ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
Rapid onset of symptoms within the past 3 weeks.
Symptoms of ear canal inflammation, including otalgia, itching, or fullness, with or without hearing loss or jaw pain.
Signs of ear canal inflammation, including tenderness of the tragus and/or pinna, ear canal edema and/or erythema, otorrhea, regional lymphadenitis, tympanic membrane (TM) erythema, or cellulitis of the pinna and adjacent skin.
Acute or chronic otitis media with eardrum rupture, furunculosis of the ear canal, herpes zoster oticus, mastoiditis, referred temporomandibular joint pain, and chronic otitis externa.
Otitis externa (OE) is a cellulitis of the soft tissues of the external auditory canal (EAC), which can extend to surrounding structures such as the pinna, tragus, and lymph nodes. Humidity, heat, and moisture in the ear are known to contribute to the development of OE, thus it is more common in the summer months and in humid climates. Cerumen serves as a hydrophobic protective barrier to the underlying skin and its acidic pH inhibits bacterial and fungal growth. Trauma to the ear canal skin can break this skin-cerumen barrier, which is the first step in developing OE. Sources of trauma can include cotton swab use, earbuds, digital manipulation (scratching), and ear plugs. Dermatologic conditions such as atopic dermatitis can also predispose one to OE. The most common organisms causing OE are Staphylococcus aureus, Staphylococcus epidermidis, and Pseudomonas aeruginosa. However, anaerobic bacteria are also seen. Fungal infection occurs in 2%–10% of patients, usually following treatment for a bacterial OE.
Symptoms include acute onset of pain, aural fullness, decreased hearing, and itching in the ear. Symptoms tend to peak within 3 days. Manipulation of the pinna or tragus causes considerable pain. Discharge may be clear or purulent and may also cause secondary eczema of the auricle. The EAC is typically swollen and narrowed, and the patient may resist any attempt to insert an otoscope. Debris is often present in the canal, and it may be difficult to visualize the TM. However, it is important to determine the status of the eardrum to rule out secondary OE caused by middle ear drainage that may need to be managed differently.
If untreated, cellulitis of neck and face may result. Immunocompromised individuals can develop malignant OE, which is a spread of the infection to the skull base with resultant osteomyelitis. This is a life-threatening condition and should be evaluated emergently if suspected.
Management of OE includes pain control, removal of debris from the canal, topical antimicrobial therapy, and avoidance of causative factors. Cultures are not routinely sent on initial presentation as most cases will resolve with these primary interventions. Fluoroquinolone eardrops alone are first-line therapy for OE in the absence of systemic symptoms. The topical therapy chosen must be nonototoxic because a perforation or patent tube may be present; if the TM cannot be visualized, a perforation should be presumed to exist. If the ear canal is too edematous to allow entry of the eardrops, a Pope ear wick (expandable sponge) should be placed to ensure antibiotic delivery. Oral antibiotics are indicated for any signs of invasive infection, such as fever, cellulitis of the face or auricle, or tender periauricular or cervical lymphadenopathy. In such cases, in addition to ototopical therapy, cultures of the ear canal discharge should be sent, and an antistaphylococcal antibiotic prescribed while awaiting culture results. The ear should be kept dry until the infection has cleared. If drainage persists despite therapy, cultures should be sent. Patients can have difficulty applying drops to the affected ear canal, which may cause prolonged symptoms and treatment failures. If this is suspected, thoroughly explain and demonstrate the application of the topical therapy as outlined in the Clinical Practice Guideline on Acute Otitis Externa. A new formulation of ciprofloxacin suspension has recently become available; it is liquid at cooler temperatures and thickens into a gel at body temperature. This allows a single application to work for multiple days and may be useful when there are compliance problems.
et al: Clinical practice guideline: acute otitis externa executive summary. Otolaryngol Head Neck Surg 2014 Nov;150(2):161–168
Acute otitis media (AOM) is the most common reason antibiotics are prescribed for children in the United States. It is an acute infection of the middle ear space associated with inflammation, effusion, or, if a patent tympanostomy tube or perforation is present, otorrhea (ear drainage).
ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
Moderate to severe bulging of the TM or new otorrhea not associated with OE.
Mild bulging of the TM and less than 48 hours of otalgia (ear-holding, tugging, or rubbing in a nonverbal child) or intense erythema of the TM.
Middle ear effusion (MEE), proven by pneumatic otoscopy or tympanometry, must be present.
Otitis media with effusion (OME), bullous myringitis, acute mastoiditis, and middle ear mass.
Two findings are critical in establishing a diagnosis of AOM: a bulging TM and a MEE. The presence of MEE is best determined by visual examination and either pneumatic otoscopy or tympanometry (Figure 18–1). To distinguish AOM from OME, signs and symptoms of middle ear inflammation and acute infection must be present. Otoscopic findings specific for AOM include a bulging TM, impaired visibility of ossicular landmarks, yellow or white effusion (pus), an opacified and inflamed eardrum, and occasionally squamous exudate or bullae on the eardrum.
A. Pathophysiology and Predisposing Factors
1. Eustachian tube dysfunction (ETD)
The Eustachian tube regulates middle ear pressure and allows for drainage of the middle ear. The ciliated respiratory epithelium of the Eustachian tube also defends against pathogens by producing lysozyme and mucus, which helps rid the ear of microorganisms. It must periodically open to prevent the development of negative pressure and effusion in the middle ear space. If the Eustachian tube does not work properly, negative pressure leads to transudation of cellular fluid into the middle ear, as well as influx of fluids and pathogens from the nasopharynx and adenoids. Middle ear fluid may then become infected, resulting in AOM. The Eustachian tube of infants and young children is more prone to dysfunction because it is shorter, more compliant, and more horizontal than in adults. The Eustachian tube reaches its adult configuration by the age of 7 years. Children with craniofacial differences, such as those with Trisomy 21 or cleft palate, may be particularly susceptible to ETD because of abnormal anatomy of the Eustachian tube.
2. Bacterial colonization
Nasopharyngeal colonization with S pneumoniae, Haemophilus influenzae, or Moraxella catarrhalis increases the risk of AOM, whereas colonization with normal flora such as viridans streptococci may prevent AOM by inhibiting growth of these pathogens.
