Week 6: Middle Ear Disorders/Diseases (II)

Question 1

What is Otosclerosis?

Answer 1

a disease of the bony labyrinth—bone tissue of the bony labyrinth begins to grow abnormally

Question 2

Where is the word Otosclerosis derived from, and what does it mean?

Answer 2

derived from Greek, it means abnormal hardening (sclerosis) of the ear (oto)

Question 3

Describe what happens in the bone in Otosclerosis

Answer 3

the “new” bone that grows is soft (otospongiosis) but with time, the soft areas scar and harden (otosclerosis)

Question 4

What happens when the “new” bone grows over the footplate of the stapes?

Answer 4

the stapes becomes partially or completely fixed

Question 5

When the “new” bone grows over the footplate of the stapes and caused the stapes to become partially/completely fixed, what does it limit and what type of hearing loss does it result in?

Answer 5

this limits the movement (vibration) of the ossicular chain (decreasing the transmission of sound to the inner ear) resulting in a Conductive hearing loss

Question 6

When the “new” bone grows over the stapes and inside the cochlea, what type of hearing loss does it result in?

Answer 6

Mixed hearing loss

Question 7

When the “new” bone only grows inside the cochlea, what type of hearing loss does it result in? How often does this happen?

Answer 7

Sensorineural hearing loss

less frequently

Question 8

True or False. Otosclerosis tends to run in families (~50% of cases)

Answer 8

true

Otosclerosis tends to run in families (~50% of cases)

Question 9

Otosclerosis is inherited in an autosomal dominant pattern with variable penetrance, what does it mean?

Answer 9

this means that there is a 50% chance of getting the gene for otosclerosis if one parent has it (dominant), but not everyone with the gene develops symptoms (variable penetrance)

Question 10

But why do some people with no family history of otosclerosis develop it?

Answer 10

perhaps, in some cases, an autosomal dominant disorder results from a new (de novo) mutation of the gene

Question 11

What is the female to male ratio of Otosclerosis?

Answer 11

2:1

Question 12

Otosclerosis is bilateral in up to what percentage of cases?

Answer 12

75%

Question 13

Otosclerosis occurs most frequently between what ages?

Answer 13

20 and 50 years of age

Question 14

Initial awareness of hearing loss or an accelerated progression of an existing hearing loss is a common observation when?

Answer 14

during or following pregancy

Question 15

True or False. Hormonal changes during pregnancy do not stimulate the new bone formation in otosclerosis disease.

Answer 15

false

hormonal changes during pregnancy may stimulate the new bone formation in otosclerosis disease

Question 16

Several studies have shown that otosclerosis is most common in?

Answer 16

Caucasians, about half as common in the Oriental population, and very rare among Blacks and American Indians

Question 17

True or False. Otosclerosis will progress to different degrees and at different rates for different people

Answer 17

true
otosclerosis will progress to different degrees and at different rates for different people (not everyone will develop moderate to severe hearing loss)

Question 18

What is the typical (primary) form of otosclerosis?

Answer 18

gradually progressive
bilateralconductive hearing loss
most frequently between 20 and 5o years of age
the “new” bone grows over the footplate of the stapes, and the stapes becomes partially or completely fixed

Question 19

Describe the pure-tone audiometry for the early stage of otosclerosis

Answer 19

bilateral conductive mild low-frequency hearing loss (stapes is partially fixed)

Question 20

Describe the pure-tone audiometry for the late stage of otosclerosis

Answer 20

conductive hearing loss increases (and more frequencies are affected)
there is frequently a Carhart’s notch

Question 21

What is Carhart’s notch?

Answer 21

a depression in the bone conduction audiogram (an increase in bone-conduction threshold) most at 2 kHz

Question 22

Is Carhart’s notch an indicator of cochlear hair loss damage? Why or Why not?

