Nebraska Early Hearing Detection and Intervention Program

Jeffrey Hoffman, MS, CCC-A

Edited by Patricia Burk, M.S., CCC-SLP, LSLS Cert. AVT

The Nebraska Early Hearing Detection and Intervention (NE-EDHI) Program is under the direction of the Newborn Screening and Genetics Program, within Lifespan Health Services, Division of Public Health in the Nebraska Department of Health and Human Services.  The NE-EHDI Program Manager is directly supervised by Julie Miller, Program Manager of the Newborn Screening and Genetics Program.  Collaboration occurs on a daily basis as information is shared between the two programs to facilitate tracking and follow-up.  Collaborative projects include retrieval of the dried blood spot to determine etiology of congenital hearing loss and exploration of approaches to integrate and link data systems. 

The NE-EHDI Program continues to build on the accomplishments and resources of the early hearing detection and intervention (EHDI) system that has been developed in Nebraska in the last eight years.  The Infant Hearing Act of 2000 (Neb. Rev. Stat. § 71-4735) specifies four key provisions for the EHDI system:  1. Hearing screening during birth admission is the standard of care.  2. Birthing facilities are to educate parents about hearing, hearing loss, and hearing screening.  3. Regulations must be written if the inpatient screening rate falls below 95%.  4. The Department of Health and Human Services would develop a tracking system and annual aggregate reports are required for birthing and confirmatory test facilities.  Required activities of the NE-EHDI Program include development, implementation, and monitoring of statewide systems to track newborns with or at-risk of hearing loss; gathering required data and generating annual reports; and establishing guidelines for referral to early intervention services.

The newborn hearing screening reporting system is an integrated module of the State of Nebraska Vital Records Electronic Registration System (ERS-II).  At the birthing facility, a clerk creates an individual hearing information (HINFO) record from the birth certificate which includes identifying data of the newborn and parents.  Multiple detail records (HSCREENING) contain information about hearing screening, audiologic diagnostic, risk factors, medical and early intervention services.  The data system includes basic search functions, work queues in which pending records are placed and aggregate quality assurance and status reports. 

The NE-EHDI Program tracking procedure for newborns who did not pass or were discharged prior to receiving a newborn hearing screening includes an initial letter to the primary care provider (PCP) reporting the newborn hearing screening results and any recommendations made by the birthing facility.  The letter requests that the PCP complete the recommended follow-up protocol and submit the results to the NE-EHDI program. Included with the PCP letter is a parent education brochure (available in ten languages), an algorithm that displays the recommended follow-up protocol and a list of audiology testing sites.  If the results of the outpatient follow-up, either a screening or audiologic diagnostic evaluation, have not been received within three weeks, a second request is sent to the PCP and a letter and parent education brochure are also sent to the mother explaining the hearing screening results, encouraging follow-up and talking with the baby’s PCP about the hearing screening.  If results are still not received within one month, a third request is faxed to the PCP, a second letter is sent to the mother, and the file is coded as lost-to-system.

The feasibility of retrieving the newborn dried blood spot (DBS) for identification of congenital cytomegalovirus (CMV), Connexin 26 and 30, mitochondrial, and Pendred syndrome continues to be evaluated.  Identification of these factors, which are risk factors for progressive or later-onset hearing loss, can also assist in establishing the etiology of a congenital hearing loss.  A work group of the Newborn Screening (blood-spot) Advisory Committee recommended the implementation of procedures to encourage physicians to get the audiological evaluation and diagnosis completed before 90 days (at which time regulations require that the DBS be destroyed) so that they could request that the dried blood spot be returned so it could be tested for genetic causes of hearing loss, or at a minimum, CMV for those infants diagnosed with sensorineural hearing loss.  Educational materials were developed and mailed to physicians, physician assistants and nurse practitioners.  Beginning in October, 2007, materials for parent consent and physician retrieval of the dried blood spots for babies identified with sensorineural hearing loss before three months of age were included with the basic information provided to the PCP.    

Nebraska has recently created a statewide hearing aid loaner bank, Nebraska Children’s Hearing Aid Loaner Bank, to provide amplification within one week of the request for an initial loan period of six months. A special taskforce is also working to identify audiologists who are capable of providing appropriate, comprehensive audiologic diagnostic evaluations for babies up to six months of age. A listing will be included in NE-EHDI Resource Guide, along with revised protocols, to provide parents and PCPs with the information necessary to make an informed decision.    

The biggest challenge facing the NE-EHDI Program is nurturing the ongoing development of an integrated, comprehensive and seamless system of diagnostic and intervention services for babies who don’t pass the newborn hearing screening and for those later identified with a permanent hearing loss.  Strides have been made in recent years to improve the system of care through refinement of educational materials; engaging parents and professionals in the exploration, development and implementation of changes; and keeping a central focus on the desired outcomes for young children and their families as approaches for improvement are considered  and the work continues.