The importance of pediatricians and family physicians having an understanding of and experience with otolaryngologic problems and being skilled in techniques of examination of the head and neck region cannot be overemphasized. One study revealed that more than one third of all visits to pediatricians’ offices were prompted by ear symptoms. When nasal and oral symptoms are included, ear, nose, and throat pathology accounts for more than 50% of all visits. With patience and proper equipment, pediatricians can complete a thorough examination on almost all children. If a disorder fails to respond to therapy or becomes chronic or recurrent, or if an unusual problem is encountered, then consultation with a pediatric otolaryngologist should be sought.
Successful examination of the ears, nose, and oropharynx of a young child can present some challenges, especially with older infants and toddlers, who fail to appreciate the need for (and thus often vigorously resist) examination. This can be a particular problem in children who have had previous bad experiences. Patience, warmth, humor, and careful explanation on the part of the examiner help reduce fear and enhance cooperation.
Whenever possible, the child should be allowed to sit on the parent’s lap. Pacifiers, puppets and other toys, and tongue blades with faces drawn on them can all serve to reduce anxiety, enlist the child’s trust, and distract attention. Gradual introduction of the equipment can also be helpful, especially if done in a playful way. The child can be asked to blow out the otoscope light while the examiner turns it off, urged to catch the light spot as the examiner moves it around, and even allowed to look in the parent’s or examiner’s ears (Fig. 23-1, A-D). Parents can also help demonstrate maneuvers for opening the mouth, panting to depress the tongue, and holding the head back. Although this may take a little additional time at the outset, it often saves considerable time in the long run and makes future follow-up examinations far easier.
Figure 23-1 Techniques to assist examination of a child’s ears, nose, and oropharynx. A, Young infants often can be examined on their mother’s lap, with gentle immobilization provided by the parent and the examiner’s hand. B, Having a toddler or preschooler sit on the mother’s lap and using puppets, other toys, and tongue blades with faces drawn on them while gradually introducing the examining instruments reduces anxiety and enlists cooperation. C and D, Making a game of blowing out the otoscope light and allowing the patient to check the examiner first convey that otoscopy does not have to hurt.
Ear pain (otalgia), discharge from the ear (otorrhea), and suspected hearing loss are three of the more common and specific otic symptoms for which parents seek medical attention for their children. Less specific symptoms such as pulling or tugging at the ears, fussiness, and fever are also frequently encountered, particularly in children younger than 2 years of age.
History should center on the nature and duration of symptoms, character of the clinical course, and possible antecedent treatment. Because many infections of the ear are recurrent and/or chronic, the parent should be asked about previous medical or surgical therapy (e.g., antibiotics, myringotomy, tube insertion).
A brief review of the anatomy of the ear is helpful in developing a logical approach to any clinical abnormalities that may be encountered. The ear is conveniently divided into the following three regions (Fig. 23-2):
Figure 23-2 Anatomy of the ear. A, A normal external ear (auricle or pinna) is shown, with its various landmarks labeled. It is helpful to refer to such a diagram in assessing congenital anomalies. B, This coronal section shows the various structures of the hearing and vestibular apparatus. The three main regions are the external ear, middle ear, and inner ear. The eustachian tube connects the middle ear and the nasopharynx and serves to drain and ventilate the middle ear.
The examination should include inspection of the auricle, periauricular tissues, and external auditory canal and visualization of the entire tympanic membrane, including assessment of its mobility in response to positive and negative pressure with pneumatic otoscopy. This necessitates clearing the canal of cerumen or discharge by using a curette, cotton wick, lavage, or suction, and presumes that the tympanic membrane is intact (Fig. 23-3, A and B). Use of a surgical otoscope head or an examining microscope assists visualization during the cleaning process. These procedures should be performed carefully and gently and attempted only after the child has been carefully immobilized to avoid trauma (Fig. 23-4). It is extraordinarily easy to injure the canal during the process of cleaning the external ear. Hence great care must be taken; otherwise bleeding from the ensuing trauma obscures the examination and upsets the patient and parent. Steadying of the examiner’s hands on the child’s head while using the otoscope and instruments is an essential skill to avoid ear trauma. Both the patient and parent should be given a clear explanation of the procedure beforehand. Allowing older children to handle and look through the equipment before cleaning the ear reduces anxiety and enhances cooperation (see Fig. 23-3, C).
Figure 23-3 A, Equipment for cleaning the external auditory canal. The curette (1) is the implement most commonly used to remove cerumen. Use of a surgical otoscope head (2) makes the process considerably easier. Additional implements include cotton wicks (3) and a suction tip (4) for removal of discharge or moist wax, alligator forceps (5) for removing foreign bodies, and an ear syringe (6) and motorized irrigation apparatus (7) for removing firm objects or impacted cerumen. Lavage is contraindicated when there is a possible perforation of the tympanic membrane. If the motorized apparatus is used for irrigation, it must be kept on the lowest power setting to avoid traumatizing the eardrum. B, Use of suction is often necessary when there is copious exudate. C, Allowing the child to look through the examining microscope may help him or her cooperate with the examination.
Figure 23-4 Method of immobilization for cleaning. An assistant holds the child’s arms and simultaneously immobilizes the child’s head with the thumbs. The parent firmly holds the hips and thighs. This critical immobilization prevents dangerous motion by the child during cleaning of the ear canal and is also useful for otoscopy in young children.
Because the external auditory canal is often angulated in infants and young children, gentle lateral traction on the pinna is frequently necessary to assist visualization of the eardrum itself (Fig. 23-5). In infants the tympanic membrane tends to be oriented at a greater angle (Fig. 23-6, A and B); the landmarks are less prominent; and the skin that lines the canal, being loosely attached, moves readily on insufflation of air, simulating a normally mobile eardrum. To avoid confusion, the canal should be inspected as the speculum is inserted to ensure that the transition between canal wall and tympanic membrane is visualized.
Figure 23-5 Because the external auditory canal usually is angulated in children, lateral traction on the pinna is often required to straighten the canal and improve visualization of the tympanic membrane.
