Kj Lee - Set #5

Front 1

What kind of EMG potentials are seen in regeneration of the facial nerve?

Back 1

Polyphasic potentials

Front 2

What are indications for imaging the facial nerve?

Back 2

"Progression of palsy over 3 weeks, recurrent palsy, facial hyperkinesia, development of associated cranial neuropathies (think cancer)"

Front 3

What are causes of acute facial palsy?

Back 3

"Bells (70%), Herpes Zoster (Ramsay Hunt) - 15%"

Front 4

What is the onset of Bell's palsy?

Back 4

Rapid - <48hours

Front 5

What nerves can be affected with Bell's Palsy?

Back 5

V to X

Front 6

How can Ramsay Hunt be differentiated from Bell's palsy?

Back 6

1. Cutaneous vesicles of EAC and conchal bowl 2. Otalgia 3. Higher incidence of cochlear and vestibular disturbances

Front 7

Where is the site of injury in acute facial paralysis from Bell's palsy/Ramsay Hunt?

Back 7

Meatal foramen - just proximal to the labyrinthine portion of the Facial nerve

Front 8

Which antivial is used for treating ramsay hunt?

Back 8

Valacyclovir - believed to be more effective than acyclovir

Front 9

16 causes of facial nerve palsy besides bell's and ramsay hunt

Back 9

Diabetes Mellitis Guillain-Barre Hyperthroidism Lyme disease Melkersson-Rosenthal syndrome Mobius syndrome Mononucleosis Multiple Sclerosis Mumps Myasthenia gravis Neoplasia Otitis Media Perinatal Sarcoidosis Trauma Wegener's Granulomatosis

Front 10

Cause of lyme disease?

Back 10

Borrelia burgdorferi

Front 11

Ratio of unilateral to bilateral facial paralysis with lyme disease?

Back 11

0.125694444

Front 12

Treatment of lyme disease

Back 12

Tetracycline for adults Penicillin for children

Front 13

What is Melkersson - Rosenthal syndrome?

Back 13

Unilateral facial palsy Facial edema Fissured tongue (lingua plicata)"

Front 14

What is Mobius syndrome?

Back 14

bilateral facial and abducens nerve palsies

Front 15

Why is the facial neve particular at risk perinatally?

Back 15

1. No mastoid tip 2. Compression by mothers sacrum or delivery forceps

Front 16

What is Heerfordt disease?

Back 16

uveoparotid fever - associated with sarcoidosis - uveitis, fever, parotidis and CN paralysis

Front 17

What confirms the diagnosis?

Back 17

Elevated ACE levels

Front 18

What are crocodile tears?

Back 18

Cross inervation of the lesser superficial petrosal nerve with the greater superficial petrosal nerve

Front 19

What are the audiometry options for an infant less than 6 months?

Back 19

1. ABR 2. DPOAE 3. behavioral observation audiometry (warble tones)

Front 20

What are the audiometry options for a child 6 months to 3 years?

Back 20

1. ABR 2. DPOAE 3. visual response audiometry (child localizes to an object)

Front 21

What are the audiometry options for a child 3-6 years?

Back 21

conventional play audiometry (child performs an activity every time a sound a heard)

Front 22

What are the audiometry options for a child over 6 years?

Back 22

standard audiometry

Front 23

4 common pathogens for tonsilitis?

Back 23

"S. pyogenes, S. Viridins, S. aureus, H. influenzae"

Front 24

Pathogen for adult tonsils?

Back 24

mixed infections with 3/4 having beta lactamase producing organisms

Front 25

What is a classic finding in S. pyogenes tonsilitis?

Back 25

tonsillar exudate

Front 26

What else besides S. pyogenes causes an abudant exudate with tonsilitis?

Back 26

EBV

Front 27

Treatment of tonsilitis?

Back 27

"Keflex +/- flagyl, Augmentin, Clindamycin"

Front 28

What are the indications for tonsillectomy?

Back 28

"1. 6-7 episodes of acute tonsillitis in one year, 5 episodes per year for 2 years, 3 episodes for 3 years; 2. Peritonsillar abscess; 3. Chronic tonsilitis; 4. OSA; 5. Adenotonsillar hypertrophy with dysphagia, speech abnormalities and occlusive abnormalities"

Front 29

"With caustic ingestion in kids, what causes the most esophageal injuries?"

Back 29

"Bases - sodium, potassium and ammonium hydroxide"

Front 30

What kind of damage do bases causes in esophageal injury?

Back 30

Liquefactive necrosis with full-thickness burns

Front 31

Are acids or bases worse for esophageal injury?

Back 31

Bases

Front 32

What kind of damage do acids cause in esophageal injury?

Back 32

Coagulative necrosis

Front 33

Why don’t you get esophageal full thickness burns with acids?

