Neurofibroma around the Ear Canal: Report of Three Cases

Abstract

Facial nerve neurofibromas are an uncommon entity, particularly in the intraparotid region. Three cases are presented herein, including the first reported case of both an intratemporal and intraparotid facial nerve malignant neurofibroma. To the best of our knowledge, this is the first medical publication to describe a neurofibroma involving both the intratemporal and intraparotid regions. The aim of this clinical record was to present the surgical experiences in three cases of intratemporal and intraparotid facial neurofibroma. Of the three cases of neurofibroma included in this report, the first case was a 30-year-old male who initially presented with a parotid mass without facial dysfunction. The other two cases were intratemporal neurofibromas, one of which was confirmed as a plexiform neurofibroma by pathology. These tumors often involve two or more segments of the facial nerve. The outcomes of these tumors are closely related to nerve integrity, preoperative facial nerve function level, and the follow-up period. Neurofibromas are most commonly found with neurofibromatosis type 1 and are characterized by incorporation of the nerve fibers within their matrix. Presentation of the intratemporal facial neurofibromas is variable, with facial palsy most common. Furthermore, the incidence of a solitary neurofibroma of the facial nerve originating in the parotid region is extremely low.

Solitary Neurofibroma of External Aural Canal

“…Neurofibromas are most commonly found in males aged between 14 and 45 years [3]. However, in the ear, the female sex seems to be more affected: 4 out of 7 reported cases are female [5]- [9]. Our patient was a young woman aged 45.…”

“…Ear canal neurofibromas can develop on the auricular branch of the X or branches of the VIIth and IXth pairs of cranial nerves [9]. The nerves are responsible for innervation of the auditory canal.…”

“…Clinicals signs are non-specific, but conductive hearing loss is consistently reported in all cases [5] [6] [8] [9]. The hearing threshold typically range between 30 and 40 dB and depends on the degree of ear canal obstruction [9]. A CT scan reveals a heterogeneous contrast-enhanced tissue mass that is not specific to neurofibroma.…”