3. Viral upper respiratory infections
Upper respiratory infections (URIs) impair Eustachian tube function by causing adenoid hypertrophy and edema of the Eustachian tube itself. Viral infections also inhibit the antibacterial properties of mucus and mucociliary clearance.
Passive smoke increases the risk of persistent MEE by enhancing colonization, prolonging the inflammatory response, and impeding drainage of the middle ear through the Eustachian tube. For infants aged 12–18 months, cigarette exposure is associated with an 11% per pack increase in the duration of MEE.
5. Impaired host immune defenses
Immunocompromised children such as those with selective IgA deficiency usually experience recurrent AOM, rhinosinusitis, and pneumonia. However, most children who experience recurrent or persistent otitis only have selective impairments of immune defenses against specific otitis pathogens.
Bottle feeding especially with bottle propping in the crib or carseat increases the risk of AOM because of aspiration of contaminated secretions into the middle ear space. Breastfeeding reduces the incidence of acute respiratory infections and provides immunoglobulin A (IgA) antibodies that reduce colonization with otitis pathogens.
The incidence of AOM correlates with the activity of respiratory viruses, accounting for the annual surge in otitis media cases during the winter months in temperate climates.
Children exposed to large groups of children have more respiratory infections and OM. The increased number of children in day care over the past three decades has undoubtedly played a major role in the increase in AOM.
9. Genetic susceptibility
Genetics is thought to play a role in 40%–70% of ear infections. Most of the genes responsible regulate immunity. However, environmental and pathogen-related causes also play a role. The role of genetics in AOM is an area of active research.
Children ages 1–3 years are at greatest risk for AOM.
B. Microbiology of Acute Otitis Media
Bacterial or viral pathogens can be detected in up to 96% of middle ear fluid samples from patients with AOM. Peak activity of respiratory syncytial virus, metapneumovirus, and influenza A corresponds with increased visits for AOM and 71% of middle ear aspirates from children undergoing ear tube surgery for recurrent OM contain viruses. Polybacterial infections are seen in up to 55% of cases, with bacterial and viral coinfections occurring in up to 70%. S pneumoniae and H influenzae account for 35%–40% and 30%–35% of isolates, respectively. With widespread use of the pneumococcal conjugate vaccine starting in 2000, the incidence of AOM caused by H influenzae rose while that of the S pneumoniae vaccine serotypes declined. However, there has been an increase in disease caused by S pneumoniae serotypes not covered by the vaccine, as well as S aureus. The third most common pathogen cited is M catarrhalis, which causes 15%–25% of AOM cases in the United States (Table 18–1). The fourth most common organism in AOM is Streptococcus pyogenes, which is found more frequently in school-aged children than in infants. S pyogenes and S pneumoniae are the predominant causes of mastoiditis.
Table 18–1.Microbiology of acute otitis media (AOM). ||Download (.pdf) Table 18–1. Microbiology of acute otitis media (AOM).
|Organism ||Percentage of AOM Cases |
Drug-resistant S pneumoniae is a common pathogen in AOM and strains may be resistant to only one drug class (eg, penicillins or macrolides) or to multiple classes. Children with resistant strains tend to be younger and to have had more unresponsive infections. History of antibiotic treatment in the preceding 3 months increases the risk of harboring resistant pathogens.
C. Examination Techniques and Procedures
AOM is overdiagnosed, leading to inappropriate antibiotic therapy, unnecessary surgical referrals, and significant associated costs. Contributing to errors in diagnosis is the temptation to accept the diagnosis without removing enough cerumen to adequately visualize the TM, and the mistaken belief that a red TM establishes the diagnosis. Redness of the TM is often a vascular flush caused by fever or crying.
A pneumatic otoscope with a rubber suction bulb and tube is used to assess TM mobility. When used correctly, pneumatic otoscopy can improve diagnostic ability by 15%–25%. The largest possible speculum should be used to provide an airtight seal and maximize the field of view. When the rubber bulb is gently squeezed, the TM should move freely with a snapping motion; if fluid is present in the middle ear space, the mobility of the TM will be absent or resemble a fluid wave. The ability to assess mobility is compromised by failure to achieve an adequate seal with the otoscope and poor visualization of the TM.
In order to adequately visualize the TM, cerumen (ear wax) removal is an essential skill for anyone who cares for children. Please refer to section on cerumen impaction below.
Tympanometry can be helpful in assessing middle ear status, particularly when pneumatic otoscopy is inconclusive or difficult to perform. Tympanometry can reveal the presence or absence of a MEE but cannot differentiate between acutely infected fluid (AOM) and a chronic effusion (also referred to as OME).
Tympanometry measures TM compliance and displays it in graphic form. It also measures the volume of the ear canal, which can help differentiate between an intact and perforated TM.
Standard 226-Hz tympanometry is not reliable in infants younger than 6 months. A high-frequency (1000 Hz) probe is used in this age group.
Tympanograms can be classified into four major patterns, as shown in Figure 18–2. The pattern shown in Figure 18–2A, characterized by maximum compliance at normal atmospheric pressure, indicates a normal TM, good Eustachian tube function, and absence of effusion. Figure 18–2B identifies a nonmobile TM with normal volume, which indicates MEE. Figure 18–2C indicates an intact, mobile TM with excessively negative middle ear pressure (> –150 daPa), indicative of poor Eustachian tube function. Figure 18–2D shows a flat tracing with a large middle ear volume, indicative of a patent tube or TM perforation.
Four types of tympanograms obtained with Welch-Allyn MicroTymp 2. A: Normal middle ear. B: Otitis media with effusion or AOM. C: Negative middle ear pressure due to ETD. D: Patent tympanostomy tube or perforation in the TM. Same as B except for a very large middle ear volume.
Pain is the primary symptom of AOM, and the 2013 clinical practice guidelines emphasize the importance of addressing this symptom. As it may take 1–3 days before antibiotic therapy leads to a reduction in pain, ibuprofen or acetaminophen should be administered as needed to relieve discomfort. Topical analgesics have a very short duration and studies do not support efficacy in children younger than 5 years.
