Infection section
Name that syndrome
Eponym party
OH BABY (peds)
100

Meningitis after cochlear implantation is most commonly caused by what organism?

S. pneumoniae

Kids 2-4 yo: prevnar series; >5 yo: pneumovax 

100

Temporal bone anomalies, syndactyly, craniosynostosis, midface anomalies (hypertelorim, high arch palate, proptosis)


Apert syndrome

Causes of hearing loss: recurrent middle ear effusions (partially due to ETD), stapes fixation 

100

Increased vascularity of the promontory in otosclerosis


Schwartz sign

100

Most common cause of genetic hearing loss

Connexin 26 mutation

(Autosomal recessive)

200

4 year old presents with recurrent middle ear effusions and TM perforations despite appropriate otic drops and PO abx. Stain of otorrhea shown below. What is the next best test?


PPD or quantiferon 

(concern for mycobacterial infection)

200

Myopia and cataracts, hypermobile joints with early arthritis, cleft palate, and hearing loss

Stickler syndrome 

Autosomal dominant: COL2A1, COL11A1, and COL11A

Autosomal recessive: COL9A1

200

Triad of diplopia, retro-orbital pain, and otorrhea

Gradenigo's syndrome (petrous apicitis)

200

Name one syndrome associated with congenital aural atresia

Treacher Collins, Crouzon, Goldenhar

  

300

What other physical exam finding may this patient present with?


Facial paralysis

Ramsey Hunt is the second most common cause of acquired facial paralysis/paresis; 40% can have SNHL

300

Retinitis pigmentosa and congenital deafness

Usher syndrome (autosomal recessive)

Hearing loss is due to atrophy of the organ of Corti; most likely of all congenital deafness to manifest with vestibular symptoms

300

The anatomic space bounded by pars flaccida and neck of the malleus and enclosed by the lateral malleolar fold

Prussak’s space (most common site of an acquired epitympanic cholesteatoma)


300

An infant fails their newborn hearing screen and also has cataracts and cardiac anomalies. What does this constellation of findings suggest?

Congenital rubella

400

(1) Abnormal central incisors (2) Interstitial keratitis of the eye (3) Deafness

This triad is associated with what infection?


Syphilis (Hutchinson’s triad)

400

Sensorineural hearing loss and enlarged thyroid (euthyroid goiter)

Pendred syndrome (autosomal recessive)

enlarged vestibular aqueduct, Mondini cochlear hypoplasia, and often atrophy of organ of corti

400

Tympanic branch of the glossopharyngeal nerve (CN IX)

Jacobson nerve (neuroendocrine cells from here give rise to glomus tympanicum)


400

The most severe congenital malformation with complete absence of the bony and membranous labyrinth

Michel aplasia

Associated with thalidomide exposure


500

Name the imaging modalities to (1) confirm and (2) clinically follow malignant otitis externa?

(1) CT and technetium-99 scans (high sensitivity for osteomyelitis)

(2) CT and gallium-67 scans (uptake decreases as infection is cleared)

500

Profound sensorineural hearing loss and prolonged QTc with risk of life-threatening arrhythmias

Jervell and Lange-Nielsen Syndrome (autosomal recessive)

500

Blanching of this lesion with pneumatic otoscopy


Brown's sign: blanches glomus tympanicum, most common tumor of the middle ear

Most common symptom is pulsatile tinnitus. May also cause hearing loss (conductive > sensorineural).

500

What is the most common middle ear abnormality associated with aural atresia?

Fusion of the malleus and incus