w3 Flashcards

1
Q

what is otosclerosis?

A

A metabolic bone disease of ossicles & otic capsule
fixation of ossicles (stapes)
- Spongy bony growth occurs and then solidifies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what hearing loss does otosclerosis cause?

A

conductive or mixed hearing loss or cochlear

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what is cochlear otosclerosis

A

hen boney growth degrades into cochlea and the loss is purely sensorineural

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Clinical otosclerosis:

A

symptomatic and presenting with combination of hearing loss and tinnitus (rarely vertigo)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Histological otosclerosis

A

Asymptomatic more common than clinical, typically diagnosed postmortem

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

classification:site of lesion

A
  • Which structure is affected (less used because clinicians see subtypes as a continuum rather than 2 distinct entities)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Fenestral otosclerosis

A

stapes predominately affected

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

retrofenestral otosclerosis

A

cochlea predominantly affected

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Prevalence otosclerosis

A

Caucasian 10%, Asian 5% African American 1% native American 0%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

onset of otosclerosis

A

15-45 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

clinical sex ratio otosclerosis

A

M:F 1:2.5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

histological sex ratio otosclerosis

A

1:1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Hereditary otosclerosis

A

family history 8 genes implicated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Endocrine Otosclerosis

A

increase prevalence in women

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Autoimmune otosclerosis

A

immune system genes found associated with otosclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

metabolic otosclerosis

A

genes in bone remodeling pathway found associated (not causative) with otosclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Osteosclerotic process

A

resorption and formation of new bone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

early phase otosclerosis

A

otospongiosis: vascular spongy bone growth

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

late phase otosclerosis

A

dense sclerotic bone in areas of earlier resorption & otospongiosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Cochlear dysfunction otosclerosis

A

invade membranous labyrinth
Atrophy of spiral ligament in lateral wall

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

site of dysfunction (stapes & otic capsule)

A

Middle ear (conductive component): bone remodeling of oval window & foot plate(anterior focus, most common; round window less frequent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Cochlea: sensorineural component (otosclerosis)

A
  • Perilabyrinthine decalcification
  • Osteosclerotic bone of otic capsule
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

for otosclerosis with SNHL (cochlea) Damage occurs via

A

(1) bony invasion of membranous labyrinth (2) enzymes produced in osteosclerotic foci adjacent to lateral wall of cochlea, infiltrate membranous labyrinth
- Disrupts cochlear fluid homeostasis
- Eventual degeneration of organ of corti

24
Q

case history and symptoms otosclerosis

A

Progressive HL: conductive, mixed, Sensorineural, Carhart notch
- Initially unilateral but often progress to bilateral (80%)
- Tinnitus common (75%)
- Vestibular complaints (25%)

25
Q

medical diagnosis otosclerosis

A

based on case history, symptoms and audiological assessment
- Physical exam limited, most patients have normal EAC and TM
- Schwartze sign: reddish hue around TM due to highly vascularized otoscpongeosis and can sometimes be visualized through the TM
- Imaging not usually required: high res CT used for surgical planning

26
Q

Audiogram otosclerosis

A

variable presentation
- Unilateral initial, progress to bilateral
Common presentation: conductive HL in low freq
- With progression configuration flattens
- further progression=decline in BC threshold in high freq &
increased conductive component in low freq

27
Q

carharts notch otosclerosis

A
  • 2KHz Carhart notch (not always present)
  • Primary resonance of the ossicular chain for BC signals approx.. 1.7KHz
  • Otosclerosis causes reduction in BC activated ossicular motion affecting resonance notch appears at 2KHz
28
Q

immitance otosclerosis

A

Type A or As
Acoustic reflexes absent or unusual (reverse or “on-off” or diphasic)

29
Q

interventions for otosclerosis

A

Stapedectomy, stapedotomy
Medical treatments: floride (not effective) or Bisosphonate (vary range of success)
Amplifications: for those not wanting/fit for surgery
Cochlear implant: advanced otosclerosis invading cochlea causing SNHL

30
Q

Stapedectomy:

A

older traditional technique where entire footplate removed, prosthetic piston used to replace entire stapes

31
Q

Stapedotomy

A

more common, footplate remains intact, less trauma to oval window, less possibility of damaging inner ear, incus vibroplasty

32
Q

goal of otosclerosis surgery

A

close air bone gap
- Otosclerosis can recur and cause displacement of prosthesis
- Revision surgery may be required
- May not restore hearing to initial post-op level