B. The Observation Option
The choice to observe an episode of AOM and not treat with antibiotics is an option in otherwise healthy children with mild to moderate otitis media without other underlying conditions such as cleft palate, craniofacial abnormalities, immune deficiencies, cochlear implants, or tympanostomy tubes. The decision should be made in conjunction with the parents, and a mechanism must be in place to provide antibiotic therapy if there is worsening of symptoms or lack of improvement within 48–72 hours. A Safety-Net Antibiotic Prescription (SNAP) given to parents with instructions to be filled only if symptoms do not resolve lowered overall antibiotic use in a large pediatric practice-based research network. The American Academy of Pediatrics clinical practice guidelines include age, presence of otorrhea, severity of symptoms, and laterality as criteria for antibiotic treatment versus observation (Table 18–2).
Table 18–2.Recommendations for initial management of uncomplicated AOM.a ||Download (.pdf) Table 18–2. Recommendations for initial management of uncomplicated AOM.a
|Age ||Otorrhea With AOMa ||Unilateral or Bilateral AOMa With Severe Symptomsb ||Bilateral AOMa Without Otorrhea ||Unilateral AOMa Without Otorrhea |
6 mo to 2 y
Antibiotic therapy or additional observation
≥ 2 y
Antibiotic therapy or additional observation
Antibiotic therapy or additional observationc
Antibiotics have been shown to shorten the duration of AOM. Therefore, high-dose amoxicillin remains the first-line antibiotic for treating AOM, even with a high prevalence of drug-resistant S pneumoniae, because data show that isolates of the bacteria remain susceptible to the drug 83%–87% of the time.
Amoxicillin-clavulanate enhanced strength (ES), with 90 mg/kg/day of amoxicillin dosing (14:1 ratio of amoxicillin:clavulanate), is an appropriate choice when a child has had amoxicillin in the last 30 days, or is clinically failing after 48–72 hours on amoxicillin (Table 18–3) or has concomitant conjunctivitis. Purulent conjunctivitis is often caused by nontypeable H influenzae. The regular strength formulations of amoxicillin-clavulanate (7:1 ratio) should not be doubled in dosage to achieve 90 mg/kg/day of amoxicillin, because the increased amount of clavulanate will cause diarrhea.
Table 18–3.Antibiotic therapies for AOM. ||Download (.pdf) Table 18–3. Antibiotic therapies for AOM.
|A. Initial Immediate or Delayed Antibiotic Treatment |
Alternative Treatments (if Penicillin-Allergic)
| || |
Cefdinir (14/mg/kg/day in 1 or 2 doses)
Cefuroxime (30 mg/kg/day divided BID)
Cefpodoxime (10 mg/kg/day in two divided doses)
Ceftriaxone (50 mg IM or IV per day for 1 or 3 days)
For children with severe penicillin allergies (IgE-mediated events) or known cephalosporin allergy:
|B. Antibiotic Treatment After 48–72 h of Failure of Initial Antibiotic |
Amoxicillin-clavulanate (90 mg/kg/day or amoxicillin, with 6.4 mg/kg/day of clavulanate in two divided doses)
Ceftriaxone (50 mg IM or IV per day for 3 days)
Ceftriaxone (50 mg IM or IV per day for 3 days)
Clindamycin (30–40 mg/kg/day, divided TID) with or without a third-generation cephalosporin
|C. Recurrence > 4 wk After Initial Episode |
A new pathogen is likely, so restart first-line therapy.
Be sure diagnosis is not OME, which may be observed for 3–6 mo without treatment.
Three oral cephalosporins (cefuroxime, cefpodoxime, and cefdinir) are more β-lactamase–stable and are alternative choices in children who develop a papular rash with amoxicillin (see Table 18–3). Of these, cefdinir suspension is most palatable; the other two have a bitter aftertaste which is difficult to conceal.
A second-line antibiotic is indicated when a child experiences symptomatic infection within 1 month of finishing amoxicillin; however, repeated use of high-dose amoxicillin is indicated if more than 4 weeks have passed without symptoms. Macrolides are not recommended as second-line agents because S pneumoniae is resistant in approximately 30% of respiratory isolates, and because virtually all strains of H influenzae have an intrinsic macrolide efflux pump, which pumps the antibiotic out of the bacterial cell. However, if there is a history of type 1 hypersensitivity reaction, macrolides may be used.
Reasons for failure to eradicate a sensitive pathogen include drug noncompliance, poor drug absorption, or vomiting of the drug. If a child remains symptomatic for longer than 3 days while taking a second-line agent, a tympanocentesis is useful to identify the causative pathogen. If a highly resistant pneumococcus is found or if tympanocentesis is not feasible, intramuscular ceftriaxone at 50 mg/kg/dose for 3 consecutive days is recommended. If a child has experienced a severe reaction, such as anaphylaxis, to amoxicillin, cephalosporins should not be substituted. Otherwise, the risk of cross-sensitivity is less than 0.1%. Multidrug-resistant S pneumoniae poses a treatment dilemma and newer antibiotics, such as fluoroquinolones or linezolid, may need to be employed. However, these drugs are not approved by the U.S. Food and Drug Administration (FDA) for the treatment of AOM in children.
In patients with tympanostomy tubes with uncomplicated acute otorrhea, ototopical antibiotics (fluoroquinolone eardrops) are first-line therapy. The eardrops serve two purposes: (1) They treat the infection and (2) they physically “rinse” drainage from the tube which helps prevent plugging of the tube. Oral antibiotics are not indicated in the absence of systemic symptoms.
If a TM perforation has occurred with otorrhea then topical antibiotics are recommended as first-line agents due to the ability to provide high concentrations of antibiotic directly to the middle ear. Topical fluoroquinolone otic drops (ofloxacin and ciprofloxacin) with or without steroids are considered safe for administration into the middle ear. If there is a large amount of debris or drainage in the ear canal, the canal may need to be suctioned first to allow drops to gain access to the middle ear.
Tympanocentesis is performed by placing a needle through the TM and aspirating the middle ear fluid. The fluid is sent for culture and sensitivity. Indications for tympanocentesis are (1) AOM in an immunocompromised patient, (2) research studies, (3) evaluation for presumed sepsis or meningitis, such as in a neonate, (4) unresponsive otitis media despite courses of two appropriate antibiotics, and (5) acute mastoiditis or other suppurative complications.
E. Prevention of Acute Otitis Media
1. Antibiotic prophylaxis
Strongly discouraged due to poor efficacy and concern for antibiotic resistance.
2. Possible lifestyle modifications
Parental education plays a major role in decreasing AOM.