Back 33

Coagulum limits the penetration of the acid and prevents full thickness burns

Front 34

How do you diagnose caustic ingestion?

Back 34

"1. Evaluate chin, lips, tongue and palate for burn evidence 2. CXR for free air 3. Esophagoscopy within 24-48 hours 4. Esophagram in kids presenting after 48hours"

Front 35

Treatment of caustic ingestions?

Back 35

"Steroids, antibiotics"

Front 36

What are two clinical tests of ciliary function?

Back 36

methelene blue and saccharin

Front 37

What is Kartagener syndrome?

Back 37

"Recurrent sinusitis, bronchiectasis and situs inversus - lack dynein side arms on A-tubules"

Front 38

What is the inheritance of cleft lip/palate?

Back 38

Multifactorial inheritance and etiology

Front 39

What are risk factors for cleft lip/palate?

Back 39

"1. Single gene transmission 2. Chromosome aberrations 3. Teratogens (ETOH, thalidomide, vit A) 4. Environmental (amniotic band syndrome, maternal diabetes"

Front 40

When does a family have the highest risk for cleft lip/palate?

Back 40

When one child and one parent have cleft (18%)

Front 41

Describe the nasal ala and caudal septal abnormality in cleft lip

Back 41

Nasal ala on affected side is displaced inferolaterally; Caudal septum displaced to contralateral side

Front 42

Describe the TVP and LVP in cleft palate

Back 42

"Levator Veli Palatini, which normally forms a sling across the palate is oriented parallel to cleft; Tensor Veli Palatini runs in a more anterior posterior direction resulting in ETD and need for tubes"

Front 43

When do you repair a cleft lip?

Back 43

"Rules of 10: 10 weeks, 10 lbs, hemoglobin of 10"

Front 44

What type of flap is used to repair a cleft lip?

Back 44

Millard rotation advancement flap

Front 45

When do you repair a cleft palate?

Back 45

10 to 18 months - when deciduous molars arrive

Front 46

What is the vascular pedicle for cleft palate flaps?

Back 46

Decending palatine

Front 47

"What are ""lengthening"" cleft palate flaps?"

Back 47

V to Y and Furlow Z plasty

Front 48

"What are ""posterior"" cleft palate flaps?"

Back 48

Pharyngeal flap and sphincter pharyngoplasty

Front 49

Inheritance pattern for cystic fibrosis?

Back 49

Autosomal recessive

Front 50

Seven features of cystic fibrosis

Back 50

1. Pancreatic insufficiency; 2. COPD/bronchiectasis/pneumonia; 3. Malabsorption; 4. Cirrhosis of the liver; 5. Nasal polyps/chronic sinusitis; 6. High sweat chloride/salt wasting; 7. Dehydration

Front 51

How do you diagnose cystic fibrosis?

Back 51

sweat chloride > 60 meq/L

Front 52

How do you manage cystic fibrosis polyposis?

Back 52

"steroids, regular sinus irrigation with tobramycin and endoscopic sinus surgery"

Front 53

What palatal muscle has poor tone in Down syndrome?

Back 53

Tensor Veli Palatini

Front 54

What is the most common inner ear abnormality in children with congenital hearing loss?

Back 54

Enlarged vestibular aqueduct

Front 55

What malformations/syndromes is an Enlarged Vestibular Aqueduct associated with?

Back 55

"Mondini malformation, Pendred syndrome, branchio-oto-renal syndrom"

Front 56

What is upper limit of normal for vestibular aqueduct?

Back 56

1.5mm

Front 57

What is the characteristic SNHL seen with enlarged vestibular aqueduct?

Back 57

Progressive SNHL that progresses in a stepwise fashion

Front 58

What anatomic abnormality can cause hearing loss that can worsen after minor trauma?

Back 58

Enlarged vestibular aqueduct

Front 59

What is the treatment for enlarged vestibular aqueduct?

Back 59

"Hearing aids, cochlear implant"

Front 60

What is esophageal atresia and tracheoesophageal fistula associated with?

Back 60

"VACTERL - vertebral, anal, cardiac, tracheoesophageal, renal, limb abnormalities"

Front 61

What are 5 types of esophageal atresia/tracheoesophageal fistula?

Back 61

1. Esophageal atresia with distal TEF (85%); 2. Esophageal atreasia w/o TEF (7%); 3. TEF w/o atresia; 4. Esophageal atresia with proximal and distal TEF; 5. Esophageal atresia with proximal TEF

Front 62

What must you rule out in a child with unilateral wheezing?

Back 62

Foreign body

Front 63

What are signs of a tracheal/bronchial foreign body in children?

Back 63

"stridor/wheezing, cough w/o associated illness, recurrent or migratory pneumonia; acute aphonia"

Front 64

What are signs of an esophageal foreign body in children?

