Search

PURPOSE The purpose of this study was to describe the MRI characteristics of intralabyrinthine schwannoma (ILS) on post contrast three-dimensional (3D) fluid-attenuation-inversion-recovery (FLAIR) images obtained four hours after intravenous administration of a gadolinium-based contrast agent (4h-3D-FLAIR). MATERIALS AND METHODS This IRB-approved retrospective multi-center study included patients presenting with typical ILS from January 2016 to October 2020. All medical charts were systematically collected. All MRI examinations, including 4h-3D-FLAIR images, were reviewed by two board-certified neuroradiologists. Main outcome measures were location, signal intensity and associated anomalies of ILS. RESULTS Twenty-seven out of 8730 patients (0.31%) referred for the investigation of a cochleovestibular disorder had a final diagnosis of ILS. There were 13 men and 14 women with a mean age of 52 ± 17 (SD) years (age range: 20-86 years). The most common clinical presentation was unilateral progressive sensorineural hearing loss (16/27; 59%). All ILS were unilateral and 15 (15/27; 55%) were intracochlear. All ILS presented as a hypointense filling defect within the labyrinth on T2-weighted images that enhanced on post-contrast T1-weighted images. On 4h-3D-FLAIR images, all ILS presented as a hypointense filling defect, associated with diffuse perilymphatic hyperintensity. Two patients (2/27; 7%) presented with ipsilateral endolymphatic hydrops. CONCLUSION ILS displays consistent features on post-contrast 4h-3D-FLAIR images. ILS should not be confused with endolymphatic hydrops and requires a systematic analysis of the corresponding T2-weighted images.

International audience; Our objective was to identify a sensitive marker of disease progression in Friedreich’s ataxia. We prospectively evaluated speech, voice, and oromotor function in 40 patients at two timepoints. The mean disease duration was 20.8 ± 9.8 years and mean SARA score 23.7 ± 8.6 at baseline. Oral motor mobility, assessed by a combination of movements of the face, eyes, cheeks, lips, and tongue, decreased significantly after 1 year (P < 0.0001). The standardized response mean over 12 months was considered as large for oral mobility (1.26) but small for SARA (0.12). Oral mobility could therefore be a sensitive marker in therapeutic trials.

Objective To investigate the hearing performance of adult patients presenting unilateral deafness with contralateral fluctuating hearing loss who received a cochlear implant on the deaf side. Study Design Case series with chart review. Setting University tertiary referral center. Subjects and Methods Preoperatively and at 6 and 12 months postoperatively, 23 patients underwent pure tone audiometry and speech audiometry with disyllabic and monosyllabic words in a quiet environment and sentences in quiet and noisy (signal-to-noise ratio +10 dB SPL) environments under best-aided conditions. The Abbreviated Profile of Hearing Aid Benefit (APHAB) inventory was evaluated preoperatively and at 6 and 12 months postoperatively. Results No difference was found between pre- and postoperative tests for disyllabic and monosyllabic words. For sentences in quiet and noisy environments, a difference between pre- and postoperative performance was present at 1 year ( P = .002 and P = .02, respectively). In a noisy environment, a difference was present at 6 and 12 months postoperatively as compared with the preoperative value (mean ± SD: 6 months: 42% ± 7.1% vs 61% ± 6.5%, P = .016). A significant improvement in the APHAB score was found at 6 and 12 months postimplantation (Friedman's 2-way analysis of variance by ranks, P < .001). The number of years of hearing deprivation of the deaf ear was not correlated with performance. Conclusion When incapacitating fluctuating hearing loss occurs in patients presenting a contralateral deaf ear, a cochlear implant is indicated in the latter ear, significantly improving performance in noisy conditions and allowing a better quality of communication to be achieved.

The impact of hearing loss and of auditory rehabilitation (hearing aid, cochlear implant) on quality of life is a crucial issue. Commonly used questionnaires to assess quality of life in these patients (Nijmegen, APHAB, GBI) are time-consuming, difficult for patients to fill out, and show poor sensitivity to small improvements or deterioration. The objective of the present study was to validate a dedicated quality of life scale for hearing-impaired adults with or without auditory rehabilitation.ERSA (Évaluation du Retentissement de la Surdité chez l'Adulte: Evaluation of the Impact of Hearing Loss in Adults) is a self-administered questionnaire. It is divided into 4 domains, each comprising 5 questions graded from 1 to 10. The questions are simple, and formulated so patients will answer according to how they feel at the actual time of the session. Test-retest reliability was measured in 38 patients. Internal coherence and validity against the APHAB questionnaire as gold standard and in relation to hearing performance were measured in 122 patients at auditory assessment. Sensitivity to change in hearing was measured in 36 cochlear implant patients, before and 6 or 12 months after implantation.Test-retest reliability was very satisfactory (ρ=0.88). Internal coherence was good for all questions. External validity, comparing ERSA to APHAB scores in the same non-implanted hearing-impaired patients, was good (ρ=0.52). Additionally, ERSA scores correlated with hearing performance in adverse conditions (monosyllabic words: ρ=0.22; sentences in noise: ρ=0.19). In patients tested before and after cochlear implantation, improvement in hearing performance in silence and in noise correlated with an improvement in ERSA score (ρ=0.37 to 0.59, depending on the test), but not to GBI score.The ERSA questionnaire is easy and quick to use, reliable, and sensitive to change in hearing performance after cochlear implantation.

