking about needing “big crayons”),
 an inability to touch type (she joked about how slow it was to hunt and peck),
 occasional trouble swallowing,
 occasional nausea,
 feeling that her eyes weren’t working together,
 being more sensitive to motion than usual (being a passenger in a car making turns was very
distressing and increased her dizziness to almost unendurable levels),
 perceiving an increase in her hearing (sounds seemed louder than normal),
 feeling that her husband was now walking too fast at the supermarket (previously she had been
frustrated with his slow pace).

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 Fig. 1: Alexis about one month before going to her doctor with symptoms.

Question Set (1)

Q1: What parts of Alexis’ nervous system are most likely affected? Which region of the brain
coordinates muscular activity? What cranial nerves are most likely involved? Be sure to link each
part to one or more symptoms. There are a total of 12 symptoms listed above, discuss at least 5 of
them.

Q2: Is there additional information you think would be useful before any type of clinical testing is
performed? If so, please indicate what else you would like to know about Alexis and/or her
history.

Notes:

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----------------------------------------------------------------Step 2---------------------------------------------------------------

Alexis’ doctor first thought that the dizziness was due to benign paroxysmal positional vertigo (BPPV),
which is the sudden sensation that you are spinning causing brief episodes of mild to intense dizziness.
She was instructed to do the Epley maneuver. Despite doing this maneuver multiple times, the dizziness
remained. The next step was to check her vestibulo-ocular reflex (VOR). Once again, negative results.

Question Set (1)

Q3: Describe the structure of the inner ear. Indicate which structure(s) would most likely be involved
with the symptoms described.

Q4: Alexis received negative results for both tests. What does that tell you? What do you think the next
steps or tests would be?

Notes:

Question Set (2)

Q5: What is the Epley maneuver and how does it work? Describe the procedure for the test.

Notes for additional research and/or questions for Alexis:

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----------------------------------------------------------------Step 3---------------------------------------------------------------

Finally, her doctor ordered an MRI. The MRI revealed a vestibular schwannoma, also known as an
acoustic neuroma; spanning her right cranial nerves IV-XI as well as an arachnoid cyst (Fig. 2a and 2b).
The tumor also extended to her right inner ear.

A neurosurgeon and a neurologist who specialized in the ear, nose and throat (neuro ENT), completed a
marathon 17-hour surgery to remove the tumor and cyst. During the surgery, Alexis was positioned in a
special device to hold her head steady during surgery and the surgeons used microscopes guided by new
MRI images. While they were operating, the 7th cranial nerve (CN VII) on the right side was aggravated.

Fig. 2a: The tumor. Fig. 2b: The tumor + cyst (the white ovoid).

Question Set (1)

Q6: What symptoms might occur from a tumor pressing on CN IV-XI?

Q7: What symptoms might occur from the aggravation of CN VII during surgery?

Notes:

Question Set (2)

Q8: Look up vestibular schwannomas. What causes them?

Q9: What are the typical symptoms patients present?

Q10: Did Alexis have the typical symptoms?

Notes for additional research and/or questions for Alexis:

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----------------------------------------------------------------Step 4---------------------------------------------------------------

When the tumor was removed, the doctors had to sever CN VIII (vestibulocochlear). They also removed
her semicircular canals, utricle and saccule (all on the right side).

By the afternoon after surgery, Alexis was sitting up and the grogginess from the anesthesia was wearing
off. The next day the nurses had her up for her first walk (Fig 3). She had to wear a gait belt so the nurses
could keep her upright if she lost her balance and special yellow hospital socks to indicate she was a high
fall-risk patient. One of the highlights of her recovery was a visit from a therapy dog the day after her first
walk. Her husband spotted a woman visiting rooms with a dog and arranged for Alexis to get a visit.

While recovering from surgery, Alexis felt that her eyes still didn’t seem to be working together.
Unfortunately, she wasn’t able to explain it well and failed to mention it to the nurses or doctors who
tended to make rounds in the morning. The best way she could explain it was, “it feels like my eyes are
fighting each other rather than cooperating.” The medical staff, however, seemed pleased with her ability
to follow their fingers during the tracking portion of the cranial nerve exams they conducted at the
beginning of each new shift. The exams also included questions checking general orientation (e.g., date,
location, reason for being there). When asked, “Why are you in the hospital?” during her first exam, she
replied, with great detail, telling the nurse how she had a vestibular schwannoma. The nurse laughed and
said that most people just said they had a “brain tumor.” Alexis was just being her usual thorough self.

Initially her facial muscles worked well, but she developed paralysis of the right side of her face (Fig. 4).
Her doctors told her they had checked facial nerve function during surgery and it was working well so
they were convinced that any paralysis would ultimately resolve itself and she should get close to full
function back.