Figure 23-6 Angulation of the tympanic membrane in infancy. A, The relationship between the ear canal and eardrum is different in the infant, with the drum being tilted at an angle of 130 degrees. B, Greater care is required in examining an infant’s eardrum because of this angulation and because the landmarks are less prominent.
The pneumatic otoscope is the most valuable diagnostic tool when signs or symptoms of otitis media are present. Pediatricians, family practitioners, and otolaryngologists who treat children should be skilled in its use. Practical advice on the use of this instrument is as follows:
When otoscopic findings are unclear or when it is difficult to obtain a good air seal for pneumatic otoscopy, tympanometry can be highly useful in evaluating patients older than 8 months of age (Fig. 23-7). The procedure is not of value in young infants because the abundance of loose connective tissue lining the ear canal and the laxity of the cartilage at the entrance increase canal wall compliance and invalidate the results.
(Courtesy Mrs. Ruth Bachman, Pittsburgh, Pa.)
Because otitis media can be a reflection of both immunologic and anatomic abnormalities, the practitioner should be suspicious of possible underlying immune or temporal bone defects when seeing patients with chronic or frequently recurrent otitis media. The temporal bone is the bony housing for the auditory and vestibular systems. In addition, it provides bony protection for the facial nerve as it crosses from the brainstem to the facial muscles. The growth and development of this bone are affected in syndromes such as Treacher Collins that are characterized by altered midface growth (Fig. 23-8). The soft tissues attached to the temporal bone, such as the muscles controlling eustachian tube function, can be abnormal in children with cleft palates (see Palatal Disorders, later). As a result, children with these disorders tend to have an increased incidence of otitis media.
Figure 23-8 Syndromes affecting the growth of the temporal bone and midface that predispose patients to recurrent or chronic otitis media and chronic recurrent sinus infections. A and B, Treacher Collins syndrome. Note the maxillary hypoplasia, micrognathia, and auricular deformity. C, Apert syndrome. D and E, Crouzon syndrome. Both Apert and Crouzon syndromes are characterized by severe maxillary and midfacial hypoplasia. F, Hemifacial microsomia with unilateral hypoplasia.
(B–F, Courtesy Wolfgang Loskin, MD, University of North Carolina, Chapel Hill, N.C.)
Children with chronic effusions who complain of hearing loss, those whose parents complain that they do not listen, those with speech delays, or those with suspected congenital malformations must have their hearing evaluated by audiometry, evoked otoacoustic emissions, or brainstem evoked potentials. Patients with vertigo and/or problems of balance and those with facial weakness or asymmetry warrant testing of hearing, facial nerve, and vestibular function. These children, and those suffering from malformations, may require computed tomography (CT), magnetic resonance imaging (MRI), or genetic studies in select cases to clarify the nature of the problem.
Examination of each child’s ear begins with inspection of the auricle and periauricular tissues for four important signs—discharge, displacement, discoloration, and deformity (the “four D’s”). The canal is normally smooth and angulated slightly in an anterior direction. Cerumen is often present; it varies in color from yellowish-white to tan to dark brown. It is secreted from glands interspersed among the hair follicles at the entrance to the ear canal, and it may have some bacteriostatic activity. When cerumen obstructs the view, it must be removed to allow adequate visualization of the canal and tympanic membrane. When soft and moist, cerumen is easily removed with a curette. Removal may be more difficult if the cerumen is dry and flaky and may require removal with otologic forceps or, at times, instillation of drops. In some children, cerumen solidifies or has been packed in with cotton-tipped applicators, forming a firm plug that impedes sound conduction and requires softening for removal. Irrigation is contraindicated if there is any possibility that the eardrum may not be intact, as infection may be driven into the ear by blind irrigation.
Discharge is a common complaint with a number of possible causes. When there is thick, white discharge and erythema of the canal wall, the physician should gently pull on the pinna. If this maneuver elicits pain and the canal wall is edematous, primary otitis externa is the likely diagnosis (Fig. 23-9), although prolonged drainage from untreated otitis media with perforation may present a similar picture (see Disorders of the Middle Ear, later). When the middle ear is the source of otic discharge, the tympanic membrane is abnormal and should show evidence of perforation (see Fig. 23-26). The major predisposing condition to primary otitis externa is prolonged excessive moisture in the ear canal, which promotes bacterial or fungal overgrowth. Thus this is a common problem in swimmers. Another major source is the presence of a foreign body in the ear canal (see Fig. 23-19), which stimulates an intense inflammatory response and production of a foul-smelling purulent discharge. Thus when otic drainage is encountered, the discharge must be gently removed under appropriate magnification to assess the condition of the tympanic membrane and to rule out the presence of perforation and foreign objects. This can be accomplished either by gentle siphoning and wiping with cotton wicks or by careful suctioning (see Fig. 23-3).
Figure 23-9 External otitis. Acute bacterial external otitis is characterized by intense pain that is worsened by traction on the pinna, along with purulent exudate and intense canal wall inflammation with normal tympanic membrane mobility on pneumatic otoscopy.
If the history indicates that the drainage is persistent or recurrent despite therapy, a culture should be obtained to determine both the causative organism and its sensitivity to antimicrobial agents. Treatment consists primarily of topical otic antimicrobial/steroid preparations. Systemic antibiotics should be given when pain is severe; when there is evidence of otitis media; or when, despite attempts at cleaning, there is still uncertainty about an infection of the middle ear. Parenteral antibiotics may be required when the process has extended, producing cellulitis of the periauricular soft tissues or frank mastoiditis.
