Cochlear Implantation and Other Treatments in Single-Sided Deafness and Asymmetric Hearing Loss: Results of a National Multicenter Study Including a Randomized Controlled Trial.

INTRODUCTION: Cochlear implantation is a recent approach proposed to treat single-sided deafness (SSD) and asymmetric hearing loss (AHL). Several cohort studies showed its effectiveness on tinnitus and variable results on binaural hearing. The main objective of this study is to assess the outcomes of cochlear implantation and other treatment options in SSD/AHL on quality of life. METHODS: This prospective multicenter study was conducted in 7 tertiary university hospitals and included an observational cohort study of SSD/AHL adult patients treated using contralateral routing of the signal (CROS) hearing aids or bone-anchored hearing systems (BAHSs) or who declined all treatments, and a randomized controlled trial in subjects treated by cochlear implantation, after failure of CROS and BAHS trials. In total, 155 subjects with SSD or AHL, with or without associated tinnitus, were enrolled. After 2 consecutive trials with CROS hearing aids and BAHSs on headband, all subjects chose any of the 4 treatment options (abstention, CROS, BAHS, or cochlear implant [CI]). The subjects who opted for a CI were randomized between 2 arms (CI vs. initial observation). Six months after the treatment choice, quality of life was assessed using both generic (EuroQoL-5D, EQ-5D) and auditory-specific quality-of-life indices (Nijmegen Cochlear implant Questionnaire [NCIQ] and Visual Analogue Scale [VAS] for tinnitus severity). Performances for speech-in-noise recognition and localization were measured as secondary outcomes. RESULTS: CROS was chosen by 75 subjects, while 51 opted for cochlear implantation, 18 for BAHSs, and 11 for abstention. Six months after treatment, both EQ-5D VAS and auditory-specific quality-of-life indices were significantly better in the "CI" arm versus "observation" arm. The mean effect of the CI was particularly significant in subjects with associated severe tinnitus (mean improvement of 20.7 points ± 19.7 on EQ-5D VAS, 20.4 ± 12.4 on NCIQ, and 51.4 ± 35.4 on tinnitus). No significant effect of the CI was found on binaural hearing results. Before/after comparisons showed that the CROS and BAHS also improved significantly NCIQ scores (for CROS: +7.7, 95% confidence interval [95% CI] = [4.5; 10.8]; for the BAHS: +14.3, 95% CI = [7.9; 20.7]). CONCLUSION: Cochlear implantation leads to significant improvements in quality of life in SSD and AHL patients, particularly in subjects with associated severe tinnitus, who are thereby the best candidates to an extension of CI indications.

Treatment choice in single-sided deafness and asymmetric hearing loss. A prospective, multicentre cohort study on 155 patients.

OBJECTIVES: To describe the treatment choice in a cohort of subjects with single-sided deafness (SSD) and asymmetric hearing loss (AHL). To assess the reliability of the treatment trials. DESIGN: In this national, multicentre, prospective study, the choice of subjects was made after two consecutive trials of Contralateral Routing Of the Signal (CROS) hearing aids and a Bone Conduction Device (BCD) on a headband. Subjects could proceed with one of these two options, opt for cochlear implantation or decline all treatments. SETTING: Seven tertiary university hospitals. PARTICIPANTS: One hundred fifty-five subjects with SSD or AHL fulfilling the candidacy criteria for cochlear implantation, with or without associated tinnitus. MAIN OUTCOME MEASURES: After the two trials, the number of subjects choosing each option was described. Repeated assessments of both generic and auditory-specific quality of life were conducted, as well as hearing assessments (speech recognition in noise and horizontal localization). RESULTS: CROS was chosen by 75 subjects, followed by cochlear implantation (n = 51), BCD (n = 18) and abstention (n = 11). Patients who opted for cochlear implantation had a poorer quality of life (P = .03). The improvement of quality of life indices after each trial was significantly associated with the final treatment choice (P = .008 for generic indices, P = .002 for auditory-specific indices). The follow-up showed that this improvement had been overestimated in the CROS group, with a long-term retention rate of 52.5%. CONCLUSIONS: More than one third of SSD/AHL subjects are unsatisfied after CROS and BCD trials. Repeated quality of life assessments help counselling the patient for his/her treatment choice.

