OTOLOGIC DISEASE: A PEDIATRICS PERSPECTIVE
Kenneth M. Grundfast, MD, Professor and Chair, Department of Otolaryngology–Head and Neck Surgery, Boston
University School of Medicine, Boston, MA
| MODERN MANAGEMENT OF OTITIS MEDIA (OM)
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| Defining disorder: acute otitis media (AOM)—characterized by pain, fever, and objective findings (eg, red bulging
eardrum); persistent otitis media with effusion (POME)—possible erythematous eardrum (not necessarily pain or fever);
goal of therapy to diminish duration of effusion; frequent recurrent episodes of AOM—goal to diminish number and frequency
of episodes; parameters—ears involved (1 or 2; left or right); if child has AOM and persistent effusion in, eg,
right ear (and never left ear), problem may be cholesteatoma; severity (duration or frequency of episodes)
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Evidence-Based OM
| Decongestants: no evidence that decongestants significantly benefit patients with AOM or POME
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| Allergy and OME: some evidence of association, but not worthwhile to pursue extensive allergy work-up in every child
with OM; OM common, and allergy difficult to treat in early childhood (most OM occurs in children <3 yr of age)
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| Corticosteroid therapy: no definitive data to show that steroids reduce duration of effusion; risks involved
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| Antibiotics for OME: no evidence that any antibiotic hastens resolution of POME
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| Prophylaxis for AOM: some evidence that small daily dose of medication effective for preventing OM; however, not
treatment of choice in new era of conjugate vaccine
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| Tympanostomy tubes: does insertion of tympanostomy tubes reduce frequency of AOM? conflicting data; may
shorten length of time OM present (more study needed)
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| Tonsillectomy and adenoidectomy: no evidence for role of tonsillectomy in management of OM; adenoidectomy
may be beneficial (especially in children ≥4 yr of age); newer studies show that flora of nasopharynx can cause middle
ear infection; removal of adenoids diminishes pathogens and may have beneficial effect
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| Speech and language development: latest data do not show strong relationship among POME, conductive hearing
loss, and speech and language delays or delays in cognition
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Agency for Healthcare Research and Quality (AHRQ) Guidelines, 2004
 | 1) To diagnose AOM, confirm history of acute onset, and identify signs and symptoms of middle ear inflammation
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| 2) Management should include assessment of pain
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| 3) (a) Observation without use of antimicrobial agents in child with uncomplicated AOM option for selected children,
based on diagnostic certainty, age, severity of illness, and assurance of follow-up; (b) if decision to treat, clinician
should prescribe amoxicillin for most children; caveat — treatment questioned by some experts in pediatric infectious
disease
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| 4) If patient fails to respond to initial management within 48 to 72 hr, reassess and possibly prescribe antibiotic, or if
antibiotic prescribed and patient unresponsive, consider switching to different antibiotic; response defined as reduction
of fever (if present) and pain; persistent pain merits change in antibiotic
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| 5) Clinician should encourage prevention of AOM through reduction of risk factors (eg, attending day care with ≥6
children in group, passive smoke exposure)
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| 6) Evidence insufficient to recommend use of complementary and alternative medicine for AOM
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| OME (clinical practice guidelines)
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| Target population: children 2 mo to 12 yr of age; children with or without developmental disabilities or underlying conditions
that predispose to development of OME
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| Recommendations
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| 1) Document laterality, duration of effusion, and presence and severity of associated symptoms each time child with
OME evaluated
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| 2) Distinguish child at risk for speech, language, and learning problems, and more promptly evaluate speech and language
and possible necessity to intervene if child slightly delayed developmentally
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| 3) If child not at risk, manage with watchful waiting for 3 mo from date of onset of OME
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| 4) Test hearing when OME persists 3 mo, or at any time that language delay, learning problems, or significant hearing
loss suspected