Displacement of the pinna away from the skull is a worrisome sign. The most severe condition causing displacement is mastoiditis, resulting from extension of a middle ear infection through the mastoid air cells and out to the periosteum of the skull. In addition to displacement, important clinical signs of mastoiditis include erythema and edema and possibly fluctuance of the skin overlying the mastoid, exquisite tenderness on palpation of the mastoid process, a sagging ear canal, purulent otorrhea, fever, and usually toxicity (Fig. 23-10). This condition is now considered unusual and is seen mainly in patients with long-standing, untreated, or inadequately treated otitis media. Recognition, prompt institution of parenteral antibiotic therapy, and myringotomy with culture and sensitivity testing are crucial because there is significant risk of central nervous system (CNS) extension. The use of a CT scan will delineate the extent of involvement and assist the surgical approach (Fig. 23-11). Mastoidectomy is indicated in cases complicated by bone erosion, or CNS extension, and in those in whom intravenous (IV) antibiotics and myringotomy fail to produce complete resolution.
Figure 23-10 Mastoiditis. A, This frontal photograph clearly shows the left auricle displaced anteriorly and inferiorly. B, In another patient, viewed from the side, erythema can be appreciated over the mastoid process. C, On otoscopy, erythema and edema of the canal wall are evident and the posterosuperior portion of the canal wall sags inferiorly.
(C, Courtesy Michael Hawke, MD.)
Other conditions characterized by displacement of the pinna away from the head include parotitis, primary cellulitis of the periauricular tissues, and edema secondary to insect bites or contact dermatitis. Parotitis is differentiated by finding prominent induration and enlargement of the parotid gland anterior and inferior to the external ear, together with blunting of the angle of the mandible on palpation (see Chapter 12). Primary cellulitis is characterized by erythema and tenderness but can often be distinguished clinically from mastoiditis by the presence of associated skin lesions that antecede the inflammation (Fig. 23-12). In cases secondary to untreated external otitis or otitis media with perforation, the picture may be clinically similar. Localized contact dermatitis and angioedema may be erythematous, but they are also pruritic and nontender. The former condition is characterized by microvesicular skin changes (Fig. 23-13), whereas in the latter condition, a precipitating insect bite can often be identified on inspection (Fig. 23-14).
Figure 23-12 Periauricular and auricular cellulitis. This infant had mild postauricular seborrhea and developed varicella. The vesicular varicella lesions that clustered at sites of prior skin irritation became secondarily infected with group A β-streptococci, resulting in cellulitis with intense erythema, edema, and tenderness of the auricle and periauricular tissues. In this case the external canal was normal.
(Courtesy Ronald Chludzinski, MD.)
Figure 23-13 This young girl became sensitive to the nickel posts of her earrings and developed periauricular contact dermatitis. The auricle and periauricular skin are erythematous and covered by a weeping, pruritic microvesicular eruption.
(Courtesy Michael Sherlock, MD, Lutherville, Md.)
Figure 23-14 Angioedema. This youngster had pruritic, nonpainful, nontender erythema and swelling of his ear and infraorbital region. Close examination of the latter revealed the punctum of an insect bite. Another punctum on his ear was obscured by crusting following scratching.
(Courtesy Michael Sherlock, MD, Lutherville, Md.)
Discoloration is another important sign and is commonly a feature of conditions producing displacement. Erythema of the pinna is common when there is inflammation, with or without infection (see Fig. 23-10, B and Figs. 23-12 to 23-14). Ecchymotic discoloration may be encountered with trauma. When this overlies the mastoid tip, the area immediately posterior to the pinna, it is termed a Battle sign (Fig. 23-15, A) and usually reflects a basilar skull fracture. In such cases the canal wall should be checked for tears and the tympanic membrane for perforation or a hemotympanum (blood behind the tympanic membrane; Fig. 23-15, B). These findings are generally more helpful in making the diagnosis than routine skull x-rays, which are often inconclusive. CT can usually confirm the diagnosis of a basilar skull fracture. Recognition that bruising of the pinna and/or postauricular area can be a manifestation of child abuse is most important (see Chapter 6).
Figure 23-15 Basilar skull fracture. A, The presence of a basilar skull fracture involving the temporal bone is often signaled by postauricular ecchymotic discoloration, termed the Battle sign. B, The force of the blow may also cause tearing of the ear canal or, as shown here, middle ear hemorrhage with hemotympanum. Depending on the timing of examination, this may appear red or blue.
(B, Courtesy Michael Hawke, MD.)
When the external ear is grossly misshapen or microtic, associated anomalies of middle ear structures are common and hearing loss may be significant (Fig. 23-16, A and B). Severe deformities stem from developmental anomalies of the branchial arches, which contribute to both external ear and middle ear structures. Such abnormalities warrant a thorough evaluation in infancy to ensure early recognition and treatment of hearing loss. Deformity of the pinna can be the result of hereditary factors or exposure to teratogens, but at times it is simply produced by unusual intrauterine positioning. Most deformities are minor and represent isolated malformations of mostly cosmetic significance (Fig. 23-17, D). In some instances they may be part of a picture of multiple congenital anomalies (Fig. 23-17, A-C; see also Fig. 23-8 and Chapter 1).
Figure 23-16 A, Grade 3 microtia and congenital aural atresia of the right external ear. In this otherwise normal child, the pinna failed to develop properly and the external canal was atretic. Audiometric testing revealed a 60-dB hearing loss. B, Grade 2 microtia. Note deficiency of superior portion of auricle. Such isolated deformities stem from abnormal development of the first and second branchial arches.
Figure 23-17 Minor congenital auricular deformities. A, In this infant the superior portion of the helix is folded over, obscuring the triangular fossa; the antihelix is sharply angulated; and there are three preauricular skin tags. B, This neonate with orofaciodigital and Turner syndromes has a simple helix and a redundant folded lobule. The ear is low set and posteriorly rotated, and the antitragus is anteriorly displaced. C, This infant with Rubinstein-Taybi syndrome has an exaggerated elongated intertragal notch. D, Prominent ear in an otherwise normal child. The auricular cartilage is abnormally contoured, making the ear protrude forward.
(C, Courtesy Michael Sherlock, MD, Lutherville, Md.)
Trauma to the pinna may result in a hematoma between the skin and cartilage, the recognition of which requires otolaryngologic consultation. An unrecognized hematoma may result in a permanent deformity if left undrained, or if superinfection of the hematoma results. Either complication can result in permanent cosmetic damage to the pinna.