Objectives The present study assessed the interest of a contralateral hearing aid (HA) in adult cochlear implant (CI) bearers. Material and methods The study recruited 10 French-speaking adult HA bearers with postlingual bilateral hearing loss, fitted for at least 2 years with a unilateral CI after loss of benefit from HA in one ear but continuing to use their contralateral HA: 4 male, 6 female; mean age, 58 years. All had regularly used bilateral HAs prior to CI. Audiometric assessment comprised: (1) individual ear hearing assessment on pure-tone audiometry and speech discrimination; and (2) free-field testing without aid, with CI only, with HA only and with CI plus HA, on pure-tone audiometry and speech discrimination with quiet background and on speech discrimination in noise. Results Speech discrimination was significantly improved in the bimodal condition (CI plus HA) as compared to CI alone, on all tests. In quiet, discrimination for disyllabic words was > 50% in 7 cases with HA alone, in 2 cases with CI alone and in 1 case in with HA + CI. Under 0 dB signal-to-noise ratio, discrimination was > 50% in 1 case with HA alone, in 3 cases with CI alone and in 6 cases with HA + CI. Conclusion The present results showed benefit in auditory perception in quiet and in noise with bimodal stimulation. When there is residual hearing in the non-implanted ear, a HA should be fitted; and in progressive bilateral hearing loss, CI should be suggested when HA benefit decreases in one ear.

OBJECTIVE The relationship between hearing loss and cognitive function has already been established. The objective of our study was to determine whether the two short cognitive tests, COgnitive Disorders EXamination (CODEX) and Montreal Cognitive Assessment (MoCA), could be used in daily clinical practice to detect cognitive impairment, and its changes after cochlear implantation. PATIENTS Eighteen patients with severe to profound postlingual progressive hearing loss (mean age ± SEM: 64 ± 3.5 yr; range, 23-83 yr) were tested before, and 12 months after cochlear implantation, with adapted visual presentation of CODEX and MoCA tests. Auditory performance was tested under best-aided conditions in quiet and noise. RESULTS Twelve months after cochlear implantation, hearing performance had clearly improved (paired t tests, p

Hearing loss and visual impairment in childhood have mostly genetic origins, some of them being related to sensorial neuronal defects. Here, we report on eight subjects from four independent families affected by auditory neuropathy and optic atrophy. Whole-exome sequencing revealed biallelic mutations in FDXR in affected subjects of each family. FDXR encodes the mitochondrial ferredoxin reductase, the sole human ferredoxin reductase implicated in the biosynthesis of iron-sulfur clusters (ISCs) and in heme formation. ISC proteins are involved in enzymatic catalysis, gene expression, and DNA replication and repair. We observed deregulated iron homeostasis in FDXR mutant fibroblasts and indirect evidence of mitochondrial iron overload. Functional complementation in a yeast strain in which ARH1, the human FDXR ortholog, was deleted established the pathogenicity of these mutations. These data highlight the wide clinical heterogeneity of mitochondrial disorders related to ISC synthesis.

International audience; Most cochlear implantations are unilateral. To explore the benefits of a binaural cochlear implant, we used water-labelled oxygen-15 positron emission tomography. Relative cerebral blood flow was measured in a binaural implant group (n = 11), while the subjects were passively listening to human voice sounds, environmental sounds non-voice or silence. Binaural auditory stimulation in the cochlear implant group bilaterally activated the temporal voice areas, whereas monaural cochlear implant stimulation only activated the left temporal voice area. Direct comparison of the binaural and the monaural cochlear implant stimulation condition revealed an additional right temporal activation during voice processing in the binaural condition and the activation of a right fronto-parietal cortical network during sound processing that has been implicated in attention. These findings provide evidence that a bilateral cochlear implant stimulation enhanced the spectral cues associated with sound perception and improved brain processing of voice stimuli in the right temporal region when compared to a monaural cochlear implant stimulation. Moreover, the recruitment of sensory attention resources in a right fronto-parietal network allowed patients with bilateral cochlear implant stimulation to enhance their sound discrimination, whereas the same patients with only one cochlear implant stimulation had more auditory perception difficulties.

Our objective is to prospectively report very early complications and outcomes of cerebellopontine angle (CPA) surgery. Between January and August 2007, 72 patients were operated on by different transpetrosal approaches in a tertiary referral center. During preoperative assessment, facial nerve function (House-Brackmann grading system), the presence of vertigo or tinnitus and caloric test results were recorded and correlated with complications and symptoms occurring daily from day (D) 1 to D 8. The overall number of complications did not differ from those of former retrospective studies; nevertheless, the prospective feature of this study prompts several comments. Even slight (grade II) preoperative facial impairments increased the risk of severe postoperative facial dysfunction. Keratitis was frequent (42%) even in patients with normal facial function. Thrombo-embolic complications only occurred after long air-travel (≥5 h). Preoperative caloric test status was predictive of postoperative vestibular disturbance occurrence. With respect to the activity recovery; younger patients (40 years old) displayed faster central compensations than the older (60 years old) patients. This study highlights several features that may be used for preoperative patient counseling and complication management. In particular, the practitioner has to pay attention to even minor preoperative clinical signs of facial dysfunction to properly inform the patient of facial outcome. Routine ophthalmologic evaluation should be practiced, even when facial function is normal or subnormal.1b.

