Ophthalmology / Otology Flashcards
Most common genetic cause for congenital visual impairment (1) and other features (3)
- Leber convenital amaurosis (retinal dystrophy)
- Features
- Visual impairment beginning a 3-4 months > nystagmus and sluggish pupils
- midface hypoplasia
- Cognitive / developmental delay
What does this show?

Optic nerve edema from optic neuritis NOT papilledema (pictured below)

What does this show?

Optic disk edema and splinter hemorrhages 2/2 AION
What does this show?

Optic atrophy (From longstanding MS)
- pale, “shrunken” appearing disc
- pallor extends beyond margins of disc
Location of vertical and horizontal Gaze Centers

Centers for:
Saccaddes
Pursuit
- Saccades
- Contralateral frontal lobe
- Pursuit
- Ipsilateral parietal / occipital
nerve most commonly injured in head trauma
CN IV

Midbrain:
Structures (7)
Locations of Injury (2)


Lesions of the third Nerve Nuclei

- Superior rectus nucleus (Contralateral)
- Levator nucleus (levator palpebrae)
- NOTE: single nuclei controls both eyes
6.
- NOTE: single nuclei controls both eyes

Patient arrives with right CN III nuclear palsy:
how do you tell if the levator nuclei was damaged?
- levator nuclei
- Bilateral ptosis (injured) or NO ptosis at all (not injured)
- Superior rectus nuclei
- Injured = left SR is damaged
- not injured: NO SR damage
Patient arrives with right CN III palsy and bilateral ptosis
what was damaged?
CN III nuclei with invovlement of levator palpebare nucleus
Pons Anatomy (9)


Patient arrives with isolated CN III palsy
What is the significance of also having:
Contralateral hemiparesis? (3)
Contralateral hand tremor? (3)
Cerebellar ataxia? (3)
Contralateral hand tremor AND cerebellar ataxia?
- Contralateral hemiparesis = Weber
- CN III fascicle
- pyramidal tract
- Contralateral hand tremor = benedikt syndrome
- CN III fascicle
- Red nucleus
- Cerebellar ataxia = Nothnagles
- CN III fascicle
- superior cerebellar peduncle
- Both 2 and 3 = claude’s syndrome
Patient arrives with Abducens palsy
what is the significance of also having:
Ipsilateral Facial palsy + Contralateral Hemiparesis (4).
Ipsilateral loss of facial sensation, facial palsy, and horners syndrome?
- VI, VII palsy + CL hemiparesis = Millard-Gubler syndrome
- VI fascicles
- VII fascicles
- Pyramidal tract
- Facial sensation, facial palsy, and horners = Fovile’s syndrome
- CN V
- CN VI nucleus
- CN VII nucleus and sympathetics
Why does blown pupil with CN III raise concerns?
Nerve fibers corresponding to pupillary constriction are in the periphery, and thus are targeted during compressing lesions (like aneurysm)

Features of Dorsal Midbrain syndrome
Vertical gaze palsy
Convergence - retraction nystagmus
lid retraction
Patient comes in with:
Vertical gaze Palsy
lid Retraction
Pulsating in-and-out movements of eyes with vertically moving OKN drum
Where is the lesion?
what are you suspecting as the cause?
what would you not expect to see?
Dorsal midbrain (Dorsal midbrain syndrome)
suspect:
OLD = Stroke
young = Pinealoma
would NOT expect to see pupillary light-near dissociation
Patient unable to look left, but eyes look left when head is passively turned to the right
what does this tell you?
This is a supranuclear lesion due to positive doll’s eye maneuver
(possibly due to frontal lobe stroke)
Horizontal or vertical gaze palsy but positive Doll’s eye in that direction suggets what?
supranuclear lesion
What does this show?
what is damaged?
What two other features will you see?

Bilateral Intranuclear ophthalmoplegia (bilateral limitation of ADDuction)
Damage to medial longitudinal fasciculus
Will also see:
beating nystagmus of adducting eye
vertical skew deviation = vertical diplopia
(#2)

What does this show?
what is the syndrome?

