ObjectivesTo report two cases of cleidocranial dysplasia in which hearing loss was the first presenting symptom.Study designRetrospective case review.PatientsTwo cases of cleidocranial dysplasia, a rare autosomal dominant skeletal dysplasia affecting both membranous and enchondral bone formation.SettingTertiary referral center.InterventionsClinical, audiometric, and imaging diagnostic procedures.ConclusionWith this report, we want to illustrate the possibility of a rare genetic disorder as the underlying cause of hearing loss. We also want to emphasize the need for a multidisciplinary approach and evaluation of unexplained hearing loss to obtain a correct diagnosis, which is important for genetic counseling and management of the patient and his or her family.