Acquired cholesteatoma after cochlear implants: case series and literature review.

PURPOSE: To assess the prevalence and management of acquired cholesteatoma after cochlear implantation in pediatric and adult patients. METHODS: Retrospective case review of pediatric and adult cochlear implants (CI) followed at a tertiary referral center and literature review of acquired cholesteatoma after CI surgery, to identify its prevalence, cause, and treatment. RESULTS: Nine pediatric CIs were diagnosed with cholesteatoma in seven patients after 6.4 ± 4 years from CI surgery, and two adults after 11.3 and 21.7 years from CI surgery. Thirty-four pediatric cases and 26 adult cases are described in the literature. Cholesteatoma has a prevalence of 0.54% in pediatric CIs, and 1.79% in adult CIs (case series and literature). Adult cases were diagnosed significantly later compared to pediatric cases (Mann-Whitney test, p = 0.0460). Three pediatric cholesteatomas were treated with conservative surgery and preservation of the CI; they all developed recurrent disease. The remaining pediatric cases underwent subtotal petrosectomy with simultaneous CI explantation and staged reimplantation. Only one case recurred. The adult cases underwent simultaneous subtotal petrosectomy, explantation, and reimplantation. Similarly, 33.3% of cases treated with conservative/reconstructive surgery in the literature required revision surgery or conversion to subtotal petrosectomy against 6.2% of subtotal petrosectomies in the literature. CONCLUSIONS: Cholesteatoma after CI is a rare and late-onset complication of CIs. It is more prevalent in the adult CI population, although it affects children significantly earlier. The treatment of choice is subtotal petrosectomy and CI explantation with simultaneous or staged reimplantation.

A Multicenter Clinical Evaluation of Data Logging in Cochlear Implant Recipients Using Automated Scene Classification Technologies.

Currently, there are no studies assessing everyday use of cochlear implant (CI) processors by recipients by means of objective tools. The Nucleus 6 sound processor features a data logging system capable of real-time recording of CI use in different acoustic environments and under various categories of loudness levels. In this study, we report data logged for the different scenes and different loudness levels of 1,366 CI patients, as recorded by SCAN. Monitoring device use in cochlear implant recipients of all ages provides important information about the listening conditions encountered in recipients' daily lives that may support counseling and assist in the further management of their device settings. The findings for this large cohort of active CI users confirm differences between age groups concerning device use and exposure to various noise environments, especially between the youngest and oldest age groups, while similar levels of loudness were observed.

A New Treatment Option in Incomplete Partition Type III: The Varese Bone-Air Stimulation (B.A.S.).

The incomplete partition type III is a severe cochlear malformation present in X-linked deafness. It is a rare, non-syndromic cause of severe to profound mixed hearing loss, often progressive. The complete absence of bony modiolus and the wide communication between the cochlea and the internal auditory canal make cochlear implantation challenging, with still no consensus on the management of these patients. To the best of our knowledge, no results have ever been published in the literature on the treatment of these patients with hybrid stimulation (bone and air). We present three cases in which this hybrid stimulation gave better audiological results then air stimulation alone. A literature review on audiological results of the current treatment options in children affected by IPIII malformation was conducted independently by two researchers. Ethical considerations on the treatment of these patients were conducted by the Bioethics department of the University of Insubria. In two of the patients, the bone-air stimulation, associated with prosthetic-cognitive rehabilitation, meant that surgery was avoided, obtaining similar communication performances of those present in the literature. We believe that, when the bone threshold appears partially preserved, a stimulation through the bone or hybrid modality, such as the Varese B.A.S. stimulation, should be attempted.

Bulging of the Oval Window in Common Cavity Deformity: A Possible Predictor of Meningitis.

OBJECTIVE: To investigate the prevalence-rate of oval window bulging in the common cavity and its association with bacterial meningitis. PATIENTS: CT and clinical files of 29 children with preliminary diagnosis of common cavity deformity were collected from 13 Italian centers. INTERVENTION: A retrospective case review study was conducted with a centralized evaluation of the temporal bone CT imaging was performed at Azienda Ospedale - Università Padova, Padova, Italy. MAIN OUTCOME MEASURE: Diagnosis of common cavity was reviewed; in addition, a fluid protrusion into the middle-ear cavity through the oval window at CT imaging was considered as oval window bulging. Its association with the history of bacterial meningitis was investigated. RESULTS: Common cavity deformity was confirmed in 14/29 children (mean-age 11.4 ±â€Š3.8; age-range 5-20; nine females) referred with this diagnosis. In 7/14 patients, the common cavity deformity was bilateral (i.e., 21 common cavities). Oval window bulging was found in 3/19 common cavities (concomitant middle-ear effusive otitis hampered the evaluation in two cases), while the internal acoustic meatus fundus was defective in 10/21 cases. History of bacterial meningitis was found in three children (21%) and two of them had oval window bulging at CT. In the case unrelated to oval window bulging, meningitis occurred late at the age of 12 during acute otitis contralateral to common cavity deformity (ipsilaterally to incomplete partition type 1). CONCLUSION: Patients harboring common cavity deformity have a high risk of meningitis in their first years of life. Oval window bulging seems to be associated with a higher risk of meningitis. This information might be important for appropriate surgical planning.