Fig. 3: Alexis’ first walk, one day post-surgery.

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 Fig. 4: Alexis is smiling (attempting full smile) 4 days post-surgery

Question Set (1)

Q11: What symptoms would you expect from the severing of CN VIII?

Notes:

Question Set (2)

Q12: What is the time frame for facial nerve healing?

Notes for additional research and/or questions for Alexis:

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----------------------------------------------------------------Step 5---------------------------------------------------------------

Approximately three weeks post-surgery, Alexis experienced clear fluid dripping from her right nostril.
She had previously been warned to watch for any leakage, which would indicate cerebral spinal fluid
(CSF) drainage from the nose. After calling the neurosurgeon’s office to report this, she was told she had
to get back to the hospital as soon as possible. Alexis called her husband and let him know he’d have to
leave work immediately to make the long drive home and from there to the hospital where a bed was
waiting.

Independent of her admission for the CSF leak, a few days after being readmitted, Alexis’ right eye moved
medially (so far that the sclera was barely visible between her eye and her nose, Fig 5). While she could
move her right eye medially (Fig 6, first image) she was unable to make her eye move very far laterally
(Fig. 6, second image). Alexis had to wear a patch over her right eye because her double vision was so
severe she couldn’t keep both eyes open. She decided to draw on the eye patches with a sharpie – a closed
eye and eyelashes one day, an open eye the next (Fig 7).

Alexis also noted that her sense of taste had changed. Her preferred drink (Coke Zero) no longer tasted
good so she went back to drinking Diet Pepsi. Not only did her sense of taste change, but she discovered
she could not sense temperature on the right side of her tongue when she had something ice cold to
drink. This taste change may have been going on for a while, but since she routinely drank her fluids
room temperature, she did not notice it.

Fig. 5: Note deviation of R eye

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Fig. 6: Note that Alexis’ right eye (on left side of pictures) moved medially (first panel), but not fully
laterally (second panel).

Fig. 7: One of Alexis’ decorated eye patches

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Question Set (1)

Q13: Which cranial nerve is responsible for Alexis’ inability to fully turn her right eye laterally?

Q14: What cranial nerve(s) would be responsible for her non-tearing [right] eye?

Q15: What cranial nerve(s) would be responsible for the changes in her taste perception?

Notes:

Question Set (2)

Q16: Why the urgency in getting back to the hospital when Alexis experienced a CSF leak from her
nose?

Q17: Explain why Alexis’ thorough explanation for why she was in the hospital (previous section) and
the humor she exhibited with the eyepatch are good signs (hint: think lobes of the brain).

Notes for additional research and/or questions for Alexis:

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----------------------------------------------------------------Step 6---------------------------------------------------------------

Facebook sites dealing with vestibular schwannomas (typically referred to as acoustic neuromas) talk
about the “new normal” that results post-surgery. Many of the people who have joined Facebook sites for
acoustic neuromas have posts that highlight their continued frustration with their “new normal.” Stories
include those of individuals who still have tinnitus, dizziness, and headaches. Luckily for Alexis, there was
never any tinnitus or headaches and her dizziness disappeared immediately after the tumor was
removed. But, she still had to cope with being completely deaf in her right ear and losing her balance
organs on the right side.

Alexis now suffers from single-sided deafness (SSD). Her brothers insist that it should be called single-
sided hearing to emphasize the positive. Alexis pointed out that single-sided hearing would have SSH as
an acronym which makes it sound like the person is being shushed. Regardless of what it’s called, there
are different solutions for dealing with deafness. Possible devices include: traditional hearing aids,
cochlear implants, brainstem implants, and bone conduction implants. Not all of these devices will work
for Alexis’ one-sided hearing loss that resulted from her surgery.

She still occasionally will say (mainly to herself, but now and then to her husband), “I’m deaf in one ear.”
Alexis knows intellectually that she is and always will be deaf in her right ear and considers it a fair trade
for her life-saving surgery (her neurosurgeon’s words). Besides SSD, Alexis has some other residual
effects which, unlike the deafness, might yet resolve (Fig. 8a and 8b). Alexis still experiences some trouble
controlling her facial muscles on the right side (including synkinesis), her sense of temperature on the
right side of her tongue is missing as is her sense of taste, her right eye needs lubricating drops several
times each night and sometimes closer to the end of the day to keep it sufficiently moist, and her right eye
will not cry. She also experiences a hoarse voice at times.

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Fig. 8a: Alexis 3 months post-surgery. Note the Fig. 8b: Alexis 16 months post-surgery. Note
asymmetric smile smile which is less asymmetric.

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Question Set (1)

Q18: What cranial nerve(s) are responsible for Alexis’ residual effects of brain surgery?