Smoking is a risk factor both for URI and AOM. Primary care physicians should provide information on smoking cessation programs and measures.
Breastfeeding protects children from AOM. Clinicians should encourage exclusive breastfeeding for 6 months.
Bottle-propping in the crib should be avoided. It increases AOM risk due to the reflux of milk into the Eustachian tubes.
Pacifiers are controversial. There may be a protective effect of pacifiers against SIDS but they may increase risk of AOM. Currently, the recommendation from the American Academy of Family Physicians is to wean pacifiers after 6 months of age to reduce the risk of AOM.
Day care is a risk factor for AOM, but working parents may have few alternatives. Possible alternatives include care by relatives or child care in a setting with fewer children.
Tympanostomy tubes are effective in the treatment of recurrent AOM as well as OME.
4. Immunologic evaluation and allergy testing
While immunoglobulin subclass deficiencies may be more common in children with recurrent AOM, there is no practical immune therapy available. More serious immunodeficiencies, such as selective IgA deficiency, should be considered in children who suffer from a combination of recurrent AOM, rhinosinusitis, and pneumonia. In the school-aged child or preschooler with an atopic background, skin testing may be beneficial in identifying allergens that can predispose to AOM.
The pneumococcal conjugate and influenza vaccines are recommended. The seven-valent pneumococcal conjugate vaccine (PCV7) was introduced in the United States in 2000, and the 13-valent pneumococcal conjugate vaccine (PCV13) in 2010. The transition from PCV7 to PCV13 has resulted in a decline of otitis media among children younger than 2 years due to decreased risk of both the first and subsequent episodes of otitis media.
et al: Panel 4: Report of the microbiology panel. Otolaryngol Head Neck Surg 2017 April; 156(4 Suppl):S51–S62
et al: The diagnosis and management of acute otitis media. Pediatrics 2013;131:e964–e999
et al: Panel 7: Otitis media and complications. Otolaryngol Head Neck Surg 2017;156(4s):S88–S105
et al: Treatment of otitis media with observation and a safety-net antibiotic prescription. Pediatrics 2003 Sep;112(3 Pt 1):527–531
et al: Changes in otitis media episodes and pressure equalization tube insertions among young children following introduction of the 13-valent pneumococcal conjugate vaccine: A birth-cohort based study. Clin Infect Dis 2019 Nov 27;69(12):2162–2169. doi: 10.1093/cid/ciz142
3. Otitis Media With Effusion
ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
MEE with decreased TM mobility as diagnosed on pneumatic otoscopy.
No signs or symptoms of acute inflammation.
OME should not be treated with antibiotics.
OME is the presence of fluid in the middle ear space without signs or symptoms of acute inflammation. This is common, with 90% of children having one episode of OME by age 5. On examination, the TM may be opacified and thickened and the middle ear fluid can be clear, amber-colored, or opaque. Pneumatic otoscopy can confirm the presence of a MEE. If the pneumatic otoscopy examination is uncertain, then tympanometry should be performed to confirm presence of middle ear fluid.
Children with OME can develop AOM if the middle ear fluid should become infected. After AOM, fluid can remain in the ear for several weeks, with 60%–70% of children still having MEE 2 weeks after successful treatment. This drops to 40% at 1 month and 10%–25% at 3 months after treatment. It is important to distinguish OME from AOM because the former does not benefit from treatment with antibiotics.
An audiology evaluation should be performed after approximately 3 months of continuous bilateral effusion in most children. However, children who are at risk of language delay due to socioeconomic circumstances, craniofacial anomalies, or other risk factors should undergo a hearing evaluation at the time that OME is diagnosed. Children with hearing loss or speech delay should be referred to an otolaryngologist for possible tympanostomy tube placement. Antibiotics, antihistamines, and steroids have not been shown to be useful in the treatment of OME.
In uncomplicated cases, OME is observed for 3 months prior to consideration for tympanostomy tube placement. Longer periods of observation may be acceptable in children with normal or very mild hearing loss on audiogram, no risk factors for speech and language issues, and no structural changes to the TM. These children should be followed every 3–6 months until the effusions clear or problems develop. Indications for tympanostomy tubes include hearing loss greater than 40 dB, TM retraction pockets, ossicular erosion, adhesive atelectasis, and cholesteatoma. In children older than age 4, adenoidectomy may be recommended with ear tubes as studies show this can reduce failure rate and need for additional surgeries.
Prognosis is variable based on age of presentation. Infants who are very young at the time of first otitis media are more likely to need surgical intervention. Other factors that decrease the likelihood of resolution are onset of OME in the summer or fall, history of prior tympanostomy tubes, presence of adenoids, and hearing loss greater than 30 dB.
et al: Clinical practice guideline: tympanostomy tubes in children. Otolaryngol Head Neck Surg 2013 July;149(1Suppl):S1–S35
et al: Clinical Practice Guideline: otitis media with effusion executive summary (update). Otolaryngol Head Neck Surg 2016 Feb;154(2):201–214. doi: 10.1177/0194599815624407.26833645
4. Complications of Otitis Media
A. Changes of the Tympanic Membrane
Tympanosclerosis is an acquired disorder of calcification and scarring of the TM and middle ear structures secondary to inflammation. If tympanosclerosis involves the ossicles, a conductive hearing loss may result. The term myringosclerosis applies to calcification of the TM only and is a fairly common sequela of OME and AOM. Myringosclerosis may also develop at the site of a previous tympanostomy tube; tympanosclerosis is not a common sequela of tube placement. Myringosclerosis rarely causes hearing loss, unless the entire TM is involved (“porcelain eardrum”).
The appearance of a small defect or invagination of the pars tensa or pars flaccida of the TM suggests a retraction pocket. Retraction pockets occur when chronic inflammation and negative pressure in the middle ear space produce atrophy and atelectasis of the TM.
Continued inflammation can cause adhesions to form between the retracted TM and the ossicles. This condition, referred to as adhesive otitis, predisposes one to formation of a cholesteatoma or fixation and erosion of the ossicles.
A greasy-looking or pearly white mass seen in a retraction pocket or perforation behind the eardrum suggests a cholesteatoma (Figure 18–3). If infection is superimposed, serous or purulent drainage will be seen, and the middle ear cavity may contain granulation tissue or even polyps. Persistent, recurrent, or foul-smelling otorrhea following appropriate medical management should make one suspect a cholesteatoma and prompt an ENT (Ear-Nose-Throat/Otolaryngology) referral.
