Facial nerve anomalies in association with congenital hearing loss

Objective: Facial nerve (FN) anomalies are exceedingly rare. The association between a hearing loss and an anomalous facial nerve has never been touched deeply in the literature. The aim of this study is to report facial nerve anomalies in patients presenting with congenital hearing loss (COHL) and/or facial nerve palsy. Methods: A retrospective chart review over 10 years from two academic tertiary referral centers. Eight patients were diagnosed with facial nerve anomalies in association with congenital hearing loss. The age ranged from 7 months to 13 years. Results: Five patients had conductive congenital hearing loss (CCHL). Two had congenital sensorineural hearing loss (CSNHL). The eighth patient had a mixed hearing loss (MHL). Surgical findings for patients with conductive congenital hearing loss included facial nerve passing inferior to oval window (OW) (two patients), facial nerve covering head of stapes (three patients), deformed stapes (two patient), deformed incus (two patient), and absent incus (one patient). Radiological findings for patients with congenital sensorineural hearing loss and mixed hearing loss include, single cavity anomaly (one patient), dilated vestibule and superior semicircular canal (one patient), and absent internal auditory canal (IAC) (two patients). For patients with conductive congenital hearing loss, the radiological findings consistency demonstrates a soft tissue opacification in the middle ear. Conclusions: Congenital hearing loss may be associated with facial nerve anomalies. Pediatric otolaryngologists should be cautious when exploring patients with conductive congenital hearing loss.