Preauricular sinuses and cysts constitute two of the more common congenital abnormalities. These are congenital remnants located anterior to the pinna with an overlying surface dimple (Fig. 23-18, A). These cysts are vulnerable to infection and abscess formation (Fig. 23-18, B), which necessitate needle aspiration or incision and drainage in conjunction with antistaphylococcal antibiotics. Once infection has occurred, recurrence is common unless the entire sinus is completely excised. This procedure should be undertaken once inflammation has subsided. A preauricular sinus may also result from branchio-oto-renal syndrome (also known as Melnick-Fraser syndrome), an autosomal dominant disorder characterized by bilateral preauricular sinuses, ear anomalies, branchial cleft anomalies in the neck, and renal problems.
It is not unusual for children to put paper, beads, and other foreign objects into their ear canals (Fig. 23-19, A). Small insects also on occasion may become trapped in the external ear (Fig. 23-19, B). In some cases small objects may be embedded in cerumen and missed on inspection. As noted earlier, if present for more than a few days, the foreign material stimulates an inflammatory response and production of a purulent discharge that is often foul-smelling and may obscure the presence of the inciting foreign body. Removal of some objects can be accomplished by use of otologic alligator forceps or by irrigation of the ear canal; others—particularly spherical objects—require use of a Day (right angle) hook or suction (Fig. 23-19, C; see also Fig. 23-3). Foreign objects may also be the cause of painful abrasions or lacerations of the external auditory canal or even perforation of the tympanic membrane. Insertion of pencils or sticks into the ear canal by the child and parental attempts to clean the canal with a cotton swab are the most common modes of such injury.
Figure 23-19 Otic foreign bodies. A, This child inserted a bead into her ear. The object must be removed carefully to prevent further trauma. B, This patient experienced a period of intense buzzing, pain, and itching in the ear that abated after a few hours. If the tympanic membrane is intact, olive or mineral oil may be used to drown the insect. C, A blunt-tipped, right-angled Day hook, small wire loop curette, Hartmann forceps, and an alligator forceps (and see Fig. 23-3, A) are useful instruments for removing foreign bodies from the external auditory canal.
Exposure to concussive forces such as a direct blow (which may be accidental or inflicted) or an explosion can also result in perforation (Fig. 23-20, A and B). Patients with traumatic perforations must be carefully assessed for signs of injury to deeper structures. If tympanic membrane perforation occurs as a result of penetration by a foreign object or of concussive forces, the physician must be particularly aware of the possibility of middle ear or inner ear damage. Evidence of hearing loss, vertigo, nystagmus, facial nerve injury, or cerebrospinal fluid leak should prompt urgent otolaryngologic consultation because an emergency surgical exploration may be indicated.
Figure 23-20 Traumatic perforations of the tympanic membrane. A, This 8-year-old boy’s tympanic membrane was perforated by a forceful slap on the ear. B, Even more severe damage with thickening and hemorrhage is seen in this victim of a blast injury caused by an explosion.
(A, Courtesy Michael Hawke, MD.)
The normal tympanic membrane is thin, translucent, neutrally positioned, and mobile. The ossicles, particularly the malleus, are generally visible through the membrane (Fig. 23-21). Adequate assessment of the tympanic membrane requires that the examiner note four major characteristics: (1) thickness, (2) degree of translucence, (3) position relative to neutral, and (4) mobility. Application of gentle positive and negative pressure, using a properly sealed pneumatic otoscope (Fig. 23-22), produces brisk movement of the eardrum when the ear is free of disease and abnormal movement when fluid is present, when the drum is thickened or scarred, or when there is an increase in either positive or negative pressure (Fig. 23-23). An abnormality in any one of the four major characteristics suggests middle ear pathology.
(Courtesy Sylvan Stool, MD.)
Figure 23-22 Pneumatic otoscopy. This procedure requires proper equipment including a pneumatic otoscope head and an appropriately sized speculum to achieve a good air seal. When a seal is difficult to obtain despite proper speculum size, the head and tubing should be checked for air leaks. If none is found, application of a piece of rubber tubing to the end of the speculum (shown attached to the otoscope) or use of a soft speculum (1) may solve the problem.
Figure 23-23 Technique and findings in pneumatic otoscopy. A, The speculum is inserted into the ear canal to form a tight seal. The bulb is then gently and slowly pressed and released while the mobility of the drum is assessed. Pressing on the bulb applies positive pressure; letting up applies negative pressure. B, A normal drum moves inward and then back. C, In cases of acute otitis media, in which the middle ear is filled with purulent material, the drum bulges toward the examiner and moves minimally. D, In cases of acute otitis media with an air–fluid level, mobility may be nearly normal. In some patients, however, the drum may be retracted, indicating increased negative pressure. If this is the case, mobility on positive pressure may be reduced whereas movement on negative pressure is nearly normal or only mildly decreased. E, This is the same pattern as that seen commonly in children with chronic serous otitis. F, In cases of high negative pressure and no effusion, application of positive pressure produces little or no movement, but on negative pressure the drum billows back toward the examiner.
Acute otitis media is the term used to describe acute infection and inflammation of the middle ear. Associated inflammation and edema of the eustachian tube mucosa appear to play key roles in the pathogenesis by impeding drainage of the middle ear fluid. In some children, anatomic or chronic physiologic abnormalities of the eustachian tube predispose to infection. The problem is commonly seen in conjunction with an acute upper respiratory tract infection, and its onset is often heralded by a secondary temperature spike one to several days after the onset of respiratory symptoms. The major offending organisms are bacterial respiratory pathogens. The most commonly isolated organisms and their relative frequency, shown in Table 23-1, demonstrate the rise of penicillin-resistant Streptococcus pneumoniae. A small proportion of cases constitute an exception to these percentages, that is, those in which otitis is accompanied by conjunctivitis. Nontypable Haemophilus influenzae is found to be causative in 70% to 75% of these cases. Increasing rates of β-lactamase positivity in these organisms, as well as the rising incidence of penicillin-resistant S. pneumoniae, has necessitated the use of high-dose amoxicillin and/or greater use of β-lactamase–resistant antibiotic regimens whenever this scenario is seen. Sulfa drugs may also be useful for therapy of community-acquired methicillin-resistant Staphylococcus aureus, which is becoming increasingly common.
