Clinical presentation of intralabyrinthine schwannomas: a multicenter study of 110 cases.

OBJECTIVE: To describe the presentation of intralabyrinthine schwannomas (ILSs). STUDY DESIGN AND SETTING: Retrospective multicenter study involving 12 European skull base surgery tertiary referral centers. PATIENTS: One hundred ten patients with the diagnosis of ILS, either labyrinth confined or extending into the internal auditory meatus for less than 50% of their volume. MAIN OUTCOME MEASURES: Data collected were age, sex, nature and timing of presenting symptoms, hearing (according to the AAO-HNS grading system), results of vestibular tests (caloric tests and cervical vestibular-evoked myogenic potentials [c-VEMPs]), and tumor localization. Presenting symptoms and laboratory test results were studied according to the extension of the lesion into the cochlea (C) and vestibule (V), on one hand, and according to unifocal (L1) or plurifocal (L2) extension into the labyrinth, on the other. RESULTS: Intracochlear type was more common (50%) than vestibular (19.1%) and more diffuse forms (30.9%). The mean delay for diagnosis was long (72.5 mo; SD, 76.6). Mean age was 53.9 years (SD, 13.2). Deafness was the most common symptom (77.8 dB HL [SD, 33.6], with only 24.6% of patients keeping viable hearing. Caloric tests (65.5% of patients) were abnormal in 77.8% of cases. c-VEMPs were abnormal in 65.7% of the 36 cases analyzed. In V forms, hearing was significantly better (class A + B in 21.1% in C and 45.8% in V forms) (p = 0.03), and vestibular function was more altered (C: 57%, V: 100%, p = 0.0009*). L2 forms were diagnosed later (L1: 59.1 mo, L2: 104.5 mo; p = 0.004*) and were associated more frequently with a dead ear (L1: 13.1%, L2: 41.2%, p = 0.002*) than L1 forms. CONCLUSIONS: This series, which is the largest in the literature, demonstrates that even very small and localized ILSs heavily compromise labyrinthine functions.

Clinically isolated syndrome manifested as acute vestibular syndrome: bedside neuro-otological examination and suppression of transient evoked otoacoustic emissions in the differential diagnosis.

A case of a 34-year old woman with acute vestibular syndrome caused by a demyelinating lesion in the root entry zone of the 8th cranial nerve is presented. Neuro-otological bedside examination and suppression of transient evoked otoacoustic emissions provided objective clinical evidence of a retrolabyrinthine lesion. Magnetic resonance imaging and the presence of oligoclonal IgG bands in cerebrospinal fluid analysis established the diagnosis of clinically isolated syndrome. This case report highlights the clinical information provided by the neuro-otologist in the differential diagnosis of the acute vestibular syndrome and the diagnosis of possible multiple sclerosis.

Glomus tumor of the cheek: a case report.

Glomus tumors are benign, subcutaneous neoplasms of the perivasculature. Though facial location is rare, the diagnosis of a glomus tumor should be considered in cases of undiagnosed painful facial nodules or chronic facial pain. Imaging aids in defining the tumor and planning a complete excision in order to avoid recurrence. Histological examination is mandatory after every attempted excision. A case of glomus tumor of the cheek along with the possible pitfalls of diagnosis and treatment and a brief review of the limited associated literature are presented.