OBJECTIVE To evaluate the auditory brainstem implant (ABI) performances in neurofibromatosis type 2 (NF2) and non-NF2 patients. STUDY DESIGN Retrospective cohort study. SETTING Tertiary referral center. PATIENTS Between 1996 and 2006, 31 adult patients (mean age, 41 yr; range, 17-65 yr) were implanted with a 21-electrode Nucleus device (Cochlear Inc., Lane Cove, Australia). The population comprised 23 NF2, 3 postmeningitis bilateral ossified cochleas, 3 solitary vestibular schwannomas on the only hearing ear, 1 inner ear malformation, and 1 bilateral cochlear destruction by otosclerosis. INTERVENTION Auditory brainstem implant was placed through a translabyrinthine or a retrosigmoid approach. MAIN OUTCOME MEASURES Auditory brainstem implant was evaluated by open-set words and sentences in sound, vision, and sound-plus-vision modes. RESULTS In NF2 patients, 16 (70%) were daily users of their implants. In these patients, the open-set dissyllabic word recognition was 36 +/- 6.0 % for vision-only mode, 33 +/- 6.5 % for sound-only mode, and 65 +/- 8.0 % in vision-plus-sound mode with a high interindividual variation. Negative prognostic factors were long duration of total hearing loss (>10 yr), low number of active electrodes (

Objective To evaluate the functional results of otosclerosis surgery using diode laser. Study design Retrospective cohort analysis. Patients One hundred seven patients operated on for otosclerosis with a diode laser (119 ears, all primary cases) and 141 patients operated on with a conventional technique (141 ears, all primary cases). Revision cases using the diode laser were also described. Methods Preoperative tomographic computed scan findings and intraoperative observations were collected. Pure-tone and vocal audiometry was performed preoperatively and postoperatively (at 3 mo and 1 yr). Results In the laser group, the air-bone gap was 29 +/- 0.8 dB (n= 112) preoperatively and 9 +/- 0.6 dB (n = 58) at 1 year. Air conduction was improved by 22 +/- 1.7 dB at 1 year (n = 58). In the conventional group, the air-bone gap was 32 +/- 0.9 dB (n=127) preoperatively and 10 +/- 0.6 dB (n = 127) at 1 year. Air conduction was improved by 25 +/- 1.1 dB (n = 127) at 1 year. No difference of hearing gain was observed between the 2 groups at 1 year. A decreased rate of footplate fracture was observed with the diode laser (3.6%) compared with the conventional technique (21.3%). Conclusion Diode laser is a reliable and safe device for otosclerosis surgery. The functional results were similar to those reported in other series.

Auditory brainstem responses (ABRs) associated with other audio-vestibular examinations and a thorough clinical examination should allow detection of the majority of cerebellopontine angle (CPA) lesions (99.2-100%).The increasing quality of MRI in the detection of CPA lesions, and the reports of false negative ABRs have raised issues concerning the value of ABR in the diagnosis and preoperative assessment of CPA lesions. The aim of this work was to assess the value of the ABR in the diagnosis of vestibular schwannomas (VS) and other CPA lesions.This retrospective study included 676 solitary VS (548 operated on and 128 followed up) and 70 other CPA tumours (72% meningiomas, 11% cholesteatomas, 3% ependymomas, 15% miscellaneous) managed between 1990 and 2001. All patients underwent clinical examination, audiometry, ABR, vestibular caloric tests and MRI.ABRs were normal in 4.8% of VS. Association of normal ABR, vestibular caloric tests and audiometry (AAO-HNS class A) represented only 0.7% of VS. In other CPA lesions, ABR were normal in 15% of cases and the association of the three above-mentioned examinations was encountered in 10%. However, in the latter cases the clinical examination showed an abnormality in all cases.

Objective: To describe and analyze a series of intracochlear schwannomas. Methods: From 1987 to 2005, 19 patients with schwannomas involving the cochlea were included in this series. Clinical, audiovestibular, and imaging data concerning initial and follow-up visits were collected. Results: The mean age was 54 years (range, 25-71 yr). The series comprised 10 women and 9 men, 18 solitary tumors and 1 neurofibromatosis Type 2 patient. The mean diagnosis delay was 11 years (range, 1-31 yr). At diagnosis, a total or profound hearing loss (Class D of American Academy of Otolaryngology-Head and Neck Surgery classification) was noted in 17 patients (89%), and a severe loss (Class C) was reported in 2 patients (11%). Facial paresis was reported in 2 patients (11%; Grades 2 and 4 of House and Brackmann classification). Magnetic resonance imaging showed an involvement of the posterior labyrinth in 8 patients (37%), an extension of the schwannoma to the internal auditory meatus in 8 patients (37%), and a cerebellopontine angle extension in 7 patients (32%). In 11 patients (58%), the schwannoma was removed through a transotic route. In the 8 remaining patients, a watch-and-rescan policy was decided. The postoperative course was uneventful. Postoperative follow-up period was 27 months (range, 6-88 mo). Postoperative facial function was assessed as Grade 1 in 7 patients, as Grade 2 in 4, and as Grade 4 in 1 patient. Conclusion: The diagnosis of intracochlear schwannomas can be difficult on magnetic resonance imaging. The diagnosis should be considered in all unilateral hearing losses.

