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Chapter 17. Congenital Hearing Loss

Which of the following is false?

A. 50% of congenital hearing loss is secondary to environmental causes.

B. 70% of nonsyndromic congenital hearing loss cases are inherited via an autosomal dominant pattern.

C. 30% of syndromic congenital hearing loss cases are inherited via an autosomal recessive pattern.

D. CMV infection is the most common infectious cause of congenital hearing loss.

E. The vast majority of infants with CMV infection are asymptomatic.

According to the EHDI principles, which of the following is false?

A. All infants should be screened by 1 month of age.

B. There are different recommended screening protocol for well-infants and NICU-infants.

C. Diagnostic ABR is the superior audiologic screening modality.

D. Referral for genetic testing should only be offered in select cases.

E. Infants with confirmed hearing loss should receive intervention no later than 6 months of age.

Match the inner ear malformation with respective name:

Pars inferior dysplasia

A. Mondini

B. Schiebe

C. Michel

D. Alexander

Match the inner ear malformation with respective name:

Absent interscalar septum

A. Mondini

B. Schiebe

C. Michel

D. Alexander

Match the inner ear malformation with respective name:

Limited cochlear duct differentiation

A. Mondini

B. Schiebe

C. Michel

D. Alexander

Match the inner ear malformation with respective name:

Complete agenesis

A. Mondini

B. Schiebe

C. Michel

D. Alexander

The most commonly affected protein implicated in genetic hearing loss is

A. Connexin 30

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