Appendix 2:

Annotated Risk Indicators Associated with Permanent, Congenital, Delayed-Onset or Progressive Hearing Loss in Childhood Risk indicators that are marked with a " " are associated with delayed-onset hearing loss. 1. Any caregiver concern regarding hearing, speech, language, or developmental delay. 2. A family history of permanent childhood hearing loss. 3. A newborn intensive care stay of more than 5 days or any of the following regardless of length of stay: children who have had  ECMO (ExtraCorporeal Membrane Oxygenation- a heart-lung machine used for babies with severe heart or lung problems at birth);  ventilation (to assist or replace spontaneous breathing);  exposure to ototoxic medications (certain antibiotics that can cause hearing loss, such as  loop diuretics (certain medications, e.g., furosemide/Lasix that help get rid of sodium in the  hyperbilirubinemia (when there is too much bilirubin – a normal part of the breakdown of blood cells, in the blood) that requires exchange transfusion (removal and replacement of blood cells). 4. Maternal (Mother’s) infections (or illnesses that the mother can pass along to the baby) during pregnancy, such as  Cytomegalovirus (CMV, a [herpes] virus that many mothers do not know they have; the symptoms are flu-like. This is one of the leading causes of hearing loss.)  Herpes,  Rubella (also known as German Measles),  Syphilis (a sexually transmitted disease or STD), and  Toxoplasmosis (a parasitic disease – with cat feces being a primary site of the parasite). 5. Craniofacial (of the skull and face) anomalies, including those that involve the pinna (outer ear), ear canal, ear tags, ear pits (small pin-like holes near the ear), and temporal bone anomalies (malformed or misshapen bones around the ears). 6. Physical findings, such as white forelock (white patches of hair near the forehead), that are associated with a syndrome known to include a sensorineural or permanent conductive hearing loss. 7. Syndromes associated with hearing loss or progressive or late-onset hearing loss, such as  Neurofibromatosis (a genetically caused disease in which multiple tumors grow),  Osteopetrosis (a rare inherited disorder in which the bones harden), and  Usher syndrome (a disease in which both hearing and vision are gradually lost, not usually  Other frequently identified syndromes include Waardenburg (an inherited syndrome with characteristics that include some , if not all of the following: white forelock, different colored eyes, wide-spaced eyes, changes in skin pigmentation and low hairline and/or eyebrows that grow together), Alport (inherited kidney disease and hearing loss), Pendred (a genetic disease characterized by enlargement of the thyroid gland and hearing loss) and Jervell and Lange-Nielson (hearing loss and cardiac problems – specifically disruption in the heart’s normal rhythm). 8. Neurodegenerative disorders, such as Hunter syndrome (a hereditary disease in which the mucopolysaccharides -- a common chemical usually found inside the cells is found outside of the cells), or sensory motor neuropathies, such as Friedreich ataxia (inherited disorder that involves progressive damage to the nervous system) and Charcot-Marie-Tooth syndrome (a genetic disease of the nerves). Annotated Risk Indicators Associated with Permanent, Congenital, Delayed-Onset or Progressive Hearing Loss in Childhood 9. Culture-positive postnatal infections associated with sensorineural hearing loss, including confirmed bacterial and viral (especially herpes viruses and varicella—chicken pox) meningitis. 10. Children who have had head trauma, especially basal skull/temporal bone fracture that requires hospitalization. 11. Children who have had chemotherapy. (Adapted from Joint Committee on Infant Hearing, Year 2007 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs. PEDIATRICS, 120(4), October 2007, pp. 898-921.)


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