Intratemporal facial nerve schwannomas: multicenter experience of 80 cases.

PURPOSE: To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making. METHODS: A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients' demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered. RESULTS: At presentation, transient or persistent facial palsy was the most common symptom, followed by hearing loss. The schwannoma involved more than one segment in the majority of the patients with the geniculate ganglion being most commonly involved. Initial treatment consisted of a wait and scan approach in 67.5%, surgery in 30% and radiation therapy in 2.5% of the patients. Tympanic segment schwannomas caused mainly conductive hearing loss and were more prone to develop facial palsy at follow-up. Internal auditory canal or cerebellopontine angle schwannomas presented with significantly more sensorineural hearing loss. CONCLUSIONS: Although modern imaging has improved diagnosis of this tumor, choosing the best treatment modality remains a real challenge. Based on the literature review and current findings, more insights into the clinical course and the management of facial nerve schwannomas are provided.

Pediatric thyroid surgery: experience in 75 consecutive thyroidectomies.

OBJECTIVES: The main objective of this study is to report our experience in a university hospital in the surgical management of thyroid disease in pediatric patients. We also aim to analyze the results and evaluate the efficacy of fine-needle aspiration biopsy (FNAB) and frozen section analysis. METHODS: A retrospective review of thyroid surgeries from January 1997 to December 2017 was conducted. Patients aged under 18 who underwent total or partial thyroidectomy were included. The indication of the surgery, the investigation results, the surgical technique used and the final diagnosis were reviewed and analyzed. RESULTS: A total of 75 patients were included. 80% (n = 60) were females and 20% (n = 15) were males. The average age was 13.3 years ± 3.76 and age range was 3-18 years. The indication for thyroidectomy was thyroid nodule in 80% (n = 60), Grave's disease in 13.3% (n = 10), multiple endocrine neoplasm type II (MEN II) in 6.7% (n. 5). FNAB was done in 42.7% (n = 32/75) with sensitivity and specificity of 62.50% and 94.4%, respectively, the positive predictive value was 83.3% and the negative predictive value was 85%. Frozen section, which is a quick intraoperative micro and/or macroscopic examination, was done in 66.7% (n = 50/75). It was malignant in 12% (n = 6/50) and was benign in 88% (n = 44/50). Among benign results, nine (n = 9/44) patients had malignant disease in final pathological diagnosis. Its sensitivity and specificity were 40% and 100%, respectively, the positive predictive value and the negative predictive value were 100% and 79.6%, respectively. Total thyroidectomy was done in 54.7% (n = 41/75) and partial thyroidectomy was done in 45.3% (n = 34/75). Of the 75 patients, 29.3% (n = 22) had thyroid carcinoma on final pathological analysis. CONCLUSION: Thyroid diseases that require surgical intervention are rare in pediatric populations. However, when surgery is indicated, there is higher risk of malignancy compared to adults. Our study showed that FNAB has a lower specificity and sensitivity compared to adults, and that surgical decision should be made considering all investigations. We also showed that frozen section is considered primarily for papillary thyroid carcinoma but is not reliable for follicular carcinoma or Bethesda IV. Finally, thyroid surgery in children and adolescents must be part of global multidisciplinary management.

Cortical Processing of Vocal and Nonvocal Sounds in Cochlear-Implanted Children: An Electrophysiological Study.

OBJECTIVES: For prelingually deaf children, cochlear implants (CIs) can restore auditory input to the auditory cortex and the ability to acquire spoken language. Language development is strongly intertwined with voice perception. The aim of this electrophysiological study was to investigate human voice processing using measures of cortical auditory evoked potentials (AEPs) in pediatric CI users. DESIGN: Cortical AEPs were measured in 8 CI children (4 to 12 years old) with good auditory and language performance and 8 normal-hearing (NH) age-matched controls. The auditory stimuli were nonspeech vocal sounds (laughing, sighing, coughing) and environmental sounds (e.g., telephones, alarms, cars, bells, water, wind). Independent component analysis was used to minimize the CI artifact in cortical AEPs. RESULTS: Fronto-temporal positivity to vocal sounds was found in NH children, with a significant effect in the 140 to 240 msec latency range. In CI children, there was a positive response to vocal sounds in the 170 to 250 msec latency range, with a more diffuse and anterior distribution than in the NH children. CONCLUSIONS: Cortical responses to vocal sounds were recorded in CI children. The topography and latency of response to voice differed from that of NH children. The results suggest that cortical reorganization for processing vocal sounds may occur in congenitally deaf children fitted with a CI.

