F. Venail

Factors Affecting Auditory Performance of Postlinguistically Deaf Adults Using Cochlear Implants: An Update with 2251 Patients

Objective: To update a 15-year-old study of 800 postlinguistically deaf adult patients showing how duration of severe to profound hearing loss, age at cochlear implantation (CI), age at onset of severe to profound hearing loss, etiology and CI experience affected CI outcome. Study Design: Retrospective multicenter study. Methods: Data from 2251 adult patients implanted since 2003 in 15 international centers were collected and speech scores in quiet were converted to percentile ranks to remove differences between centers. Results: The negative effect of long duration of severe to profound hearing loss was less important in the new data than in 1996; the effects of age at CI and age at onset of severe to profound hearing loss were delayed until older ages; etiology had a smaller effect, and the effect of CI experience was greater with a steeper learning curve. Patients with longer durations of severe to profound hearing loss were less likely to improve with CI experience than patients with shorter duration of severe to profound hearing loss. Conclusions: The factors that were relevant in 1996 were still relevant in 2011, although their relative importance had changed. Relaxed patient selection criteria, improved clinical management of hearing loss, modifications of surgical practice, and improved devices may explain the differences.

Pre-, Per- and Postoperative Factors Affecting Performance of Postlinguistically Deaf Adults Using Cochlear Implants: A New Conceptual Model over Time

Objective To test the influence of multiple factors on cochlear implant (CI) speech performance in quiet and in noise for postlinguistically deaf adults, and to design a model of predicted auditory performance with a CI as a function of the significant factors. Study Design Retrospective multi-centre study. Methods Data from 2251 patients implanted since 2003 in 15 international centres were collected. Speech scores in quiet and in noise were converted into percentile ranks to remove differences between centres. The influence of 15 pre-, per- and postoperative factors, such as the duration of moderate hearing loss (mHL), the surgical approach (cochleostomy or round window approach), the angle of insertion, the percentage of active electrodes, and the brand of device were tested. The usual factors, duration of profound HL (pHL), age, etiology, duration of CI experience, that are already known to have an influence, were included in the statistical analyses. Results The significant factors were: the pure tone average threshold of the better ear, the brand of device, the percentage of active electrodes, the use of hearing aids (HAs) during the period of pHL, and the duration of mHL. Conclusions A new model was designed showing a decrease of performance that started during the period of mHL, and became faster during the period of pHL. The use of bilateral HAs slowed down the related central reorganization that is the likely cause of the decreased performance.

Comparison of Echo-Planar Diffusion-Weighted Imaging and Delayed Postcontrast T1-Weighted MR Imaging for the Detection of Residual Cholesteatoma

BACKGROUND AND PURPOSE: Echo-planar diffusion-weighted imaging (DWI) and delayed postcontrast T1-weighted MR imaging (DPI) have been proposed in previous studies to detect residual middle ear cholesteatomas, with varying results. We assessed and compared these 2 techniques in patients with canal wall-up tympanoplasty. MATERIALS AND METHODS: This was a prospective cohort study. Patients who underwent surgery for middle ear cholesteatoma had CT scanning 9 months after the surgery. If opacity was observed (64%) on CT scans, DWI and DPI were performed before second-look surgery. CT, MR imaging, and surgical data were available for 31 patients. Charts were reviewed independently by 3 blinded examiners. Interobserver agreement for MR imaging was calculated (Cohen κ). Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated for these techniques: 1) alone or in association, and 2) according to the residual cholesteatoma size measured during surgery. RESULTS: Interobserver agreement was better for DWI (κ = 0.81) than for DPI (κ = 0.51). Sensitivity, specificity, PPV, and NPV values were 60%, 72.73%, 80%, and 50%, respectively, with DWI; and 90%, 54.55%, 78.26%, and 75%, respectively, with DPI. With cholesteatomas >5 mm, the sensitivity and specificity of DWI reached 100% and 88%, respectively, with values for DPI reaching 100% and 80%, respectively. The association of both techniques only allowed improvements in the specificity for lesions >5 mm. CONCLUSIONS: Both techniques gave acceptable results for residual cholesteatoma detection. DWI is more specific but less sensitive than DPI. Their concurrent use may benefit patients by avoiding undue surgery.

New perspectives for middle ear implants : First results in otosclerosis with mixed hearing loss

Middle ear implantation is an efficient procedure to restore moderate to severe sensorineural hearing loss (HL) in selected patients. Implantation of such devices requires ossicular chain integrity. Patients suffering from otosclerosis with mixed HL should be eligible for this treatment after stapes surgery with air‐bone gap closure. To address this issue, we report four cases of middle ear implantation after or during stapes surgery. Results and complications obtained with Vibrant SoundBridge, MedEl and Middle Ear Transducer, Otologics are reported. Audiologic results were similar to those obtained in cases of sensorineural HL. One case of postoperative labyrinthitis was observed.

