Clinical Hearing Disorders

Front 1

What are the ABCDs of congenital hearing loss?

Back 1

1. Affected family members

2. Bilirubin

3. Congenital intrauterine causes/CMV

4. Deafness

5. Environmental

Front 2

What is the MCC of non-syndromic congenital SNHL?

Back 2

1. CMV

Front 3

What are the ssx of Rubella syndrome?

Back 3

1. Congenital cataracts

2. CV anomalies

3. Mental retardation

4. Retinitis

5. Deafness

Front 4

What is damaged in rubella syndrome?

Back 4

1. Degeneration of organ of Corti

2. Adhesion between organ of Corti and Reissner's membrane

3. Rolled-up tectorial membrane

4. Partial or complete stria atrophy

Front 5

How do you test for CMV deafness?

Back 5

1. OAEs--- clicks

Front 6

What are the ssx of kernicterus?

Back 6

1. Deafness--- 2o to damge to cochlear nuclei and colliculi

Front 7

What type of deafness is associated with kernicterus?

Back 7

1. High-frequency

Front 8

What is the indication or an exchange transfusion in kernicterus?

Back 8

1. Serum bilirubin> 20

Front 9

What are autosomal dominant loci associated with non-syndromic hearing loss? Recessive? X-linked?

Back 9

1. DFNA

2. Recessive--- DFNB

3. X-linked---- DFN

Front 10

What makes up a connexon?

Back 10

1. 6 connexins

Front 11

What is the MCC of non-syndromic genetic hearing loss?

Back 11

1. DFNB1 locus--- GJB2 mutation for connexin 26***

Front 12

What is the MCC of autosomal dominant hearing loss?

Back 12

1. DFNA6/14-- WFS1

Front 13

What is the MCC of x-linked non-syndromic deafness?

Back 13

1. DFN3 POU3F4 mutation

2. Perilymphatic gusher

Front 14

What is Alport syndrome?

Back 14

1. Affects collagen of basement membranes of kidneys and inner ear

2. Hearing loss may not become clinically evident until second decade of life

Front 15

What is pendred syndrome?

Back 15

1. Thyrid goiter

2. Profound sensorineural hearing loss

3. Mondini aplasia-- incomplete turn in cochlea

Front 16

With what is hearing loss associated in Pendred syndrome?

Back 16

1. Abnormal iodine metabolism--- euthyroid goiter

Front 17

How do you dx Pendred syndrome?

Back 17

1. Perchlorate discharge test--- abnormal organification of nonorganic iodine

Front 18

What is the MCC of autosomal recessive syndromic hearing loss?

Back 18

1. Usher syndrome

Front 19

What are the ssx of Usher syndrome?

Back 19

1. Sensorineural hearing loss

2. Retinitis pigmentosa

Front 20

What are the ssx of Waardenburg syndrome?

Back 20

1. White forelock

2. Heterchromia irides

3. Premature graying

4. Hearing impairment

5. Synophrys

Front 21

What is the MCC of AD form of deafness?

Back 21

1. Waardenburg syndrome

Front 22

How many turns in the cochlea?

Back 22

1. 2.5-2.75

Front 23

What are the ssx of Michel aplasia?

Back 23

1. Complete agencies of the petrol portion of the temporal bone

2. No inner ear structures--- anacusis

3. NO cochlear implants

Front 24

When is Michel aplasia caused?

Back 24

1. Prior to end of 3rd gestational week

Front 25

Whata re the ssx of Mondini aplasia?

Back 25

1. Only basal coil of cochlea can be identified

2. Endolymphatic duct enlarged

3. Asymmetric, but affects both ears

4. Deafness to normal hearing

Front 26

What are the ssx of Scheibe aplasia?

Back 26

1. Bony labyrinth and superior portion of membranous labyrinth are normal

2. Organ of Corti is poorly differentiated

3. Deformed tectorial membrane

4. Collapsed Reeissner's membrane

Front 27

What is the MCC of inner ear aplasia?