Answer 22

this is not an indicator of cochlear hair cell loss damage because it may be corrected by surgical intervention

Question 23

(blank) is an “artefact” that happens when testing bone conduction

Answer 23

Carhart’s notch

Question 24

Describe the other result (not the conductive hearing result) pure-tone audiometry for the late stage of otosclerosis

Answer 24

when the “new” bone grows over the stapes and inside the cochlea, a Mixed hearing loss results
audiogram: bilateral slightly asymmetrical mixed hearing loss, moderate to severe (right ear), moderately-severe to severe (left ear)

Question 25

Describe the hearing assessment done to a patient who suffers from otosclerosis (tympanogram, ECV)

Answer 25

Tympanogram: Type As ECV: normal

Question 26

Who is the most acclaimed and influential composer of all time?

Answer 26

Ludwig van Beethoven (1770-1827), a german composer and pianist

Question 27

Ludwig's hearing began to deteriorate when and what did he continued to do?

Answer 27

1796 (when he was 26 years old) | he continued to compose

Question 28

Provide the timeline of Ludwig's hearing loss

Answer 28

1796 (26 years old): Beethoven presents first signs of hearing loss 1801 (31 years old): Complains of “buzzing” in ears (tinnitus) and hearing continues to deteriorate 1816 (46 years old): Uses ear trumpets 1818 (48 years old): Uses conversation books—conversation had to be written 1822-24 (52-54 years old): Beethoven composed Symphony #9 in D minor (“Choral”)

Question 29

In Otosclerosis, the growth of the new bone from the (blank) over the (blank), in the cochlea or both

Answer 29

growth of new bone from the bony labyrinth over the footplates, in the cochlea or both

Question 30

True or False. Chronic consumption of wine tainted with lead can have an effect on the cochlea and cochlear nerve

Answer 30

true | chronic consumption of wine tainted with lead (affects cochlea and cochlear nerve)

Question 31

What is Barotrauma?

Answer 31

is sudden changes in air pressure (when flying or diving) and poorly functioning Eustachian tubes (allergies, cold, sinusitis

Question 32

What barotrauma may cause?

Answer 32

conductive or mixed hearing loss ear pain fluid accumulation bleeding

Question 33

What is Cholesteatoma?

Answer 33

is a pseudotumor, composed of keratin (protein) mixed with squamous epithelium (skin cells) and cholesterol extremely dangerous, it is highly erosive and causes destruction of bone and other tissues

Question 34

Where is Cholesteatoma located?

Answer 34

located in the middle ear (tympanic cavity)

Question 35

What does Cholesteatoma present as?

Answer 35

``` white mass (otoscopy) otorrhea (foul-smelling discharges) conductive hearing loss ```

Question 36

Cholesteatoma may cause destruction of bone and other tissues, given an example discussed in class

Answer 36

labyrinthitis facial palsy (paralysis) mastoiditis meningitis

Question 37

What is Labyrinthitis?

Answer 37

the inflammation (or infection) of the inner ear (labyrinth), clinically this condition produces disturbances of hearing and balance

Question 38

What may Labyrinthitis causes?

Answer 38

mixed hearing loss vertigo (a sensation of turning in space or a feeling that the room is spinning—from Latin “vertere” to turn) nausea or vomiting imbalance

Question 39

What is Facial palsy (paralysis)?

Answer 39

the loss of muscle function on one side of the face, making it difficult to smile or close the eye on the affected side

Question 40

What is Mastoiditis

Answer 40

an inflammation (or infection) of the mucosa lining of the aditus, antrum and mastoid air cell system

Question 41

What is Meningitis?

Answer 41

an infection of the coverings (meninges) of the brain

Question 42

How many layers are meninges and what does it protect?

Answer 42

meninges are three layers of membrane that protect the brain and spinal cord

Question 43

What are the three layers of meninges?

Answer 43

dura mater arachnoid pia mater

Question 44

What are the symptoms of meningitis?

Answer 44

``` headache fever neck stiffness sensitivity to light nausea and vomiting drowsiness confusion delaying treatment (increases the risk for permanent brain damage or death) ```