Objective Vertigo and dizziness, which are major symptoms of diseases affecting the vestibular system, drastically impair patients' health-related quality of life (QoL). Patient's perspectives are thus essential to symptom assessment. We sought to make a critical review of published questionnaires measuring vertigo or dizziness, and/or their impact on QoL. Methods Twenty-nine articles reporting the validation or use in clinical trials of vertigo- or dizziness-specific questionnaires were identified over the 1991–2004 period, and reviewed using a methodological and a Patient-Reported Outcomes specific checklist. Questionnaires were classified into three categories according to content: QoL (or handicap), mixed (assessing both symptoms and QoL), and symptom questionnaires. Results Four QoL, three mixed questionnaires, two symptoms, and one Meniere's disease-specific questionnaire were identified. QoL questionnaire validation was usually not complete. The structural validity of the Dizziness HandicapInventory is not established, although this questionnaire is considered to be the reference questionnaire in the QoL domain. Moreover, QoL questionnaires were not very specific to vertigo or dizziness. Similarly, the Vertigo Handicap Questionnaire appeared to have the most pertinent content, but its validation remains to be completed. Mixed questionnaires have the same imperfections. The Vertigo, Dizziness, Imbalance (VDI) Questionnaire had the best validation score from the checklist, but its responsiveness appears to be weak. Regarding symptom questionnaires, the European Evaluation of Vertigo questionnaire evaluated the five major symptoms of vestibular syndrome satisfactorily. Conclusion The present literature review failed to find any relevant and validated questionnaire assessing the impact of vertigo or dizziness on QoL.

Background. A consensus conference recommended empirical antibiotic therapy for all patients with postoperative meningitis and treatment withdrawal after 48 or 72 h if cerebrospinal fluid culture results are negative. However, this approach is not universally accepted and has not been assessed in clinical trials. Methods. We performed a cohort study of all patients who received a diagnosis of postoperative meningitis from January 1998 through May 2005 in a teaching hospital. From January 1998 through September 2003 (control period), guidelines were lacking or were not implemented. From October 2003 through May 2005 (interventional period), all patients received a predefined intravenous antibiotic therapy that was discontinued on the third day if the meningitis was considered aseptic. Clinical outcome and duration of antibiotic therapy were analyzed for each patient. Results. Seventy-five episodes of postoperative meningitis (21 cases of bacterial meningitis and 54 cases of aseptic meningitis) were investigated. Patients with aseptic meningitis received antibiotic treatment for a mean ± standard deviation duration of 11 ± 5 days during the control period and 3.5 ± 2 days during the intervention period (P = .001). The duration of antibiotic treatment for bacterial meningitis was not significantly different between the 2 periods. All episodes of bacterial and aseptic meningitis were cured, and complications were rare during both periods. Conclusions. Stopping antibiotic treatment after 3 days is effective and safe for patients with postoperative meningitis whose cerebrospinal fluid culture results are negative.

Hearing rehabilitation by cochlear implantation is not always possible in case of total ossification after pneumococcal meningitis. We report 3 cases of postmeningitis profound hearing loss with total cochlear ossification in adults who underwent auditory brainstem implantation (Nucleus 22®, Cochlear Inc., Lane Cove, Australia) between 1999 and 2004. The postoperative follow-up period ranged from 1 to 6 years. Eleven to 15 out of 22 electrodes were activated. All patients had significant speech discrimination in the sound-only mode and an enhanced lip-reading performance with the implant. Auditory brainstem implants are an efficient means of auditory rehabilitation and may be considered in selected cases of bilateral profound hearing loss with the impossibility of cochlear implantation.

A high rate of deterioration in hearing function and the loss of patient compliance during conservative management should be taken into account when considering hearing preservation strategies for patients with vestibular schwannoma (VS). To compare conservative management with surgery for solitary small VS. Among 693 patients followed up for VS between 1991 and 2002, 114 (16%) intracanalicular VSs (stage 1) and 302 (44%) VSs measuring15 mm in the cerebellopontine angle (stage 2) were included in this study. Initially, surgery was performed in 305 (73%) cases (50 stage 1, 255 stage 2) and 111 (27%) were managed conservatively (64 stage 1, 54 stage 2) by means of annual MRI scans and audiometry. Conservative management was chosen in patients aged60 years and in those who refused surgery. In this subgroup, the mean follow-up period was 33 months (range 6-111 months). In the conservative management group, 47% of VSs showed significant growth, 47% were stable and 6% showed regression. Seventeen patients (15%) were operated on secondarily for tumour growth and 1 (1%) was irradiated for tumour growth and because surgery was contraindicated. Deterioration of hearing function byor =1 class was observed in 56% of cases, 34% of patients were initially in hearing class D and only 10% showed stable hearing function. Of the conservative management group, 17% were lost during follow-up. After surgery, grade 1 or 2 facial function was obtained in 86% of cases. Following hearing preservation attempts (n=137), 54% of patients were in hearing classes A-C.