Back 27

1. Scheibe aplasia

Front 28

How do you tx Scheibe aplasia?

Back 28

1. Conventional amplification

2. Rehab intervention

Front 29

How do you tx Mondini aplasia?

Back 29

1. Cochlear implant

Front 30

What are the ssx of Alexander aplasia?

Back 30

1. Cochlear duct differentiation at level of basal coil is limited

2. High-frequency hearing loss

Front 31

How do you tx Alexander aplasia?

Back 31

1. Amplification

Front 32

What are the ssx of enlarged vestibular aqueduct syndrome?

Back 32

1. SNHL, bilateral and progressive

2. Vertigo/incoordination

3. Vestibular aqueduct measuring 1.5 mm or greater

Front 33

What causes enlarged vestibular aqueduct syndrome?

Back 33

1. Hydrodynamic changes

2. Labyrinthine membrane disruption

Front 34

How do you tx enlarged vestibular aqueduct syndrome?

Back 34

1. Conservative--- avoid head trauma/contact sports

Front 35

What is the imaging of choice in unilateral hearing loss?

Back 35

1. HRCT

Front 36

What is the highest yield assay in bilateral hearing loss?

Back 36

1. GJB2

Front 37

What are the ssx of otosclerosis?

Back 37

1. Hardening of spongy bone near anterior oval window/antefenestrum

2. Progressive conductive or mixed hearing loss

Front 38

How do you dx otosclerosis?

Back 38

1. Type A tympanogram

2. Schwartze's sign-- promontory hyperemia

Front 39

How do you tx otosclerosis?

Back 39

1. Observation

2. Hearing aid

3. Stapedectomy

Front 40

What are the MCC of ossicular discontinuity?

Back 40

1. Trauma

2. Chronic OM

3. Eustachian tube dysfunction

4. Previous surgery

Front 41

How do you dx ossicular discontinuity?

Back 41

1. Hyperobile TM and malleus

2. Conductive hearing loss with Ad tympanogram

Front 42

How do you tx ossicular discontinuity?

Back 42

1. Tympanoplasty with ossicular chain reconstruction

Front 43

What causes congenital cholesteatoma?

Back 43

1. Epithelial cells in middle ear adjure to malleus and posterior mesotympanum

Front 44

What are the ssx of congenital cholesteatoma in 2-6 years of age?

Back 44

1. TM white mass

Front 45

What are the ssx of congenital cholesteatoma in 4-12 y/o?

Back 45

1. White mass

2. White TM

Front 46

What are the ssx of congenital cholesteatoma in 12-30 y/o?

Back 46

1. White mass

2. Hearing loss

3. Vertigo

Front 47

How do you tx What are the ssx of congenital cholesteatoma?

Back 47

1. Surgery

Front 48

What is acquired cholesteatoma?

Back 48

1. Middle ear epithelium and teratoma formation with secondary enzymatic erosion

2. Usually through TM perforation ro epitympanic restriction pocket

Front 49

How do you tx acquired cholesteatoma?

Back 49

1. Surgery

Front 50

What are the ssx of Meniere's disease?

Back 50

1. Aural fullness

2. Roaring tinnitus

3. Fluctuating hearing loss

4. Episodic vertigo

Front 51

What is Lermoyez syndrome?

Back 51

1. Increasing fullness and hearing loss relived with vertigo attack

Front 52

What is Crisis of Tumarkin?

Back 52

1. Otolithic crisis

2. Drop attacks

Front 53

What is Cogan syndrome? Ssx?

Back 53

1. Nonsyphilitic interstitial keratitis

2. Vertigo/bilateral progressive SNHL

3. Bilateral Meniere's disease

Front 54

What are the ssx of idiopathic sudden SNHL?

Back 54

1. Abrupt or rapidly progressing hearin gloss over minutes or days

2. Caused by viral, immune complex, vascular issue

Front 55

What drugs cause ototoxicity?

Back 55

1. Aminoglycosides

2. Macrolides

3. Drugs

4. Salicylates