Figure 1. Source: http://www.infanthering.org

Infants who are readmitted within the first month of life and have a condition associated with potential hearing loss (eg, hyperbilirubinemia that requires exchange transfusion or culture-positive sepsis) should be rescreened prior to discharge. Re-evaluations for children with risk factors should be customized depending on the likelihood of a subsequent delayed-onset hearing loss. Children with cytomegalovirus (CMV) infection, syndromes associated with hearing loss, neurodegenerative disorders, trauma, certain culture-positive infections, those who have received extracorporeal membrane oxygenation (ECMO), chemotherapy or a family history of hearing loss should be evaluated as often as every three months for a period of two years.

An otolaryngologist who has knowledge of pediatric hearing loss should evaluate every infant with confirmed hearing loss. These infants should also have at least one evaluation to assess visual acuity by an ophthalmologist, who is experienced in evaluating infants.

All families of infants with permanent hearing loss should be considered eligible for early intervention services. Both home-based and center-based intervention options should be offered. Infant's developmental milestones, auditory skills, parental concerns and middle ear status should be monitored in the medical home, consistent with the American Academy of Pediatrics (AAP) periodicity schedule.

Infants and children whose parent's opt for amplification should be fit with appropriate hearing aids within a month of the diagnosis. Behind-the-ear (BTE) hearing aids must be sturdy, dependable, and flexible, should a change in hearing status be documented. Compatible technology such as Bluetooth and FM should be considered during the selection of the hearing aids. For those who cannot be fit with traditional hearing aids due to a permanent, conductive hearing loss (atresia, draining ears), a BAHA (osseointegrated cochlear stimulator), including an implanted abutment or softband option for those below the age of 5, should be considered. BAHA is also a good option for those with permanent, single-sided deafness.

Hearing aids should be fit utilizing appropriate verification measures, including real-ear-to-coupler difference (RECD) to ensure precise amplification, as infants and children have much smaller ear canals than adults.   Children with hearing loss should have a comprehensive speech and language evaluation immediately following the diagnosis of hearing loss and should receive intensive therapy such as Auditory Verbal Therapy (AVT) on a consistent basis. Cochlear implantation should be considered for those children with severe to profound hearing loss who are not progressing with speech and language development, despite intensive therapy with parental involvement, and who demonstrate aided hearing thresholds below the range of audibility. Children can be implanted within the first year of life. The shorter the duration of deafness, the more likely a child is to benefit from a cochlear implant and develop spoken language similar to those peers with normal hearing.

Children with any degree of hearing impairment should have adequate access to auditory information in their educational settings and require the expertise of an educational audiologist.   Students requiring special services are supported under the Individuals with Disabilities Education Act (IDEA, 2004) or section 504 of the Rehabilitation Act (1973).

References:

Year 2007 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs. Joint Committee on Infant Hearing. Journal of the American Academy of Pediatrics. Vol. 120, No. 4, 2007

Kimberly A Orr, AuD

Director, Division of Audiology