[Long term results of congenital middle ear cholesteatoma in children]. / Résultats à long-terme des cholestéatomes congénitaux de l'oreille moyenne de l'enfant.

OBJECTIVES: To report the long term results of congenital cholesteatoma of the middle ear in children. PATIENTS AND METHODS: Retrospective study of thirty two children with 33 congenital cholesteatoma (1 bilateral cholesteatoma) operated on by the same surgeon. The mean age was 6 years. The most common clinical presentation was unilateral hearing loss. A trans-canal approach was performed in 4 cases and an intact canal wall technique in 29 cases. A two staged surgery was necessary in 28 patients, whom a residual cholesteatoma was observed in 7 patients. A third stage was performed in 11 patients because of a residual cholesteatoma in 2 two cases and a functional failure in 9 cases. RESULTS: At five years postoperative audiometry (air bone gap inferior or equal to 20 dB HL), a functional success was obtained in 50% of cases. CONCLUSION: The diagnosis of congenital cholesteatoma must be evoked in every atypical otitis media with effusion or unilateral transmission hypoacusis. The intact canal wall technique in two stages is the most appropriate treatment, since congenital cholesteatoma in children appears aggressive in a well pneumatized mastoid. The choice for an intact canal wall technique is also justified by the ambition of a conservative surgery in the young child. The functional results are generally satisfactory in early diagnosed cases. Thus, ENT and pediatric physicians have a unique role in detecting congenital cholesteatoma in the early period of life, and in ensuring a long term follow-up.

Clinical experience with the Vibrant Soundbridge implant device.

OBJECTIVE: To evaluate the full degree and range of benefits provided by the Vibrant Soundbridge (VSB; Symphonix Devices, Inc., San Jose, CA, U.S.A.) and analyze pre-and postoperative results of audiologic tests. STUDY DESIGN: Single-subject study with each subject serving as his or her own control. SETTING: Multicenter clinical study conducted at 10 centers in Europe. PATIENTS: 47 patients who met the selection criteria for participation in the study. INTERVENTIONS: Implantation of the VSB direct-drive middle ear hearing device. MAIN OUTCOME MEASURES: Average change in unaided thresholds with the patient wearing headphones at each frequency pre-and postsurgery was measured. A mean threshold change less than 5 dB across all frequencies was considered clinically nonsignificant. RESULTS: 47 patients had successful surgery for implantation and fitting with the VSB device. CONCLUSION: The VSB is a new middle ear implant device that can be used safely in the treatment of patients with moderate to severe sensorineural hearing loss.

Diagnostic value of vibration-induced nystagmus obtained by combined vibratory stimulation applied to the neck muscles and skull of 300 vertiginous patients.

On subjects with unilateral vestibular dysfunction, the application of a vibratory stimulation (100 Hz) to the two mastoids and the vertex, and to the right and left dorsal neck muscles produces a nystagmus directed towards the good ear in 85% of patients. Fixation must be suppressed by Frenzel's glasses or video nystagmoscopy. To be significant this nystagmus must appear in at least 3 of the 5 vibratory stimulated sites. On healthy subjects nystagmus is present in 6% of cases but never in those below 30 years. In subjects affected by central vertigo, nystagmus was elicited in 10% of cases and in subjects suffering from vertigo of unknown origin in 6% of cases. Vibration nystagmus which stops immediately after stimulation differs from head shaking nystagmus which is present in only 34% of unilateral vestibular dysfunctions. Vibration occasionally produces a pseudo-caloric nystagmus which persists after stimulation. We believe that vibratory stimulation is a useful test, quick and easy to perform. In conjunction with questionnaires, clinical examination, positional testing and the results of audiometry, it gives an immediate indication of a peripheral lesion when the vertigo is seen for the first time. With unilateral deafness, a positive test leads one to suspect an acoustic neuroma. Conversely if the test becomes negative after a vestibular neuritis when it was initially positive, it is a sign of recovery.

Multicenter audiometric results with the Vibrant Soundbridge, a semi-implantable hearing device for sensorineural hearing impairment.