The aim of this study was to evaluate the functional outcome of facial nerve repair with fibrin glue in end-to-end anastomosis and intermediate nerve graft. Thirty-six patients undergoing facial nerve repair by end-to-end anastomosis or facial nerve grafting using exclusively fibrin glue between 1986 and 1999 were included in this retrospective study. The population comprised ten vestibular schwannomas (28%), nine temporal bone fractures (25%), seven facial nerve schwannomas (19%), four facial nerve hemangiomas (11%), two iatrogenic facial nerve interruptions (6%) and four miscellaneous facial nerve lesions (11%). Data were reviewed concerning etiology, location of the nerve interruption, type of repair and postoperative facial function according to the repaired facial nerve recovery scale (A: normal; B: independent movements of eyelid and mouth; C: strong closure of eyelids and mouth; D: incomplete eyelid closure; E: minimal movement; F: no movement). Eleven patients (31%) underwent end-to-end nerve anastomosis and 25 (69%) underwent intermediate facial nerve grafting. The mean follow-up period was 50 months (range: 3-95). Among patients followed-up more than 18 months (n = 20), a score of B or C was obtained in 16 patients (80%), a score D in 2 cases (10%) and a score E in 2 cases (10%). The type of repair and the site of interruption did not influence the results. Fibrin glue is a simple, rapid and efficient means of facial nerve repair. In case of intraoperative facial nerve interruption, this type of repair can be attempted in any location at the time of the tumor removal.

OBJECTIVE To assess the value of cerebrospinal fluid pressure as a decisional factor for immediate surgical revision in cerebrospinal fluid leakage after acoustic neuroma removal. STUDY DESIGN Prospective study. SETTING Tertiary referral center. PATIENTS Between 1998 and 2001, 220 patients were operated on for acoustic neuroma by different transpetrosal approaches. Among 24 patients (12%) presenting postoperative cerebrospinal fluid leakage, those with meningitis or with hydrocephalus were excluded. Fifteen patients were included in this study. METHODS Each patient had initial conservative treatment with serial depletive lumbar punctures and cerebrospinal fluid pressure measurements associated with oral acetazolamide. Surgical revision was decided on in case of persistent cerebrospinal fluid leakage. RESULTS In eight patients with high cerebrospinal fluid pressure (18+/-1.4 cm H2O; range, 14-28 cm H2O), cerebrospinal fluid leak disappeared in 3 days after conservative treatment. Seven other patients required surgical revision for persistent cerebrospinal fluid leakage. Revision surgery was efficient in six patients with low cerebrospinal fluid pressure (8+/-1.3 cm H2O, range, 3-12 cm H2O). In the remaining patient with high cerebrospinal fluid pressure (18 cm H2O), cerebrospinal fluid leakage continued despite surgical revision, requiring lumboperitoneal shunting. CONCLUSION The cerebrospinal fluid pressure value may be used as a decisional indicator for cerebrospinal fluid leakage treatment after acoustic neuroma surgery. Low cerebrospinal fluid pressure leakage would imply a revision surgery procedure without delay, whereas high cerebrospinal fluid pressure leakage would imply conservative treatment.

To evaluate the results of partial and total ossicular reconstruction prostheses entirely made of hydroxylapatite.A retrospective review of cases followed-up between 1997 and 2000.Tertiary referral center.One hundred adult patients (60 men and 40 women) were studied. The mean age was 55 years (range, 25 to 65 years). Fifty patients had a previous tympanoplasty. The series included 45 cholesteatomas (45%), 37 chronic otitis media without cholesteatoma (37%), 13 retraction pockets (13%), and 5 posttraumatic ossicular displacements (5%).A one-stage ossiculoplasty with a partial ossicular reconstruction prosthesis (n = 65) or a total ossicular reconstruction prosthesis (n = 35) was performed. This was associated with a simple mastoidectomy in 70 cases and a radical mastoidectomy in 10 cases.All patients were followed-up at 1 year, and 50 were reexamined 2 years after surgery. Clinical and audiometric data were collected in a database at each visit.A prosthetic extrusion was noted in two cases (2%). A prosthetic displacement occurred in 10 cases (10%). A revision surgery was carried out in 10 cases because of an ossiculoplasty failure or a disease recurrence. At 1 year, the residual air-bone gap was lower in cases with a partial ossicular reconstruction prosthesis than those with a total ossicular reconstruction prosthesis (16 +/- 9.3 versus 23 +/- 13.7 dB, p0.05). The gain in mean air conduction threshold was 21 +/- 12.8 dB in cases of partial ossicular reconstruction prosthesis and 19 +/- 11.3 dB in cases of total ossicular reconstruction prosthesis (not significant). Revision surgery, canal wall-down mastoidectomy, or cholesteatoma yielded poorer functional results.Partial ossicular reconstruction prosthesis and total ossicular reconstruction prosthesis entirely made of dense hydroxylapatite yielded high rates of air-bone gap reduction and high anatomic stability.

