Isolated synchronous meningioma of the external ear canal and the temporal lobe.

BACKGROUND: Extracranial meningiomas are common tumours but may occur in unexpected locations. Awareness can avoid misdiagnosis and inappropriate management of these tumours. We report the case of a 54-year-old Caucasian male with en plaque meningioma of the external ear canal and an intracranial temporal lobe meningioma. INTERVENTION: The patient underwent extended canal wall down atticomastoidectomy with preservation of the ossicular chain and tympanic membrane and en bloc resection of the bony posterior canal wall, tumour, and overlying skin. RESULTS: Radical removal of a grade I meningothelial meningioma was achieved. The tegmen tympani and dura were not breached. No connection with the temporal lobe meningioma was demonstrated. The patient recovered completely and experienced a marked improvement in hearing. No clinical signs of recurrent or residual tumour have occurred. CONCLUSION: Careful clinical examination and extensive radiological workup is required to avoid missing the unusual diagnosis of concurrent meningioma of the temporal bone and temporal lobe, and miss the chance to treat this disease adequately.

Superior semicircular canal dehiscence: prevalence in a population with clinical suspected otosclerosis-type hearing loss.

Superior semicircular canal dehiscence (SSCD) can present with a variety of symptoms that can be predominantly auditory, predominantly vestibular or both. It can mimic a wide range of otological disorders, in particular otosclerosis-like stapes fixation. Our study revealed that, in 5.3% of our patients with clinically suspected otosclerosis, SSCD was detected in high-resolution multi-detector computed tomography (HRMDCT) of the temporal bone. We therefore emphasise the value of HRMDCT with reconstructions in the plane of the superior semicircular canal and perpendicular to the superior semicircular canal in the diagnostic work-up of each patient with a tentative diagnosis of otosclerosis-type stapes fixation. Where there are doubts, VEMP testing should be performed. We believe that a number of unexplained complications after an uneventful stapedotomy procedure might be explained by a pre-operatively undetected dehiscent superior semicircular canal and therefore unwarranted surgery. The typical feature of the conductive hearing loss due to SSCD compared to otosclerosis is an air-bone gap which is much more prominent at low frequencies than at high frequencies.

Meniett therapy: rescue treatment in severe drug-resistant Ménière's disease?

CONCLUSION: Our data indicate that Meniett therapy is unlikely to be helpful in the long-term treatment of patients with severe, drug-resistant Ménière's disease (MD) in whom injection of intratympanic gentamicin (ITG) or another destructive procedure would otherwise be performed. OBJECTIVE: To investigate the value of Meniett therapy in patients with drug-resistant MD referred for injection of ITG. MATERIAL AND METHODS: Twelve patients referred for ITG treatment were followed during a 2-month period of Meniett therapy. Symptoms, functional level and hearing status were evaluated using a standardized staging system. Disease-specific quality-of-life measures were obtained before and after Meniett therapy. At the end of the study period, patients were followed for a mean of 37 months, thus providing long-term outcome data. RESULTS: In two patients, Meniett treatment was interrupted after 1 month because of persistent severe vertigo. In the remaining 10 subjects, we found a significant decrease in the median number of vertigo spells from 10.0/month (25th-75th percentile 4.0-19.0) prior to treatment to 3.0/month (25th-75th percentile 1.5-4.5) after treatment (p = 0.02). There was, however, no improvement in hearing status, tinnitus, functional level or self-perceived dizziness handicap. Long-term (>1 year) follow-up data revealed that only 2 subjects preferred to continue Meniett therapy and that ablative surgery had to be performed in 6/12 study patients.

Preoperatively diagnosed utricular neuroma treated by selective partial labyrinthectomy.

Unlike the acoustic neuroma that occupies the internal acoustic meatus and extends into the cerebellopontine angle, the true intralabyrinthine neuroma is an extremely uncommon finding. The majority of the published cases were found at autopsy or during destructive labyrinthectomy for uncontrollable Meniere's syndrome. To the best of our knowledge, we present the smallest exclusively intrautricular neuroma that has ever been diagnosed preoperatively. Detailed magnetic resonance imaging studies allowed the detection of this tumor, measuring only 3.5 mm in diameter. The tumor was removed via atticomastoidectomy and partial labyrinthectomy. The diagnostic workup, radiographic characteristics, and surgical treatment are discussed, together with a review of the literature.

Facial nerve neuroma. Report of two cases including histological and radiological imaging studies.

Unlike the acoustic neuroma, a facial nerve neuroma is an uncommon finding, even in referral clinical centers. Two cases of facial nerve neuromas are presented, with special focus on the importance of adequate radiological imaging techniques and histological characteristics of tumor specimens. Surgical modalities regarding tumor removal as well as facial nerve repair are discussed and the current world literature reviewed.

MRI of acoustic schwannoma: a retrospective study of 89 tumours.

Magnetic resonance imaging (MRI) is now considered the diagnostic study of choice for evaluation of patients with suspected acoustic schwannoma. This retrospective study of 118 MR examinations presents an analysis of the MR findings of 89 acoustic tumours in 86 patients. The method of examination included precontrast and gadolinium(Gd)-enhanced MRI in 72 and plain MRI in 14 patients. The common MR-appearances of acoustic schwannomas were: on T1-weighted images (WI) isointense (36%) or slightly hypointense (64%) relative to the brainstem; intense and homogeneous contrast enhancement in 62%; the shape of the tumour was round or oval in 71%; the tumour was centred at/or located in the internal auditory canal (IAC) in 80%. The small acoustic schwannomas were mostly round or oval in shape and showed homogeneous signal intensity (SI) both before and after Gd. The larger acoustic tumours were more heterogeneous in morphology and SI. The significance of these and other signs, early diagnosis and differential diagnosis are discussed.