Audiometric evaluation in patients with Alzheimer's disease.

The objective of this study is to assess the validity of ASSR as a complementary diagnostic test for peripheral hearing loss by proving a significant correlation between behavioral thresholds and ASSR. The design used in this study is monocentric prospective study from November 2014 to April 2015. The setting used in this study is the ENT-Head and Neck Surgery Department and Geriatrics Department in a French Regional and University Hospital. The participants are patients over 75 years with cognitive impairment (Alzheimer's disease or mild-cognitive impairment) with a Mini-Mental State Examination score under 27/30 and without hearing aids. Exclusion criteria were: otoscopic and middle ear abnormalities, retro-cochlear lesion, other types of dementia, and central nervous system disease altering cerebral lateralization. The intervention used in this study is pure-tone audiometry, speech audiometry, dichotic listening test, and auditory steady-state responses recording. The correlations between these exams were studied with Pearson's correlation coefficient and Student's t test. Results were significant if p < 0.05. Twenty-three ears were analyzed from 12 patients. There were six women and six men with cognitive impairment, mean age 82.1 (±4.6) years, and mean MMSE score that was 21.3/30 (±5.7). The correlation between pure-tone audiometry and ASSR was significant for all frequencies: r = 0.55 (p = 0.006) for 500 Hz, r = 0.58 (p = 0.005) for 1000 Hz, r = 0.61 (p = 0.003) for 2000 Hz, and r = 0.66 (p = 0.002) for 4000 Hz. There was no significant correlation between the MMSE and the difference between ASSR and PTA on each frequency. The dichotic listening test showed a right ear advantage (50.9 %, p = 0.039). The ASSR in patients with cognitive impairment and understanding troubles is a promising complementary technique to estimate the hearing thresholds.

Management of laryngoceles by transoral robotic surgery.

We herein report our experience with the management of laryngoceles using transoral robotic surgery (TORS). A bicentric retrospective study was conducted from November 2009 to September 2015. The inclusion criteria were treatment of a laryngocele by TORS and no malignancy on definitive histopathology. Surgery was performed using the Da Vinci (Intuitive®) surgical robot. Surgical methods and post-operative outcomes were evaluated. Eight patients (four men and four women) presenting with a laryngocele (one bilateral case) were included (mean age 61.8 years). There was one covering tracheotomy. The average post-operative stay was 3.75 days. Three patients treated for a combined laryngocele had a nasogastric feeding tube inserted for 5 days. One patient experienced late laryngeal bleeding that required surgical treatment. TORS may offer an efficient treatment option for laryngoceles. The use of precise and flexible instruments and a three-dimensional camera allow fine dissection of these tumours, preserving the glottic space and vocal function, even for combined laryngoceles extending deep within the neck.Evidence level: 4.

Day-case otology: Special attention to the pediatric cochlear implantation procedure.

OBJECTIVE: Assess the feasibility of cochlear implantation as day-surgery in children and identify variables influencing admission, readmission, and unplanned postoperative consultation. METHODS: This retrospective observational monocentric study was conducted according to the STROBE recommendations. Between January 2017 and July 2022, all medical records of children who underwent cochlear implantation were analyzed. Eligible children were admitted for the first time to the pediatric day-surgery unit. Exclusion criteria were children planned for inpatient procedure, bilateralization or explantation-reimplantation. Sex assigned at birth, analgesic medication, anesthesia and complications were related to categorical variables. Age, duration of anesthesia, length of stay and ASA score were related to continuous variables. RESULTS: We included 66 children from a total of 106. Mean age was 53 months [SD: 46 months, range 8-184 months]. Successful day-surgery management was observed in 86% of cases. In 14% of cases, children were admitted to the pediatric ENT unit for the following reasons: late awakening in 6%, non-controlled pain in 4.5%, postoperative nausea and vomiting in 3.5% of cases. Univariate analysis did not observe any factor promoting success of day-surgery regarding anesthetic agents. Age was not statistically significant as a factor of ambulatory failure. Time spent in the operating room was not a determining factor (p = 0.559). None of the children were rehospitalized. Early unplanned consultations were observed in 3% of cases for vertex edema in 1 case and uncomplicated otorrhea in 1 case. CONCLUSION: This study adds to the knowledge on pediatric cochlear implantation and suggests that this procedure is suitable for day-surgery at any age.