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| 5) Child with persistent effusion who is not at specific risk for delay can be seen at 3- to 6-mo intervals; look for
structural abnormalities of eardrum or middle ear if suspected (child may be at risk for cholesteatoma)
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| 6) When surgery indicated, insertion of tubes preferred initial procedure
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| Options: 1) tympanometry to confirm diagnosis of OME; 2) physician referring child with OME should document duration
of effusion and reasons for referral, and provide additional relevant information
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Conjugate Pneumococcal Vaccine
| Coverage: Food and Drug Administration approved heptavalent (PCV7) vaccine in 2000; 7 serotypes; 84% of OM pneumococcal
organisms covered (95% of drug-resistant isolates were of serotypes covered); Northern California Kaiser
Permanente vaccine study—episodes of OM reduced 7% (recurrent episodes reduced 23% over 6 mo); vaccination associated
with 20% reduction in need for tympanostomy tubes; summary—vaccine helpful, but not highly effective in preventing
OM and not definitive cure
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| Pneumococcal capsular polysaccharides conjugated to protein D (Prymula et al, 2006): vaccine efficacy
for episodes of AOM (52.6% for first episode, 57.6% for any episode); efficacy against episodes of AOM caused by nontypeable
Haemophilus influenzae, 35%; vaccine reduced frequency of infection from vaccine-related cross-reactive
pneumococcal serotypes by 65%; using H influenzae–derived protein D as carrier protein for pneumococcal polysaccharides
protects against pneumococcal OM, and AOM due to nontypeable H influenzae (predominant organism in most episodes
of AOM)
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| EVALUATION OF THE CHILD WITH SUSPECTED HEARING LOSS
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| Timing of detection: in current era, hearing screening for newborns widespread practice (48 of 50 states have mandatory
universal newborn hearing screening); hearing impairment may be detected within first few weeks after birth (in past,
detection at ≈3 yr of age); increasing ability to confirm underlying gene mutation; evaluation conducted earlier, and cochlear
implant surgery possible at ≤1 yr of age (in past, hearing aid, possible cochlear implant at 3 yr of age)
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| Common causes: in past, meningitis leading cause of severe sensorineural hearing loss in children (diminished with advent
of H influenzae type B vaccine); trauma due to auto or bicycle accidents diminished with use of helmets; noise-induced
hearing loss diminished because of public information campaigns; infectious causes reduced with administration
of measles, mumps and rubella (MMR) vaccine; today, congenital and hereditary types of hearing loss more prevalent;
complete shift in past 10 to 15 yr
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| Congenital vs hereditary hearing impairment
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| Definitions: congenital means present at birth; hereditary means caused by genetic factors
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| Congenital but not hereditary: rubella (largely eradicated by vaccine); factors related to low birth weight (cytomegalovirus
infection, fetal alcohol syndrome [FAS]); for every child with syndrome as cause of hearing impairment, 2 others
have hereditary hearing impairment with no associated findings (genetic mutation with no phenotypic signs beyond
hearing impairment)
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| Hereditary but not congenital: more hereditary hearing impairment autosomal recessive than autosomal dominant (≈80%
autosomal recessive; ≈18% autosomal dominant; ≈2% X-linked recessive; mitochondrial [virtually immeasurable]);
otosclerosis—hearing loss in one or both ears at 30 to 40 yr of age; hearing progressively worsens; conductive hearing
loss; cure stapes surgery (stapedectomy); 50% penetrance, but not present at birth; pattern of progression (loss begins
in 20s and 30s; by 40-50 yr of age, significant hearing loss); delayed-onset nonsyndromic autosomal dominant progressive
sensorineural hearing loss—individual born with normal hearing; hearing impairment beginning at 10 to 20
yr of age; in midlife, progressive hearing loss; runs in families; no phenotypic abnormalities except hearing loss;
Usher’s syndrome type 2—theoretically, individual born with normal hearing (loss progresses with age)
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| Clues to gene mutation: type—if conductive hearing loss, possible persistent middle ear effusion; if sensorineural,
suspect genetic mutation; if hearing loss mixed, different genes involved; timing of onset—at birth or after birth? (different
genes involved); family history—look for inheritance pattern (caveat, many children with hearing impairment have
no affected family members); physical findings—syndrome present or absent? some syndromic findings not evident on
physical examination; other findings—proteinuria, hematuria, or abnormal electrocardiography (ECG); stability—
hearing loss stable or progressive?