|Bacterial Pathogen||Number of Isolates (% of Total)||Number Resistant (%)|
|Streptococcus pneumoniae||49 (44)||18 (37)|
|Haemophilus influenzae||46 (41)||21 (46)|
|Moraxella catarrhalis||16 (14)||16 (100)|
|Staphylococcus aureus||0 (0)||0 (0)|
|Total||111 (100)||55 (50)|
In acute otitis media the classic findings on inspection of the tympanic membrane are erythema and injection; bulging that obscures the malleus; thickening, often with a grayish-white or yellow hue, reflecting a purulent effusion; and reduced mobility (Fig. 23-24, A). However, crying produces erythema of the eardrum, and thus tympanic erythema in a crying child is of little diagnostic value. The patient is usually febrile and, if old enough, typically complains of otalgia. However, in many cases this “textbook picture” is not seen. This is probably due in part to time of presentation, the virulence of the particular pathogen, and host factors.
Figure 23-24 Acute otitis media. A, This is the textbook picture: an erythematous, opaque, bulging tympanic membrane. The light reflex is reduced, and the landmarks are partially obscured. Mobility is markedly reduced. B, In this acutely febrile child who complained of otalgia, the presence of both air and fluid formed bubbles separated by grayish-yellow menisci. Even though the drum was not injected, this finding, combined with fever and otalgia, is consistent with acute infection. C, In this child the tympanic membrane was injected at the periphery, and a yellow purulent effusion caused the inferior portion to bulge outward. Mobility was markedly reduced.
(A, Courtesy Michael Hawke, MD.)
Accuracy in diagnosis necessitates meticulous inspection during otoscopy and knowledge of the various modes of presentation. Children may have fever of a few hours’ duration and otalgia (or if very young, fever and irritability) yet have no abnormality on otoscopy. If re-examined the next day, many of these patients have clear evidence of acute otitis media. Some have erythema and bubbles or air–fluid or air–pus levels (a result of venting by the eustachian tube) without bulging and with nearly normal mobility of the eardrum (Fig. 23-24, B). In still other cases the drum may be full and poorly mobile with cloudy fluid behind it but with minimal erythema (Fig. 23-24, C). In some patients the drum is retracted, moves primarily or only in response to negative pressure, and shows signs of inflammation and/or a cloudy effusion.
On occasion the signs and symptoms of otitis media may be accompanied by formation of a bullous lesion on the surface of the tympanic membrane, a condition termed bullous myringitis (Fig. 23-25). These children usually complain of intense pain. Whereas this phenomenon is most commonly associated with Mycoplasma infection in adults, any of the usual pediatric pathogens (see Table 23-1) can be causative in children. Finally, acute otitis media may, by virtue of increasing middle ear pressure, result in acute perforation of the tympanic membrane. On presentation the canal may be filled with purulent material; however, tugging on the pinna usually does not elicit pain, and erythema and edema of the canal wall are minimal or absent. Cleansing with a cotton wick or suction usually reveals an inflamed drum with a barely visible perforation (Fig. 23-26).
Figure 23-25 Acute otitis media with bullous myringitis. This patient was febrile and extremely uncomfortable. On otoscopy an erythematous bullous lesion is seen obscuring much of the tympanic membrane. This phenomenon, called bullous myringitis, is caused by the usual pathogens of otitis media in childhood. The bullous lesion often ruptures and drains spontaneously, providing immediate relief of pain.
Figure 23-26 Acute otitis media with perforation. In this child, increased middle ear pressure with acute otitis resulted in perforation of the tympanic membrane. The drum is thickened, and the perforation is seen at the 9 o’clock position.
Just as clinical findings of acute otitis media vary, so do symptoms. Although some patients have severe otalgia, others may complain of sore throat, mild ear discomfort, ear popping, or decreased hearing yet have floridly inflamed eardrums. Fever may be absent. As a guideline, the American Academy of Pediatrics and the American Academy of Family Physicians recommend that the diagnosis of acute otitis media be made with (1) a history of acute onset of signs and symptoms, (2) the presence of middle ear effusion, and (3) signs and symptoms of middle-ear inflammation.
Radiographic studies are generally of little value in the diagnosis of acute otitis media. When a temporal bone CT scan is obtained in a patient with acute otitis media and fluid in the middle ear, fluid is often present in the mastoid cavity. This will be interpreted by a radiologist as opacification of the mastoid because it may be difficult to distinguish between the CT findings of acute otitis media and those of acute mastoiditis. In such instances it is important that the physician look at the patient’s clinical signs rather than rely on radiographic findings to make the diagnosis.
Treatment for acute otitis media may include the option of observation without use of antibiotics based on diagnostic certainty, age, illness severity, and assurance of follow-up. In addition to treating patients with an appropriate antimicrobial agent and analgesics when necessary, follow-up examination is important. This is best done 2 to 3 weeks after diagnosis, when complete resolution can be expected in more than 50% of children. The purpose of reevaluation is to identify those patients who have persistent serous effusions and require ongoing surveillance. Selected older patients with mild, uncomplicated acute otitis media may be observed without antimicrobial therapy, but follow-up examination is still necessary.
Serous effusion in the middle ear may result from an upper respiratory tract infection, or it may be the residual of treated acute otitis. In many instances this effusion is not spontaneously cleared but instead remains in the middle ear for weeks or months, resulting in a persistent clear gray or yellow effusion behind the eardrum (Fig. 23-27). Persistence appears to result in part from eustachian tube dysfunction with poor drainage and ventilation. Pneumatic otoscopy often reveals poor mobility of the tympanic membrane, and mobility is noted primarily on negative pressure. The latter is thought to develop as a result of absorption of middle ear gases by mucosal cells, creating a vacuum that persists with the fluid because of failure of ventilation by the eustachian tube. Such long-standing effusions impair hearing. Persistence of bilateral serous effusions for more than 3 to 4 months with hearing loss is an indication for myringotomy and insertion of tubes (see Fig. 23-29, A and B) to improve hearing.