The Vibrant Soundbridge, a semi-implantable hearing device for subjects with moderate to severe sensorineural hearing impairment was introduced commercially. First audiologic results are presented on 63 patients from 10 European implant centers. Hearing loss was at 0.5, 1, 2, and 4 kHz varying between 43 and 81 dB HL. The patients used the analogue audio processor, type 302. Measured sound-field gain was compared with NAL-R target values. For most patients an acceptable agreement was found. There was a subgroup of patients, however, with relatively low gain. The results suggest that this was related to the suboptimal positioning and fixation of the transducer to the incus.

[Middle ear cholesteatoma surgery]. / La chirurgie des cholestéatomes de l'oreille moyenne.

UNLABELLED: To verify the efficiency of surgical techniques used by the senior author in cholesteatoma surgery, 1,048 cases operated on between 1966 and 1997 are reviewed: 739 in adults and 309 in children. In adults, the majority of cases were between 16 and 40 (64.5%) without difference between both sexes. On the contrary in children, boys were 200 and girls 109; 30 cases were less than 4. In adults only 312 cases were operated on first hand, instead in children almost all cases were operated on first hand (273 cases). The beginning of the cholesteatoma was usually the Shrapnell membrane in adults (58%) while in children the postero-superior region was as common as the Shrapnell membrane (34% 35%); the cholesteatoma was congenital in 13% of cases. Surgical techniques: Mostly two techniques were used: closed technique in two stages and obliteration technique in one or two stages. The closed technique requires remodeling of the postero-superior region of the bony canal, performing a correct posterior tympanotomy, placing a silastic sheeting in all the middle ear to prevent fibrosis and finally rebuilding all the bony defects and a large part of the tympanic membrane with thin cartilage with its perichondrium. The second stage is performed 18 months later in children, 24 months in adults. The ossiculoplasty is often performed at the first stage if the stapes arch is intact and safe. The obliteration technique requires also a precise removal of the lesions, a lowering of the facial ridge, the rebuilding of the tympanic membrane with fascia and the posterior cavities obliteration with Palva flap and free connective grafts. If the tympanic and posterior cavities are reasonably safe, and if the stapes arch is intact, one stage is performed. Otherwise a second stage is performed usually 18 months later, ossiculoplasty only or combined with a second look of posterior cavities under the flap. RESULTS: Residual cholesteatoma was noticed in adults in 16% of first hand closed technique and 19% of second hand closed technique. It is only of 7% in first hand obliteration technique and 13% in second hand obliteration technique. It was 16% in radical cavity reconstruction. Retraction pocket was almost only observed in closed technique. In adults, an early retraction pocket was observed in 3.5% of cases and in children 12.5% of cases at the moment of the second stage. A larger cartilage more precisely fixed with fibrin glue seems to have almost suppressed the early retraction pocket. Some late retraction pockets nevertheless appeared: 6 cases in adults over 394 and 5 cases in children over 236. Hearing results in adult were the best with first hand closed technique 84% of good results if the stapes was intact and 51% if the crura were missing. If the closed technique was performed second hand, these results were only 57% if the stapes was intact and 29% if the crura were missing. For the obliteration technique these results are for the first hand 70% of good result if the stapes was intact and 60% if the crura were missing, for the second hand, only 36% if the stapes was intact and 39% if the crura were missing. In radical cavity reconstruction 63% of good results were achieved when the stapes was intact and 48% if the crura were missing. The good result is an air-bone gap between 0 and 20 dB for the three frequencies 0.5, 1 and 2 kHz.

[The facial nerve and the retrosigmoid approach]. / La facial et la voie rétrosigmoide.

The author gives an account of his experience with the retrosigmoid approach, and emphasizes the preservation of the facial nerve and hearing. A detailed description of the operative technique is given. This approach is used when it is intended to conserve the hearing. The series consists of 2 stage III cases and 26 stage I and II cases. The two stage III cases had total deafness. For the other stages, 25 patients had normal facial function postoperatively. The hearing was preserved in 9 cases in the frequency range 500-1000-2000 Hz, with thresholds of less than 30 dB, and in 7 of cases these criteria were also true for the 4000 Hz frequency. The author feels that the retrosigmoid approach with facial nerve monitoring provides the same degree of safety for the facial nerve as the translabyrinthine approach for surgery of stage I and II tumours.