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| Behavioral audiography: u-shaped or “cookie-bite” audiogram showing worse hearing in mid-frequencies highly suggestive
of hereditary hearing impairment; clue in older child (cooperation with test usually requires age ≥3 yr); if not severe,
problem might not be detected early on
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| Syndromic vs nonsyndromic: in United States, most hereditary hearing impairment nonsyndromic (connexin-26 mutation
most common genetic nonsyndromic cause); identification of syndrome may lead to detection of renal or cardiac abnormality
(not common); Usher’s, Pendred’s, and Jervell and Lange-Nielsen syndromes mentioned frequently in literature, but
not most common; clues to syndrome not manifest at or soon after birth—subtle abnormalities difficult to detect at 3 or 4
wk of age; distinguishing physical features more apparent at 6 to 12 mo of age; abnormal computed tomography (CT) or magnetic
resonance imaging can help diagnose some syndromes; developmental delay key component of some syndromes; diagnosis
of syndrome helps identify affected organ systems (eg, kidney, heart) and helps explain to parents reason for hearing
impairment
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| Usher’s syndrome: autosomal recessive disorder characterized by retinitis pigmentosa; type 1—congenital; vestibular
function absent (look for semiataxic gait); type 2—“progressive”; recent study (Reisser et al, 2002) suggests that visual
impairment progressive but hearing loss stable
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| Pendred’s syndrome: autosomal recessive disorder; nontoxic goiter; sensorineural hearing loss (usually congenital);
patients usually euthyroid; newborn hearing screening helpful; previously, perchlorate discharge test used (new genetic
test easier); associated with Mondini’s deformity (detectable on CT); if gene mutation present, confirm diagnosis with radiography
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| Jervell and Lange-Nielsen syndrome: syncopal episodes with arrhythmia and risk for sudden death; prolonged Q-T
interval; abnormality evident on ECG (needed for diagnosis); severe sensorineural hearing loss
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| Goldenhar’s syndrome: oculoauriculovertebral dysplasia; facial asymmetry; unilateral mandibular hypoplasia; relatively
common (prevalence 1 in 5600 live births); case—child failed screening hearing test in left ear; on examination,
could not get speculum in to see ear canal and eardrum (subtle diagnosis); conductive hearing loss due to ossicular abnormality;
more flagrant form—newborn with micropsia and epibulbar dermoid in left eye; nondevelopment of left ear (no
ear canal or middle ear bones)
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| Treacher Collins syndrome: another facial bone abnormality associated with profound hearing impairment; mixed or
conductive hearing loss; “Jiminy Cricket” facies; anti-Mongoloid eye slant; cup-shaped ears; constricted midface; autosomal
dominant disorder; widely variable expression; stenosis or atresia of ear canal; congenital stapes fixation; poorly
pneumatized mastoid
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| Waardenburg’s syndrome: pigmentary disorder involving eye; variable expression (from near-normal hearing to
profoundly deaf); 4 types (types 1 and 2 most common); type 1 (case)—white forelock; widely spaced eyes; classic blue
eyes; synophrys (confluent eyebrows); type 2—no dystopia canthorum; in hypotelorism, cranial bone abnormality (orbits
laterally displaced); in dystopia canthorum, look closely at medial canthi; case—mother <30 yr of age has poliosis
(early graying); medial canthi widely spaced; confluent eyebrows
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| X-linked recessive hearing loss: occurs in male patients (female individuals carriers); mixed hearing loss (conductive
and sensorineural); clinical diagnosis (case)—boy between 4 and 5 yr of age; history of frequent episodes of OM; poor
academic performance in comparison to peers; teacher considers child inattentive; mother says child cannot hear properly;
few ear infections, but usually presents with small amount of fluid in one ear; after placement of tubes, conductive
and mild sensorineural hearing loss still present; radiographic diagnosis—distinct abnormality on CT
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| Branchio–oto–renal (BOR) syndrome: relatively common; autosomal dominant; preauricular pits and branchial
cleft cysts; wide variation in hearing levels; caveat (preauricular pits not necessarily associated with hearing impairment)
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| Fetal alcohol syndrome: congenital, not hereditary; facial appearance—elongated distance between columella of
nose and upper lip; long forehead; poorly defined philtrum; case—boy had facial signs and history consistent with FAS
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| Cytomegalovirus (CMV) infection: not common but still presents; many women of childbearing age carry CMV;
chorioretinitis, poor neuromuscular control, and cognitive disabilities; polymerase chain reaction test used to make diagnosis;
difficult to diagnose at birth; case—CMV diagnosed in girl after birth; left-ear hearing impairment detected at 4 yr
of age; by age 6 yr, progression from unilateral to bilateral hearing loss
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| Enlarged vestibular aqueduct: most common radiographic abnormality detected in children with hearing impairment;
