Ear

Question 1

What is diagnostic rate from genetic testing for those with congenital deafness?

Answer 1

53.6%

CT 30%
Mri 26%
Eye exam 8%
Renal us 4%
ECG 1%

Question 2

Most common gene associated with snhl

Answer 2

Gap junction beta 2 (GJB2) gene at DFNB1A locus

— accounts for 50% of all auto recessive snhl and up to 20% of all cong HL

Question 3

How does gJB2 snhl generally present?

Answer 3

Cong, bilateral, mild to profound

Question 4

What does GJB2 do?

Answer 4

Encodes the gap junction protein connexin 26- a component of the gap junction potassium recycling pathway in cochlea

Question 5

Most common cause deaf-blindness

Answer 5

Ushers syndrome

Question 6

In patients with cong HL what is prevalence of vestibular impairment

Answer 6

54%

Question 7

What is risk to vestibular function on side of cochlear implant for SSD

Answer 7

10% total loss
Change in vestibular responses in 40%
No change 50%
In case of inner ear malformation prevalence of complete loss of vest function after implantation increases to 35%

Question 8

What is risk of vestibular impairment in profound cong HL pts undergoing CI

Answer 8

50% normal
20% complete loss
22% asymmetrical vI
7% symmetrical vi

Question 9

Imaging abnormalities in HL

Answer 9

44% of pts with normal CT had auditory nerve abnormality.

Most common finding in Ped SSD:
Cochlear nerve deficiency- 50%
Cochlear dysplasia 40%
Enlarged vest aqueduct 26%

Question 10

Syndromes with associated hearing loss- autosomal dominant

Answer 10

Noonan syndrome
Treacher Collins
bOR
Stickler

Question 11

Vaccine recs for CI

Answer 11

Prevnar prior to 2 years of age

Question 12

Stapes fixation surgery in kids - outcome is poorer if cause is

Answer 12

Tympanosclerosis

Question 13

Which canal is affected in bppv?

Answer 13

Posterior- 80%

Question 14

Describe vestibular neuonitis

Answer 14

Sup vestibular nerve
Hsv1? 
Resolves in a few days. 
Be active to speed compensation
Mri if deficits
Less than 5% of dizzy pts.