Incidence of Hearing Loss in Corpus Callosum Agenesis

Congenital corpus callosum agenesis (CCA) is one of the congenital anomalies in newborns, which usually presents with syndromic features. It can be asymptomatic or have variable neurological deficits. Some studies demonstrated that hearing loss can occur in patients with CCA; however, the exact prevalence remains unclear. Therefore, we aimed to investigate the prevalence of hearing loss in CCA using data from newborn hearing screening in a single tertiary referral center.

A total of 126 patients with CCA combined with hearing loss diagnosed at our hospital from November 2005 to November 2022 were retrospectively included in our study. All patients had at least one screening or diagnostic auditory brainstem response result. Brain ultrasonography and magnetic resonance imaging were used to diagnose CCA.

Among 126 patients, 93 had automated auditory brainstem response within a month from birth. Of the 93 patients, 20 (21.5%) had a "refer" result in the screening tests in at least one ear. The final incidence of hearing loss in patients with CCA was 16.1%. We observed no hearing loss in 22 patients with isolated CCA.

Patients with CCA have a higher incidence of hearing loss. However, this is likely related to the concurrent condition of patients. CCA seems not to be a risk factor for hearing loss.