Right horizontal Gaze palsy + Right medial rectus palsy when looking left
One-and-a-half-syndrome

“Painful horners”
Make sure to rule out what?
What should you also see (3)?
Carotid Dissection
Vision loss IL to horners
Headache IL to horners
CL hemiparesis
What is damaged here?
What will worsen the pupillary finding?

Sypathetic innervation to the right eye (horners)
Anisocoria will be more evident in DIM light (sympathetics / Dilation is what is affected)

How would we confirm this diagnosis?
Option 1:
what drug?
what do you see?
Option 2:
what drug?
what do you see?

Option 1: Cocaine
- Affected Pupil will NOT dilate
Aproclonidine
- Affected WILL dilate

Threshold for physiologic anisocoria
<1-2 mm
What is this showing?
What does this suggest?
If this wasn’t present, what would you want to exclude?

Horners syndrome with hypochromia of the iris
congenital horners (also feed into melanocytes of eye)
pediatric acquired horners = r/o pineoblastoma
Woman with this eye finding and “wiggly” pupillary movements.
What other finding would you see?
How would you test?

Light-near dissociation (picture)
Dilute Pilocarpine
Will cause constriction (because it’s hypersensitive)

Woman with this eye finding and “wiggly” pupillary movements.
what is injured?
what are the two most common causes?

Ciliary gangion
“wiggly” = vermiform movements
most common causes:
post-viral
trauma / surgery to eye
What does this show?
What conditions can you see it in (3) ?

Light-Near dissociation
Seen in:
Argyll-Robertson pupil (tertiary syphilis)
Parinaud’s dorsal midbrain syndrome (stroke in adult, pinealoma in children)
Adie’s tonic pupil (post-viral or post-surgery)
(accomodates but does not react)
Label the Following:


Diseases associated with
Unilateral central scotoma
Bilateral central scotoma
Unilateral central scotoma = optic neuritis / MS
Bilateral = toxic / medication
- Ethambutol for tuberculosis
- Thiamine deficiency
- Leber’s hereditary neuropathy
leber’s hereditary neuropathy:
Vision problems are worse with _____
Smoking
Difference between pituitary microadenoma and pituitary macroadenoma and why is it imporant?
Microadenoma = \<10 mm macroadenoma = \>10 mm
> 10 mm and you are more likely to have visual impairment

Patient reports loss of vision only when sketching fine details in sketchbook
what tumor could cause this?
what is it typically associated with?
Tumor in optic chiasm (i.e. Pineal tumor)
typically associated with bitemporal hemianopsia
when convergin, “blind” temporal fields overlap

What should this make you worry about?
Central lesion (bow-tie atrophy)

What is this showing?
what causes this finding?

Junctional scotoma
central scotoma in one eye + suprerotemporal defect in other eye
Lesion to anterior part of chiasm
infranasal fibers move up to opposite optic nerve before decussating (Von Wilbran’s knee)

What is this showing?
what is it suggestive of?
what should NOT be affected?

Homonimous field defect with weird wedge
damage to lateral geniculate nucleus lesion
pupillary light reflex should NOT be affected

What is this showing?
What does this suggest?

“pie in the sky” defect
right temporal lobe defect
What oculomotor finding are suggestive of parietal lobe lesion?
Defects in OKN drum
(persuit / saccade)
Label the following and the associated Visual fields


Match the following with the visual field defect


What is this?
features (2)
Vision?
What is it seen in (3)
Epidemiology note

Morning glory Disk
Funnel-shaped staphyloma
radial vessels
Poor vision (hand motion)
Seen in:
serous retinal detachment
basal encephalocele
signifies vascular development failure
What causes this?

Incomplete closure of embryonic fissure
Also associated with
visual field defects
relative Afferent pupillary defect RAPD
What non-neurologic evaluation should this person get?