Attic cholesteatoma, formed from an in-drawing of an attic retraction pocket.
C. Tympanic Membrane Perforation
Occasionally, an episode of AOM may result in rupture of the TM. Discharge from the ear is seen, and often there is rapid relief of pain. Perforations due to AOM usually heal spontaneously within a couple of weeks. Ototopical antibiotics are recommended for a 10- to 14-day course and patients should be referred to an otolaryngologist 2–3 weeks after the rupture for examination and hearing evaluation.
When perforations fail to heal, surgical repair may be needed. TM repair is generally delayed until the child is older and Eustachian tube function has improved. Repair of the eardrum (tympanoplasty) is generally deferred until around 7 years of age, which is approximately when the Eustachian tube reaches adult orientation.
In the presence of a perforation, water activities should be limited to surface swimming, preferably with the use of an ear plug.
D. Facial Nerve Paralysis
The facial nerve traverses the middle ear as it courses through the temporal bone to its exit at the stylomastoid foramen. Normally, the facial nerve is completely encased in bone, but occasionally bony dehiscence in the middle ear is present, exposing the nerve to infection and making it susceptible to inflammation during an episode of AOM. The acute onset of a facial nerve paralysis should not be deemed idiopathic Bell palsy until all other causes have been excluded. If middle ear fluid is present, prompt myringotomy and tube placement are indicated. CT is indicated if a cholesteatoma or mastoiditis is suspected.
E. Chronic Suppurative Otitis Media
ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
Ongoing purulent ear drainage.
Nonintact TM: perforation or tympanostomy tubes.
May be associated with cholesteatoma.
Chronic suppurative otitis media (CSOM) is present when persistent otorrhea occurs in a child with tympanostomy tubes or TM perforation for greater than 6–12 weeks. It starts with an acute infection that becomes chronic with mucosal edema, ulceration, granulation tissue, and eventual polyp formation. Risk factors include a history of, multiple episodes of otitis media, living in crowded conditions, day care attendance, and being a member of a large family. The most common associated bacteria include P aeruginosa, S aureus, Proteus species, Klebsiella pneumoniae, and diphtheroids.
Visualization of the TM, meticulous cleaning with culture of the drainage, and appropriate antimicrobial therapy, usually topical, are the keys to management.
Occasionally, CSOM may be a sign of cholesteatoma or other disease process such as foreign body, neoplasm, Langerhans cell histiocytosis, tuberculosis, granulomatosis, fungal infection, or petrositis. If CSOM is not responsive to culture-directed treatment, imaging and biopsy may be needed to rule out other possibilities. Patients with facial palsy, vertigo, or other CNS signs should be referred immediately to an otolaryngologist.
Suppurative infections of the middle ear can spread into the membranous labyrinth of the inner ear. Symptoms include vertigo, hearing loss, and fevers. The child often appears extremely toxic. Intravenous antibiotic therapy is used, and intravenous steroids may also be used to help decrease inflammation. Sequelae can be serious, including a condition known as labyrinthitis ossificans, or bony obliteration of the inner ear, including the cochlea, leading to profound hearing loss.
ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
AOM is almost always present.
Postauricular pain and erythema.
Ear protrusion (late finding).
Mastoiditis occurs when infection spreads from the middle ear space to the mastoid portion of the temporal bone, which lies just behind the ear and contains air-filled spaces. Mastoiditis can range in severity from inflammation of the mastoid periosteum to bony destruction of the mastoid air cells (coalescent mastoiditis) with abscess development. Mastoiditis can occur in any age group, but more than 60% of the patients are younger than 2 years. Many children do not have a prior history of recurrent AOM.
Patients with mastoiditis usually have postauricular pain, fever, and an outwardly displaced pinna. On examination, the mastoid area often appears indurated and red and with disease progression, it may become swollen and fluctuant. The earliest finding is severe tenderness on mastoid palpation. AOM is almost always present. Late findings include a pinna that is pushed forward by postauricular swelling and an ear canal that is narrowed due to pressure on the posterosuperior wall from the mastoid abscess. In infants younger than 1 year, the swelling occurs superior to the ear and pushes the pinna downward rather than outward.
The best way to determine the extent of disease is by CT scan. Early mastoiditis is radiographically indistinguishable from AOM, with both showing opacification but no destruction of the mastoid air cells. With progression of mastoiditis, coalescence of the mastoid air cells is seen with bone destruction. Mastoiditis is a clinical diagnosis, based on pain as well as physical exam findings.
The most common pathogens are S pneumoniae followed by H influenzae and S pyogenes. Rarely, gram-negative bacilli and anaerobes are isolated. In the preantibiotic era, up to 20% of patients with AOM developed mastoiditis requiring mastoidectomy. Antibiotics decrease the incidence and morbidity of acute mastoiditis. However, acute mastoiditis still occurs in children who are treated with antibiotics for an acute ear infection. In the Netherlands, where only 31% of AOM patients receive antibiotics, the incidence of acute mastoiditis is 4.2 per 100,000 person-years. In the United States, where more than 96% of patients with AOM receive antibiotics, the incidence of acute mastoiditis is 2 per 100,000 person-years. Despite the routine use of antibiotics, the incidence of acute mastoiditis has been rising in some cities. The pattern change may be secondary to the emergence of resistant S pneumoniae.
Lymphadenitis, parotitis, trauma, tumor, histiocytosis, OE, and furuncle.
Meningitis can be a complication of acute mastoiditis and should be suspected when a child has associated high fever, stiff neck, severe headache, or other meningeal signs. Lumbar puncture should be performed for diagnosis after imaging. Brain abscess occurs in 2% of mastoiditis patients and may be associated with persistent headaches, recurring fever, or changes in sensorium. Facial palsy, sigmoid sinus thrombosis, epidural abscess, cavernous sinus thrombosis, and thrombophlebitis may also be encountered.
Intravenous antibiotic treatment alone may be successful if there is no evidence of coalescence or abscess on CT. However, if there is no improvement within 24–48 hours, surgical intervention should be undertaken. Minimal surgical management starts with tympanostomy tube insertion, during which cultures are taken. If a subperiosteal abscess is present, incision and drainage is also performed, with or without a cortical mastoidectomy. Intracranial extension requires complete mastoidectomy with decompression of the involved area.
