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Didier Bouccara

Publications and source records attributed to Didier Bouccara.

14 recordsLinked to original sources

Auditory brainstem implantation to rehabilitate profound hearing loss with totally ossified cochleae induced by pneumococcal meningitis.

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.

Adult↗

Atypical haemangioma of the internal auditory meatus: a case report.

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.

Adult↗

[Effects of noise on inner ear].

The effects of noise on health depend both on individual factors and characteristics of sound exposure. In case of acoustic trauma, reversible or irreversible lesions of inner ear components are possible. Most often there is immediately an acute tinnitus and hearing loss. Audiometric tests demonstrate hearing loss on the high frequency, generally focused on 4 kHz. Immediate treatment is recommended even there is no currently indicator of the ability to restore hearing. New perspectives on treatment are directed to local treatment and/or using new procedure as antioxidative treatment. Occupational and leisure are the two conditions in which chronic exposure to noise is found. Detection and prevention of noise-induced hearing loss is easier in case of industrial workers than in case of noise exposition for musicians and other sounds and stage technicians or concert managers, and of course non-professional with exposure to amplified music.

Ear, Inner↗

Intratumoral and brainstem hemorrhage in a patient with vestibular schwannoma and oral anticoagulant therapy.

OBJECTIVE: To report the first case of spontaneous intratumoral and brainstem hemorrhage in a patient with a vestibular schwannoma. STUDY DESIGN: Case report and review of the literature. SETTING: University-based, tertiary referral center. PATIENT: A 73-year-old woman with a vestibular schwannoma, anti-vitamin K overdose, and arterial hypertension experienced a rapid onset of headache, facial palsy, diplopia, and hoarseness. INTERVENTIONS: The patient was admitted to the intensive care unit and treated with vitamin K and corticoids. MAIN OUTCOME MEASURES: Clinical and radiologic findings. RESULTS: At admission, the magnetic resonance imaging and computed tomographic scans showed an intratumoral hemorrhage. The patient entered a coma 24 hours after the onset of the symptoms. At that time, the computed tomographic scan demonstrated a massive intratumoral and brainstem hemorrhage. The patient died 3 days later. CONCLUSION: Intratumoral, subarachnoid, and brainstem hemorrhages can occur in patients with vestibular schwannoma. Oral anticoagulant therapy is a risk factor for tumor-related hemorrhage.

Administration, Oral↗

[Auditory rehabilitation].

Auditory rehabilitation depends of the cause and the severity of the hearing loss (or deafness). Hearing losses dues to middle ear pathologies can beneficiate of medical or surgical treatments, by ossicular prostheses, if it is necessary to restore the function of the ossicles chain. In the sensorineural hearing losses, with inner ear pathology, the use of auditory aid is immediately considered. In the cases for which they are insufficient because of severity of the hearing loss or not suitable because of local non-tolerance, it is possible to use middle ear implant or cochlear implant. The indications of the auditory brainstern implants remain at this day limited to the total bilateral hearing losses due to a complete destruction of cochleae and auditory nerves. These therapeutic orientations are selected after a multidisciplinary evaluation of the deaf person, evaluation that allows the characterization of the hearing loss and its repercussion. In all the cases, the restoration of a bilateral hearing has to be done if possible, making an improvement of the speech comprehension, mainly in the noisy situations, as well as the localization of the sound sources.

Cochlear Implants↗

Conservative management versus surgery for small vestibular schwannomas.

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 measuring <15 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 aged >60 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 by > or =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.

Adolescent↗

Long-term functional outcome in facial nerve graft by fibrin glue in the temporal bone and cerebellopontine angle.

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.

Adolescent↗

Large deletion of the GJB6 gene in deaf patients heterozygous for the GJB2 gene mutation: genotypic and phenotypic analysis.

Recent investigations identified a large deletion of the GJB6 gene in trans to a mutation of GJB2 in deaf patients. We looked for GJB2 mutations and GJB6 deletions in 255 French patients presenting with a phenotype compatible with DFNB1. 32% of the patients had biallelic GJB2 mutations and 6% were a heterozygous for a GJB2 mutation and a GJB6 deletion. Biallelic GJB2 mutations and combined GJB2/GJB6 anomalies were more frequent in profoundly deaf children. Based on these results, we are now assessing GJB6 deletion status in cases of prelingual hearing loss.

Connexin 26↗

Opening cerebrospinal fluid pressure guides the management of cerebrospinal fluid leakage after acoustic neuroma surgery.

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.

Acetazolamide↗

Cochlear implants in elderly people: preliminary results.

The objective of this study was to evaluate the benefit of cochlear implantation in adults aged 60 years and over. Twenty-eight patients, older than 60 years and with profound bilateral sensorineural hearing loss, received a cochlear implant between 1991 and 2001. The mean age was 66 years and the median follow-up was 22.5 months. Speech perception scores before and after implantation were analyzed retrospectively in order to evaluate the benefit of cochlear implantation. There was a significant improvement of the disyllabic words and sentences scores after implantation. The patients who were over 70 years performed as well as those who were younger. The surgical procedure was well tolerated in all patients. One patient developed a postoperative vertigo due to a perilymphatic fistula. In conclusion, cochlear implantation offers improvement in speech perception to the elderly population, as in the younger population. A careful assessment of the physical status of these patients remains essential in order to evaluate the risk-benefit of this procedure.

Aged↗

Auditory brainstem implant in bilateral and completely ossified cochleae.

OBJECTIVE: To report and evaluate the results of auditory brainstem implantation in a case of postmeningitis hearing loss with totally ossified cochleae on both sides. STUDY DESIGN: Case report. SETTING: Tertiary referral center. PATIENT: A 49-year-old man was referred to the authors' department for bilateral total hearing loss subsequent to bacterial meningitis 2 years earlier. Pure tone audiometry, auditory brainstem response tests, and promontory tests did not reveal any cochlear activity. Computed tomography showed bilateral and totally ossified cochleae. Magnetic resonance imaging confirmed the diagnosis by showing no cochlear signal on T2-weighed images and ruled out brainstem and cerebellopontine angle abnormalities. INTERVENTION: A left auditory brainstem implantation was performed through a translabyrinthine route, using a Nucleus 22-channel device. MAIN OUTCOME MEASURES: Word and sentence recognition tests in sound-only and sound plus vision modes. RESULTS: No postoperative complication was observed. Twelve electrodes could be activated, and their tonotopy was defined. Nine other electrodes were inactivated because of an absence of auditory response (4 electrodes) or paresthesia (5 electrodes). At the last follow-up visit, 26 months after the implantation, 50% of disyllabic word scores and 60% of sentence scores were achieved using auditory brainstem implant sound only. These scores reached 80% and 93%, respectively, with lip-reading. CONCLUSION: Auditory brainstem implantation is an efficient means of auditory rehabilitation in cases of bilateral total hearing loss with totally ossified cochleae. It should be considered in cases of predictable failure in cochlear implantation.

Brain Stem↗

Ossiculoplasty with total hydroxylapatite prostheses anatomical and functional outcomes.

OBJECTIVE: To evaluate the results of partial and total ossicular reconstruction prostheses entirely made of hydroxylapatite. STUDY DESIGN: A retrospective review of cases followed-up between 1997 and 2000. SETTING: Tertiary referral center. PATIENTS: 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%). INTERVENTION: 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. MAIN OUTCOME MEASURES: 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. RESULTS: 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, p < 0.05). 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. CONCLUSION: 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.

Adult↗

Two large French pedigrees with non syndromic sensorineural deafness and the mitochondrial DNA T7511C mutation: evidence for a modulatory factor.

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.

Adolescent↗