(optic nerve hypoplasia)
Endocrine evaluation (due to midline defects)
Conditions associated with this finding

(optic nerve hypoplasia)
Associated with:
midline hemispheric defects
maternal DM, drug, EtOH
Retina Versus Visual field
Nasal retina
Temporal retina
Nasal retina = Temporal visual field
Temporal retina = nasal visual field
What tests could you order to confirm this finding?

calcified nodules (drusen)
CT

What can cause this?
Bilateral lateral compression of chiasm
such as calcivfied internal carotid arteries
Function of inferior oblique
inferior rectus
Inferior oblique: Elevates and Extorts
inferior rectus: depresses and extorts
“inferiors extort”
what structure is responsible for circadian rhythms?
Suprachiasmic nucleus (hypothalamus)
Terms:
internal ophthalmoplegia
VS
external ophthalmoplegia
Internal ophthalmoplegia: loss of parasympathetic innervation to sphincter papillae (results in miadriasis)
External ophthalmoplegia = paralysis of external ocular muscles
Cranial nerve Palsies which spare the pupil (3)
“Inside the I, it is DiM
- *I_ncomplete _I**schemic lesions
- *D**iabetes, Myasthenia
Ciliary nerves responsible for:
pupil Dilation
pupil constriction
Pupil dilation: Long ciliary nerve
Pupillary constriction: Short ciliary nerve
Emboli to the Eye and what they signify:
Small, shiny emboli to the eye lodged in retinal arteriole
Gray, globular emboli completely occluding retina
Long, gray emboli
- Hollenhorst plaque:
- signififies prior ischemic damage to eye (treating upstream vessel may prevent further embolization injury)
- Calcific emboli: disease heart valves
- Platelit-fibrin emboli: endogenous
Baby comes in with Infantile spasms and Agenesis of the corpus callosum
Why is this in the “ophthalmology” slide deck?
Aicardi syndrome can have chorioretinal Lacunae, look for it.
AICardi
Ageneis of corpus callosum
Infantile spasms
Chorioretinal Lacunae
Lens Deviation:
upwards deviation, think _____
downwards, think ____
Upwards = marfans
Downwards = Homocystinuria
Dancing eyes, dancing feet
What do you look for in child (2)
what do you look for in adult (4)
Child
- Urine catecholimines due to neuroblastoma
Adult
- Anti-Ri due to cancer of (anti-R_epublican _BL_o_G)
- Breast
- Lung
- Gynecologic
Slow vertical saccades + square-wave jerks suggest what?
Progressive supranuclear palsy
Woman with MS-like lesions but bilateral retinal artery occlusions:
What do you think about?
what are it’s features?
Susac’s syndrome
Bilateral retinal artery occlusions
Encephalopathy
sensorineural hearing loss
Mnemonic for Whipple’s disease
My Super Duodenum Smells Like We Can’t Digest Nothing
Classic triad:
- Myoclonus
- supranuclear palsy
- dementia
Other features
- Steatorrhea
- Lymphadenopathy
- Weight loss
- Convergence/Divergence Nystagmus (pendular nystagmus with simultaneous jaw movements; whipple’s eyes)
Caused by trophermya whippelii (PAS +)
Hallucination roundup:
- Formed hallucinations
- unformed visual hallucinations
- real objects look malformed, smaller, or larger
- Hypnogogic / hypnopompic
- pendular hallucinations
- Formed hallucinations = Temporal lobe epilepsy
- unformed visual hallucinations = occipital lobe epilepsy
- Malformed (metamorphosia), smaller (micropsia), larger (macropsia) = migraine
- Hypnogogic / hypnopompic = narcolepsy
- Pendular = midbrain injury
Thalalamic nuclei for vision and hearing
L = Light (lateral geniculate nucleus)
M = Music (Medial geniculate nucleus)
Dix Hallpike Maneuver:
How do you do it?
What constitutes a “positive” test?
How to do it:
- Start in reclining position with head back roughly 20 degrees
- Tilt head 45 degrees and bring patient up from reclining to sitting position
Positive result:
- Upward nystagmus when patient is reclining
- downward nystagmus when sitting up
45- year old man complains of Vertigo, tinnitus and “fullness” in ears
What does he have?
what else would you expect (2)?
What 3 things should he avoid?
Menier’s disease u
- Features
- Vertigo with “fullness” in ears
- Tinnitus
- Low-frequency hearing loss (compared to high frequency associated with loud sound exposure)
- +/- horizontal nystagmus
- Risk factors
- Excessive salt intake
- head trauma
- Cigarette / EtoH use
Infant presents with sensorineural hearing loss:
- Name 4 congential infections that could cause this
- If also has retinitis pigmentosa, what could this be?
- If no RP but instead Goiter is present, what could this be?
- Congential infections causing sensorineural hearing loss
- Toxoplasmosis
- CMV
- Rubella
- HSV
- Sensorineural hearing loss + RP suggests usher syndrome
- Sensorineural hearing loss + goiter suggest Pendred syndrome.
Older man comes in with unsteady gait, incontinence, dementia, and tinnitus.
What does the tinnitus sound like?
Venous Hum (pulsatile tinnitus with NPH)
What common prophylactic drug can result in subjective tinnitus
Aspirin
Differe AION versus NAION
AION = more likely to have superior altitudinal defects (also seen with glaucoma)
NAION more likely to have inferior
Label the following and important elements of each

