ENT/ophtho

Question 1

Sensory neural hearing loss (syndromic vs. non)

Answer 1

Syndromic 25% - AD 75%, AR 25%

Non synd 75% - AD 25%, AR 75%

Question 2

most common cause of SNHL (congenital)

Answer 2

#1 CMV: infect 1/100, about 75% of affected have SNHL - can be progressive 
#2 AR, 1/500 - 75% of non-syndromic deafness due to mutation, GJB2 most common

Question 3

cochlear implant indications

Answer 3

<1 year (or younger with meningitis) –> cochlea ossifies and electrode won’t fit after
profound bilateral SNHL
no medical contraindication
high motivation from entire family
enrolment in program to emphasize auditory skills
results are age dependent (MUST BE <2 years)

Question 4

Neonate hearing screen indications

Answer 4

Fam Hx SNHL
TORCH infection
craniofacial anomalies, esp involving pinna or ear canal
BWt <1500g
hyperbili requiring exchange transfusion
ototoxic meds (aminoglycosides, loop diuretics)
bacterial meningitis
apgar 0-4 at 1 min or 0-6 at 5 min
mechanical ventilation > 5 days
findings associated with a known syndrome

Question 5

hearing screen in infants (29 d-2 yrs)

Answer 5

parent/caregiver concern
bacterial meningitis
other acquired infection (lye, EBV, H flu, lassa virus, measles, enterovirus, malaria, VZV)
head trauma with LOC or skull #
ototoxic meds/chemo meds
recurrent or persistent OM with effusion > 3months

Question 6

causes of delayed onset SNHL

Answer 6

family history of childhood hearing loss
in utero infection (TORCH)
NF 2 (acoustic neuroma)

Question 7

when to refer for hearing assessment (by age)

Answer 7

12 mo: no differentiated babbling or vocal imitation
18 mo: no use of single words
24 mo: single word vocab <10 words
30 mo: <100 words, no 2 word combos, unintelligible
36 mo: <200 words, no use of telegraphic sentences, <50% intel
48 mo: <600 words, no simple sentences, <80% clear

Question 8

waardenburg syndrome

Answer 8

deafness associated with pigmentary anomalies and defects in neural crest-derived tissues (white forelock, heterochromia)
prominent nasal root, hypertrichosis of medial part of eyebrow

Question 9

heterochromia

Answer 9

irises of 2 different colours, or a portion of the iris is different from remainder. Can be:
AD, part of waardenburg syndrome

Question 10

laryngomalacia vs. tracheomalacia and red flags

Answer 10

laryng: inspiratory, common, starts first few weeks, outgrow, usually fine feeding and growing
tracheo: expiratory component, collapse during exp with increased air flow (crying, feeding, coughing etc), can have feeding difficulties, FTT

Red flags: Severity, Progression, Eating/feeding, Cyanosis, Sleep, Radiology (SPECS-R)

Question 11

tracheomalacia 3 types

Answer 11

type 1: congenital/intrinsic - may be associated with TEF or EA
type 2: extrinsic defects (e.g. vascular ring)
type 3: prolonged intubation, chronic tracheal infection, inflammatory conditions (e.g. relapsing polychondritis)

Question 12

most common congenital neck mass

Answer 12

thyroglossal duct cysts

Question 13

thyroglossal duct cysts

Answer 13

most common cong neck mass, 3/4 midline, moves with up/down with tongue protrusion/swallowing 
surgical removal (after resolution of any infection)

Question 14

epidermal and sebaceous cyst

Answer 14

round, mobile, smooth surfaced
slow growing 5mm to 5 cm
may see pore in center of lesion

Question 15

branchial cleft cyst

Answer 15

lateral neck mass
2nd brnachial cleft cyst most common at border of SCM
2nd branchial fistula: associated with syndromes
1st branchial cyst: recurrent parotid swelling

Question 16

cystic hygroma

Answer 16

congenital lymphatic malformation (lymph vessel fails to connect and drain), large multiloculated cyst
mass transilluminates, mal fluctuate, Sx excision (risk of airway comp)

Question 17

ranula

Answer 17

large soft, mucous containing swelling in the floor of mouth, occurs at any age
if large can present as neck mass if they extend through the melohyhoid musc
cyst should be excised

Question 18

common head and neck tumors

Answer 18

1. lymphoma/leukemia (48%)
2. rhabdomyosaarcoma (most common soft tissue malignancy of head/neck)
3. CNS - neuroblastoma
4. teratoma
5. thyroid: familial (MEN II, papillary), if familial the prophylactic throidectomy by age 5

Question 19

Ramsay Hunt syndrome

Answer 19

VSV oticus - acute peripheral facial neuropathy associated with erythematous vesicular rash of the skin of the ear canal, auricle
50% don’t recover facial nerve function completely
treat: antivirals and steroids (both systemic)

Question 20

Sinus development

Answer 20

born with ethmoid
maxillary present at birth but pneumatized at 4 yrs
sphenoid @5yrs
Frontal bigin at 7-8 years and complete by teens

Question 21

Sinusitis

Answer 21

fever, purulent nasal discharge >10days, periorbital tenderness, halitosis
treat with same abc for OM, may need longer course (3 weeks) + decongestant/saline spray/stroid spray

Question 22

sinusitis complications

Answer 22

meningitis
abscess (frontal, epidural, brain)
periorbital cellulitis
orbital cellulitis 
cavernous sinus thrombosis
blindness

Question 23

allergic fungal sinusitis

Answer 23

10-15% chronic rhinosinusitis in adolescents (usually bipolar is or curvularia)

• production of eosinophili containing noninvasive fungal hyphae
• nasal polyposis
• unilateral sinus disease
• immunocompetence
• IgE to fungi with skin testing of serum IgE

treat: oral steroids after surgical debridement

Question 24

chronic sinusitis predisposing factors

Answer 24

- adenoidal hypertrophy
allergic fungal sinusitis 
allergic rhinitis 
anatomic 
chronic bacterial 
CF, PCD
humoral immunodeficiency 
sarcoid
Wegeners granulomatosis (associated with nasal polyposis)