Pediatric Examination and Board Review (86 page)

15.
Treacher-Collins                   (B) sensorineural

16.
Pierre-Robin

17.
Hunter-Hurler

18.
Alport

19.
Crouzon

20.
Which of the following does not indicate a need for hearing screening if newborn hearing screening was performed?

(A) recognition of developmental delay

(B) meningitis

(C) post-natal exposure to cytomegalovirus (CMV)

(D) otitis media with effusion for 4 months

(E) skull fracture

ANSWERS

1.
(E)
Both otitis externa and otitis media with a perforated TM can cause purulent discharge and cannot always be differentiated by history alone. The term
otitis media with effusion
is used to refer to an effusion behind the TM without signs of acute inflammation. The differential diagnosis for ear discharge also must include foreign body in the external auditory canal, chronic suppurative otitis, and acquired cholesteatoma. Tuberculous otitis media should also be considered but is unlikely a process of 2 days.

2.
(A)
This scenario depicts otitis externa, which presents with the main complaint of ear pain, especially with movement of the pinna or tragus, and thick white discharge from the external auditory canal.
P. aeruginosa
is the most common isolate when the onset of symptoms follows swimming (80%), but
S aureus
,
Streptococcus pyogenes
(group A Streptococcus), gram negatives such as
Proteus
or
Klebsiella
, and fungi such as
Candida
or
Aspergillus
are also possibilities. The last is rare and presents with a more insidious ear discharge that is white and fluffy, sometimes with small black spores visible along the ear canal.

3.
(E)
Anything that causes trauma to the external canal (cotton swabs, foreign body) or changes the microbial environment (swimming, and thus the designation “swimmer’s ear” for otitis externa) predisposes to bacterial infection of the external canal. Otitis media can cause otitis externa when the TM ruptures and secondarily infects the ear canal.

4.
(D)
Topical therapy is the first line treatment for uncomplicated otitis externa. Oral antibiotics are reserved for those with chronic otitis externa, concomitant otitis media, or a patient with an underlying immunodeficiency. Topical antibiotics should have activity against
P. aeruginosa
and
S. aureus
. Acidifying solutions such as acetic or boric acid lower the pH, thereby making the environment less favorable to the causative organisms. Topical steroids may decrease the associated inflammation and pruritus with otitis externa but are not necessary for a mild to moderate otitis externa.

5.
(E)
Both otitis media and otitis externa can present with bloody otic discharge. Likewise, rupture of a bulla when bullous myringitis is present may produce bloody discharge. Langerhans cell histiocytosis is a spectrum of disorders, the most systemic of which is Letterer-Siwe disease, often associated with chronic otitis externa.

6.
(B)
The most important condition to rule out in the presence of clear otorrhea is a leakage of cerebrospinal fluid (CSF). Measurement of glucose (CSF glucose concentration is 45-70 mg/100 mL), protein (<200 mg/mL), and beta
₂
-transferrin levels may be of assistance because very low concentrations of these should be present in inflammatory fluid. In addition, imaging of the area is warranted to rule out congenital or malignant causes of CSF leak.

7.
(E)
Infections of the inner (media) or outer (externa) ear typically cause purulent discharge or otorrhea. In bullous myringitis, the middle ear is filled with clear fluid, so if the bulla ruptures, clear otorrhea is possible. In addition, a CSF leak through a perforated TM produces clear otorrhea.

8.
(C)
Because the main diagnostic possibilities include AOM with TM perforation, otitis externa, and the presence of a foreign body, and because the discharge is prohibiting you from making a diagnosis, attempting gentle water lavage is the best next step. The foul-smelling discharge should alert you to the possibility of a foreign body, which should be removed in as timely a manner as possible to prevent secondary infection and puncture of the TM. Sometimes small foreign bodies can be removed simply by gentle warm water lavage.

9.
(B)
Of the foreign bodies that 3-year-olds place in their ear, batteries are the most urgent. As they degrade, they leak an ototoxic fluid that can be damaging to the ear canal. They must be removed as promptly as possible. Both topical lidocaine and topical antibiotics are often used for treatment. Lidocaine can numb the ear canal for removal of the foreign body, and antibiotics may be useful for infection of the canal associated with the foreign body. Neither should be prescribed until the procedure for battery removal is imminent.

