Posterior Cranial Fossa Meningioma Presenting With Hearing Impairment and Recurrent Vertigo
An 18-year-old female patient presented with several months of hearing impairment in the right ear and recurrent attacks of vertigo. She had no history of other medical illness, head trauma, or surgery. During the previous 6 months, she had frequently experienced tiredness. She underwent an audiogram that indicated a moderate sensorineural hearing loss in the right ear. Physical examination and routine vestibular function assessment revealed a gaze-evoked and direction-changing nystagmus. Magnetic resonance imaging (MRI) scans of the brain were obtained for further evaluation. A huge extraparenchymal mass with hypointensity in the posterior cranial fossa (PCF), especially the right cerebellopontine angle (CPA), was visible on axial T1-weighted images (T1WIs; Figure 1). A gadolinium-enhanced sagittal T1WI demonstrated a strong enhancing heterogeneous mass, 4 cm in size, with well-defined margins (Figure 2). To facilitate surgical removal by reducing the vascularity of the tumor and to decrease intraoperative blood loss, we performed a transfemoral cerebral angiography. However, the angiogram revealed neither feeding vessels nor the characteristic tumor blush (Figure 3). Gross total resection of the tumor was carried out through a midline suboccipital approach. Postoperatively, the patient developed right-sided facial (House-Brackmann grade IV) and lower cranial nerve paralyses. After 6 months, these symptoms significantly improved.
Figure 1. Radiological findings on MRI of the brain. An axial T1-weighted image reveals an extraparenchymal mass with hypointensity in the posterior cranial fossa and the right cerebellopontine angle. MRI indicates magnetic resonance imaging.
Figure 2. Radiological findings on MRI of the brain. A gadolinium-enhanced sagittal T1-weighted image demonstrates a strong enhancing heterogeneous mass, 4 cm in size, with well-defined margins. MRI indicates magnetic resonance imaging.
Figure 3. Radiological findings on transfemoral cerebral angiography. Lateral view of the vascular supply to the tumor from the right vertebral artery displays neither the main feeding artery nor the characteristic tumor blush.
Meningiomas arise from arachnoid cap cells that are sprinkled on the outer surface of the meninges. They account for 13% to 37% of all primary brain tumors.1–3 They are the second most common primary intracranial tumor and frequently originate from the parasagittal, falcine, or convexity regions. Meningiomas are diagnosed twice as frequently in female patients. Age-specific incidence rates show an increase with age.2,4,5 The number of meningiomas that are detected incidentally have increased recently because of extensive precautionary neuroimaging evaluations.
Most meningiomas grow slowly and compress the central nervous system (CNS) at such a gradual rate that the CNS is able to accommodate this and any associated damage without displaying any symptoms for several years. When clinical manifestations develop, they depend on the anatomical location of the lesion. Fifteen percent of all intracranial meningiomas are located in the PCF. Patients with meningiomas in the PCF might present with vague, nonspecific vertiginous symptoms, tinnitus, and progressive sensorineural hearing loss because of compression of adjacent seventh and/or eighth cranial nerves. This happens regardless of the grade of the tumor.6 As a result, they are often difficult to differentiate from vestibular or glossopharyngeal schwannomas in the CPA through their clinical manifestations alone. Without neuroimaging, they might be misdiagnosed as Meniere’s disease, labyrinthitis, or delayed endolymphatic hydrops. Therefore, it is incumbent upon the clinician to rule out PCF meningiomas, especially when patients present with hearing loss and frequent vertigo.
In the present case, hypofunction of vestibular ocular reflex (VOR) presented with a direction-changing and gaze-evoked nystagmus. This suggested damage to the floccular region. The flocculus of the vestibulocerebellum contributes to horizontal smooth gaze pursuit and cancellation of VOR by a fixed target.7,8
According to the World Health Organization grading system for CNS tumors, meningiomas are histologically classified into 3 grades: grades I (benign), II (atypical), and III (malignant or anaplastic). Benign (grade I) meningiomas constitute approximately 75% of the total. They include meningotheliomatous, fibrous, and transitional subtypes. Atypical (grade II) meningiomas account for 20% to 35% and include choroid plexus and clear cell subtypes. Malignant or anaplastic (grade III) meningiomas include papillary, rhabdoid, and anaplastic subtypes and comprise only 1% to 3% of the total.9
For diagnosis and evaluation of meningiomas, MRI is the most frequently selected neuroimaging technique. Advances in MRI such as high resolution, multiplanar views, and reconstructed 3-dimensional simulations have improved its ability to predict outcomes for complete removal of tumors. The dural tail sign occurs because of thickening and enhancement of the dura and is a characteristic imaging feature. Magnetic resonance angiography can be performed to help surgeons plan embolization. This can reduce the extensive blood supply to the tumors and decrease intraoperative bleeding.
Several treatment options are available for meningiomas. Treatment modalities include observation, radiotherapy, surgery, and any combination of these.2 Although some meningiomas might be inoperable, surgical resection would be the first choice of treatment if the tumors were located in an easily accessible area of the brain. For inoperable tumors that cannot be safely removed, atypical and malignant, or residual or recurrent tumors, radiation therapy such as stereotactic radiosurgery could be considered.4 Stereotactic radiosurgery is a highly effective alternative to surgery, as well as an adjuvant treatment for residual or recurrent tumors.10 The “wait-and-monitor” option requires serial imaging and regular follow-ups. It is a reasonable approach for small tumors that do not show evidence of growth or related symptoms.2 In deciding the treatment, the relative benefits of each treatment option or their combinations are weighed against the associated risks.
Declaration of Conflicting Interests
The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Dr Se-Hyung Kim is Assistant Professor in the department of Otorhinolaryngology-Head and Neck Surgery, Jeju National University School of Medicine.
The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Dr Se-Hyung Kim is Assistant Professor in the department of Otorhinolaryngology-Head and Neck Surgery, Jeju National University School of Medicine.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
The author(s) received no financial support for the research, authorship, and/or publication of this article.
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