Antibiotic therapy (intravenous and topical ear drops) is instituted along with surgical management and relies on culture-directed antibiotic therapy for 2–3 weeks. An antibiotic regimen should be chosen which is able to cross the blood-brain barrier. After significant clinical improvement is achieved with parenteral therapy, oral antibiotics are begun and should be continued for 2–3 weeks. A patent tympanostomy tube must also be maintained with continued use of otic drops until drainage abates.
Prognosis for full recovery is good. Children that develop acute mastoiditis with abscess as their first ear infection are not necessarily prone to recurrent otitis media.
et al: Medical versus surgical treatment of pediatric acute mastoiditis: a systematic review. Laryngoscope 2019 Mar;129(3):754–760
et al: What is the best practice for acute mastoiditis in children? Laryngoscope 2014 May;124(5):1057–1058
ACUTE TRAUMA TO THE MIDDLE EAR
Head injuries, a blow to the ear canal, sudden impact with water, blast injuries, or the insertion of pointed objects into the ear canal can lead to perforation of the TM, ossicular chain disruption, facial nerve injury, hearing loss, vertigo, and hematoma of the middle ear. One study reports that 50% of serious penetrating wounds of the TM are due to parental use of a cotton swab.
If there is facial paralysis, severe vertigo or subjective hearing loss after ear trauma, urgent otolaryngology consultation is warranted. Middle ear trauma can lead to a perilymphatic fistula which is a breach of the inner ear that causes sensorineural (nerve) hearing loss and vertigo. This hearing loss can be prevented or reversed with emergent surgery. Facial nerve injury in the setting of middle ear trauma also often needs to be addressed emergently with possible facial nerve decompression. Other sequelae of middle ear trauma may be treated with observation. Blood collecting in the middle ear space may cause a conductive hearing loss that will resolve with time. Antibiotics are not necessary unless signs of infection appear. The patient needs to be followed with audiometry or by an otolaryngologist until hearing has returned to normal, which is expected within 6–8 weeks. If the conductive hearing loss does not resolve, there may be injuries to the ossicular chain. A CT scan may be needed to evaluate the middle ear structures in this case.
Traumatic TM perforations should be referred to an otolaryngologist for examination and hearing evaluation. Spontaneous healing may occur within 6 months of the perforation. In the acute setting, antibiotic eardrops are often recommended to provide a moist environment which is thought to speed healing.
EAR CANAL FOREIGN BODY & CERUMEN IMPACTION
Foreign bodies of the ear canal, both intentional (placement by patient or other person) or accidental (eg, insect, playground mulch), are common in childhood. Cerumen can also be obstructive, acting like a foreign body. These objects can be removed if they are easy to visualize and there is appropriate instrumentation. Factors that may make them difficult to remove include size of foreign body, particularly when large enough to obscure the TM, rounded or globular objects, and objects deep in the ear canal adjacent to the TM. If these conditions exist or you are unable to remove the object on the first attempt, an ENT referral is often necessary for resolution. Vegetable matter should never be irrigated as it can swell and become more difficult to remove. An emergency condition exists if the foreign body is a disk-type battery. An electric current is generated in the moist canal, and a severe burn can occur in less than 4 hours. If the TM cannot be visualized, assume a perforation and avoid irrigation or ototoxic medications.
Cerumen impaction is common in children and using cotton swabs or other devices in the ear canal predisposes to the problem. Not only do these objects block the natural outflow of cerumen, they can also increase cerumen production from irritation. Cerumen impaction should be removed if it is symptomatic or obstructs visualization of the TM. Education on the proper techniques of ear cleaning (such as only cleaning the external meatus) is important to avoid impactions. Explaining to parents that cerumen production is normal and cerumen protects the ear canal skin can help reinforce proper ear care.
LC: Foreign bodies of the ear, nose and throat. Emerg Med Clin North Am 2019 Feb;37(1):121–130
SR: Clinical practice guideline (update). Otolaryngol Head Neck Surg 2017 Jan;156(1 suppl):S1–S29. doi: 10.1177/0194599816671491.28045591
GA: Pediatric battery-related emergency department visits in the United States, 1990–2009. Pediatrics 2012 Jun;129(6):1111–1117
Trauma to the outer ear can result in formation of a hematoma between the perichondrium and cartilage of the pinna. This is different from a bruise, which does not change the ear shape and where the blood is in the soft tissue outside of the perichondral layer. A hematoma appears as a boggy purple swelling of the cartilaginous auricle, and the normal folds of the ear are obscured. If untreated, neocartilage is deposited after 7–10 days resulting in “cauliflower ear.” To prevent this cosmetic deformity, physicians should urgently refer patients to an otolaryngologist for drainage and application of a pressure dressing.
CONGENITAL EAR MALFORMATIONS
The external ear and EAC start to develop at 3 weeks gestation. Variable anomalies can present depending on timing of abnormal development. Atresia is failure of the ear canal to form. This results in conductive hearing loss and should be evaluated within the first 3 months of life by an audiologist and otolaryngologist. Microtia is the term used for an external ear that is small, collapsed, or only has an earlobe present. Usually there is a deficiency of cartilage and tissue. Anotia refers to an absent external ear. Often, there is an associated atresia with microtia and anotia. Reconstruction of the auricle usually occurs around ages 6–8 years and requires introduction of additional tissue or implants.
Malformations of the auricle can occur that are not due to a deficiency of tissue. This can range from ears that lack the proper folds and therefore protrude from the skull (prominotia) to ears that are folded over due to lack of cartilage stiffness or in utero positioning. Taping of the ears into correct anatomic position is very effective if performed in the first 72–96 hours of life. Tape is applied over a molding of wax or plastic and continued for at least 2 weeks. If this window of opportunity is missed or taping is unsuccessful, surgical correction, called an “otoplasty,” can be performed at school age.
An ear is considered “low-set” if the upper pole is below eyebrow level. This condition is often associated with other congenital anomalies, and in these patients a genetics evaluation should be considered.
