Management of Hearing Loss in Patients with Treacher-Collins Syndrome and Hemifacial Microsomia

Treacher-Collins syndrome (TCS) and hemifacial microsomia (HFMS) are associated with anomalies of the outer and middle ear. Anomalies of the inner ear are occasionally observed in TCS but are rare in HFMS. Hearing loss in TCS is largely bilateral, whereas hearing loss is generally asymmetric or unilateral in HFMS. In both syndromes, the hearing loss is largely conductive in nature, but some patients also have a mixed hearing loss. Sensorineural hearing loss alone is uncommon. A thorough history and physical examination is necessary in these patients to identify any anatomic abnormalities of the external and middle ears as well as identification of associated abnormalities. A thorough audiologic assessment is also required to evaluate for hearing loss. Treatment options for hearing loss associated with TCS and HFMS include bone conduction hearing aids as well as implantable hearing devices. Traditional hearing aids may also be helpful in some circumstances but have limited application due to the predominance of external ear malformations in these patients.