CEA-Inserm U1000 Neuroimaging and Psychiatry, Service Hospitalier Frederic Joliot, Orsay, France, CEA, DRM, DSV, Service Hospitalier Frederic-Joliot, Orsay, France, Laboratoire de Correction Auditive, Eric Bizaguet, Paris, France, Inserm, UMR-S 867, Paris, France, AP-HP, Hopital Beaujon, Service d’ORL et de Chirurgie cervico-faciale, Clichy, France, Universite Paris 7 Denis Diderot, Paris, France, AP-HP, Hopital Louis Mourier, Service d’ORL et de Chirurgie cervico-faciale, Colombes, France, AP-HP, Hopital Pitie-Salpetriere, Service Urgences cerebro-vasculaires, Paris, France, Universite Paris 6 Pierre et Marie Curie, Paris, France

Cochlear implantation in elderly patients is a questionable subject. The purpose of this study was to evaluate the procedure and its outcome, the postoperative course, and the audiologic and social benefits of cochlear implantation in this population. Twenty-seven patients older than 60 years were compared with a control group of 15 adult patients. This retrospective study analyzed data concerning the outcome of the procedure, postoperative course, postoperative orthophonic test results, and answers of a questionnaire assessing the changes in communication, perception, and social outcomes. The procedure was uneventful in both groups. Minor complications were not more frequent in elderly patients. Orthophonic test results were comparable in both groups. At 12 months, 83% of the elderly patients had an open-set speech discrimination score above 60%. The benefits of cochlear implantation in terms of the quality of life are not statistically different with younger patients. Cost-utility analysis might support these findings.

The aim of this retrospective study was to evaluate the functional results of surgical lowering of the high jugular bulb in the treatment of patients with Ménière's disease and pulsatile tinnitus. Fifteen patients with disabling Ménière's disease associated with pulsatile tinnitus and a high and medial jugular bulb were included in this study. As treatment a complete mastoidectomy was performed, after which the jugular bulb was freed by an infralabyrinthine and subfacial approach. The bulb was then displaced downwards with surgical wax. Functional results of surgery were assessed by a questionnaire according to the 1995 guidelines of the United States American Academy Committee on Hearing and Equilibrium, audiometric and vestibular tests, and by magnetic resonance and computed tomographic imaging with vascular sequences. Surgical treatment was contraindicated in two cases: one had hypoplasia of the contralateral sigmoid sinus and the other a small petrous hemangioma located around the jugular bulb that was discovered peroperatively. Among the 13 patients treated by definitive surgery, attacks of vertigo were reported as disabling in 12 cases preoperatively (92%) versus 1 (8%) after surgical treatment. No significant change in hearing was observed after surgery. Tinnitus had been reported in all patients preoperatively and decreased in intensity in four (31%) and disappeared in three (23%) after surgery.

Menière's disease and fluctuating hearing loss are related to labyrinthine fluid pressure variations. The development of a new indirect method of analysis of the tympanic membrane displacement during the stapedial reflex, using the Marchbanks Measurements System (MMS 10), allows us to study inner ear fluid pressure during these pathological conditions. In this study, measurements with this method were made in four groups of patients: a control group with normal hearing (n=7), stable sensorineural hearing loss (n=9), fluctuating hearing loss (n=8), and Menière's disease (n=25). Results show, first, a good relationship between the recording of negative curves, suggesting a high pressure, and the acute episodes of fluctuating hearing loss; and, secondly, in the case of Menière's disease two types of situation: positive and negative curves suggesting normal and high pressures, respectively.

Residual hearing could be preserved with various arrays ranging from 16 to 18 mm in insertion length and 0.25 to 0.5 mm tip diameter. Whether array insertion is performed through a cochleostomy or a round window, tip diameter is an essential criterion for the array design to improve hearing preservation results.The goal of this study was to report the outcome of patients implanted with electric acoustic cochlear implants with various surgical techniques and array designs.Thirty-two implanted ears (30 patients) were included in this retrospective study. Three array models were inserted: Contour Advance implant (n = 16), Nucleus Hybrid-L (n = 12), and Med-El Flex EAS (n = 4). Postoperative pure tone audiometry was performed at 3 and 12 months after implantation.Three months postoperatively, hearing preservation within 30 dB was achieved in 50%, 50%, and 84% cases of patients implanted with a Contour Advance, Flex-EAS, and Hybrid-L, respectively. Two patients (Hybrid-L group) had a delayed sudden hearing loss (30 dB) 3 months postoperatively and three patients (Contour Advance group) had total hearing loss at 1 year. Best results were achieved using arrays with small tip diameters. Cochleostomy or round window insertion did not affect hearing preservation results.

The objective of the study was to compare the performance of cochlear implantation between post-meningitic and non-meningitic patients, and to evaluate the impact on hearing outcome of technical advances in cochlear implant technology. Retrospective chart review was used as the study design. Twenty adults with post-meningitic profound hearing loss receiving unilateral or bilateral cochlear implants between 1990 and 2008 were tested. Results were compared to a control group of 46 adults implanted for a non-meningitic hearing loss, with the same pre-operative speech scores. Speech scores were poorer in post-meningitic patients compared to those of control group, whatever the duration after implantation (p < 0.0001). Speech scores of subjects implanted and fitted before 2001 were compared to those of subjects implanted after 2001, with the same duration of hearing loss. Performance improved with implants and processors available after 2001, with a magnitude of improvement higher in post-meningitic patients (p < 0.0001 and p < 0.05 in post-meningitic and control groups, respectively, two-way ANOVA). Consequently, speech scores of post-meningitic patients implanted after 2001 achieved those of control subjects (two-way ANOVA). Advances in cochlear implant technology and coding strategy improve hearing outcome in post-meningitic adult patients, who now achieve similar performance as those of non-meningitic patients.