Q19: What is synkinesis?

Notes:

Question Set (2)

Q20: For each assistive device: indicate whether or not it could be used in Alexis’ case and why.
1. traditional hearing aids
2. cochlear implant
3. brainstem implant
4. bone anchored hearing aid (now called a bone anchored hearing system)

Q21: What do you think is the best option? Explain your choice.

Notes for additional research and/or questions for Alexis:

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 Scenarios: Anatomy and Physiology Focus

Place yourself in the following (true) scenarios. What might you feel if you were Alexis? What might you do? Using
your knowledge of structure and function relationships, discuss the structural adaptations that may occur in Alexis
to overcome these new challenges.

Scenario A:

Alexis does not have the balance she used to. She routinely leaves a night light on in the bathroom and
tries to avoid being in dark areas. Being in the dark causes her to lose her balance. Recently, as she was
walking across campus in the evening (after the sun went down), she took a longer path to stay on
sidewalks rather than cut across the grass. Before surgery, she would have cut across the grass, but now
she was worried she’d trip on some uneven ground.

Scenario B:

Alexis cannot cry with her right eye. She has discovered that she does not tear up for emotional reasons
(e.g., a sad story) or for physical reasons (e.g., onions). In fact, she still uses lubricating eye drops in her
right eye (typically at bedtime and in the morning when she wakes up). She’s noticed that her right eye
will tear occasionally. This tends to happen when she’s exercising; not immediately, but after some time.
She also still has vertical displacement of her vision. Her glasses have 4 diopters of prism ground in (2 up
and 2 down) so that images are on the same horizontal level regardless of which eye looks at them.

Scenario C:

Alexis has some trouble keeping food and drink in her mouth. This makes eating with others challenging.
She has to be especially careful with the amount of liquid she puts in her mouth. She tries to compensate
for this by putting a forefinger to her lips to help them stay closed while eating and drinking. She also has
trouble saying certain letters/letter combinations. Alexis feels that her muscles are fighting to say words
such as “speaking” (the “sp” sound is hard to make).

Scenario D:

Alexis has a very difficult time localizing sound, if she can at all. A person who didn’t know about her
surgery called to her saying “Over here” when she didn’t immediately look in the correct direction. She
just chuckled and continued to turn around trying to figure out where “over here” was. Alexis has told her
students about her localization problems and has asked them to raise their hand when they speak so she
can direct her response to the appropriate person.

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 Scenarios: Psychological Focus

Alexis now has a hearing impairment. She is deaf in her right ear and has trouble understanding people
who are talking on her right side, if she is able to hear them at all via her left ear. Place yourself in the
following (true) scenarios. What might you feel if you were Alexis? What might you do?

Scenario A:

Alexis started wearing a pin that tells people, who read it, that she’s deaf on the right side. Now she has an
obvious sign of a problem. Do you think people will treat her differently? If so, how might their actions
differ from interacting with a non-hearing impaired individual?

Scenario B:

While at a hotel, approximately 4 months after surgery, Alexis asked the concierge to find a number for a
guest’s room. The concierge dialed the phone then handed her the receiver. She took it with her dominant
hand (right) and out of habit put the phone to her (now deaf) right ear. Alexis asked the concierge,
“Should I be hearing something? I don’t hear any ringing.” After realizing she was using her deaf ear, she
switched ears, had her conversation, then handed the phone back to the concierge and simply said,
“Sorry, newly deaf.” What type of reaction do you think this social interchange would yield?

Scenario C:

While working in group settings, Alexis has had several “misadventures” interacting with others. One
instance occurred at a busy lunch table and the other while grading standardized tests with other faculty
in a grading room.

At the lunch table, Alexis was speaking over others in the group. She realized there was someone else
talking when she turned her head at one point in her conversation and then heard the other person
speaking. Alexis stopped speaking and apologized for talking over the other person. In the grading room,
Alexis started talking not realizing that a person on her deaf side was speaking so she ended up speaking
over them. Her partner tapped her on the shoulder and pointed to the person speaking. In both
situations, not everyone present knew that Alexis was deaf on the right side. What types of assumptions
do you think were made by the people who knew she is deaf vs. those who did not?

Scenario D:

Alexis now has a bone-anchored hearing system (BAHS). Even though Alexis now has a BAHS, she still
orients herself so her left ear is pointed toward whatever she is listening to. This has caused some
problems. In the classroom, for example, when students ask questions during tests she insists on hearing
them with her left ear. When sitting with others at a round table (like at a restaurant) she finds it difficult
to hear someone on her right side. But, if she plugs her left ear, she does hear via the BAHS. Discuss what
options she has for dealing with these situations.

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