Preauricular tags, ectopic cartilage, fistulas, and cysts require surgical correction primarily for cosmetic reasons. Since the inner ear forms in conjunction with the outer ear children with ear malformations should have their hearing tested. Renal ultrasound should be considered, as external ear anomalies can also be associated with renal anomalies, as both structures form during the same period of embryogenesis. Most preauricular pits are asymptomatic but may become infected and require antibiotic treatment and possible surgical management.
et al: Neonatal ear molding: timing and technique. Pediatrics 2016 Mar;137(3): e20152831
et al: Microtia reconstruction. Facial Plast Surg Clin North Am 2016 Nov;24(4):577–591
IDENTIFICATION & MANAGEMENT OF HEARING LOSS
Hearing loss is classified as being conductive, sensorineural, or mixed in nature. Conductive hearing loss occurs when sound transmission is blocked somewhere between the opening of the external ear and the cochlear receptor cells. The most common cause of conductive hearing loss in children is fluid in the middle ear. Sensorineural hearing loss (SNHL) is due to a defect in the neural transmission of sound, arising from a defect in the cochlear hair cells or the auditory nerve. Mixed hearing loss is characterized by elements of both conductive and sensorineural loss.
Hearing is measured in decibels (dB). The threshold, or 0 dB, refers to the level at which a sound is perceived in normal subjects 50% of the time. Hearing is considered normal if an individual’s thresholds are within 20 dB of normal. In children, severity of hearing loss is commonly graded as follows: 20–40 dB mild, 41–55 dB moderate, 56–70 dB moderately severe, 71–90 dB severe, and 91+ dB profound.
Hearing loss can significantly impair a child’s ability to communicate and hinder academic, social, and emotional development. Studies suggest that periods of auditory deprivation may have enduring effects on auditory processing, even after normal hearing is restored. Even a unilateral loss may be associated with difficulties in school and behavioral issues. Early identification and management of any hearing loss is therefore critical.
The most common cause of childhood conductive hearing loss is otitis media and related conditions such as MEE and ETD. Other causes may include EAC atresia or stenosis, TM perforation, cerumen impaction, cholesteatoma, and middle ear abnormalities, such as ossicular fixation or discontinuity. Often, a conductive loss may be corrected with surgery.
MEE may be serous, mucoid, or purulent, as in AOM. Effusions are generally associated with a mild conductive hearing loss that normalizes once the effusion is gone. The American Academy of Pediatrics recommends that hearing and language skills be assessed in children who have recurrent AOM or MEE lasting longer than 3 months.
Sensorineural Hearing Loss
SNHL arises due to a defect in the cochlear receptor cells or the auditory nerve (cranial nerve VIII). The loss may be congenital (present at birth) or acquired. In both the congenital and acquired categories, the hearing loss may be either hereditary (due to a genetic mutation) or nonhereditary. It is estimated that SNHL affects 2–3 out of every 1000 live births, making this the most common congenital sensory impairment. The incidence is thought to be considerably higher among the neonatal intensive care unit population. Well-recognized risk factors for SNHL in neonates include positive family history of childhood SNHL, birthweight less than 1500 g, low Apgar scores (0–4 at 1 minute or 0–6 at 5 minutes), craniofacial anomalies, hypoxia, in-utero infections (eg, TORCH syndrome), hyperbilirubinemia requiring exchange transfusion, and mechanical ventilation for more than 5 days.
A. Congenital Hearing Loss
Approximately 50% of congenital hearing loss is nonhereditary. Examples include loss due to infection, teratogenic drugs, and perinatal injuries. The other 50% is attributed to genetic factors. Among children with hereditary hearing loss, approximately one-third of cases are thought to be due to a known syndrome, while the other two-thirds are considered nonsyndromic.
Syndromic hearing loss is associated with malformations of the external ear or other organs, or with medical problems involving other organ systems. Over 400 genetic syndromes that include hearing loss have been described. All patients being evaluated for hearing loss should be also evaluated for features commonly associated with these syndromes. These include branchial cleft cysts or sinuses, preauricular pits, ocular abnormalities, white forelock, café au lait spots, and craniofacial anomalies. Some of the more frequently mentioned syndromes associated with congenital hearing loss include the following: Waardenburg, branchio-oto-renal, Usher, Pendred, Jervell and Lange-Nielsen, and Alport.
Over 70% of hereditary hearing loss is not related to a syndrome (ie, there are no associated visible abnormalities or related medical problems). The most common known mutation associated with nonsyndromic hearing loss is in the GJB2 gene, which encodes the protein Connexin 26. The GJB2 mutation has a carrier rate of about 3% in the general population. Most nonsyndromic hearing loss, including that due to the GJB2 mutation, is autosomal recessive.
Hereditary hearing loss may be delayed in onset, as in Alport syndrome and most types of autosomal dominant nonsyndromic hearing loss. Vulnerability to aminoglycoside-induced hearing loss has also been linked to a mitochondrial gene defect.
Nongenetic etiologies for delayed-onset SNHL include exposure to ototoxic medications, meningitis, autoimmune or neoplastic conditions, noise exposure, and trauma. Infections such as syphilis or Lyme disease have been associated with hearing loss. Hearing loss associated with congenital cytomegalovirus (CMV) infection may be present at birth, or may have a delayed onset. The loss is progressive in approximately half of all patients with congenital CMV-associated hearing loss. Other risk factors for delayed-onset, progressive loss include a history of persistent pulmonary hypertension and extracorporeal membrane oxygenation therapy.
C. Congenital CMV Infection and Hearing Loss
Congenital CMV (cCMV) associated hearing loss can be congenital, but is more often acquired. It deserves special mention as it is believed to be the most common cause of nongenetic hearing loss in the US pediatric population, and it can present at birth or have a delayed onset as long as several years. Congenital CMV is the most common in utero infection in the United States today, and an estimated 0.5%–1% of all newborns are infected in the prenatal period. While some manifest severe symptoms at birth such as petechiae, hyperbilirubinemia, hepatosplenomegaly, seizures, neurologic deficits and retinitis, 90%–95% of newborns with cCMV infection are completely asymptomatic. Thirty to fifty percent of symptomatic and 8%–12% of asymptomatic infants will go on to develop SNHL in early childhood. Congenital CMV infection can only be confirmed in the newborn period, so given that most newborns are asymptomatic during this period, it has been very difficult to accurately determine the percentage of SNHL attributable to cCMV. There is currently no universal screening for cCMV, but with the increased awareness of this problem, some centers are now performing targeted screening on newborns who fail newborn hearing screening. There is no consensus on the management of cCMV but trials of valganciclovir have shown promise in the treatment of cCMV hearing loss.
