Aplasia and hypoplasia of the vestibulocochlear nerve: diagnosis with MR imaging.

PURPOSE: To introduce aplasia or hypoplasia of the vestibulocochlear nerve (VCN) as a possible cause of hearing loss and to identify the magnetic resonance (MR) imaging characteristics of this entity. MATERIALS AND METHODS: In seven patients with congenital deafness or unexplained sensorineural hearing loss, MR imaging enabled diagnosis of aplasia or hypoplasia of the VCN. Axial (0.7-mm) three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) images and parasagittal reconstruction images perpendicular on the course of the VCN were obtained. Twenty normal inner ears were also studied; their findings were compared with those of the patients. RESULTS: The facial nerve and inferior and superior vestibular and cochlear branches of the VCN were identified on the MR images in the 20 normal inner ears. Aplasia of the VCN was detected in two patients with normal labyrinths but with a severe stenosis of the internal auditory canal. A common VCN with absence of the cochlear branch was found bilaterally in two patients with a congenital malformation of the labyrinth. A common VCN with absence or hypoplasia of the cochlear branch was found in three patients with normal internal auditory canals and labyrinths. CONCLUSION: Submillimetric gradient-echo images (eg, 3DFT-CISS) should always be used to exclude aplasia or hypoplasia of the cochlear branch of the VCN in all cochlear implant candidates and patients with congenital deafness. This entity, which can occur with or without associated labyrinthine malformation, should be confirmed in two planes.

Inner ear malformations in patients with sensorineural hearing loss: detection with gradient-echo (3DFT-CISS) MRI.

The sensitivity of different MRI sequences in the detection of inner ear malformations in patients presenting with sensorineural hearing loss (SNHL) and/or vertigo was evaluated. We studied 650 patients presenting with SNHL and/or vertigo, clinically not suspected of having inner ear malformations. The sensitivity of T1-weighted, Gd-enhanced T1-weighted and (when available) T2-weighted spin-echo images, and three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) gradient-echo images, to unexpected malformations was assessed. Inner ear malformations were found in 15 (2.3%) of these patients. Enlargement of the endolymphatic duct and sac was the most frequent malformation, found in 11 patients. The 3DFT-CISS images showed all lesions; the other sequences were less sensitive and the pathology was missed, partially or only retrospectively seen in 11 of the 15 patients. Therefore, in addition to the routine unenhanced and Gd-enhanced T1-weighted and T2-weighted images, thin gradient-echo (3DFT-CISS) images are necessary to detect all clinically unexpected inner ear malformations in patients presenting with vertigo and/or SNHL.

[Hearing loss following lumbar puncture: case report and literature study]. / Gehoorsdaling na lumbaal punctie: gevalsbespreking met litteratuurstudie.

This report describes a patient with permanent hearing loss after spinal anesthesia. The literature is reviewed, particularly concerning incidence and possible causes. The incidence is surprisingly high and related to the size of the needle. The role of the cochlear aqueduct as a transmitter of a decreased fluid pressure in the subarachnoid space to the perilymph of the labyrinth is discussed.

MRI in craniofacial fibrous dysplasia.

Five patients with biopsy-proven craniofacial fibrous dysplasia underwent MRI with T1- and T2-weighted sequences and a gadolinium-enhanced T1-weighted spin-echo sequence. Low to intermediate signal intensity was usually seen in the largest part of the lesion on both spin-echo sequences, but smaller regions of hyperintensity on T1- and T2-weighted images and intermediate signal intensity throughout a lesion on T1-weighted images were also seen. All lesions enhanced but only two became iso- or hyperintense compared to fat. High clinical and pathological activity in three cases correlated with high signal intensity on both spin-echo sequences and with strong enhancement in two of the three. The presence of large veins or sinusoids on pathological examination did not correlate with the enhancement pattern.

Long-term (2-year) isradipine data in the treatment of mild-to-moderate hypertension.