International audience; The superior temporal sulcus (STS) is specifically involved in processing the human voice. Profound acquired deafness by post-meningitis ossified cochlea and by bilateral vestibular schwannoma in neurofibromatosis type 2 patients are two indications for auditory brainstem implantation (ABI). In order to objectively measure the cortical voice processing of a group of ABI patients, we studied the activation of the human temporal voice areas (TVA) by PET H(2)(15)O, performed in a group of implanted deaf adults (n=7) with more than two years of auditory brainstem implant experience, with an intelligibility score average of 17%+/-17 [mean+/-SD]. Relative cerebral blood flow (rCBF) was measured in the three following conditions: during silence, while passive listening to human voice, and to non-voice stimuli. Compared to silence, the activations induced by voice and non-voice stimuli were bilaterally located in the superior temporal regions. However, compared to non-voice stimuli, the voice stimuli did not induce specific supplementary activation of the TVA along the STS. The comparison of ABI group with a normal-hearing controls group (n=7) showed that TVA activations were significantly enhanced among controls group. ABI allowed the transmission of sound stimuli to temporal brain regions but lacked transmitting the specific cues of the human voice to the TVA. Moreover, among groups, during silent condition, brain visual regions showed higher rCBF in ABI group, although temporal brain regions had higher rCBF in the controls group. ABI patients had consequently developed enhanced visual strategies to keep interacting with their environment.

International audience; UNLABELLED: Cochlear implants may improve the medical and social prognosis of profound deafness. Nevertheless, some patients have experienced poor results without any clear explanations. One correlate may be an alteration in cortical voice processing. To test this hypothesis, we studied the activation of human temporal voice areas (TVA) using a well-standardized PET paradigm adapted from previous functional MRI (fMRI) studies. METHODS: A PET H(2)(15)O activation study was performed on 3 groups of adult volunteers: normal-hearing control subjects (n = 6) and cochlear-implanted postlingually deaf patients with >2 y of cochlear implant experience, with intelligibility scores in the "Lafon monosyllabic task" >80% (GOOD group; n = 6) or

Objective: To evaluate speech performance, in quiet and noise, and localization ability in adult patients who had undergone bilateral and simultaneous implantation. Study Design: Prospective multi-center study. Methods: Twenty-seven adult patients with profound or total hearing loss were bilaterally implanted in a single-stage procedure, and simultaneously activated (Med-El, Combi 40/40+). Subjects were assessed before implantation and at 3, 6 and 12 months after switch-on. Speech perception tests in monaural and binaural conditions were performed in quiet and in noise using disyllabic words, with speech coming from the front and a cocktail party background noise coming from 5 loudspeakers. Sound localization measurements were also performed in background noise coming from 5 loudspeakers positioned from –90° to +90° azimuth in the horizontal plane, and using a speech stimulus. Results: There was a bilateral advantage at 12 months in quiet (77 ± 5.0% in bilateral condition, 67 ± 5.3% for the better ear, p < 0.005) and in noise (signal-to-noise ratio +15 dB: 63 ± 5.9% in bilateral condition, 55 ± 6.9% for the better ear, p < 0.05). Considering unilateral speech scores recorded in quiet at 12 months, subjects were categorized as ‘good performers’ (speech comprehension score ≥60% for the better ear, n = 19) and ‘poor performers’ (n = 8). Subjects were also categorized as ‘asymmetrical’ (difference between their 2 unilateral speech scores ≥20%, n = 11) or ‘symmetrical’ (n = 16). The largest advantage (bilateral compared to the better ear) was obtained in poor performers: +19% compared to +7% in good performers (p < 0.05). In the group of good performers, there was a bilateral advantage only in cases of symmetrical results between the 2 ears (n = 10). In the group of poor performers, the bilateral advantage was shown in both patients with symmetrical (n = 6) and asymmetrical results (n = 2). In bilateral conditions, the sound localization ability in noise was improved compared to monaural conditions in patients with symmetrical and asymmetrical performance between the 2 ears. No preoperative factor (age, duration of deafness, use of hearing aids, etiology, etc.) could predict the asymmetrical performance, nor which ear would be the best. Conclusion: This study demonstrates a bilateral advantage (at 12 months after the implantation) in speech intelligibility and sound localization in a complex noisy environment. In quiet, this bilateral advantage is shown in cases of poor performance of both ears, and in cases of good performance with symmetrical results between the 2 ears. No preoperative factor can predict the best candidates for a simultaneous bilateral implantation.

We compared the indices of satisfaction and use among patients wearing an osseo-integrated prosthesis BAHA (bone anchored hearing aid) according to the indications: conductive or mixed hearing loss (CHL) and patients with single side deafness (SSD). The study was carried out among patients wearing a BAHA fitted in one of three French departments between November 2001 and November 2005. Each patient received a postal questionnaire relating to the ease of use and the daily utilization period of the prosthesis, as well as a satisfaction rating (from 1 to 10) evaluating improvement in quality of life, overall satisfaction, improvement in sound localization and satisfaction from the aesthetic point of view. In total, 170 out of 231 patients responded to the questionnaire (response rate of 73.5%). The average age at the time of fitting of the BAHA was 56 years (18–79 years). The SSD group was composed of 118 patients (69.4%): 92 following surgery for vestibular schwannoma, 2 following surgery for meningioma and 24 with “other” causes (e.g. idiopathic sudden deafness, sensori neural hearing loss complicating surgery of the middle ear). The CHL group was composed of 52 patients (30.5%): 44 patients with a chronic otitis and 8 with a malformation of the middle ear. The average duration of use of the prosthesis was 22 months (3–72 months). The average utilization period was higher than 8 h per day in 48.5% of cases. There was a significant difference between the two groups concerning the quality of life (p

