Notification To:

Parents
Primary Care Provider
Colorado Hearing Resource Coordinator (CO-Hear Coordinator)*

Primary Care Provider: Initiates and supervises evaluation and the referral process to ENT otology, genetics, audiologists, and therapists.

History:

Prenatal

Ototoxic medication exposure
Any significant complications during pregnancy
Immunization to Rubella
Syphilis screening
Maternal drug use
Frequent spontaneous abortions

Perinatal

*High Risk Factors

Family

*Other family members with hearing loss with onset of age less than 30
*Desire to have other children

Physical:

Minor Anomalies: Unusual morphologic features occurring in less than 5% of the population with no cosmetic or functional significance.

Major Anomalies: Those causing cosmetic and/or functional abnormality i.e. cleft palate, cardiac, limb or skeletal deformities.

Poor growth and/or microcephaly, abnormal neurologic examination

Lab:

Urine culture for CMV prior to 3 weeks of age, if possible.

ANY of the above positive: ENT/Otology and Genetics referrals
ALL of the above negative: ENT/Otology referrals

Prenatal, Perinatal, Family and Behavioral

Head & Neck examination
Head circumference

Review of prior testing i.e. ABR and OAE results

Evaluation:

Infectious Diseases: CMV, Rubella, Syphilis, Toxoplasmosis
Urinalysis
Ophthalmologic examination
CT high resolution of temporal bones
ABR for threshold (if not previously done)
Otoacoustic emissions (if not previously done)

Referrals:

Genetics
Audiology Assessment (see Audiology guide lines)
Colorado Hearing Regional Coordinator
Early Intervention - Language/Speech Therapy
If not currently being provided

History:

Pregnancy
Family pedigree
Developmental

Physical:

General Pediatric examination
Careful dysmorphologic examination
Neurologic/developmental evaluation

Diagnostic Tests:

Hearing tests on first degree relatives (parents and siblings)
Ophthalmologic examination by 6 months of age.
TORCH titers or CMV specific IgG and IgM if under 6 months.
All other laboratory tests depend upon clinical evaluation and history but may include the following:

1. Chromosomes if dysmorphic

2. EKG

3. Skeletal survey if there is short stature or disproportional growth.

4. Evaluation of other systems: renal, cardia, skin

5. CT or MRI of brain if neurologically abnormal

6. Specialized genetic studies: molecular, gene testing, etc.

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