33
Q

what are Temporal bone paragangliomas

A

(Aka glomus tumours)
Neuroendocrine glomus cells cluster to form paraganglia in parasympathetic nervous system
- Clusters reside in adventitia (connective tissues) of blood vessels and parasympathetic nerves

34
Q

where are Temporal bone paragangliomas distributed

A

ear, larynx, along vagus nerve & associated with carotid artery & aorta

35
Q

4 types of head and neck glomus tumours

A

Carotid body paragangliomas (CBP)
Glomus jugulare (GP)- occur within temporal bone
Glomus tympanicum (GT)
Glomus vagale (GV)

36
Q

age for temporal bone paraganglioma

A

40-60 years

37
Q

temporal bone paraganglioma sex related assocition

A

4:1 ratio of females to males

38
Q

temporal bone paraganglioma etiology

A
  • Sporadic
  • Genetic: Familial autosomal dominant disorder (<10% cases; chromosome 11q23)
39
Q

Glomus tympanicum (GT) originate

A

from promontory in ME
a. Course of tympanic branch of glossopharyngeal nerve (Jacobsen’s nerve

40
Q

Glomus jugulare originate

A

Arise in area of jugular bulb below the floor of the ME
a. Aurical branch of the vagus nerve (Arnold’s nerve) or Jacobsen’s nerve
b. May compress cranial nerves IX and XII
c. Additional growth can lead to compression of the brainstem

41
Q

glossopharyngeal and vagus nerves affect

A

swallowing and horse voice

42
Q

hypoglossal nerve affect

A

loss of sensation & paralysis of tongue

43
Q

symptoms glomus tumour

A

Vary with tumor origin and spread
Earliest signs: hearing loss and pulsatile tinnitus
Additional symptoms: headache, vertigo, otalgia, aural fullness, cranial nerve compression etc.,

44
Q

role of audiologist in glomus tumour detection

A

aware of unilateral symptoms associated with vestibular schwannoma (acoustic neuroma) and need for referral
- Glomus tumours associated with similar symptoms/complains but different clinical presentation
- Aware of potential comorbidities of these tumours

45
Q

pathology of glomus tumour

A
  • Firm red mass, slow growing, typically benign, non metastazing
  • Locally destructive: multi-directional spread along paths of least resistance, erode mastoid bone, invade ME, eustachian tube, brain cavity, cochlea and vestibular end organs, rupture TM
46
Q

Glomus jugulare incidence

A

paraganglioma 1-3/100 000 of which 16%-24% are Glomus jugulare

47
Q

Glomus Tympancium incidence

A

paraganglioma 1-3/100 000 of which 20% are GT

48
Q

GT can cause

A

conductive loss: impaired ossicular motion, abnormal ME pressure/aeration
- SNHL and/or dizziness: tumour can invade inner ear

49
Q

Glomus Jugulotympanicum

A

For large tumours, site of origin may be uncertain as it involves:
- Jugular bulb & fossa
- ME promontory
Ex., tumour grows superior-laterally from jugular formen to invade ME

50
Q

Medical examination for Paraganglioma

A

Otoscopy findings
Inspect oral cavity and pharynx for pulsing contractions
Pulsatile tinnitus: decreases with head rotation ipsilaterally
Neurological exam
Radiology: CT & MRI

51
Q

Otoscopy findings for Paraganglioma

A
  • Brown sign= red “blush” on TM; blanches with ear canal pressure
  • Red mass behind TM (rising sun)
  • Biopsy not advised on outpatient basis (bleeding)
52
Q

Medical management GT:

A

surgical excision
- Tympanoplasty; Mastoidectomy if more extensive

53
Q

Medical management GJ:

A

depends on tumour extent and patient factors
- Wait and see approach (patient risk factors: age, anaesthetic risk, tumour location)
- Surgical excision: challenging surgical procedure (ENT & neurosurgery) – risk of cranial nerve damage & excessive bleeding
- Radiation may be preferable
- Radiation & surgery may be combined

54
Q

audiology assessment glomus tumour

A
  • Conventional audiometric assessment
  • Variable audiometric and immittance results
  • Immittance: pulsatile admittance synced to heartbeat
  • Referral for medical assessment if classic signs (pulsatile tinnitus, red mass noted on otoscopy)
55
Q

audiology managment glomus tumour

A
  • Monitoring for recovery post-treatment & recurrence
  • Aural rehabilitation if residual hearing loss following medical management
56
Q

aural rehabiliation glomus tumour

A
  • Counselling for HL & tinnitus
  • Conventional amplification & ALDs
  • Cochlear implant
  • Bone conduction aid