The inner ear can be the target of autoimmune disorders. Recognition of autoimmune inner ear disease is important, as it is one of the very few forms of sensorineural hearing loss (HL) that can be successfully treated by medical therapy. The aim of this study was to evaluate whether the detection of antibodies to myelin protein P0 (MPZ) could be a diagnostic test for inner ear disease of autoimmune cause.This multicentric prospective study included 129 patients: patients with progressive sensorineural HL or with Menière's disease, together with their control group corresponding to patients with similar symptoms, but of presumably known origin. Detection of antibodies to myelin P0 protein was performed by using western blots. NORMAL: The prevalence of antibodies to myelin P0 protein in patients with rapidly progressive HL was not statistically different from that of the control group corresponding to genetic HL patients (30 versus 28%). In patients with Menière's disease, the prevalence was lower than that of the control group corresponding to patients with benign paroxysmal positional vertigo (5.4 versus 18.7%). No patient with auto-immune disease had antibodies to myelin P0 protein.The sole presence of antibodies to myelin P0 may not be used as a marker of inner ear disease of autoimmune origin.

Haemangiomas involving the internal auditory meatus (IAM) are rare and can mimic other frequent lesions of the IAM such as schwannomas by their clinical and imaging aspects. The case of a patient with an atypical IAM haemangioma fluctuating in size is reported to highlight this diagnostic possibility. A 36-year-old female presented with a sudden and recurrent left sensorineural hearing loss (SNHL). Three consecutive MRIs were performed in a 10-month period of preoperative observation. They showed a fluctuation of the tumour signal and size. The surgical removal was performed via a translabyrinthine approach. Pathological findings were consistent with the diagnosis of a heamangioma. MRI and CT findings suggested the diagnosis of IAM heamangioma, but the rapid variation in size and signal was misleading. This phenomenon may be due to haemorrhage or oedema, and can be accompanied by a deterioration of the hearing function. Early surgical resection is the treatment of choice and allows to confirm the diagnosis.

Hearing impairment is the most frequent sensory defect in children, with a genetic basis in about 50% of cases. Several point mutations and deletions in mitochondrial DNA (mtDNA) have been identified in non-syndromic sensorineural hearing loss (NSSNHL). Beside the frequent A1555G mutation, a number of mutations in tRNAs have been reported recently, but their incidence remains unknown. We identified the T7511C mutation in the tRNASer(UCN) gene in two French families with isolated deafness. Maternal transmission was obvious in both. The 15 patients with hearing impairment exhibited a variable disease phenotype in terms of onset, severity, and progression. T7511C was present in all the patients screened. Homoplasmic and heteroplasmic levels were observed and did not correlate with the severity of the disease. T7511C was also present in 12 hearing offspring of the oldest deaf mothers, confirming the existence of modulatory factors. Our data suggest that this mtDNA mutation should be screened for in all cases of familial NSSNHL compatible with maternal transmission.

In order to evaluate the decisional elements in the surgical strategy of deep-seated and/or extensive intratemporal lesions, a retrospective review of cases followed up between 1985 and 1996 in our department was undertaken. Eighty-one adult patients presenting temporal bone lesions located or extending beyond the middle ear limits excluding vestibular schwannomas and surgically treated were included. The population comprised 38 men and 43 women (mean age: 43 years, range: 17-81). Pre-, intra- and postoperative data were collected from medical files. The principal factors influencing the choice of the surgical approach were the location of the lesion and its presumed aggressiveness, the tumour involvement of the internal carotid artery and the labyrinth on preoperative imaging, and the preoperative hearing loss. A coherent algorithm based on these factors can be proposed for the surgical management of intratemporal lesions. High quality preoperative imaging is mandatory for the surgical planning.

✓ Extraaxial cerebellopontine angle (CPA) medulloblastomas and other primitive neuroectodermal tumors (PNETs) are rare tumors. The authors report on two patients with PNETs who presented with progressive audiovestibular symptoms. In each case magnetic resonance (MR) imaging revealed an extraaxial lesion that filled the internal auditory meatus and exhibited the neuroimaging features of a vestibular schwannoma (VS). No high signal intensity was apparent in either the brainstem or adjacent cerebellum on T2-weighted MR images. Surgery with maximum resection (total in one case and subtotal in the other) was performed, followed by craniospinal radiotherapy. One year postoperatively, both patients were free from tumor. A CPA PNET mimicking a VS is a rare entity, the diagnosis of which is important because its treatment differs dramatically from that of VS, including prescribed surgery followed by conventional craniospinal radiotherapy.

Hypertrophic neuropathy is a peripheral nerve lesion that is histologically characterized by onion bulb formations around axons. This histologic picture, which is usually seen in generalized hypertrophic neuropathies, can occasionally be observed in single nerves as localized hypertrophic neuropathy. Cranial involvement of such localized hypertrophic neuropathy represents a very rare entity; only a few cases have been reported in the literature. We report the history of a progressive facial paralysis with a tumorous enlargement of the seventh cranial nerve that was clinically suspected of being a schwannoma. Pathological examination permitted the diagnosis of hypertrophic neuropathy.