Download 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
gentimycin and tobramycin), or
 loop diuretics (certain medications, e.g., furosemide/Lasix that help get rid of sodium in the
body); and
 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
at the same time);
 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).
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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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