Hearing rehabilitation in patients with Treacher Collins syndrome

Patients with Treacher Collins syndrome primarily have bilateral conductive hearing loss, accompanied by severe craniofacial anomalies. The selection of appropriate management is essential.

To explore the suitable hearing reconstruction methods in patients with Treacher Collins syndrome (TCS).

A retrospective study was conducted on 14 TCS patients by collecting the medical history, audiological, and imaging results. The sound field and speech recognition ability from 7 follow-up patients with and without the bone conduction hearing aid (BCHA) were measured.

The HRCT anatomical variations showed facial nerve displacement (92.31%), high jugular bulb (61.54%), Low-lying middle cranial fossa (53.85%), anteriorly located sigmoid sinus (11.54%), vestibular window abnormalities(76.92%), and stenosis with cholesteatoma(30.77%). The unilateral BCHA implantation markedly improved the average sound field threshold from 62.00 ± 4.00 dB HL to 35.71 ± 4.99 dB HL with significant gains in SRT and SRS. However, hearing improvement was limited in stenosis patients with cholesteatoma after canalplasty and tympanoplasty. Postoperative complications included one case of canal restenosis and two cases of implant failure.

The BCHA can provide sufficient hearing gain and represent the preferred hearing intervention in TCS patients.