Back 64

"Odynophagia, drooling, vomiting/spitting, airway compromise due to posterior tracheal impingement"

Front 65

Radiographic signs of tracheal/bronchial foreign body?

Back 65

"1. Visualize FB, 2. Atelectasis on affected side, 3. Overinflation due to air trapping"

Front 66

Why do a lateral decubitus when looking for an airway foreign body?

Back 66

evaluates for mediastinal shift - uninvolved side down results in shift from gravity; involved side does not show shift due to air trapping

Front 67

Five levels at which a foreign body is likely to lodge in the esophagus?

Back 67

1. Cricopharyngeus; 2. Thoracic inlet; 3. Level of aortic arch; 4. Tracheal bifurcation; 5. Gastroesophageal junction

Front 68

What does a disc battery contain?

Back 68

"lithium, NaOH, KOH, mercury"

Front 69

What can ingesion of a disc battery cause after an hour?

Back 69

mucosal damage

Front 70

What can ingesion of a disc battery cause after 2-4 hours?

Back 70

damage to muscular layer

Front 71

What can ingesion of a disc battery cause after 8-12 hours?

Back 71

potential perforation

Front 72

What do you do with a disc battery that passed into the stomach?

Back 72

send home and monitor stool; repeat xray in 4-7 days; with larger batteries repeat xray in 48 hours; if still in stomach remove endoscopically

Front 73

What % of children are born with hearing loss?

Back 73

0.001

Front 74

What % of deaf children have normal hearing parents?

Back 74

0.9

Front 75

How long is hearing loss usually delayed in children?

Back 75

2.5 years

Front 76

What % of hereditary hearing loss is non-syndromic?

Back 76

0.7

Front 77

What % of hereditary deafness have recessive transmission?

Back 77

80% (DFNB - deafness gene B)

Front 78

What % of hereditary deafness have dominant transmission?

Back 78

20& (DFNA - deafness gene A)

Front 79

What is the most common cause of hereditary deafness with recessive transmission?

Back 79

Conexin 26 (GJB2) gene

Front 80

What is the typical audiogram for hereditary hearing loss?

Back 80

Cookie bite audiogram

Front 81

What % of hereditary hearing loss is syndromic?

Back 81

15-30%

Front 82

What are autosomal recessive syndromes with hearing loss?

Back 82

JUP Jerville-Lange-Nielson Usher Pendred

Front 83

What are 7 autosomal dominant syndromes with hearing loss?

Back 83

1. Achondroplasia 2. Branchio-oto-renal 3. Crouzon syndrome 4. NF-2; 5. Stickler 6. Treacher-Collins syndrome 7. Waardenburg syndrome

Front 84

Features of Jerville-Lange-Nielsen syndrome

Back 84

"SNHL, prolonged QT with syncopal events and sudden death"

Front 85

Features of Usher syndrome

Back 85

"SNHL, retinitis pigmentosa +/- vestibular symptoms. Most common syndrome to afffect the eyes and ears. Diagnosed with electroretinogram"

Front 86

Most common syndrome to afffect the eyes and ears?

Back 86

Usher syndrome

Front 87

Features of Pendred syndrome?

Back 87

"SNHL, euthyroid goiter. Patient have an abnormal perchlorate uptake test (reduced thyroid activity over time) - associated with mondini malformation and enlarged vestibular aqueduct"

Front 88

What abnormal test is seen in Pendred syndrome?

Back 88

Perchlorate uptake test

Front 89

"What syndrome is assoicated with SNHL, mondini malformation and EVA?"

Back 89

Pendred

Front 90

What is the most common skeletal dysplasia?

Back 90

Achondroplasia

Front 91

What is achondroplasia a disorder of?

Back 91

Endochondral bone formation

Front 92

Features of achondroplasia?

Back 92

"Skeletal dysplasia, Narrow foramen magnum, hydrocephalus, spinal canal stenosis, respiratory infections, apnea, otitis and Conductive Hearing Loss"

Front 93

Features of Branchio-oto-renal syndrome?

Back 93

"Branchail apparatus abnormalities (clefts, cysts, fistulas), preauricular pits, hearing loss (SNHL, CHL or mixed), renal abnormalities"

Front 94

Features of Crouzon syndrome?

Back 94

"CHL, craniosynostosis, maxillary hypoplasia, ocular hypertelorism with exopthalmos, mandibular prognathism"

Front 95

Features of Neurofibromatosis type 2

Back 95

Bilateral acoustic neuromas - candidate for auditory brainstem implants

Front 96

Features of Stickler syndrome:

Back 96

SNHL or mixed flattened facial appearance with Pierre Robin sequence myopia

Front 97

Features of Treacher-Collins syndrome

Back 97

Mandibulofaial dysostosis CHL, SNHL, mixed midface hypoplasia micrognathia malformed ears lower lid coloboma downward slanting eyes