[Gadolinium and contrast medium MRI of the acoustic nerve in patients with meningeal neuritis and acoustico-facial syndrome]. / Imagerie en résonance magnétique avec Gadolinium et prise de contraste de la VIIIe paire au cours de méningo-névrites avec syndrome acoustico-facial.

Twelve cases of vestibular neuritis were investigated in gradient echo MRI with gadolinium. Only 3 severe cases associated with an acoustico facial syndrome (2 cases of herpes zoster oticus and one case after influenzae) demonstrated focal enhancement within the internal auditory canal on post contrast T1 weighted images. This enhancement involved at least 2 differents nerves. These 3 severe cases associating sensory neural hearing loss and facial palsy revealed a meningeal reaction after cerebrospinal fluid examination. The enhancement lasted a long time (up to 10 months) in one case of RAMSAY HUNT syndrome associated with a chronic lymphocytic leukemia. The MRI was able to confirm the anatomical reality of the vestibular neuritis and more precisely of the meningoneuritis and gave arguments for the theory of the polyneuropathy of Adour. Enhancement at MRI seems correlated with the severity of the affection (permanent vestibular areflexia in 3 cases and permanent hearing loss in 1 case).

Benign positioning vertigo (BPV) and three-dimensional (3-D) eye movement analysis.

Fifty two patients with positioning nystagmus were studied with the V.N.G. three-dimentional device of ULMER. In benign paroxysmal positional vertigo (BPPV) the torsionnal component is not pure: a vertical and a less important horizontal components also exist. They are not of the same amplitude on both eyes. The torsionnal and horizontal components are more important on the eye ipsilateral to the BPPV. The vertical component is more important on the contralateral eye. The horizontal canal B.P.V. (2% of the cases of B.P.V.) is so defined by the absence of vertical and torsionnal components. Three-D Eye Movement Analysis is helpful for differential diagnosis with the positional protocol described. The characteristics of central and peripheral paroxysmal positional nystagmus are given. The head Tilt and the ocular counter rolling reflex (O.C.R.) can be quantified with this device. It will be useful in the future to better explore the inferior root of the eighth nerve and the otolith organ. For clarity we propose to describe the torsionnal nystagmus so that the results are expressed with respect to the patient: clockwise nystagmus (in reference to the patient) would thus be also right rotatory nystagmus while left rotatory nystagmus would be also anticlockwise.

A human homologue of the Drosophila eyes absent gene underlies branchio-oto-renal (BOR) syndrome and identifies a novel gene family.

A candidate gene for Branchio-Oto-Renal (BOR) syndrome was identified at chromosome 8q13.3 by positional cloning and shown to underlie the disease. This gene is a human homologue of the Drosophila eyes absent gene (eya), and was therefore called EYA1. A highly conserved 271-amino acid C-terminal region was also found in the products of two other human genes (EYA2 and EYA3), demonstrating the existence of a novel gene family. The expression pattern of the murine EYA1 orthologue, Eya1, suggests a role in the development of all components of the inner ear, from the emergence of the otic placode. In the developing kidney, the expression pattern is indicative of a role for Eya1 in the metanephric cells surrounding the 'just-divided' ureteric branches.

[Surgical treatment under endoscopic control of cerebrospinal fluid rhinorrhea of sphenoid origin. A propos of 5 cases]. / Traitement chirurgical sous contrôle endoscopique des rhinorrhées cérébrospinales d'origine sphénoïdale. A propos de 5 cas.

From 5 to 15% of cerebrospinal fluid (CSF) leaks come from the sphenoid, subdivided in two groups: traumatic and spontaneous. Many surgical approaches are used for their treatment, with consistent morbidity. Five sphenoidal CSF leaks (3 traumatic and 2 spontaneous) were operated only by endoscopic endonasal route from 1993 to 1995, after endoscopic and computerized tomography (CT) scan evaluation. A sphenoidotomy by a simple endonasal route through the spheno-ethmoidal recess was performed in 3 cases. In the other 2 cases, the sphenoidotomy required a trans-ethmoidal approach through the posterior part of the ethmoid. After identification of the leak and the removal of mucosa, the sphenoid sinus was filled up by abdominal fat kept in place by biological glue and supported by a silastic sheat. No post-operative complication appeared. The median duration of hospitalization was 6.5 days (5-13). During the follow-up (19.5 months, 8-30), 4/5 complete remission was observed. The last case needed a second obturation at 11 months, due to a retraction of the fat, without recurrence. This endonasal endoscopic approach is safe and efficient for leaks closure, with no morbidity compared with others invasive approaches.