Figure 23-27 Serous otitis media. This patient has a chronic serous middle ear effusion. The tympanic membrane is retracted, thickened, and shiny. Behind it is a clear yellow effusion. Mobility was decreased and primarily evident on negative pressure. The child was not acutely ill but did have decreased hearing.
(Courtesy Sylvan Stool, MD.)
Chronic or chronic–recurrent otitis media with effusion (COME) is common in young children. Patients subject to this condition appear to have significant and prolonged eustachian tube dysfunction. This “otitis-prone” state may be a seemingly isolated phenomenon, or it can be a feature of a number of syndromes characterized by palatal dysfunction or malformation or by facial hypoplasia or deformity. These conditions include cleft palate, Crouzon syndrome, Down syndrome, and the mucopolysaccharidoses and mucolipidoses (see Fig. 23-8; and see Palatal Disorders, later). Chronic obstructive adenoidal hypertrophy may also be a predisposing condition. Less commonly, immunodeficiency and immotile cilia syndrome are identified as underlying etiologic conditions.
Chronic otitis media is associated with significant morbidity in terms of intermittent or chronic hearing impairment, intermittent discomfort, and the sequelae of recurrent infection. Over months or years, the process produces permanent myringosclerotic changes in which the tympanic membrane becomes whitened, thickened, and scarred (Fig. 23-28, A). Chronic perforations are common (Fig. 23-28, B). Patients with persistent middle ear infections despite medical therapy, those with frequent recurrences, and children with chronic severe tympanic membrane retraction (Fig. 23-28, C) appear to benefit from surgical drainage and insertion of tympanostomy tubes that vent the middle ear (Fig. 23-29). Persistence of a serous effusion for longer than 3 to 4 months with significant hearing loss is also an indication for myringotomy and insertion of tubes. Once placed, tubes should be checked at intervals for presence and patency. Spontaneous extrusion generally occurs 6 to 24 months after insertion. When tubes have been inserted, it is wise to prevent contamination of the middle ear with water. The need for earplugs in children with tubes or a perforation is the subject of some controversy, but in general their use is still recommended.
Figure 23-28 Sequelae of chronic otitis media. A, Much of this child’s tympanic membrane is scarred and thickened, and a thinned dimeric area balloons out of the inferior central portion. B, The eardrum is markedly thickened, scarred in an arc from 12 to 5 o’clock, and has a large chronic perforation. C, Severe retraction of the tympanic membrane is seen in this patient. The membrane adheres to the malleus.
(A and B, Courtesy Sylvan Stool, MD; C, courtesy Alejandro Hoberman, MD, Children’s Hospital of Pittsburgh, Pittsburgh, Pa.)
Figure 23-29 A, Tympanic membrane of patient with a history of chronic otitis media, with tympanostomy tube in place. The tube serves to vent the middle ear, improve hearing, and reduce the frequency of infection. B, The tympanic membrane is slightly smaller than the size of a dime. A typical tube takes up approximately 15% of the tympanic membrane’s surface area. There are many different types in a variety of shapes, materials, sizes, and colors. Selection is based on specific pathology and surgeon preference.
(A, Courtesy Sylvan Stool, MD.)
Earplugs come in all shapes and sizes. They vary in cost from inexpensive, premolded earplugs to expensive, custom-molded devices. The general purpose of an earplug is to prevent water from entering the external ear canal and contaminating the middle ear space. “Wax” earplugs act like a putty that can be molded into the particular shape that comfortably blocks the individual’s ear canals. A preformed earplug is held in place by the conchal bowl and provides reasonable protection for children. Although custom-made earplugs are not of critical importance, they do provide a better fit and are more comfortable, and compliance with their use tends to be greater. Children have a propensity to lose or misplace these devices, and it is best to focus on obtaining functional earplugs that are easily replaceable at minimal cost.
The most common and one of the most serious mass lesions of the eardrum is a cholesteatoma. It can present as a defect in the tympanic membrane through which persistent drainage occurs, or it can appear as a white cystic mass behind or involving the eardrum. It consists of trapped epithelial tissue that grows beneath the surface of the membrane (Fig. 23-30). Although a few are congenital with an intact tympanic membrane, the majority are sequelae of untreated or chronic–recurrent otitis media with a tympanic membrane defect. If a cholesteatoma is not removed surgically, it continues to enlarge; becomes locally destructive; and can erode the mastoid bone, destroy the ossicles, and even invade the inner ear structures or cranium. Progressive hearing impairment is usually a feature of this condition. Congenital cholesteatoma is rare. It is seen under an intact tympanic membrane in the anterior superior quadrant of the middle ear.
Figure 23-30 Cholesteatomas. A, Congenital cholesteatoma noted in a young child with spontaneous ear drainage. No previous history of ear infections existed. B and C, Acquired cholesteatomas, which generally present after a long history of chronic middle ear disease.
Granulomas or polyps of the tympanic membrane (Fig. 23-31) can also develop in children with chronic middle ear infections. The most common cause of aural polyps in children is an old, retained tympanostomy tube. Cholesteatoma is another predisposing condition. These tissues often bleed easily, which can frighten the patient, the parent, and the physician. Left untreated, polyps can enlarge to fill the canal and by expansion can progressively damage the drum and the ossicles. Therefore prompt surgical removal is indicated if therapy with topical and oral antimicrobials is unsuccessful.
Figure 23-31 Granulomas and polyps of the tympanic membrane. A, Growth of this polypoid granuloma was stimulated by the inflammatory process of chronic middle ear infection. B, These polyps, which protrude through a tympanic membrane perforation, have enlarged to entirely fill the external ear canal. Because of the possible attachment of the polyp to the facial nerve or the ossicles of the middle ear, removal of polyps requires extreme caution.
(A, Courtesy Sylvan Stool, MD.)