Does Phonological Complexity Provide a Good Index of Language Disorder in Children With Cochlear Implants?

Purpose Phonological complexity is known to be a good index of developmental language disorder (DLD) in normal-hearing children, who have major difficulties on some complex structures. Some deaf children with cochlear implants (CIs) present a profile that evokes DLD, with persistent linguistic difficulties despite good audiological and environmental conditions. However, teasing apart what is related to auditory deficit or to language disorder remains complex. Method We compared the performance of three groups of school-age children, 33 children with CI, 22 with DLD, and 24 with typical development, on a nonword repetition (NWR) task based on phonological complexity. Children with CI were studied regarding their linguistic profile, categorized in four subgroups ranging from excellent to very poor performance. Influence of syllable length and phonological structures on the results of all the children were explored. Results The NWR task correctly distinguished children with DLD from typically developing children, and also children with CI with the poorest linguistic performance from other children with CI. However, most complex phonological structures did not reliably identify children with CI displaying a profile similar to that of children with DLD because these structures were difficult for all of the children with CI. The simplest phonological structures were better at detecting persistent language difficulties in children with CI, as they were challenging only for the children with the poorest language outcomes. Conclusions The most complex phonological structures are not good indices of language disorder in children with CI. Phonological complexity represents a gradient of difficulty that affects normal-hearing and deaf children differently.

Prospective Multicentric Follow-up Study of Cochlear Implantation in Adults With Single-Sided Deafness: Tinnitus and Audiological Outcomes.

OBJECTIVE: This study investigated the audiological and tinnitus outcomes of cochlear implantation (CI) in adults with single-sided deafness (SSD) and tinnitus. STUDY DESIGN: Multicentered prospective, non-randomized intervention study. SETTING: Six French CI centers. PATIENTS: Twenty-six patients with SSD and incapacitating tinnitus (Tinnitus Handicap Inventory [THI] >58) underwent cochlear implantation. INTERVENTIONS: First, CIs delivered only masking white noise stimulation for 1 month and then standard CI stimulation. MAIN OUTCOME MEASURES: Before and after CI surgery, patients completed the THI, Tinnitus Reaction Questionnaire (TRQ), Subjective Tinnitus Severity Scale (STSS), and two visual analogue scales quantifying tinnitus loudness and annoyance. Speech perception in spatialized noise was tested at 13 months. RESULTS: The first month of white noise stimulation triggered a significant improvement in THI scores (72 ±â€Š9 to 55 ±â€Š20, p < 0.05). No change was observed for the other measures. After 1 year of standard CI stimulation, 23 patients (92%) reported a significant improvement in tinnitus. This improvement started 1 to 2 months after CI and exceeded 40% improvement for 14 patients (54%). Average speech-in-noise perception after 1 year significantly improved for the 23 patients who completed these measures. CONCLUSIONS: CI is efficacious to reduce the handicap of patient with SSD and incapacitating tinnitus, leading to a decrease in reported tinnitus and partial restoration of binaural hearing abilities.

Relationship between Metabolomics Profile of Perilymph in Cochlear-Implanted Patients and Duration of Hearing Loss.

