3. What type of hearing loss is characteristic with CHARGE Syndrome?


Ear problems that accompany CHARGE typically vary in patients. CHARGE patients usually have a hearing loss that is sensorineural or mixed in nature. The pinna (outer ear) is often malformed. Patients exhibit a wide outer ear and a small lobe or absence of a lobe at all (Norbury & Hefner, no date).

Patients often have frequent ear infections because of poor drainage from eustachian tubes (Davenport, Hefner, & Mitchell, 1986). Interestingly found in several autopsy patients with CHARGE the bones of the middle ear are often calcified, malformed, or absent. Also other vital parts of the ear were missing such as the oval window and round window (areas where pressure is released from activity in cochlea) (Wright, Meyerhoff, Brown, & Rutledge, 1986).

Degrees of loss can range from mild to profound depending on symptoms (Norbury et al., no date). Loss for CHARGE patients may be progressive (Davenport et al., 1986). Due to the outer ear malformations hearing aids may be difficult to fit. Frequent ear infections could also inhibit proper amplification (FM systems and hearing aids) from being used (Norbury et al., no date).


As teachers we must be always aware of the implications of hearing loss on education. As with other symptoms of CHARGE, hearing loss is individual. There is no consistency "across the board" which would be strictly typical of CHARGE Syndrome. Knowing what type of hearing loss (conductive, sensorineural, or mixed) and the degree of the loss helps teachers and parents to understand where we can go with the child educationally and developmentally. Professional team members must also remember to keep in mind that each child is different and requires different services to address different needs.

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