10.
(D)
Pure tone audiometry is most appropriate for evaluation of hearing in children older than 4-5 years. Although this child may have otitis media with effusion, the larger problem is that even 3 months ago she failed a screening hearing test, suggesting there may be a more chronic problem involved. Automated brainstem response (ABR) and otoacoustic emission (OAE) tests depend less on patient cooperation and are therefore more useful in infants. Tympanometry may support your finding of middle ear effusion but will not assess hearing.

11.
(B)

12.
(B)
A middle ear effusion is the most common cause of conductive hearing loss. In addition to those listed above, other potential causes include congenital anomalies of the external auditory canal, tympanosclerosis, trauma, and masses in the inner ear (histiocytosis, rhabdomyosarcoma).

13.
(C)
Sensorineural hearing loss (involves inner ear, cochlea or auditory nerve) is more often caused by systemic infections as opposed to the localized middle or outer ear infections that lead to conductive hearing loss. The incidence of congenital CMV in the United States ranges from 0.2% to 2.0% of newborns. Of those, 10% have clinical manifestations at birth. Seventy-five percent of symptomatic infants and 5-15% of asymptomatic infants have sensorineural hearing loss that often may be progressive and present at 4-5 years old.

14.
(B)
Down syndrome is one of the most common and well-studied trisomies. The incidence is 1 in 700 births and increases in women older than 35 years. Major associations include congenital heart disease, gastrointestinal (GI) disorders (most commonly celiac disease), thyroid disease, leukemia, and mental retardation. Associated anomalies are numerous and include delayed puberty, short stature, sensorineural hearing loss, rapid aging, and sterility in males.

15.
(A)
Treacher-Collins, or mandibulofacial dysostosis, is an autosomal dominant syndrome characterized by hypoplasia of the facial bones. The clinical findings vary but often include microtia, conductive hearing loss, cleft palate, colobomas, and facial deformities.

16.
(A)
Pierre-Robin syndrome is an autosomal recessive disorder characterized by severe mandibular hypoplasia, leading to cleft palate, conductive hearing loss, congenital heart disease, and other oral deformities (see
Figure 53-1
).

FIGURE 53-1.
A child with Pierre-Robin syndrome. Note the extremely retruded chin in this child, who is being prepared for surgical tongue-lip adhesion. (Reproduced, with permission, from Lalwani AK. Current Diagnosis & Treatment in Otolaryngology: Head & Neck Surgery, 2nd ed. New York: McGraw-Hill; 2008: Fig. 19-7.)

17.
(B)
Hunter-Hurler syndrome is an autosomal recessive lysosomal disorder characterized by dwarfism, hunchback, coarse facies, mental retardation, sensorineural hearing loss, and cardiac abnormalities. Affected children may appear normal until 1 year of age.

18.
(B)
Alport syndrome is an X-linked dominant or recessive disease that causes hearing sensorineural loss, hematuria, and renal failure.

19.
(A)
In general, syndromes that involve craniofacial abnormalities are more likely to display conductive hearing loss. Both Crouzon (1/25,000 births) and Apert (1/100,000 births) syndromes have craniosynostosis as their most prominent clinical feature, associated with multiple other facial dysmorphisms. Crouzon syndrome is an autosomal dominant disorder with variable clinical presentation.

20.
(C)
Congenital CMV is associated with sensorineural hearing loss, although acquired CMV infection is not.

S
UGGESTED
R
EADING

American Academy of Pediatrics Subcommittee on Management of Acute Otitis Media. Diagnosis and management of acute otitis media.
Pediatrics
. 2004;113:1451-1465.
http://aappolicy.aappublications.org/cgi/content/full/pediatrics;113/5/1451.
Accessed June 2010.

Behrman RE, Kliegman RM, Jenson HB, et al.
Nelson Textbook of Pediatrics
. Philadelphia, PA: WB Saunders; 2007.

Gürtler N. Etiology of syndromic and nonsyndromic sensorineural hearing loss.
Otolaryngol Clin North Am.
2002;35(4):891-908.

Hone SW. Medical evaluation of pediatric hearing loss. laboratory, radiographic, and genetic testing.
Otolaryngol Clin North Am.
2002;35(4):751-764.

Sander R. Otitis externa: a practical guide to treatment and prevention.
Am Fam Physician.
2001;63(5):927-942.

CASE 54: A 2-YEAR-OLD WITH EYE REDNESS