The p.Gly130Val mutation in the GJB2 gene: A familiar case of autosomal dominant non-syndromic hearing loss.

Several forms of sensorineural hearing loss (SNHL) have been imputated to connexins mutations and prevalently to connexin 26 (Cx26), codified by the GJB2 gene (gap junction protein, beta 2). Here, we report the first familiar case (heterozygous p. G130V mutation) of non-syndromic (without any dermatological manifestation) dominant profound SNHL. Proband was a 6-years-old male with post-lingual bilateral profound SNHL, clinically identified at the age of 3 with diagnosis of severe SNHL. We confirm that the p. G130V variant of the GJB2 gene is causative of autosomal dominant form of SNHL, although it is not always associated with the presence of skin diseases.

Evaluation of speech reception threshold in noise in young Cochlear™ Nucleus® system 6 implant recipients using two different digital remote microphone technologies and a speech enhancement sound processing algorithm.

OBJECTIVE: Children affected by hearing loss can experience difficulties in challenging and noisy environments even when deafness is corrected by Cochlear implant (CI) devices. These patients have a selective attention deficit in multiple listening conditions. At present, the most effective ways to improve the performance of speech recognition in noise consists of providing CI processors with noise reduction algorithms and of providing patients with bilateral CIs. The aim of this study was to compare speech performances in noise, across increasing noise levels, in CI recipients using two kinds of wireless remote-microphone radio systems that use digital radio frequency transmission: the Roger Inspiro accessory and the Cochlear Wireless Mini Microphone accessory. METHODS: Eleven Nucleus Cochlear CP910 CI young user subjects were studied. The signal/noise ratio, at a speech reception threshold (SRT) value of 50%, was measured in different conditions for each patient: with CI only, with the Roger or with the MiniMic accessory. The effect of the application of the SNR-noise reduction algorithm in each of these conditions was also assessed. The tests were performed with the subject positioned in front of the main speaker, at a distance of 2.5 m. Another two speakers were positioned at 3.50 m. The main speaker at 65 dB issued disyllabic words. Babble noise signal was delivered through the other speakers, with variable intensity. RESULTS: The use of both wireless remote microphones improved the SRT results. Both systems improved gain of speech performances. The gain was higher with the Mini Mic system (SRT = -4.76) than the Roger system (SRT = -3.01). The addition of the NR algorithm did not statistically further improve the results. CONCLUSION: There is significant improvement in speech recognition results with both wireless digital remote microphone accessories, in particular with the Mini Mic system when used with the CP910 processor. The use of a remote microphone accessory surpasses the benefit of application of NR algorithm.

Vestibular function after cochlear implant surgery.

OBJECTIVE: The aim of this study was to analyze vestibular function in cochlear implant (CI) patients for iatrogenic damage to vestibular function. METHODS: Prospective clinical study. Tertiary care audiological center. Twenty-five subjects receiving surgery for cochlear implantation during 2012 and 2013 were analyzed. Both vestibulo-ocular and vestibulo-spinal responses before and 2 months after CI surgery were evaluated using several tests: analysis of spontaneous nystagmus, head shaking test (HST), and head impulse test recorded by videooculography; caloric stimulation at 44° and 30° in both ears; cervical evoked myogenic potentials and static stabilometry. Residual cochlear function was tested by air-conduction pure-tone audiometry. RESULTS: Our tests showed damage to vestibular receptors after CI surgery in 12% of the patients; in particular, horizontal semicircular canal function and saccular function had lower responses after surgery. Audiometric results showed poorer thresholds after CI surgery. The static stabilometry results indicate good vestibulo-spinal responses and patients did not report disequilibrium nor postural deficit. CONCLUSION: All data suggest an efficient vestibular compensation mechanism in CI patients.

Assessment of directionality performances: comparison between Freedom and CP810 sound processors.

OBJECTIVE: To compare speech recognition in noise for the Nucleus Freedom and CP810 sound processors using different directional settings among those available in the SmartSound portfolio. STUDY DESIGN: Single-subject, repeated measures study. SETTING: Tertiary care referral center. SUBJECTS AND METHODS: Thirty-one monoaurally and binaurally implanted subjects (24 children and 7 adults) were enrolled. They were all experienced Nucleus Freedom sound processor users and achieved a 100% open set word recognition score in quiet listening conditions. Each patient was fitted with the Freedom and the CP810 processor. The program setting incorporated Adaptive Dynamic Range Optimization (ADRO) and adopted the directional algorithm BEAM (both devices) and ZOOM (only on CP810). Speech reception threshold (SRT) was assessed in a free-field layout, with disyllabic word list and interfering multilevel babble noise in the 3 different pre-processing configurations. RESULTS: On average, CP810 improved significantly patients' SRTs as compared to Freedom SP after 1 hour of use. Instead, no significant difference was observed in patients' SRT between the BEAM and the ZOOM algorithm fitted in the CP810 processor. CONCLUSION: The results suggest that hardware developments achieved in the design of CP810 allow an immediate and relevant directional advantage as compared to the previous-generation Freedom device.