Perilymph metabolomic analysis is an emerging innovative strategy to improve our knowledge of physiopathology in sensorineural hearing loss. This study aims to develop a metabolomic profile of human perilymph with which to evaluate the relationship between metabolome and the duration of hearing loss. Inclusion criteria were eligibility for cochlear implantation and easy access to the round window during surgery; patients with residual acoustic hearing in the ear to be implanted were excluded. Human perilymph was sampled from 19 subjects during cochlear implantation surgery. The perilymph analysis was performed by Liquid Chromatography-High-Resolution Mass and data were analyzed by supervised multivariate analysis based on Partial Least-Squares Discriminant Analysis and univariate analysis. Samples were grouped according to their median duration of hearing loss. We included the age of patients as a covariate in our models. Statistical analysis and pathways evaluation were performed using Metaboanalyst. Nineteen samples of human perilymph were analyzed, and a total of 106 different metabolites were identified. Metabolomic profiles were significantly different for subjects with ≤ 12 or > 12 years of hearing loss, highlighting the following discriminant compounds: N-acetylneuraminate, glutaric acid, cystine, 2-methylpropanoate, butanoate and xanthine. As expected, the age of patients was also one of the main discriminant parameters. Metabolic signatures were observed for duration of hearing loss. These findings are promising steps towards illuminating the pathophysiological pathways associated with etiologies of sensorineural hearing loss, and hold open the possibilities of further explorations into the mechanisms of sensorineural hearing loss using metabolomic analysis.

[Cavernous hemangioma of the nasal cavity]. / Hémangiome caverneux de la fosse nasale.

OBJECTIVES: To study the circumstances of diagnosis, imaging techniques and therapeutic management of cavernous hemangioma of the nasal cavity. MATERIAL AND METHOD: We report the case of a 60-year-old woman, who presented epistaxis CT scan and nasal obstruction. RESULTS: Rhinoscopy showed a purplish, regular mass that filled the left nasal cavity. Computed tomographic and MRI images showed the tumor in contact close to the middle turbinate. An arteriography with selective embolization was performed. The lesion was completely removed by endonasal endoscopic surgery. The histological examination showed a cavernous hemangioma of the nasal cavity. CONCLUSION: Even if cavernous hemangiomas are rare, the practitioner must suggest the diagnosis if the patient has a purplish bleeding mass of the nasal cavity. CT scan, MRI, and arteriography can help make the diagnosis. Before surgical management, embolization is recommended.

Long-Term Cognitive Prognosis of Profoundly Deaf Older Adults After Hearing Rehabilitation Using Cochlear Implants.

OBJECTIVES: To analyze long-term cognitive status and function after cochlear implantation in profoundly deaf individuals. DESIGN: Prospective observational longitudinal study. SETTING: Ten academic medical centers referent for cochlear implantation. PARTICIPANTS: Individuals aged 65 and older who qualified for cochlear implantation (N=70). MEASUREMENTS: Cognitive tests were administered before cochlear implantation and 1 and 5 or more years after cochlear implantation. Evaluation consisted of 6 tests assessing attention, memory, orientation, executive function, mental flexibility, and fluency. Cognitive status was determined as normal, mild cognitive impairment (MCI), or dementia. Speech perception in quiet and noisy conditions was assessed using disyllabic words, and quality of life was assessed using the Nijmegen Cochlear Implant Questionnaire. RESULTS: Mean follow-up was 6.8 years (range 5.5-8.5 years). Speech perception scores and quality of life remained stable from 1 to 7 years after cochlear implantation. Of 31 participants (45%) with MCI before cochlear implantation, 2 (6%) developed dementia during follow-up, 19 (61%) remained stable, and 10 (32%) returned to normal cognition. None of the 38 with normal cognition developed dementia during follow-up, although 12 (32%) developed MCI. CONCLUSION: MCI is highly prevalent in older adults with profound hearing loss. Nevertheless, we observed a low rate of progression to dementia, and cognitive function improved in some individuals with MCI at baseline. These results highlight that cochlear implantation should be strongly considered in profoundly deaf individuals, even those with MCI, who may have a specific subtype of MCI, with a possible positive effect of hearing rehabilitation on neurocognitive functioning.

Serous otitis media revealing temporal en plaque meningioma.

