Summary
Introduction: Treacher-Collins syndrome is characterized by craniofacial malformations, narrowing
of the external auditory canal (EAC), and, in 30% of cases, agenesis of the canal
and ossicular chain defects. The use of hearing aids (HA) is not possible in cases
in which agenesis or stenosis of the EAC accompanies conductive deafness. In contrast,
bone conduction implants such as the Bone Anchored Hearing Aid (BAHA®) allow direct stimulation of the cochlea and are thus superior to conventional hearing
aids in cases of severe conductive hearing loss.
Objective: To present 2 cases of patients with Treacher-Collins syndrome who underwent implantation
of BAHA®.
Cases Reports: The first patient was a 52-year-old woman diagnosed with Treacher-Collins syndrome
who presented with severe bilateral mixed hearing loss and a history of unsuccessful
previous use of a bone contact conduction device. The BAHA® implantation was uneventful, and the post-operative results were good. The second
patient was a 14-year-old girl who was also diagnosed with Treacher-Collins Syndrome
with bilateral moderate conductive hearing loss by audiometry. The use of a bone vibrator
contact device did not improve her hearing; however, implantation of a BAHA® resulted in a decreased gap postoperatively.
Final comments: BAHA® hearing devices provide adequate rehabilitation and consequent improvement of the
quality of life in patients with Treacher-Collins syndrome.
Keywords
mandibulofacial dysostosis - hearing loss - conductive - bone conduction