Coexistence of plasma cell granulomas of lung and central nervous system.

A rare case of concurrent plasma cell granulomas (PCG) of the lung and the central nervous system (CNS) is reported. A 30-year-old man was presented with recurrent left headaches lasting for two years. Computerized tomographic (CT) scan and magnetic resonance imaging (MRI) of the head disclosed a process extending from the lateral aspect of the left cavernous sinus to the tentorium cerebelli and the infratemporal fossa through the foramen ovale. At the same time, chest-X ray and CT scan showed three symptomless masses of the pulmonary right lower lobe. Histological examination of cerebral samples and of one of the pulmonary nodules revealed the presence of a fibrous tissue containing numerous lymphocytes and plasma cells as well as remnants of vascular and respiratory structures. Immunohistochemical study proved these cells to be polyclonal. Ultrastructural analysis confirmed the presence of lymphoid cells and failed to disclose any argument for meningioma or histiocytosis X. The differential diagnostic problems of PCG are discussed as well as considerations about clinicopathological features, histogenesis and pathogenesis of inflammatory pseudotumours (IPT).

Nucleus 20-channel and 21-channel auditory brain stem implants: first European experiences.

Central electrical stimulation of the auditory pathway can allow hearing in patients suffering from deafness localized in the auditory nerve. Developments in a multichannel auditory brain stem implant based on the Nucleus Mini 22 cochlear implant with transcutaneous signal transmission is discussed. The device has been implanted in seven European patients who suffered from neurofibromatosis type 2. Preliminary speech perception results and patient satisfaction are encouraging, and the data presented include some limited open-set speech recognition.

Immunohistochemical detection of p53 protein in preneoplastic lesions and squamous cell carcinoma of the head and neck.

Alterations in the p53 tumor suppressor gene are the most frequent genetic abnormalities in human cancers. The p53 protein is present in normal cells, and is assumed to induce G1 arrest or apoptosis in the presence of DNA lesion. The mutant protein lacks this property. Squamous cell carcinomas of the head and neck (SCCHN) are related to carcinogens in tobacco and alcohol, and provide a good model of multiple-step carcinogenesis in association with DNA damage and p53-related tumorigenesis. Stabilization of the mutant p53 protein allows immunohistochemical analyses (IHC) to be routinely used to demonstrate the mutant p53 protein in tissue samples, whereas normal p53 protein is undetectable. Ninety-nine squamous cell carcinomas, 8 in situ carcinomas, 31 preneoplastic lesions and 79 normal carcinogen-exposed mucosas of the head and neck from a total of 107 patients were examined for the expression of p53 tumor suppressor gene protein. Samples were collected before treatment, and stained with p53 specific mono- and polyclonal antibodies (DO-7, Pab 1801 and 240, CM1) using an indirect immunoperoxidase technique. Proliferating cell nuclear antigen (PCNA) provided semiquantitative estimates of proliferation. The main localizations were the pharynx (64/107) and the larynx (21/107). Positive IHC detection of p53 was observed in 9% of normal-appearing carcinogen-exposed mucosas, 37% of hyperplasias, 68% of dysplasias, 75% of in situ carcinomas, and 56/99 (56.5%) of primary tumor samples. Mucosas from 15 control patients under 10 years of age were negative. There was no correlation between p53 IHC and localization, differentiation or TNM staging.(ABSTRACT TRUNCATED AT 250 WORDS)

[Minor malformations of the ear ossicles. New classification and therapeutic results]. / Les malformations mineures de la chaine ossiculaire. Nouvelle classification et résultats thérapeutiques.