Acoustic schwannoma: MR findings in 84 tumors.

MR imaging is the study of choice for the examination of patients with suspected acoustic schwannoma, because of its high sensitivity, especially after the use of contrast material. This essay illustrates the common MR features of acoustic schwannomas as seen in a study of 84 tumors. We pay special attention to the role of MR imaging in the distinction between acoustic schwannoma and meningioma.

[Facial neurinoma: a possible surprise in the diagnostic approach to Bell's palsy]. / Facialisneurinoma: een mogelijke verrassing bij de diagnostische oppuntstelling van een "Bell's palsy".

A neuroma of the facial nerve is a rather uncommon finding. Two cases of facial nerve neuromas, with special focus on the importance of high-definition imaging techniques and on the histologic characteristics are presented. Some surgical modalities regarding tumor removal as well as facial nerve repair are discussed.

[Problems with flute playing: an otological problem? Case report of a peculiar cerebellar astrocytoma]. / Problematisch dwarsfluitspel: een otologisch probleem? Case report van een bijzonder cerebellair astrocytoma.

A rare case of cerebellar astrocytoma presenting as a cerebellopontine angle tumour is discussed. A 35-year old woman noticed a bizarre twitching and fatigability of the left upper lip while playing the flute. There was also a mild hearing loss on the left side and she sometimes felt unsure of herself when walking. A thorough examination by means of speech audiometry, electronystagmography, ABR, CT-scan and MRI revealed a large, partly calcified mass occupying the cerebellopontine angle. Only the histological examination of the surgical specimen revealed the true nature of the tumour. The special characteristics of this tumour and the unusual clinical course are discussed. The importance of a good histological diagnosis is stressed.

Neurofibroma of the vagus nerve in the head and neck: a case report.

A case of a tumor of the vagus nerve, showing typical features of a neurofibroma, is presented. The natural history is reviewed, and several technical investigations, with particular reference to the contributions of magnetic resonance imaging and immunohistochemical techniques in the differential diagnosis, are discussed.

The solitary schwannoma of the eighth cranial nerve. An immunohistochemical study of the cochlear nerve-tumor interface.

Given recent controversy concerning hearing preservation surgery of the acoustic neurinoma, an immunohistochemical study was undertaken to investigate the cochlear nerve-tumor interface. Ten intact medium-sized acoustic neurinomas were studied by means of classic staining procedures and an immunohistochemical technique using monoclonal mouse antibodies to human neurofilaments. Our observations indicate that the cochlear nerve is histologically involved in the tumoral process in those cases in which macroscopically visible adherences between the cochlear nerve and the tumor are present. We were not able to discern a clear cleavage plane. Six of the 10 specimens showed tumoral invasion of the cochlear nerve. Several therapeutic attitudes are discussed in view of these observations and reports from the international literature. In conclusion, the principle of hearing preservation surgery is rejected in favor of total tumor removal in every case in which surgery is indicated.

[Acoustic neuroma: a histopathological study]. / Het acousticusneurinoma: een histopathologische studie.

In view of recent controversy concerning the preservation of hearing in acoustic neuroma surgery, a histologic study of the nerve-tumour interface was undertaken in order to investigate cochlear nerve involvement. Twelve intact acoustic neuromas were studied by means of Masson's trichrome stain, the Luxol fast blue technique, Verhoeff's stain and an immunohistochemical technique using monoclonal antibodies to human neurofilaments. In nine out of twelve specimens, macroscopically visible adherences were present between the cochlear nerve and the tumour. The microscopical correlate was found to be the absence of a clear cleavage plane between the cochlear nerve and the tumour on the one hand, and the presence of cochlear nerve fibers, surrounded by tumoural cells, past the assumed nerve-tumour interface on the other hand.

The involvement of the cochlear nerve in neurinomas of the eighth cranial nerve.

In view of recent controversies concerning the preservation of hearing in acoustic neurinoma surgery, we examined the courses of the vestibular and cochlear nerve fibers in 12 intact acoustic neurinomas studied in our department. Due to its lack of specificity, the Luxol fast blue stain was found to be inadequate for our study of the nerve fibers. In contrast, Verhoeff's stain proved to be satisfactory when combined with a highly specific immunohistochemical technique. There were macroscopically visible adherences between the tumor and the cochlear nerve in 9 out of the 12 specimens. From those specimens, histological sections were obtained in which both the cochlear nerve and tumor could be clearly identified. In these specimens the cochlear nerve was involved in the tumoral process and there was no clear cleavage plane between the nerve and the tumor. However, all these patients suffered only from minimal losses of hearing as a result of their tumors.

Differentiation of cartilage and bone in human fetal temporal bones with Luxol fast blue stain.

A modified Luxol fast blue technique was used to study the development of the temporal bone. This staining method makes it possible to make a clear distinction between the primitive cartilage present and the new forming bone. Although these tissues both contain a significant amount of collagen, their staining properties with the Luxol dyes are widely dissimilar, due to the different physicochemical properties of the collagen types involved in these tissues. The differentiation of mesenchymatous tissue into ligaments and joints can also be very clearly demonstrated with this technique. In studying the endochondral ossification process of the otic capsule and middle ear, the modified Luxol fast blue stain is a valuable technique that is complementary to more conventional staining methods.