Lip-reading abilities in a subject with congenital prosopagnosia.

We present the case of an individual with congenital prosopagnosia or "face blindness", a disorder where the ability to recognize faces is impaired. We studied the lip-reading ability and audiovisual perception of this subject using a DVD with four conditions (audiovisual congruent, auditory, visual, and audiovisual incongruent) and compared results with a normal patient cohort. The patient had no correct responses in the visual lip-reading task; whereas, he improved in the audiovisual congruent task. In the audiovisual incongruent task, the patient provided one response; thus, he was able to lip-read. (He was able to use lip-reading/to use labial informations) This patient perceived only global dynamic facial movements, not the fine ones. He had a sufficient complementary use of lip-reading in audiovisual tasks, but not visual ones. These data are consistent with abnormal development of the pathways used for visual speech perception and associated with second-order face processing disorders and normal development of the audiovisual network for speech perception.

Intraparotid facial nerve schwannoma: case report and literature review.

A 68-year-old female presented with a right parotid mass and partial frontal peripheral facial nerve palsy. Pre-operatively, the mass was thought to be benign. Surgical intervention consisted of parotidectomy with sacrifice of the facial nerve due to the nerve's involvement in the tumoural process and its intrapetrosal course. Immediate nerve reconstruction was performed by an interposition graft of the great auricular nerve. Pathological examination revealed a schwannoma of the facial nerve. Review of the literature on this rare parotid gland tumour is reported.

A pilot study of the safety and performance of the Otologics fully implantable hearing device: transducing sounds via the round window membrane to the inner ear.

OBJECTIVES: The safety and performance of the Otologics fully implantable hearing device were assessed in adult patients with mixed conductive and sensorineural hearing loss. METHODS: The subcutaneous microphone of this fully implantable device picks up ambient sounds, converts them into an electrical signal, amplifies the signal according to the user's needs, and sends it to an electromechanical transducer. The transducer tip is customized with a prosthesis in order to be in contact with the round window membrane and is protected by fascia; this translates the electrical signal into a mechanical motion that directly stimulates the round window membrane and enables the user to perceive sound. The implanted battery is recharged daily via an external charger and the user can turn the implant on and off as well as adjust the volume with a hand-held remote control. In this pilot study, 6 patients with mixed conductive and sensorineural hearing loss were implanted with the Otologics fully implantable hearing device. Pre- and postoperative air conduction, bone conduction, as well as aided and unaided thresholds and speech scores were measured. RESULTS: No significant differences between preoperative and postoperative pure-tone averages were noted. Average improvement ranged from 19.16 to 35.8 dB of functional gain across audiometric frequencies with a mean of 26.17 +/- 5.15 dB. Long-term average functional gain at 12 months was 20.83 +/- 6.22 dB. Word recognition scores demonstrated significant differences between unaided and implant-aided conditions. CONCLUSIONS: Preliminary results of this trial of the Otologics fully implantable hearing device provide evidence that this fully implantable device is capable of efficiently transferring the sound to the inner ear via the round window membrane in patients with mixed hearing loss.

Tinnitus and otosclerosis.

OBJECTIVE: To establish the origin of tinnitus in patients with otosclerosis. METHODOLOGY: We performed a literature review to identify the characteristics of tinnitus in the general population and among patients with otosclerosis. RESULTS: Characteristics of tinnitus among patients with otosclerosis differ from those among the general population, suggesting mechanisms other than those implicated in presbycusis. Considerable improvements in the prevalence of tinnitus following corrective surgery demonstrate the limited contribution of diffuse labyrinth lesions. Middle ear and possibly secondary inner ear dysfunction produced by stapes blockage appear to be major etiopathogenic factors in the development of tinnitus in patients with otosclerosis. CONCLUSION: The conductive hearing deprivation produced by otosclerosis is associated with limited and reversible modifications in the central auditory pathway that are linked to tinnitus.

[TRT: results after one year treatment]. / TRT: efficacité après un an de traitement.