At the end of a short-term (3-month) study of antihypertensive treatment of mild-to-moderate hypertension, 141 of the 200 study patients continued into a 2-year follow-up of isradipine as monotherapy or in combination with other antihypertensive agents. Although all 141 patients completed the first year, only 102 completed the study. Twenty-four patients dropped out: 2 with flushing; 1 each with arrhythmia, edema, angina, and headache; 12 who were noncompliant; 2 with disease unrelated to the study drug; and 4 for reasons unknown. Before the follow-up, 70% of the 141 patients were taking isradipine; after 2 years, 63% were still taking isradipine as monotherapy. During the follow-up study, the blood pressure remained stable (142.9/86.8 mm Hg after 3 months, and 142.9/86.2 mm Hg after 2 years), whereas the normalization rate was only slightly changed (73 vs. 75.2%). The incidence of reported adverse events decreased with time. At the end of the short-term study, 44.7% of patients had reported one or more adverse events; after 2 years of treatment, only 14.4% reported adverse events. Two patients had ECG signs of left ventricular hypertrophy: one showed no relevant changes while the other presented clear signs of regression. No clinically relevant laboratory abnormalities were noted during the study. In conclusion, isradipine is effective, well tolerated and safe in the long-term treatment of mild-to-moderate hypertension.

[Orbital involvement of sinusitis]. / Orbitale verwikkelingen van een sinusitis.

Sinusitis can spread to the orbital tissues. 'Preseptal' soft-tissue bacterial infections are relatively common during childhood and with appropriate antimicrobial therapy they usually resolve quickly. 'Orbital' soft-tissue infections, in contrast, are relatively rare and frequently cause serious morbidity. Four cases are presented. Symptomatology and pathogenesis are reviewed. Diagnostic procedures, especially CT-scan, are discussed and recommendations are given for medical and surgical management.

[Ototopic agents]. / Ototopica.

In treating ear disease every otolaryngologist is familiar with topical medications. The choice of the ideal agent for a given clinical situation may be difficult. In order to make this decision more easy, we overlook the composition of ear-drops. The most important disadvantages are ototoxicity, allergy and pain. The place of ototopical agents in otitis externa, otitis media and otomycosis is discussed.

[Nasal endoscopic approach to sinus diseases]. / Nasale endoscopische benadering van sinusaandoeningen.

The endoscopical endonasal surgery is in vogue. Today it has become a functional surgery. A thorough knowledge of the endonasal anatomy is difficult but essential. The antral ostium is reached after infundibulotomy, from which starts the inspection of the locoregional pathology.

[Mesenchymal chondrosarcoma]. / Mesenchymaal chondrosarcoma.

A case is presented of mesenchymal chondrosarcoma. This extremely rare tumor has a characteristic histology. The prognosis is poor. Radical surgery is essential. A lifelong follow-up is recommended.

Vascular tinnitus and arteriovenous meningeal fistula.

With the development of improved angiographic techniques, arteriovenous meningeal fistulas are being recognized more and more as an important cause of vascular tinnitus. Vascular tinnitus is generally considered to be objective and pulse-synchronous. This report concerns three cases of vascular tinnitus due to arteriovenous meningeal fistulas. They all could not or only hardly be objectified by auscultation and in one of them any pulse-synchronism was denied. The pathophysiology of vascular tinnitus is reviewed, followed by a more detailed discussion about the pathogenesis, the symptomatology and the radioanatomical aspects of arteriovenous meningeal fistulas. Finally, the report deals with the treatment of those fistulas.

[The CO2 laser in otorhinolaryngology. Advantages as opposed to conventional surgery and current possibilities]. / De CO2-laser in de otorhinolaryngologie. Voordelen ten opzichte van conventionele chirurgie en nieuwe mogelijkheden.

The future of lasers in medicine in general and in otorhinolaryngology in specific appears to be bright. It's use is very simple, but may not weaken the wakefulness of its user. Skill and experience remain very important. The role of the laser is clearly defined. The laser is not the only surgical tool to be used for any given operative procedure.

A survey of electro-oculography.

The vestibulometric examination in the ORL office is done by means of the registration of eye movements of vestibular origin, the electro-nystagmography. With the ENG-equipment one can perform electro-oculography, examining eye movements in general. EOG is quite helpful in neurologic diagnosis and important in ORL practice too. The adjustment of the ENG-equipment to EOG is explained, after some words on the physiology and the clinical examination of ocular motor function. Than a short survey is given of the abnormalities that may be met in EOG.

Spontaneous cerebrospinal fluid otorrhea.