Thin, dimeric portions of the eardrum may be observed in patients with chronic middle ear disease, or they may develop after extrusion of a tympanostomy tube (Fig. 23-32; see also Fig. 23-28, A). These thinned areas are the result of abnormal healing of perforations and are hypermobile on pneumatic otoscopy. The important points to note on examination are whether the full depth of the pocket is visible or partly hidden, its location with respect to the ossicles, and whether or not it is dry. If the ear canal and drum are not dry, an active infection and/or cholesteatoma is present. In cases of severe deformity, aggressive therapy including ventilation of the middle ear and surgical excision of the pocket may be necessary.
Figure 23-32 Dimerism of the tympanic membrane. Otoscopy demonstrates a severely retracted atrophic segment of the eardrum that also has multiple white scars (tympanosclerosis). The thinned portions are the result of abnormal healing of perforations and tend to be hypermobile on otoscopy.
(Courtesy Sylvan Stool, MD.)
An otoscope with a wide speculum (≥4 mm) is the most practical instrument. The examiner should gently brace his or her free hand on the child’s upper lip to prevent sudden head movement from pushing the speculum tip into the nose, which could lead to nasal trauma, and should try to look toward the back of the nose rather than up into the nose. If the child is old enough to comply, he or she is asked to breathe through his or her mouth so as not to fog up the lens on the otoscope. If a nasal spreader-type speculum is used, a headlight is desirable. The septum, the anterior edges of the middle and inferior turbinates, and the nasal floor are inspected, and the quality of nasal secretions is noted. With practice and when there is minimal congestion, adenoidal size can be assessed.
A more thorough examination is possible with a nasal endoscope; this enables full visualization of internal nasal structures. Before starting, the nose is sprayed with a decongestant to shrink the nasal mucosa and with a topical anesthetic. With patience, older children can be coaxed through the insertion and examination. Allowing them to hold and inspect the device, test the light, look at themselves on the monitor, and even insert the tip into their nose assists cooperation (Fig. 23-33, A-C). Most children younger than 5 years of age require immobilization, either on a papoose board or with parental and nursing assistance. The nasal endoscope is a useful tool, and this type of examination can be done readily in the otolaryngologist’s office.
In infancy and early childhood the nasal passages are small and easily obstructed by processes that produce mucosal edema and coryza, whether infectious, “allergic,” or traumatic. In the first 1 to 3 months, infants are obligate nose breathers and therefore can have significant respiratory distress from nasal congestion alone. Young infants with upper respiratory tract infections may, in addition to nasal discharge, have tachypnea and mild retractions and often have to interrupt feeding to breathe, which can result in the swallowing of significant amounts of air, leading to a secondary increase in spitting up after feeding and to intestinal gas pain. These secondary problems can be minimized by instructing parents to hold these infants up on their shoulders and burp them for 10 to 15 minutes after feedings. Instillation of saline nose drops to loosen secretions, followed by nasal suctioning before meals and naps, also provides a measure of relief. Oral decongestants are ineffective and often produce marked irritability when given to infants in the first year of life; they are no longer approved for pediatric use because of serious adverse events including deaths in children under 2 years of age. Fortunately, upper respiratory tract infections are generally brief and clear within a few days.
On occasion, infants with upper respiratory tract infection go on to have persistent, purulent, or serosanguineous nasal discharge. Culture of discharges persisting longer than 10 to 14 days may disclose heavy growth of a single pathogen. Preliminary studies of empirical antimicrobial therapy in such infants suggest that this produces rapid and effective resolution of symptoms when compared with a placebo. Thus this picture of prolonged nasal discharge probably represents a bacterial ethmoiditis, the infant equivalent of sinusitis.
Infants with gastroesophageal reflux disease (GERD) may develop persistent nasal congestion and rhinorrhea with varying degrees of mucosal inflammation and edema in response to exposure to gastric contents. Snoring and/or coughing during sleep are common associated symptoms. Although vomiting or frequent spitting, sometimes with passage of regurgitated material through the nose, may be reported, often such symptoms are absent. The section Otolaryngologic Manifestations of Gastroesophageal Reflux Disease near the end of this chapter details the spectrum of signs and symptoms that may assist in diagnosis of GERD-related nasal disorders.
Choanal atresia may be bony (90%) or membranous (10%), bilateral or unilateral. Newborns with bilateral choanal atresia manifest severe respiratory distress at delivery, with cyanosis that is relieved by crying and returns with rest (paradoxic cyanosis). The true nature of the problem can elude detection if the physician relies solely on passing soft feeding catheters through the nose to determine patency, because these can buckle or curl within the nose. The correct diagnosis is best made with a van Buren urethral sound or a firm plastic suction catheter (both no. 8 French). This is passed gently along the floor of the nose, close to the septum. If bony resistance is encountered, the diagnosis of choanal atresia is suspected (Fig. 23-34, A) and can be confirmed by endoscopy or by obtaining a CT scan of the nose and nasopharynx with fine overlapping cuts (Fig. 23-34, B).
Figure 23-34 Choanal atresia. A, This infant manifested severe respiratory distress at delivery, with paradoxic cyanosis. Attempts to pass a urethral sound suggested bony obstruction of the choanae bilaterally. B, A CT scan done after instillation of radiopaque dye reveals pooling of the dye within the nose anterior to the choanae, confirming complete obstruction.
Immediate relief of neonatal respiratory distress from bilateral choanal atresia may be accomplished by insertion of an oral airway (or a firm nipple from which the tip has been cut away) into the mouth. Definitive studies can then be performed to aid in planning surgical correction. Infants with unilateral choanal atresia (Fig. 23-35) are usually asymptomatic at birth; however, with time they develop a persistent unilateral nasal discharge.
Choanal stenosis or anterior nasal (piriform aperture) stenosis is also generally asymptomatic in the newborn period, but acquisition of an upper respiratory tract infection can result in significant respiratory compromise. When either lesion is suspected, nasal endoscopy or probing with a urethral sound is indicated. If the sound meets resistance, further evaluation is required. In most cases symptomatic therapy using saline nose drops and nasal suctioning is sufficient to help the infant through the upper respiratory tract infection. With growth, the problem usually abates, but in some cases surgery may be required.