ENT and Speech Disorders in Children with Down’s Syndrome: an Overview of Pathophysiology, Clinical Features, Treatments, and Current Management

Down’s syndrome is the most commonly occurring genetic abnormality, involving about 1 in 600 births. The increasing life expectancy of individuals with Down’s syndrome has revealed the presence of several unexpected pathological processes. Among these, ENT disorders hold an important place because of their high incidence and severity. Accurate knowledge of the pathophysiology underlying ENT disorders (facial dysmorphism, ear abnormalities, upper airway abnormalities, and immunodeficiency) allow an understanding of the reasons for the development of the upper airway obstruction, obstructive sleep apnea syndrome, subglottic stenosis, deafness, speech delay, and ENT infections that occur frequently in these children. Early screening and specific treatment may allow some of the long-term sequelae to be avoided, or at least their prognosis to be improved. In order to help health care professionals in their daily practice, this review makes a series of recommendations to allow them to develop a master plan for the ENT management of children with Down’s syndrome. In children with Down’s syndrome, ENT disorders occur frequently and are often severe They develop owing to craniofacial, functional, and immune system abnormalities. Early screening and treatment allow improvements in long-term outcomes.

Educational and Employment Achievements in Prelingually Deaf Children Who Receive Cochlear Implants

OBJECTIVE To investigate the educational and employment achievements of prelingually deaf children who undergo cochlear implantation. DESIGN Prospective study. Data were examined within groups defined by current age and additional disabilities. Multivariate analyses were used to identify variables influencing grade failure and communication mode. SETTING Tertiary referral center. PARTICIPANTS One hundred prelingually deaf children who received cochlear implants before 6 years of age and who also had at least 4 years of follow-up. INTERVENTION Interview of parents using a standardized questionnaire. MAIN OUTCOME MEASURES The type of schooling, form of communication, employment status, age at which the child learned to read and write, number of grade failures, and educational support required. RESULTS Mainstream schooling, regardless of educational level, was the standard experience for children without additional disabilities (16 of 24 [67%] in the 12- to 15-year age group to 20 of 24 [83%] in the 8- to 11-year age group). Four of 8 participants older than 18 years (50%) had a university-level education; the remainder had vocational training. Delayed reading and writing skills were experienced by 19 of 74 participants (26%) and, depending on the age group, 42% to 61% of participants (10 of 24 in the 8- to 11-year age group to 11 of 18 in the 16- to 18-year age group) had failed a grade. The number of grade failures was associated with communication mode at the time of the survey. Age at implantation, preoperative communication mode, and educational support influenced the final communication mode. In children with additional disabilities, the level of academic achievement and employment status varied. CONCLUSIONS Despite significant differences in the grade failure rate between the children with cochlear implants and the general population, the participants in the present study ultimately achieved educational and employment levels similar to those of their normal-hearing peers. To minimize these delays and improve academic success in mainstream education, early oral education and early cochlear implantation are important.

Impact of early cochlear implantation on the linguistic development of the deaf child.

Objective: The purpose of this study was to examine prognostic factors of cochlear implantation and to evaluate the impact of early implantation on linguistic development in deaf children. Study Design: Retrospective study. Setting: Tertiary referral center. Patients: Seventy-four prelingually deafened children implanted before the age of 5 years. Intervention: Annual follow-up after cochlear implantation. Main Outcome Measures: Speech perception, intelligibility, and expressive and receptive language scores from age 3 to 8 years were globally compared between 4 subgroups of children. Significant differences were further explored by intergroup comparisons. Stepwise logistic linear regression was performed using the following variables: age at implantation, duration of cochlear implant (CI) use, preoperative hearing levels, age of hearing aid (HA) fitting, and age at time of the evaluation. Preoperative data were not available. Results: Between group comparisons displayed significant differences according to age at implantation. Multivariate analysis demonstrated the positive impact of early implantation on receptive language. Moreover, duration of CI use and preoperative hearing levels were statistically correlated with performance on speech perception, intelligibility, and expressive and receptive language. Age of HA fitting was associated with speech intelligibility. Conclusion: Age at implantation, duration of CI, preoperative hearing levels, and age of HA fitting may each be useful in predicting linguistic success after cochlear implantation. Other factors such as preoperative linguistic development may also influence postoperative outcomes, but the lack of tests suitable for use with very young children makes such a hypothesis difficult to confirm.