A case of spontaneous cerebrospinal fluid otorrhea due to a congenital tegmen tympani defect is reported. The etiology and pathogenesis of cerebrospinal fluid otorrhea and of the spontaneous congenital form in particular is discussed, together with the clinical picture and the methods of diagnosis. Reviewing the literature, a middle ear exploration is considered to be the procedure of choice to ascertain the presence and determine the exact locus of the cerebrospinal fluid leak.

Nodular thyroid disease and cancer.

Different possibilities of solitary or multinodular involvement of the thyroid gland are described. Because of the broad spectrum of malignancy there exists a controversy and widespread divergence of opinion regarding the incidence, malignancy, classification and treatment of thyroid cancer.

[Wegener's granulomatosis in the ORL area]. / Wegener's granulomatosis in het ORL-gebied.

Overlooking the literature, Wegener's granulomatosis often seems to have manifestations in the ear-nose-throat region, especially in the beginning. The usual clinical findings are mentioned, to put the attention of the ENT-surgeon on this affection. Indeed, by early recognition and proper treatment of this disease, the prognosis is clearly improving. A case-history is illustrating the clinical picture.

[Role of air-CT-meato-cisternography in the diagnosis of acoustic neuroma]. / De plaats van de luchtmeatocisternografie met CT-scan in de diagnose van acousticusneurinoma.

The CT-meato-cisternography with air is at the moment the examination of choice in the diagnosis of the intra-canalicular acoustic neuroma. Every time there is a possibility of acoustic neuroma the air-CT-meato-cisternography has to be performed after the CT-scan was negative. The air-CT-meato-cisternography is easily performed on an outpatient basis and complications are never seen. Filtrated air seems to be the best contrast material.

[Study of the ciliary motility and the mucus in sinusitis]. / Het onderzoek van de ciliare motiliteit en de mucus bij sinusitis.

The white mucus or the opaque mucus lump found in chronic sinusitis may be considered a consequence of hypersecretion, or/of a poor drainage due to ostial dyspermeability, caused by a deficiency of the ciliary system or a modification of the viscosity.

[The Noonan syndrome]. / Het Noonan-syndroom.

Two cases of Noonan syndrome are observed. The Noonan syndrome is a Turner-like syndrome with normal chromosomal constitution. Essential criteria in diagnosing the syndrome are the facial expression (hyper-telorisme, low-set and malformed ears, micrognathie, fish-like mouth). The short webbed neck and the cubitus valgus. These children are rather small and mental retardation can be present, especially in comparison with their siblings. Associated cardiac malformations occur in approximately 50% of the cases. We insist on audiologic findings. One case has a hearing impairment due to a malformation of the middle ear, recognised radiologically. The other has a deafness of perception type. Yet, the familial occurrence of last patient makes it doubtful whether this hearing loss can be linked to the Noonan-syndrome.

[Resorption of depot cortisone after its submucosal injection in the nasal concha]. / Etude de la résorption de la cortisone dépôt après son injection sous-muqueuse dans le cornet nasal

Injections of "depot" steroid preparations in the nasal mucosa are frequently used for allergic disorders. We felt it would be interesting to study the fate of these injections. Indeed, a microscopic section through the nasal concha shows a tremendous amount of large vessels and it seems possible that a quantity of the injection enters the general blood stream. The difficulties in obraining radioactive cortisone made us choose the red blood cell as a tracer. They have the same dimensions as the cortisone crystals used in the depot preparations, and are a much used tool in a department of nuclear medicine. Mostly they are marked with radioactive chromium. We modified the technique used for the determination of the blood volume by a dilution method. In this technique radioactive blood is injected and the dilution allows for calculation of the whole blood mass. We injected the radioactive blood into the nasal concha and we searched for these radioactive erythrocytes in the circulation. This gave us an idea of the total amount that escaped from the concha. Blood was taken from a patient and marked by the department of nuclear medicine; it was then injected in the concha in the way a depository cortisone preparation is injected, and after several moments blood was taken from an arm vein and the radioactivity measured. Relying upon a normal blood volume in our patient, we were able to calculate the amount that went into the circulation. As the chart shows, there is a very fast increase and after 15 minutes, practically everything was in the blood stream. Thus we conclude: 1. Intra-nasal injections of cortisone are no more useful than intra-muscular ones. 2. Some reactions after this intranasal injections could be explained by the enmasse embolisation of the crystal suspension.