INTRODUCTION: Tinnitus Retraining Therapy (TRT) (which aims to induce changes in the mechanisms responsible for transferring signals from the auditory system to the limbic and autonomic systems) is a method for treating Tinnitus and decreased sound tolerance. An individualised explanation of Jastreboff's neurophysiological model allows greater insight and motivation on the part of the patient. Previous studies have demonstrated that daytime TRT is effective. As sleep forms a significant component of the distress associated with Tinnitus however, we hypothesised that night-time TRT could represent a useful tool in the treatment of this disabling condition. MATERIAL AND METHODS: 46 patients were studied (30 male, 16 female). Patients were selected from an ENT outpatient clinic. Patients with significant psychological disability were excluded. Patients were reviewed twice by their doctor and 5 times by a therapist over 12 months. Treatment consisted of 8 hours nighttime white noise stimulation, at progressively increasing intensity. Although several objective assessments of response were undertaken, patients' subjective testimonies were considered a more accurate signal of success. RESULTS: In total, 80% of patients had a satisfactory response after 1 year of treatment. 20% had no response. Patients were subcategorised according to Jastreboff's categories as follows: 1. Tinnitus (n = 6), 100% improved; 2. Tinnitus with hearing loss (n = 16); 62% improved; 3. Hyperacusis (with or without Tinnitus) (n = 16), 88.5% improved; 4. Hyperacusis (with or without Tinnitus, exacerbated by noise) (n = 8), 75% improved. CONCLUSION: Tinnitus is a symptom rather than an illness, and TRT gives patients greater control, allowing re-integration of normal perception. Night-time TRT is an effective treatment for Tinnitus and decreased sound tolerance. It has the potential advantage over day-time TRT of rapidly improving sleep and decreasing use of sedative hypnotics, a secondary effect noted in the personal testimonies of our cohort of patients. Further studies are needed to confirm this advantage, in view of the significant risks associated with long-term use of benzodiazepines. When investigating therapies for Tinnitus, it is necessary to measure success in terms of quality of life, as it is to this that the patient attaches the most importance.

Metastatic renal cell carcinoma to the temporal bone: case report.

We report an extremely rare case of metastasic renal cell carcinoma to the temporal bone which presented initially as a jugulotympanic paraganglioma. The clinical and radiological appearances were misleading.Investigations of concomitant high blood pressure revealed a tumour of the right kidney. Biopsy of the mastoid mass was histologically compatible with a metastasis from a clear cell renal carcinoma. The patient underwent a radical nephrectomy and local external radiotherapy to the head. He also received adjuvant treatment with interferon-_ and interleukin 2. The clinical presentation, the radiological and histological features, the patterns of spread, the treatment options and the prognosis of these tumours are discussed. A review of the literature confirms the extremely unusual occurrence of this localisation.

Tragal cartilage in tympanic membrane reconstruction.

INTRODUCTION: Management of chronic perforation and severe posterior and/or attic Retraction Pocket (RP) or atelectasis of the tympanic membrane continues to be one of the most difficult problems for otologists. AIM OF THE STUDY: To analyse the usefulness of the tympanic membrane reconstruction with tragal cartilage. MATERIALS AND METHODS: The study included 27 patients with chronic perforation or severe RP operated by the same surgeon, using tragal cartilage tympanoplasty. Seven supplementary procedures were required for recurrent cholesteatoma (second-look surgery). RESULTS: There has been no recurrence of the retraction and the perforation. Nineteen ossicular reconstructions were necessary with acceptable hearing results. CONCLUSIONS: Tragal cartilage has made a significant improvement in the tympanic membrane reconstruction procedure. A large thin cartilage combined with a titanium prosthesis can provide an excellent anatomical result, perfect stability and good functional outcome. When the eardrum is intact, we still use allografts of costal cartilage for the management of the retraction pocket or titanium prosthesis.

Imaging in sensorineural deafness.

In this paper, the authors describe, in details, the techniques and the indication of CT scanner and MRI for the evaluation of neurosensory deafness. CT scanner allows to study bony and pneumatized structures of the ear. CT scanner is mostly indicated for the study of congenital deafness, post-trauma deafness and unilateral progressive deafness for which the otoscopy is normal. MRI allows the study of fluid compartments, nerves, vascularization and tissue. MRI will be chosen to evaluate sudden sensory neural deafness and unilateral deafness. However, it is worth to mention that CT scanner and MRI are complementary and that precision of the imaging system as well as a strong collaboration between the otolaryngologist and the radiologist are mandatory.

Tinnitus and otosclerosis.

Fifty patients with otosclerosis and tinnitus were studied for the course of the tinnitus after stapes surgery. Tinnitus disappeared in 64% of the cases, improved in 16%, was unchanged in 14%, and worsened in 6%. The reduction in tinnitus was more favorable after a small fenestra stapedotomy than after a partial stapedectomy with removal of the posterior half of the footplate. An unfavorable postoperative course of tinnitus did not appear to be linked to the postoperative audiometric result. In our study the subjective evaluation of pitch and loudness of the tinnitus did not provide significant information regarding its prognosis.

Labyrinthine fistula after cholesteatomatous chronic otitis media.

