Intralabyrinthine schwannomas: a case series with discussion of the diagnosis and management.

OBJECTIVE: By extracting cases of intralabyrinthine schwannomas (ILS) from the sum of all vestibular schwannomas, we aim to identify and analyze unique features of its presentation. This allows us to refine the management protocol of this rare condition.

DESIGN: This is a retrospective study of all patients seen in the Department of Otorhinolaryngology-Head and Neck Surgery, Rouen University Hospital, with either ILS or ILS with intracanalicular extension (ILS-IAC) between 2001 and 2011. A literature search was performed and results combined to draw conclusions on management strategies.

METHOD: Three patients with ILS and 6 patients with ILS-IAC were identified. We retrieved data on age, sex, symptoms, audiometry, imaging, and management. Pure tone audiometry and speech discrimination score were assessed and hearing classification recorded. Facial nerve function and vestibular function were documented throughout. The diagnostic and surveillance imaging (MRI with or without CT) were reviewed.

RESULTS: The average age at presentation was 62.8 years and the sex ratio was (male: female) 4:5. An ipsilateral hearing loss was observed in all patients. Eight of 9 patients had tinnitus at presentation, 2 had rotatory vertigo, and 1 patient had a facial palsy and hemifacial spasm. In 2 cases, the labyrinthine extension was initially missed. The patient presenting with a large tumor and facial palsy was operated on without delay. The others underwent MRI surveillance, with 4 requiring surgery at a later stage. No postoperative facial palsies were encountered other than the one that had been present preoperatively.

CONCLUSION: Frequency of ILS is underestimated because of poor diagnostic criteria. These tumors have often been described as having features, which resemble Ménière's disease, which is not found in our series. In the absence of tumor progression or disabling symptoms, their management is surveillance/medical, and when surgery is considered, facial paralysis and recurrence rates seem low. The treatment of IAC-ILS differs from that of ILS.