For children with CHL the Joint Commission on Infant Hearing (JCIH) 2000 and 2007 recommends following the 1:3:6 model- screen by 1 month, confirm diagnosis and amplify by 3-4 months and enroll in appropriate intervention by 6 months. Often though, for children with microtia/atresia, recommendations to parents center on watching and waiting to see if a delay in language occurs before amplifying or providing intervention.

JCIH 2000Defines the targeted hearing loss for UNHS programs as permanent bilateral or unilateral, sensory or conductive hearing loss…..”

Hearing loss as defined above has effects on communication, cognition, behavior, social-emotional development, and academic outcomes and later vocational opportunities (Karchmer and Allen, 1999).”

Next Steps1.How do we improve reporting of incidence of microtia/atresia by PCP?2.Diversify the N’s3.Better Education for Professionals4.Research! Research! Research!

anaging children with microtia/atresiaMichelle Kraskin, Au.D. CCC-AAssistant Director, Hearing & Speech DepartmentWeill Cornell Medical College

mlk2003@med.cornell.edu

Meredith Berger, M.S.DirectorClarke Schools for Hearing and Speech/New York

Mberger@clarkeschools.org@meredith_berger

Bone Responses

Air Responses

Baby A is born.In the delivery room, his father notices something is wrong with his right ear and asks the OBGYN about it. Dr. OB has never

seen it before so Dad looks it up and diagnoses his 30 minute old with microtia/atresia.

What happens next? Left ear (non-m/a) is screened and passes.

Right ear “refers” for further testing. EI services represent the purpose and goal of the entire EHDI process.

Screening and confirmation that a child is D/HH are largely meaningless

without appropriate, individualized, targeted and high-quality intervention.

In 2014, 4 million babies were born in the U.S. Of those, 500-600 were born with microtia/atresia(CDC,2014). When comparing the prevalence of microtia/atresia with sensorineural hearing loss (1-3/1000 well babies), it is likely that clinicians and interventionists have limited experience working withchildren with microtia/atresia. Although the JCIH of 2000 and 2007 recommendations includes the 1:3:6timeline for permanent conductive losses in children, medical professionals often recommend waitingfor a delay to develop before providing intervention. Waiting for a delay to develop, though, is thecontraindication of early diagnosis. If the JCIH guidelines are followed for children with permanentconductive hearing loss, what would the outcomes be?Definitions:

Microtia (congenital deformity affecting the outer ear) and Aural Atresia (absence or closure of the external auditory ear canal) affect the normal transmission of sound from the external ear to the internal ear resulting in a conductive hearing loss (CHL).

Follow up:1.ABR-confirms cochlear function “normal bone”

2.Ear, Nose and Throat (ENT) examination 3.Counsel family on results

1. Counsel on amplification use

2. Obtain medical clearance for a baha and fit

3. Refer to Early Intervention (EI)

1. Advise Parent that baby has 1 good ear

2. Aid if language delays appear later3. Monitor good ear4. Consider surgical options > age 55. Hope delays don’t develop

1. EI Evaluations2. IFSP developed3. Therapy with appropriate

therapist starts