more common than Mondini’s deformity; once abnormality detected on CT, child at risk for incremental decrease
in hearing; step-wise decrements associated with head trauma (these children should not play contact sports)
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| Diagnostic paradigm for childhood idiopathic sensorineural hearing loss (studies by Preciado et al,
2004 and 2005): not helpful—erythrocyte sedimentation rate, tests for syphilis; cholesterol; triglycerides; hemoglobin;
platelets; urinalysis; thyroid function tests; occasionally helpful—ECG (to detect, eg, Jervell and Lange-Nielsen
syndrome); helpful—radiographic imaging (≈27% had abnormalities); genetic testing (18% had mutations in GJB2 gene
[connexin gene]; ophthalmology evaluation
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| Speaker’s advice: know results of newborn hearing screening tests; look for dysmorphic features (consult otolaryngologist,
ophthalmologist, or clinical geneticist if not sure whether phenotypic abnormalities present); obtain urinalysis (look
for proteinuria and hematuria) and ECG; test for connexin mutations; CT usually not performed at <12 to 18 mo of age
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| Conclusions: know difference between congenital and hereditary hearing loss; look for subtle abnormalities in syndromes
(not all findings evident at birth); timely consultations may be indicated to make correct diagnoses through first,
second, and third years of life in children who have failed newborn hearing screening tests
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Suggested Reading
Coyte PC et al: The role of adjuvant adenoidectomy and tonsillectomy in the outcome of the insertion of tympanostomy
tubes. N Engl J Med 344:1188, 2001; Harrison CJ: Changes in treatment strategies for acute otitis media after full implementation
of the pneumococcal seven valent conjugate vaccine. Pediatr Infect Dis J 22:S120, 2003; Paradise JL et
al: Effect of early or delayed insertion of tympanostomy tubes for persistent otitis media on developmental outcomes at the
age of three years. N Engl J Med 344:1179, 2001; Paradise JL et al: Language, speech sound production, and cognition
in three-year-old children in relation to otitis media in their first three years of life. Pediatrics 105:1119, 2000; Paradise
JL et al: Tympanostomy tubes and developmental outcomes at 9 to 11 years of age. N Engl J Med 356:248, 2007; Poehling
KA: Reduction of frequent otitis media and pressure-equalizing tube insertions in children after introduction of
pneumococcal conjugate vaccine. Pediatrics 119:707, 2007; Preciado DA et al: A diagnostic paradigm for childhood
idiopathic sensorineural hearing loss. Otolarngol Head Neck Surg 131:804, 2004; Preciado DA et al: Improved diagnostic
effectiveness with a sequential diagnostic paradigm in idiopathic pediatric sensorineural hearing loss. Otol Neurotol
26:610, 2005; Prymula R et al: Pneumococcal capsular polysaccharides conjugated to protein D for prevention of acute
otitis media caused by both Streptococcus pneumoniae and non-typable Haemophilus Influenzae: a randomized double-
blind efficacy study. Lancet 367:740, 2006; Reisser CF et al: Hearing loss in Usher syndrome type II is nonprogressive.
Ann Otol Rhinol Laryngol 111:1108, 2002; Rosenfeld RM, Bluestone CD: Evidence-Based Otitis Media (2nd ed),
BC Decker, Inc: St Louis, MO, 2003; Rosenfeld RM et al: Clinical practice guideline: otitis media with effusion. Otolaryngol
Head Neck Surg 130:S95, 2004; Rovers MM et al: The effect of ventilation tubes on language development in
infants with otitis media with effusion: a randomized trial. Pediatrics 106:E42, 2000.
Educational Objectives
| The goal of this program is to improve management of otitis media (OM) and hearing impairment. After hearing
and assimilating this program, the clinician will be better able to:
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| 1. Explain the differential diagnosis of OM.
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| 2. Describe current recommendations from the Agency for Healthcare Research and Quality (AHRQ) for
managing acute OM.
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| 3. Describe current recommendations from the AHRQ for managing OM with effusion.
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| 4. Evaluate children for suspected hearing loss.
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| 5. Recognize syndromes associated with hearing impairment.
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Faculty Disclosure
In adherence to ACCME Standards for Commercial Support, Audio-Digest requires all faculty amd planning committee members to
disclose relevant financial relationships within the past 12 months that might create any personal conflicts of interest. Any identified
conflicts were resolved to ensure that this educational activity promotes quality in health care and not a proprietary business or commercial
interest. For this program, the following has been disclosed: Dr. Grundfast has been a paid consultant to Innovia Medical. The
planning committee reported nothing to disclose.
Acknowledgments
Dr. Grundfast was recorded at Pediatrics for the Primary Care Physician, presented June 29 to July 1, 2007, in Amelia Island,
FL, by Nemours. The Audio-Digest Foundation thanks Dr. Grundfast and Nemours for their cooperation in the production of this
program.
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