OBJECTIVES: To report on cases of labyrinthine fistula diagnosed in an ear, nose, and throat department and to study the incidence, location, pre- and postoperative symptoms (hearing loss, tinnitus, vertigo, facial palsy), preoperative diagnostic imaging, and surgical treatment of two types of cholesteatomatous labyrinthine fistulae-the extensive fistula that erodes both the bony and membranous labyrinths and the bone fistula that affects only the bony labyrinth. STUDY DESIGN: Retrospective case review. PATIENTS: Fifty-four patients with cholesteatomatous chronic otitis media with labyrinthine fistulae. SETTING: Tertiary referral center. INTERVENTIONS: Diagnosis and treatment. MAIN OUTCOME MEASURES: Clinical, imaging, and surgical correlation of extensive fistulae and bone fistulae. RESULTS: The incidence of labyrinthine fistulae was 7% in all patients who underwent surgery for chronic otitis media. The bone type (66%) is more common than the extensive type (33%). Compared with bone fistulae, the outcome for extensive fistulae is more severe in terms of hearing loss, vertigo, and facial palsy. In terms of preoperative diagnosis, computed tomography imaging ensured early diagnosis in 89% of extensive cases and in 28% of bone cases. For extensive fistulae, the surgical technique was more radical, requiring an open technique in 66% of cases versus 22% of the bone fistulae cases. The most common location is the lateral semicircular canal (61%). CONCLUSIONS: The breach in the membranous labyrinth is consistent with a more aggressive pathology, causing more severe pre- and postoperative symptoms. Preoperative computed tomography is more sensitive for diagnosing extensive fistulae, which also require a more radical treatment.

Results of cerebellopontine space imaging by magnetic resonance during one year.

The authors present the results of a retrospective study concerning 231 magnetic resonance imaging (MRI) of the cerellopontine space requested by the otologists of the department between September 1996 and September 1997. The motives of imaging were sensorineural hearing loss (44%), vertigo and unsteadiness (25.5%), tinnitus (16.5%) and other (13%). The MRI was abnormal in 18% of the cases and the diagnosis of acoustic schwannoma was made in 10% of the cases.

Our classification of tympanic retraction pockets.

A classification of tympanic retraction pockets is indispensable in order to standardise their evaluation. In this manner, the evolution of the pathology can be carefully followed and the most appropriate therapy chosen. The authors propose adapting their classification to endoscopy which permits the inspection of the depths of certain pockets, otherwise invisible to examination under microscope, thus ensuring a better follow-up.

The vestibular neurectomy by retrosigmoid approach: operative technique and results.

A vestibular neurectomy is indicated in all cases of persistent vertigo without central vestibular compensation, with or without hearing loss. The retrosigmoid approach is described, as well as the postoperative results in 12 patients. The results are compared with those of other surgical treatments.

Use of costal cartilage in middle ear surgery.

Use of costal cartilage in middle ear surgery. Tympanic membrane retraction often occurs in otologic pathology. In an effort to reduce this pathology, the surgical procedure considered incorporates the use of a new material which diminishes retraction and allows freer movement of the eardrum. Since 1991, we have been using costal cartilage allograft obtained from donors and treated to prevent disease transmission. The allografts were used for tympanoplasty, retraction pockets and attic reconstruction. More than 150 patients were treated and the results evaluated in order to determine the indications of this new material.

The relevance of using tragal cartilage in tympanoplasty.

Chronic endotympanic depression is a pathological situation which leads to tympanic atelectasis, retraction pockets and cholesteatoma. It is also at the origin of tympanoplasty failure. Tragal composite perichondrium-cartilage graft is a procedure which gives good results in these difficult surgical cases.

[Objective tinnitus and velar myoclonus: apropos of a case of a child]. / Acouphènes objectifs et myoclonies vélaires: à propos d'un cas chez l'enfant.

The syndrome in which velar myoclonus is associated with objective tinnitus is rare, and in the adult corresponds most commonly with a lesion in the dentato-rubro-olivary tract. In the child, no lesion can usually be found. The disorders of function resulting from this syndrome are very disabling. Many forms of treatment have been proposed, and the results have been found to be disappointing. The authors report the case of a child in which muscle relaxants were used to very good effect, and emphasize the fact that the natural history of the condition in childhood is one of spontaneous remission, so that aggressive treatment is not appropriate.

Intracochlear schwannoma.

In the case reported evaluation of unilateral endocochlear deafness in a 44-year-old man led to a diagnosis of a small intracanalicular neurinoma of the cochlear nerve which had invaded the first turn of the cochlear spiral.

Neuromuscular hamartoma of the internal auditory canal.

We describe the clinical presentation and surgical findings of a 42-year-old man who was found to have a unilateral sensorineural deafness due to a hamartoma of the internal auditory canal.