Identification of Hearing Loss
A. Newborn Hearing Screening
Prior to the institution of universal newborn screening programs, the average age at identification of hearing loss was 30 months. Recognizing the importance of early detection, in 1993, a National Institutes of Health Consensus Panel recommended that all newborns be screened for hearing impairment prior to hospital discharge. Today, all 50 states and the District of Columbia have Early Hearing Detection and Intervention (EHDI) laws or screening programs, and as of 2014, 97% of newborns in the United States were screened for hearing loss. The EHDI goal is loss identification and confirmation of hearing loss by 3 months of age, and appropriate intervention by the age of 6 months. Subjective testing is not reliable in infants, and therefore objective, physiologic methods are used for screening. Auditory brainstem response and otoacoustic emission testing are the two commonly employed screening modalities.
B. Audiologic Evaluation of Infants and Children
A parent’s report of his or her infant’s behavior cannot be relied upon for identification of hearing loss. A deaf infant’s behavior can appear normal and mislead parents and professionals. Deaf infants are often visually alert and able to actively scan the environment which may be mistaken for an appropriate response to sound. In children, signs of hearing loss include inconsistent response to sounds, not following directions, speech and language delays, and turning the volume up on televisions or radios. Any child who fails a hearing screening or is suspected to have hearing loss should be referred for formal audiometric testing by an audiologist who is knowledgeable about testing the pediatric population.
Audiometry subjectively evaluates hearing. There are several different methods used, based on patient age:
Behavioral observational audiometry (birth to 6 months): Sounds are presented at various intensity levels, and the audiologist watches closely for a reaction, such as change in respiratory rate, starting or stopping of activity, startle, head turn, or muscle tensing. This method is highly tester-dependent and error-prone.
Visual reinforcement audiometry (6 months to 2.5 years): Auditory stimulus is paired with positive reinforcement. For example, when a child reacts appropriately by turning toward a sound source, the behavior is rewarded by activation of a toy that lights up. After a brief conditioning period, the child localizes toward the tone, if audible, in anticipation of the lighted toy.
Conditioned play audiometry (2.5–5 years): The child responds to sound stimulus by performing an activity, such as putting a peg into a board.
Conventional audiometry (5 years and up): The child indicates when he or she hears a sound.
Objective methods such as auditory brainstem response and otoacoustic emission testing may be used if a child cannot be reliably tested using the above methods.
In addition to infants who fall into the high-risk categories for SNHL as outlined earlier, hearing should be tested in children with a history of developmental delay, bacterial meningitis, ototoxic medication exposure, neurodegenerative disorders, or a history of infection such as mumps or measles. Children with bacterial meningitis should be referred immediately to an otolaryngologist, as cochlear ossification can necessitate urgent cochlear implantation. Even if a newborn screening was passed, all infants who fall into a high-risk category for progressive or delayed-onset hearing loss, such as in utero CMV exposure, should be referred for regular audiologic monitoring for the first 3 years and at appropriate intervals thereafter to avoid a missed diagnosis.
Management of Hearing Loss
While identification of hearing loss is the critical first step, it is largely meaningless without appropriate follow-up and management. Prompt and appropriate intervention can minimize the potential lifelong detrimental effects that hearing loss can have on language, academic, emotional and social development, and hearing-related quality of life. Optimal management requires a multidisciplinary approach. The team may include audiologists, otolaryngologists, speech-language pathologists, early intervention specialists, deaf-hard of hearing educational specialists and family counselors.
Any child with confirmed hearing loss should be referred to an otolaryngologist for further evaluation and possible medical and/or surgical management. Etiologic workup can include radiographic imaging and/or laboratory tests and should be tailored to each individual patient’s history, examination, and audiometric results. Within the past decade, major advances have made comprehensive genetic testing for syndromic and nonsyndromic hearing loss using next-generation sequencing technology available to the public.
The medical management of hearing loss depends upon the type and severity. Conductive hearing loss is sometimes fixable if the point at which sound transmission is compromised can be corrected. For example, hearing loss due to chronic effusions usually normalizes once the fluid has cleared, whether by natural means or by the placement of tympanostomy tubes. As of yet, SNHL is not reversible. Most sensorineural loss is managed with amplification. Cochlear implantation is an option for some children when benefit is no longer achieved with amplification. It is FDA approved down to the age of 12 months. Unlike hearing aids, cochlear implants do not amplify sound, but directly stimulate the cochlea with electrical impulses.
When a hearing loss is diagnosed, it is not known whether it will remain stable, or if it will fluctuate or worsen. Therefore, children with hearing loss should receive ongoing audiologic monitoring, as well as periodic assessments of global development and functional performance.
The Individuals with Disabilities Education Act 2004 (IDEA 2004) is a United States law mandating free access to individualized educational opportunities with qualified instructors for children with disabilities, including hearing loss. Part C provides early intervention for children up to age 3 years. Audiologists play an important role in the transition from diagnosis to intervention, as they are required to initiate a referral to their state’s Part C program within 7 days of a new confirmed permanent hearing loss diagnosis. An Individualized Family Service Plan (IFSP) is developed and may enlist the services of audiologists, speech-language pathologists, and other related professionals. The IFSP is family-centered, and family support services are often available. Between the ages of 3 and 21 years, children are covered under Part B of IDEA 2004. Part B is managed through the local school systems’ special education programs. Under Part B, an Individualized Education Plan (IEP) is developed with a goal of maximizing a child’s academic success.
Appropriate care may treat or prevent certain conditions causing hearing deficits. Aminoglycosides and diuretics, particularly in combination, are potentially ototoxic and should be used judiciously and monitored carefully. Given the association of a mitochondrial gene defect with aminoglycoside ototoxicity, use should be avoided when possible, in patients with a family history of aminoglycoside-related hearing loss. Reduction of repeated exposure to loud noises may prevent high-frequency hearing loss associated with acoustic trauma. Any patient with sudden-onset SNHL should be seen by an otolaryngologist immediately, as in some cases, steroid therapy may reverse the loss if initiated right away.