With the execution of universal newborn hearing screening programs and implementation of early intervention programs,
pediatric audiologists often confirm type and degree of congenital hearing loss within the first few months of life. For those diagnosed with sensorineural hearing loss, recommendations for habilitation/rehabilitation are discussed with the family at the time of the diagnosis, along with a referral to an otologist or pediatric otolaryngologist for a medical evaluation. After the FDA required "medical clearance" has been obtained, the process for obtaining amplification begins. For those children identified with a conductive or mixed type of hearing loss, medical or surgical intervention would be determined prior to consideration for amplification.   

Infants and children with at least a mild degree of sensorineural hearing loss are typically fit with good quality amplification, which when programmed appropriately, allows for maximized audition while keeping amplified sounds comfortable and safe for the user. The instruments should be flexible in programming to accommodate fluctuating or progressive hearing loss. The devices should be durable, moisture resistant and contain safety locking mechanisms. Additionally, compatibility with direct audio input should be considered.

Other features to consider when selecting amplification include, directional microphones, number of software channels or bands that can be manipulated for sufficient frequency shaping, feedback suppression and digital noise reduction circuitry. Additionally, pediatric audiologists often desire frequency compression features when selecting amplification for their patients. Frequency compression features allow for compression of selected high frequency sounds such as fricative consonants /f/, /s/ and /sh/ into a lower frequency range, where both hearing sensitivity and discrimination ability are better. When selecting amplification for adolescents and teenagers, Bluetooth compatibility should be considered, which allows for wireless coupling to many phones, computers and other advanced technology.

Figure 1.  MUSC patient wearing bone conduction processor on headband

Infants and young children are typically fitted with traditional behind-the-ear (BTE) style hearing aids with custom earmolds, while receiver-in-the-canal (RIC) devices are becoming more popular with adolescents and teenagers, due to their "natural sound" and cosmetic appearance. BTEs and RICs are available in a wide variety of colors to blend with skin and hair-tones, in addition to bold and vibrant "fun" colors. Most BTEs and RICs can be re-cased to a different colored housing at the manufacturer, for a nominal fee, should the patient or family desire a change of color. Custom earmolds are fabricated when the ear grows and the current earmolds no longer fit properly, often resulting in intermittent or increased feedback.

Pediatric audiologists should use evidence based prescriptive methods when fitting traditional amplification. Verification of proper programming can be done efficiently with real-ear aided probe microphone measurements, where the output of the hearing aid is measured in the child's ear at a variety of input levels, including speech-like stimuli and maximum power output. When fitting amplification on infants or toddlers who are often moving or noisy, simulated real-ear aided response measurements obtained in the coupler using measured or age-appropriate real-ear-to-coupler difference (RECD) are typically used. Aided measures in the test booth are also obtained, when able, and compared to the child's unaided behavioral measures.

For children with permanent conductive hearing loss, where traditional hearing aids are not appropriate, such as aural atresia, grade III or IV microtia, or chronically draining ears, "bone anchored" or "osseointegrated" devices are often recommended. For those under 5 years of age, bone anchored devices are typically coupled to a softband/elastic band, which wraps tightly around the head, allowing transmission of sound through bone conduction (Figure 1). For those at or above age 5 who meet FDA criteria, both transcutaneous and percutaneous bone anchored devices are available for surgical implantation. Attachment of the external processor typically occurs 6 months post surgery for those with a percutaneous abutment, allowing appropriate time for osseointegration. For those using transcutaneous devices, which attach via a magnet, activation of the external processor can occur as early as one-month post surgery.

For children diagnosed with Auditory Neuropathy Spectrum Disorder (ANSD), a condition where sound enters the cochlea normally but the transmission from the cochlea to the brain is impaired, children may demonstrate normal hearing or hearing loss ranging from mild to severe/profound. Hearing may also fluctuate. Treatment with amplification or cochlear implantation is controversial among professionals, however many Audiologists are recommending at least a trial with traditional amplification for those children who exhibit hearing loss. Parents of children with ANSD are urged to work with a team of professionals when considering treatment options and communication approaches.

Kimberly A. Orr , M.A., Au.D., CCC-A