- Red = cochlear promontory
- site of glomus tympanic paragangliomas
- Purple = tympanic segment of facial nerve
- Blue = Tegmen tympani
- separates cranial and tympanic caveties
- Orange = Malleus
- one of the ossicles of middle ear
- Yellow = Scutum
- first bony structure to be eroded by enlarging cholesteatoma
Patient presents with peripheral vertigo
How should their eyes move?
Horizontal / rotary nystagmus directed with fast phase away from affected size
“horners but with intact sweating”
What study should he get?
MRA to check for carotid dissection
(“post-ganglionic horners”)
Child is unable to hear soft voices in quiet background, but can hear loud voices in a noisy background?
What does this suggest?
Conductive hearing loss
(opposite would be sensorineural)
Patient with Vertigo only during severe storms and conductive hearing loss
What is this called?
What does he have?
How would you confirm diagnosis?
Superior semicircular canal dehiscence syndrome
Tullio’s phenomenon (sound-induced vertigo)
Confirm with Hennebert sign (pressure-induced vertigo)
How would you treat this patient?

Iodine / steroids / immunosuppression / referral to endocrinology
(Graves disease, note sparing of lateral rectus, this is characteristic of Graves)
Patient pesents with Pigmentary retinopathy. What medications would you worry about if patient has:
Autoimmune conditions (2)
schizophrenia (2)
HIV (1)
Cancer (2)
- Autoimmune conditions
- Chloroquine
- Hydroxychloroquine
- Schizophrenia
- Thioridazine
- chlorpromazine
- HIV
- Ritonavir
- Cancer
- Cisplatin
- BCNU
Best test to localize Horners syndrome and findings
Hydroxyamphetamine
Dilates normally after administration: 1st or 2nd order motor neuron disease
Patient presents with bilateral fluctuating hearing loss, episodic vertigo, and interstitital keratitis.
How do you treat?
Cogan’s syndrome
Treat with high dose steroids followed by immunosuppression if unresolved
Patient presents with gaze-evoked and vestibular nystagmus
What is this called?
What can this indicate?
Bruns nystagmus
Larege cerebellopontine angle tumor (such as vestibular schwannoma)
Factors associated with poorer prognosis in PRES
Corpos callosum involvent
Define Saccadomania (2)
- Flutter - random horizonal eye movements (MS)
- Opsoclonus”: random, chaotic, multiplanar eye movements
Brain regions and associated wye movements
Saccades :
smooth pursuit:
Additional note for optokinetic drumq
Saccades: contralateral frontal lobe
pursuit: IL pareito/occipital lobe (ill-defined)
Note on optokinetic drum: Allows you to assess one side of brain for each (smooth pursuit followed by saccade in other direction)
Patient presents with opsoclonus. What do you think of it patient is…
A child
An adult
Child: neuroblastoma
Adult: paraneoplastic
What regions are responsible for:
Saccades
Pursuit
Saccades (FAST): contralateral frontal lobe
Pursuit (slow): ipsilateral parietooccipital region (ill-defined)

Patient presents with optokinetic nystagmus. what has been damaged?
parietal lobe lesion ipsilateral to where smooth persuit is lost.
Reaction of eye drops in horners
Aproclonidine
Cocaine
1% hydroxyamphetamine
- Aproclonidine
- affected pupil dilates, affected lid elevates
- Cocaine
- unaffected eye will dilate
- Hydroxyamphetamine
- dilation if affected eye if 1st or 2nd order neuron