A retrospective Multicenter Study comparing Speech Perception outcomes for Bilateral Implantation and Bimodal rehabilitation

Objectives: To compare speech perception outcomes between bilateral implantation (cochlear implants [CIs]) and bimodal rehabilitation (one CI on one side plus one hearing aid [HA] on the other side) and to explore the clinical factors that may cause asymmetric performances in speech intelligibility between the two ears in case of bilateral implantation. Design: Retrospective data from 2247 patients implanted since 2003 in 15 international centers were collected. Intelligibility scores, measured in quiet and in noise, were converted into percentile ranks to remove differences between centers. The influence of the listening mode among three independent groups, one CI alone (n = 1572), bimodal listening (CI/HA, n = 589), and bilateral CIs (CI/CI, n = 86), was compared in an analysis taking into account the influence of other factors such as duration of profound hearing loss, age, etiology, and duration of CI experience. No within-subject comparison (i.e., monitoring outcome modifications in CI/HA subjects becoming CI/CI) was possible from this dataset. Further analyses were conducted on the CI/CI subgroup to investigate a number of factors, such as implantation side, duration of hearing loss, amount of residual hearing, and use of HAs that may explain asymmetric performances of this subgroup. Results: Intelligibility ranked scores in quiet and in noise were significantly greater with both CI/CI and CI/HA than with a CI-alone group, and improvement with CI/CI (+11% and +16% in quiet and in noise, respectively) was significantly better than with CI/HA (+6% and +9% in quiet and in noise, respectively). From the CI/HA group, only subjects with ranked preoperative aided speech scores >60% performed as well as CI/CI participants. Furthermore, CI/CI subjects displayed significantly lower preoperative aided speech scores on average compared with that displayed by CI/HA subjects. Routine clinical data available from the present database did not explain the asymmetrical results of bilateral implantation. Conclusions: This retrospective study, based on basic speech audiometry (no lateralization cues), indicates that, on average, a second CI is likely to provide slightly better postoperative speech outcome than an additional HA for people with very low preoperative performance. These results may be taken into consideration to refine surgical indications for CIs.

Outcomes and complications of direct end-to-side facial-hypoglossal nerve anastomosis according to the modified May technique

OBJECT The aim of this study was to address the efficiency and safety of direct end-to-side facial-hypoglossal nerve anastomosis for facial palsy rehabilitation. METHODS The authors conducted a retrospective study of 12 consecutive procedures performed between December 2000 and February 2006. Facial palsies were caused by the surgical removal of tumors in the brainstem, cerebellopontine angle, or mastoid process. Direct end-to-side facial-hypoglossal anastomosis was performed in each case. Facial function (evaluated using the overall percentage of facial function and House-Brackmann scale grades), as well as tongue trophicity and mobility, were assessed at 6, 12, and 24 months after surgery. Postoperative early and late complications were systematically reviewed. RESULTS The mean delay between tumoral and reparative surgery was 15.9 +/- 4 months (median 11 months). Preoperatively, the mean percentage facial function score was 11.6 +/- 1.7% (45% of patients with House-Brackmann Grade 5 facial palsy and 55% of patients with House-Brackmann Grade 6). Mean facial function scores increased to 19.3, 32.2, and 43.8% at 6, 12, and 24 months after surgery, respectively. Twenty-four months after surgery, 50% of cases had House-Brackmann Grade 3 facial palsy and 50% had Grade 4. A significantly better recovery at 24 months was observed postoperatively for neural lesions occurring in the mastoid or the brainstem compared with those in the cerebellopontine angle. Tongue hemiparesis was observed in 5 patients (41.7%), 2 of whom had tongue hypotrophy (16.7%). No patient complained of swallowing or speech disturbance. Facial synkinesis was noted in 1 patient (8.3%). CONCLUSIONS Facial recovery after direct end-to-side facial-hypoglossal nerve anastomosis is similar to results observed with end-to-end or end-to-side facial-hypoglossal nerve anastomosis with an interpositional graft. Tongue hypotrophy and palsy were observed in a small number of cases. This procedure allows one to minimize, although not fully prevent, facial synkinesis. The site of the neural lesion appears to be an important factor in the prognosis of recovery.

Molecular and Cellular Mechanisms of Loss of Residual Hearing after Cochlear Implantation

Objectives: We describe the various molecular and cellular pathways that lead to early and delayed loss of residual hearing after cochlear implantation. Methods: We performed a systematic review using the Medline database with the key words cochlear implant, residual hearing, inflammation, apoptosis, and necrosis. Results: The mechanisms underlying the loss of residual hearing after cochlear implantation are multiple. Early hearing loss may be provoked by the surgical access to the inner ear spaces and by trauma caused by insertion of the electrode array. After the initial trauma, an acute inflammatory response promotes elevated levels of cytokines and reactive oxygen species, which in turn promote sensory cell loss by apoptosis, necrosis, and necrosis-like programmed cell death. Treatments that counteract such an inflammatory reaction, production of reactive oxygen species, and apoptosis are effective at preventing hair cell degeneration. However, delayed hearing loss appears to be a consequence of chronic inflammation with development of fibrotic tissue. The mechanisms that lead to fibrosis are poorly understood, and standard anti-inflammatory drugs are insufficient for preventing its development. Conclusions: Cochlear implantation is followed by an inflammatory response involving several pathways that lead to either short-term or long-term sensory hair cell degeneration. Future studies should focus on revealing the precise molecular mechanisms induced by cochlear implantation to allow the discovery of new targets for the effective prevention and treatment of loss of residual hearing.