Sensorineural Hearing Loss Caused by Labyrinthine Erosion Due to a Cerebrospinal Fluid Diverticulum

Abstract

Objective:To present a case of progressive sensorineural hearing loss (SNHL) caused by labyrinthine erosion secondary to expanding geniculate ganglion cerebrospinal fluid (CSF) diverticulum extending along the path of the facial nerve.Patient:Thirteen-year-old man with no past medical history or risk factors presented with unilateral progressive SNHL and no other otologic or neurologic symptoms.Interventions:Audiological data as well as imaging studies including a magnetic resonance imaging (MRI) of the brain and internal auditory canal and a temporal bone computed tomography (CT) are presented. Partial labyrinthectomy, CSF leak closure, and cochlear implantation were performed. A lumbar puncture was obtained 2 months postoperatively.Main Outcome Measure:Closure of the CSF leak.Results:Mastoidectomy revealed a wide area of CSF leak through the geniculate ganglion and fallopian canal with partial destruction of the superior and lateral semicircular canals by an expanding diverticulum.Conclusions:This is an unusual presentation of progressive SNHL due to partial labyrinthine erosion due to a meningocele of the geniculate ganglion along the fallopian canal. It is likely that the patient had a congenital open pathway through the labyrinthine segment of the facial nerve and CSF pulsations created the meningocele over a few years. This case argues for early closure of such CSF diverticula to prevent possible SNHL due to labyrinthine erosion.

Facial Nerve Meningocele Presenting as Sudden Sensorineural Hearing Loss

“…Typically, meningoceles appear as circumscribed hyperintense lesions on T2 sequences, localized within the area of facial canal dehiscence, and present as a CSF-filled sac (2,3). Our case presented with a very rare and unusual finding not yet described in the literature: additional erosion into the cochlea with resultant sudden SNHL—a manifestation not commonly documented in facial nerve meningoceles (4,5). The differential diagnosis included a facial nerve schwannoma; however, as there was no contrast enhancement on MRI (Fig.…”

Management of Fallopian Canal Spontaneous CSF Leaks, Implications of Elevated Intracranial Pressure: Case Report and Systematic Review of the Literature

“…Fallopian canal CSF leaks are a very rare cause of sCSF-L that can lead to CSF otorrhea. To date, there have been just over a dozen adult operative cases reported in the literature (19–38) (Table 1). Patients with this defect commonly present to the clinic with middle ear effusion and conductive hearing loss that is often misdiagnosed as otitis media (36).…”