Otology Flashcards
(124 cards)
What type of inheritance is aminoglycoside induced hearing loss?
Mitochondrial mutation
So passed through mom to all of her children
Schiebe aplasia
Membranous aplasia of cochlea and saccule (can’t see on CT scan)
Pendred Syndrome:
Mutation
Characteristics
AR
SNHL, Mondini malformation with enlarged vestibular aqueduct, euthyroid goiter (by age of 10)
SCL26A4 gene mutation for Pendrin - chloride/bicarb exhange protein, iodide exchange too
Most common infectious cause of congenital hearing loss
CMV - Associated with cerebral calcifications, hepatosplenomegaly, jaundice, intellectual disability
What does the inferior vestibular nerve control?
POSTERIOR SSC function + saccule
What does the superior vestibular nerve control?
Lateral and Superior SCC + Utricle
What do aminoglycosides damage?
OUTER hair cells (Amin-O for Outside)!
Medial wall of epitympanum?
Mostly the promontory
MRI findings of cholesterol granuloma
HYPERintense on T1 AND T2
MRI findings of cholesteotomas
HYPOintense T1, HYPERintense T2, diffusion restricted
Where does chorda tympani nerve pass through in the middle ear?
Between manubrium of malleus and long process of incus before exiting through the petrotympanic fissure.
ABR waveforms mnemonic
ECOLI
I+II - 8th nerve
III - Cochlear nucleus/Sup olivary complex
IV - Sup olivary complex
V - Lateral lemniscus
VI, VII - Inferior colliculus
What is Schwartze’s sign?
Red hue seen at the promontory, found in otosclerosis
Characteristics of CENTRAL vertigo etiology
Vertigo that does not extinguish with repeated stimulation, DOWN (vertical) beating nystagmus that does not improve with fixation, NOT associated with hearing loss usually, less severe.
Get MRI!
Most common cause of NON syndromic congenital SNHL?
Connexin 26 mutation, AR
Non functional gap junction protein, product of GJB2 gene
Most common cause of SYNDROMIC congenital SNHL?
Usher syndrome - AR
Usher Type I, Type II and Type III features?
AR
Type I: Congenital profound SNHL, NO vestibular fx (NO)
Type II: Normal vestibular fx, moderate-severe SNHL (YES)
Type III: Like type II but with variable vestibular fx, progressive HL (MAYBE)
Alport syndrome
X linked, mutation of type IV collagen (COL4A3), causes hearing loss and glomerulonephritis, eye lesions
What is directional preponderance and how do you measure it?
It’s the measure of the difference in total eye speeds between slow phases on each side.
Measured with caloric testing data.
What is speech detection threshold?
Level at which patient is aware of speech 50% of the time.
Explain 7 up Coch down!
Sup ant: FN
Sup Post (behind Bill’s bar): SVN
Inf ant: Cochlear nerve
Inf Post: IVN
Transvere crest is between the SVN and IVN
CHARGE Syndrome
Coloboma of eye, heart defects, atresia of nasal choanae, retardation, genital/urinary abnormalities, ear abnormalities (SCC aplasia, deafness)
-Autosomal DOMINANT
Most common cochlear malformation
Incomplete partition type 2 - arrest at 7th week, 1.5 turns of cochlea, associated with EVA, pendred syndrome, predisposition to SNHL
What % of pediatric HL is due to genetic?
50% are genetic
70% of those are NON syndromic
Most common syndromic cause - Usher
Most common NON syndromic cause - Connexin 26