OBJECTIVES: To present a series of temporal en plaque meningiomas involving the middle ear or mastoid, whose main symptoms suggested a serous otitis media. STUDY DESIGN AND SETTINGS: Multicentric retrospective study reviewing clinical records originating from eight tertiary referral centers. MATERIALS AND METHODS: The clinical records of 10 patients presenting with signs and symptoms suggesting serous otitis media and whose neuroimaging studies revealed a temporal en plaque meningioma involving the middle ear or mastoid are reported. RESULTS: All the patients were women, ranging from 49 to 71 years old. The delay between the onset of symptoms and the diagnosis of meningioma varied from 1 to 10 years. All the patients underwent various procedures usually applied for the treatment of serous otitis media, which failed in all the cases, particularly ventilating tube placement, which was followed by severe episodes of discharge. In all cases, the computed tomographic scans showed three imaging signs: soft tissue mass filling the middle ear or mastoid, hyperostosis of the petrous bone, and hairy aspect of the intracranial margins of the affected bone. This imaging triad must alert the otologist of the possibility of intracranial meningioma. Magnetic resonance imaging was the method of choice to assess the diagnosis of intracranial meningioma involving the middle ear or mastoid. When analyzing management options, it appeared that conventional middle ear procedures were inefficient. CONCLUSION: Temporal en plaque meningioma involving the middle ear or mastoid can mimic a serous otitis media. A computed tomographic scan is recommended for cases of atypical or prolonged unilateral serous otitis media to investigate indirect signs of a meningioma, which has to be confirmed with magnetic resonance imaging.

Does Checking the Placement of Ossicular Prostheses via the Posterior Tympanotomy Improve Hearing Results After Cholesteatoma Surgery?

INTRODUCTION: Posterior tympanotomy (PT) is often performed during the surgical management of middle ear cholesteatoma with extension in the retrotympanum area. This PT can also be used to control the right position of the ossicular prosthesis masked by the tympanic membrane reconstruction. OBJECTIVE: To compare audiologic results after ossiculoplasty performed via the outer ear canal and via the PT for patients with cholesteatoma. MATERIALS AND METHODS: Retrospective chart reviews were performed for 68 patients (68 ears) with cholesteatoma who underwent titanium ossicular prosthesis surgery between January 2007 and January 2011. We compared audiologic results between two groups: the WPT group (the group without checking the prosthesis via the PT) and the PT group (the group with placing and/or checking the prosthesis via the PT). A postoperative pure-tone average air-bone gap of 20 dB or less was considered a successful hearing result. RESULTS: Of the patients who underwent canal wall-up mastoidectomy for cholesteatoma with ossicular chain reconstruction by titanium prosthesis, 36 patients (20 total ossicular replacement prosthesis [TORP], 16 partial ossicular replacement prosthesis [PORP]) were in the PT group and 32 patients (16 TORP, 16 PORP) were in the WPT group. The global success rate (defined as a mean residual air-bone gap < 20  dB) was 50% in the WPT group (56% in the subgroup PORP, 44% in the subgroup TORP) and 42% in the PT group (62% in PORP, 25% in TORP). There was no case with extrusion of the prosthesis in either group. No facial palsy occurred during the postoperative period for either group. CONCLUSION: Control of ossicular prosthesis positioning via the PT does not improve hearing results after ossicular chain reconstruction in cholesteatoma surgery. However, this approach can be used during a second-stage procedure that avoids incisions within the external ear canal.

The internal acoustic meatus and its meningeal layers: a microanatomical study.

OBJECT: The authors studied the cadaveric heads of 22 adults to describe the internal acoustic meatus (IAM) and its contents. Special attention was paid to the length of the arachnoidal and dural sheaths surrounding the neural structures, including the vestibular ganglion. An additional goal of this study was to verify anatomically the concept of arachnoidal duplication, which is reputedly induced by medial growth of vestibular neuromas and helpful in atraumatic dissection. METHODS: Twelve cadaveric heads (24 IAMs) were injected with colored latex and fixed in formalin. Cautious removal of the skull vault and the brain or the skull base allowed superior and anteroinferior views of the IAM, respectively. Photographs were obtained after removal of the bone canal and dissection of the meninges with the aid of optic magnification. Ten IAMs were prepared for histological study and the osteological anatomy of the fundus was endoscopically described for the remaining 10. The dura mater covered the bone structures of the IAM, and the arachnoidal membrane of the cerebellopontine cistern invaginated into this dural cul-de-sac as a "muff." The entire neurovascular content of the IAM, including the vestibular ganglion, was surrounded by this arachnoidal sheath in which cerebrospinal fluid circulated. The length of this arachnoidal sheath was the same ventrally and dorsally and, in all specimens, the entrance of the cochleovestibulofacial complex into the subarachnoid space was located at the fundus level. CONCLUSIONS: In this study the authors demonstrated the existence of an acousticofacial cistern containing every nerve of the vestibulocochleofacial complex, including the vestibular ganglion from which acoustic neuromas develop. These findings clearly contradict the theory of the duplication of arachnoidal layers during medial growth of vestibular neuromas and may explain some of the intraoperative difficulties encountered in the atraumatic dissection of these tumors.