Congenital mass lesions are another source of nasal obstruction. These are particularly likely to become apparent during the first 2 years of life. The modes of presentation vary; some lesions are manifest primarily by symptoms of obstruction and are detected by diagnostic radiography; others become visually evident within a nostril or as a subcutaneous mass located near the root of the nose. On occasion, these patients have recurrent nasal infections and/or epistaxis. All such masses merit thorough clinical and radiographic evaluation because many have intracranial connections.
An encephalocele is an outpouching of brain tissue through a congenital bony defect in the midline of the skull. Some patients have craniofacial deformities and a rounded subcutaneous swelling between the eyes or adjacent to the nose. In other instances the neural tissue prolapses into the nasal cavity or nasopharynx, resulting in signs and symptoms of nasal obstruction without obvious external anomalies (Fig. 23-36, A). On occasion, a grapelike mass may be seen within the nares or protruding into the back of the mouth. The mass is usually identified by diagnostic radiography. CT (Fig. 23-36, B) is particularly helpful in delineating the extent of the mass and the underlying bony defect. Repair requires a collaborative effort by specialists in otolaryngology, neurosurgery, and in some cases plastic surgery.
Figure 23-36 Nasal encephalocele. A, This normal-appearing infant had signs of severe nasal obstruction, necessitating insertion of a nasopharyngeal airway to relieve distress. B, A CT scan shows a large nasal mass lesion that fills one nostril and pushes the nasal septum into the other. This lesion proved to be an encephalocele extruding through a bony defect in the skull base (extrusion seen on another CT cut).
Nasal dermoids are embryonic cysts containing ectodermal and mesodermal tissue. They present as round, firm subcutaneous masses located on the dorsum of the nose, close to the midline (Fig. 23-37, A). Examination of the overlying skin frequently reveals a small dimple, at times with extruding hair. Some of these cysts have deep extensions down to the nasal septum or through the cribriform plate into the cranium. Thorough evaluation by axial and coronal CT scans (Fig. 23-37, B) and MRI is necessary to determine extent and to plan repair. If such cysts are not removed, secondary infection is common and often results in fistula formation.
Figure 23-37 Nasal dermoid. A, A firm, round mass with a central dimple is seen over the bridge of this infant’s nose. B, CT scan demonstrates a bony dehiscence of the nasal bridge with a nasal dermoid extending into the anterior cranial vault in the area of the foramen cecum.
Papillomas (Fig. 23-38) are similar growths that occur on the distal nasal mucosa near the mucocutaneous junction. These growths should be excised to improve appearance and confirm diagnosis; they do not cause obstruction.
The lymphoid tissue that constitutes the tonsils and adenoids is relatively small in infancy, gradually enlarges until 8 to 10 years of age, and then usually begins to shrink in size. In most instances this normal process of hypertrophy results in mild to moderate enlargement of these structures and does not constitute a problem. A small percentage of children, however, develop marked adenoidal and tonsillar hypertrophy, with attendant symptoms of nasal obstruction and rhinorrhea. A few even have difficulty swallowing solid foods. Recurrent infection appears to be the most common inciting factor, although atopy may play a role in some cases. On occasion, mononucleosis is the initiating event, resulting in rapid enlargement of adenoidal and tonsillar tissues that is then slow to resolve (see Tonsillitis/Pharyngitis, later; and see Chapter 12). In most children, progressive adenoidal enlargement appears to be the cumulative result of a series of upper respiratory tract infections. The consequent obstruction to normal flow of secretions then starts a vicious circle, making the child more vulnerable to recurrent infections of the ears, sinuses, and nasopharynx, which in turn further exacerbate the adenoidal and tonsillar hypertrophy.
Regardless of the mode of origin, when adenoidal hypertrophy is marked, blockage of the nasal airway becomes severe and results in mouth breathing, chronic rhinorrhea, inability to blow the nose, and snoring during sleep (Fig. 23-39, A). Speech becomes hyponasal and muffled. The child holds his or her mouth open and has little or no airflow through the nares, and his or her tonsils may also meet at the midline (Fig. 23-39, B). A lateral neck x-ray examination reveals a large adenoidal shadow impinging on the nasal airway (Fig. 23-39, C). For many patients these features are noted primarily in the course of acute illness; however, a number of children have symptoms even when free of acute infection. When obstruction is severe, sleep disturbance may result. This is characterized by restlessness and retractions when recumbent, snoring, and frequent waking. Some patients begin to sleep sitting up, and many manifest daytime fatigue, irritability, and short attention span. Symptoms are worse during sleep because relaxation of the pharyngeal muscles further increases the degree of upper airway obstruction. In severe cases this results in periods of hypoxia and hypercarbia. Because a patient may look relatively healthy when awake (with the exception of having to breathe through the mouth), it is important to observe for retractions and to assess the pattern of breathing after the child has been recumbent for a period of time or, better still, during a nap. Use of polysomnography or continuous pulse oximetry may help confirm or establish the diagnosis and determine the severity of sleep apnea. Polysomnography may be useful to assess the severity of the sleep-disordered breathing. If severe obstruction persists for a prolonged period of time, cor pulmonale (with signs of right ventricular hypertrophy on electrocardiogram and chest radiograph) and abnormal facial growth may result (Fig. 23-39, D).
Figure 23-39 Adenoidal and tonsillar hypertrophy. A, External appearance of a child with marked enlargement of tonsils and adenoids. He must keep his mouth open to breathe and shows signs of fatigue as a result of sleep disturbance caused by his upper airway obstruction. B, On examination of the pharynx, his tonsils are seen meeting in the midline. C, A lateral neck radiograph shows a large adenoid shadow impinging on the nasopharyngeal airway. D, If obstruction is prolonged, as was the case in this patient, cor pulmonale, abnormal facial elongation, and widening of the nasal root may result. E, When the palate is retracted before adenoidectomy, the extent of overgrowth of adenoidal tissue is readily appreciated.