• Medical history significant for craniofacial abnormalities (e.g., cleft lip or palate) or syndromes associated with craniofacial anomalies (Down syndrome, Treacher Collins syndrome)

• Prematurity

• Medical history pertinent to ear conditions (e.g., central nervous system infections, otitis media, trauma)

• Pain (onset, location, quality, duration, alleviating or aggravating factors)

• Associated symptoms, such as fever, vomiting and diarrhea, nasal congestion, or other symptoms of upper respiratory infection

• Itching or discharge

• Tinnitus or hearing loss

• Exposure to risk factors: Environmental tobacco smoke (ETS), bottle propping, pacifier use, childcare, noise, swimming

• Diabetes mellitus

• Family history of ear dysfunction

• Family history of/or presence of kidney malformation

• Inspection of the external structures of the ear for symmetry, skin abnormalities, discharge, or lesions.

The inner and outer canthi of the eye should form a straight line with the superior portion of the pinna. If the pinna inserts below this line, the ear is considered low-set, which can be associated with a number of genetic and congenital syndromes and renal issues.

• Assessment of developmental milestones related to hearing and speech development (Box 29-1)

• Palpation and rotation of the external ear for tenderness and inflammation; push on the tragus and apply pressure to the mastoid process.

• Otoscopic examination, which is best accomplished in a young child at the end of the physical examination with the child on an examining table or seated on the parent’s lap. Pulling the ear downward, outward, and backward can enhance visualization of the EAC in infants and small children. In older children and adolescents, the EAC is lifted upward and backward, slightly away from the head.

• Decreased TM mobility secondary to effusion is noted through pneumatic otoscopy, tympanometry, or acoustic reflectometry.

• Examine the canal for redness, edema, or discharge. Assess all 360 degrees of the TM, the bony processes, and the cone of light (see Color Plate). Look for air-fluid level or bubbles behind the TM. Note any retraction, perforation, redness or other alteration in color, fibrosis, bulging, or retraction.

• Evoked otoacoustic emission (EOAE) testing is the method of hearing screening used for universal newborn screening. Dr. David Kemp first described the phenomenon of otoacoustic emissions in 1978. He found that the normal-hearing ear has the ability to emit detectable sounds called spontaneous otoacoustic emissions. The normal ear also emits these sounds when given a stimulus (EOAE) and provides evidence that the outer hair cells of the cochlea are functioning appropriately and hearing is likely to be intact. EOAE is efficient, highly sensitive, and easy to perform in a quiet, cooperative child, which makes it conducive for use in newborns. However, the EOAE does not quantify hearing deficit and may not identify auditory nerve dysfunction; ambient room noise and an uncooperative child may interfere with the test and provide unreliable results. Improvements in EOAE and auditory brainstem response technology have resulted in highly acceptable levels of hearing sensitivity and specificity at relatively low cost (Nelson et al, 2008).

• Auditory brainstem response (ABR) measures the initiation of sound-induced electrical signals in the cochlea. The ABR measures the functioning of the peripheral auditory system and neurological pathways related to hearing. Although it is not a direct measure of hearing, ABR allows for inferences to be made about hearing thresholds. The ABR is useful in identifying hearing loss in a young infant or in children unable to cooperate with EOAE or audiometry. Occasionally sedation is required. Neurological abnormalities may make interpretation of an ABR impossible. Automated ABR is available as a screening device.

• Audiometry, useful in assessing hearing loss in older children, measures hearing threshold via bone or air conduction, or both, in decibels at varying frequencies (Tables 29-1 and 29-2). Twenty dB is about as loud as a whisper, 40 dB is normal speaking loudness, and 90 dB produces pain. The frequencies of normal speaking range from 250 to 4000 Hz. Hearing loss, especially in the higher frequencies (2000 to 6000 Hz), can cause significant problems in understanding speech. A screening audiogram that tests each ear at 20 dB and frequencies of 500, 1000, 2000, and 4000 Hz is a useful assessment tool in office pediatrics. If a more detailed audiogram is needed, a qualified audiologist should perform it.

• Pneumatic otoscopy helps assess TM mobility. A good seal with the speculum and otoscope is required before insufflation of air into the ear canal. Brisk movement of the membrane should be seen; altered mobility suggests MEE or possible perforation.

• Tympanometry evaluates the function of the middle ear by assessing the movement of the TM by applying from −400 to +100 mm H₂O pressure to the ear canal. Movement of the TM is translated into a graph called a tympanogram (Fig. 29-1). The type A tympanogram has a compliance peak between ±100 mm H₂O and reflects a normal TM. The type B tympanogram generally has no peak or a flattened wave and suggests effusion, perforation, or the presence of a pressure-equalizing tube (see Fig. 29-2). The type C tympanogram has a sharp peak between −100 and −200 mm H₂O and reflects negative ear pressure (Fig. 29-2). Tympanograms are helpful when otitis media (OM) with effusion is persistent or a question remains regarding the results of physical examination of the eardrum. Tympanograms are of little use in children younger than 7 months old because their ear canals are hypercompliant in response to pressure from the tympanometer.

• Acoustic reflectometry is used to detect an MEE by directing a sound of varying frequency toward the TM and measuring the intensity of reflected sound. The reflectometer directs sounds of varying frequency toward the TM and measures the intensity of the reflected sound. The fluid-filled middle ear space restricts vibration of the eardrum, so sound is intensified when returning to the device. Unfortunately the reflectometer cannot distinguish if an MEE is serous or suppurative. Acoustic reflectometry is less accurate than pneumatic otoscopy.

• Tympanocentesis with aspiration of middle ear fluid is helpful for the relief of pain and identification of persistent infecting organisms. It is rarely used in clinical pediatrics and is generally considered outside the scope of practice of the primary care provider.

• Laboratory tests of blood and urine are rarely indicated unless questions remain regarding perinatal infection, systemic illness, or concomitant kidney dysfunction. Exudate from acute otitis media with perforation may be cultured.

• Genetic testing may be useful in determining if the hearing loss is inherited.

• Approximately 50% of all cases of congenital deafness are genetic. Of these 75% to 85% are inherited in an autosomal recessive pattern, 15% to 20% in an autosomal dominant pattern, and 1% X-linked (Moody and Strasnick, 2010).