Massive infra-clinic invasion of the facial nerve by a myoepithelial carcinoma of the parotid.

We report a new case of myoepithelial carcinoma of the parotid gland in an 8-year-old girl. This is the first case published in a child. The parotid tumour was slightly tender and measured almost 2 cm in diameter. There was no associated facial nerve paralysis despite surgical and histologic evidence of massive facial nerve infiltration. We performed total parotidectomy with resection of the intra-mastoid portion of the facial nerve completed with prophylactic lymph node dissection. Eight months after surgery, MRI revealed a deep-lying recurrence, which required reintervention. There has been no subsequent recurrence 18 months after surgery. Microscopic examination of operative specimens confirmed the diagnosis of parotid myoepithelial carcinoma with fusiform cells. Immunohistochemical markers were positive for cytokeratin, epithelial membrane antigen, smooth muscle actin, S-100 protein, anti-desmine and anti-vimentine. This difficult to diagnose tumour, which was individualised by the World Health Organisation in 1991, is considered a moderate to high-grade malignancy when it develops in a pleomorphic adenoma or appears de novo.

Retrospective case study of carbon dioxide laser stapedotomy with lens-based and mirror-based micromanipulators.

Most clinical studies on carbon dioxide (CO2) (lambda = 10.6 mm) laser stapedotomy have been carried out with the laser guided by a conventional lens-based micromanipulator, with the attendant risks of correct aiming (HeNe) and surgical (CO2) beam misalignment. Hence, engineering advances have attempted to improve laser targeting as well as the spot size focus. The development of the mirror-based micromanipulator was a response to this need but no data concerning its use in stapes surgery is available. We performed a retrospective case-series review of patients treated for otosclerosis between 1992 and 2000. Primary laser stapedotomy was performed in 218 consecutive patients. In the first 78 procedures, the aiming beam (HeNe, lambda = 632 nm) and surgical beam (CO2) were guided with a conventional lens-based micromanipulator whereas in the subsequent 140 procedures, they were guided by using a mirror-based micromanipulator. Hearing was tested at six and 12 months. The mean (SD) airbone gap was 5 dB (4.5) and 4.5 dB (3.9). The mean closure was 15 dB (9.9) and 14.4 dB (9.4). The mean change in the high-tone bone-conduction level was 5.5 dB (7.3) and 7.8 dB (7.5). Overheating of the facial canal produced transient facial paralysis in one case and was due to misalignment of the beams with the lens-based micromanipulator. Use of the mirror-based micromanipulator obviated the need to verify alignment. The light-weight and superior optical yield of this system made it possible to reduce the number of impacts on the footplate by the integral restitution of the energy source. This study demonstrated that the CO2 laser is an effective method for performing stapedotomy. In addition, microtrauma to the labyrinth is reduced by its ability to perform calibrated footplate fenestration without mechanical or vibrational injury to the inner ear. The optical reflection micromanipulator simplified beam alignment and enhanced surgical comfort.

Cortical electrophysiological markers of language abilities in children with hearing AIDS: a pilot study.

OBJECTIVE: To investigate cortical auditory evoked potentials (CAEPs) in pediatric hearing aid (HA) users, with and without language impairment. DESIGN: CAEPs were measured in 11 pediatric HA users (age: 8-12 years) with moderate bilateral sensorineural hearing loss (HL); participants were classified according to language ability. CAEPs were also measured for a control group of 11 age-matched, normal-hearing (NH) children. RESULTS: HL children without language impairment exhibited normal CAEPs. HL children with language impairment exhibited atypical temporal CAEPs, characterized by the absence of N1c; frontocentral responses displayed normal age-related patterns. CONCLUSION: Results suggest that abnormal temporal brain function may underlie language impairment in pediatric HA users with moderate sensorineural HL.