We tried to classify 46 minor malformations in 5 groups: 0: Normal ossicular chain, almost normal tympanic membrane but small atretic plate around malleus handle. I: Fixation of the malleus head. II: Normal ossicular chain but fixation of the footplate with or without abnormality of the facial nerve. III: Lack of a part of the ossicular chain with or without abnormality of the stapes. IV: Severe malformation of all the ossicular chain. We operated upon 4 malformations of type 0, 8 of type I, 10 of type II, 14 of type III and 10 of type IV. An air-bone gap within 20 dB was achieved in only 15 cases. As a matter of fact, in 9 cases the facial nerve covered the oval window; there were 4 pseudo-Mondini; ossiculoplasty was performed on a totally modified footplate in one case.

[Benign paroxysmal positional vertigo. Apropos of 51 cases]. / Vertige positionnel paroxystique benin. A propos de 51 observations.

51 cases of benign paroxysmal vertigo have been investigated in 3 years. The findings put in evidence: the particular frequency of this pathology (18% of BPPV in a vestibulometry consultation), its opportunist character and the possible association with other more severe pathologies (meningiomas, multiple sclerosis, cerebellar venous angioma, vascular cerebral infarction) or more benign pathologies (labyrinth malformation, middle fossa atrophia). Sémont liberatory maneuver revealed to be much efficient (50% of patients free of disease after a single repositioning manoeuvre). Only 3 cases remaining unchanged after 2 years and intractable were proposed for surgery. We mention 7% of bilateral forms and 5% of alternating recurrent forms (sometimes on the right and sometimes on the left side). The recurrence is noted in 50% of BPPV followed at least during one year. We describe a particular topographic form concerning probably a cupulolithiasis of the external canal. 53% of cases beneficiated of CT-scan or of magnetic resonance imaging. The frequency and the possibility of pathological associations with BPPV bring to a particular vigilance for the diagnosis and to a particular care in the affirmation of the benignity in the case of long lasting vertigo (superior to 6 months), or when the nystagmus duration in Hallpike position is superior to 60 seconds.

[Implantation on the cochlear nucleus. Apropos of a patient with bilateral neuroma]. / Implantation sur le noyau cochléaire. A propos d'un malade atteint de neurinome bilatéral.

A new auditory brainstem implant (ABI), manufactured by Cochlear, was implanted in a young patient suffering from a large bilateral acoustic neuroma, associated with multiple other tumors due to a tuberor sclerosis of Bourneville. The operating procedure and the first results are described. A short historical summary, the anatomical basis and the difficulties due to side effects of stimulation are exposed.

Indirect immunofluorescence in the investigation of rapidly progressive sensorineural hearing loss and Menière's disease.

Being aware of the conservation of antigenicity, we performed indirect immunofluorescence on non fixed non decalcified frozen hamster's cochlea. Once killed, the heads of the hamsters were immediately dipped into liquid nitrogen and then stored at -20 degrees C until cutting. The sections were done at the same temperature, with Tissue-Tek embedding the heads to avoid breaking, using a sharp tungsten knife. The immunofluorescence itself was performed with the sera of the patients which were revealed through donkey fluorescein-conjugated antiserum to human immunoglobulins. For counterstaining, we used Evans blue. Twenty patients with rapidly progressive sensorineural hearing loss (according to the clinical criteria of MacCabe) and 6 with Menière's disease were tested. Five had a specific fluorescence on the stria vascularis. A correlation with the lymphocyte transformation test on human inner ear antigen has been found (p = 0.055).

[Extensive cholesteatoma without intrapetrous involvement. Discussion on the surgical approach]. / Les cholestéatomes extensifs sans invasion intrapétreuse. Discussion de l'attitude chirurgicale.

Based on 8 observations, the authors analyze the extension of middle ear cholesteatomas on the anterior attic and the anterior supralabyrinthine cellular groove, the internal retrotympanum and the posterior sublabyrinthine cellular groove, the hypotympanum and the anterior sublabyrinthine groove, the retrolabyrinthine region and the translabyrinthine and posterior supralabyrinthine grooves. Despite progress in imaging and optical instrumentation, exact precision of the lesions sometimes remains impossible. The patient must be warned of a possible enlargement of the operative pathway and its consequences.