Rehabilitation of Severe to Profound Sensorineural Hearing Loss in Adults: Audiological Outcomes.

The aim of this article is to describe the audiological patterns of 71 adult patients presenting severe to profound sensorineural hearing loss, who were rehabilitated by cochlear implants (CIs) and hearing aids. This is a retrospective study in a university setting, where the clinical records of 71 adult patients were reviewed and processed. Speech intelligibility was evaluated at one aided ear (CI) or at both aided ears (double CI or a combination of CI and hearing aid [HA]). Patients with a bilateral CI or with a bimodal hearing setup (CI and HA) performed better than those with a single CI; data from the phonetic matrices test showed that there was a statistically significant difference among patients aided by a single CI versus binaural setup (double CI or CI + HA). In particular, patients aided by a bilateral CI, or by a CI and HA, showed an improvement in the functional results of the speech tests, compared to patients using a single CI. Binaural hearing (either with a bilateral CI or bimodal) allows an improvement in the functional results at the speech tests, compared to the use of a CI only.

Outcome of cochlear implantation in children with congenital Cytomegalovirus infection: A retrospective case control study.

INTRODUCTION: To date, cCMV represents the most frequent non-genetic congenital cause of permanent sensorineural hearing loss (SNHL) in childhood and the leading infectious cause of developmental and neurologic disabilities. The aim of this paper is to describe the outcome of cochlear implantation in children affected by severe-to-profound sensorineural hearing loss, due to a symptomatic or asymptomatic cCMV infection, particularly comparing their performance results to that of matched mutated Connexin 26 (Cx26) implanted patients. METHODS: Retrospective case control study. The clinical data of symptomatic cCMV and asymptomatic cCMV patients were collected and compared to those of Cx26 patients matched for age and pre-CI (cochlear implant) linguistic category; all subjects were affected by bilateral severe-to-profound SNHL and were treated by CI and speech therapy rehabilitation. The Speech Perception Category, the language stage and the linguistic level scores, at 6 months, 1 year, and 3-4 years after CI of the three groups (symptomatic cCMV, asymptomatic cCMV and Cx26 mutation) were collected and compared. RESULTS: Statistical analysis did not show any significant difference in pre-CI perception category and linguistic level among the three groups; the symptomatic cCMV group showed a statistically worse performance of the language stage over time (p = 0.017). CONCLUSIONS: Our data support that children affected by cCMV have improved language abilities over time, although the symptomatic cCMV group achieved a lower language stage 3-4 years after CI compared to the asymptomatic cCMV and Cx26 mutation groups. Nonetheless, to date, CI supported by speech therapy can be considered an effective intervention for children affected by cCMV-related severe-to-profound hearing loss.

Cochlear Implants in Subjects Over Age 65: Quality of Life and Audiological Outcomes.

BACKGROUND Cochlear implants (CIs) have been recognized as a safe and effective means for profound hearing loss rehabilitation in children and adults and recently their use has been extended to subjects over 65 years of age. The aim of this paper was to assess indices related to changes in the quality of life (QoL) in elderly CI recipients. MATERIAL AND METHODS A case-control paradigm was used to assess the effects of CIs on the QoL. Forty-two subjects were assigned to the Case group and 15 subjects to the Control group. All 57 subjects were affected by profound hearing loss and had received a CI. Audiological data were collected from both groups at: (i) 1 month pre-implantation [T1]; (ii) 1 day pre- implantation [T2]; (iii) 30 days post-implantation, with CI used in free field [T3]; and (iv) 12 months post-implantation, with CI used in a free field [T4]. The QoL was assessed via a Glasgow Benefit Inventory (GBI) questionnaire, adapted to otolaryngology. To compare subjects across different ages with varying degrees of speech development, a perception parameter was used from the Speech Perception Categories test developed by Geers and Moog. RESULTS Hearing performance was considerably improved after CI. In relation to the hearing performance at time T1, statistically significant threshold gains were observed in both groups in the T3 and T4 observation windows. At time T4, a threshold gain of 70 dB HL in the Case group and a gain of 84 dB HL in the Control group were observed. With speech therapy rehabilitation, a perception level of 6 was reached by 80.0% of patients in the Case group and by 100% of patients in the Control group. In terms of QoL, both groups showed improved post-CI scores. Statistical differences were observed between the 2 groups, with the Control group outperforming the Case group in all but the social section. CONCLUSIONS Despite age-related changes in auditory system and prolonged hearing deprivation, CIs offer audiological and QoL benefits in the elderly.

Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies.

BACKGROUND: Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. METHODS: The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. RESULTS: Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). CONCLUSIONS: Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies represent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity and stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent.

Remote programming of MED-EL cochlear implants: users' and professionals' evaluation of the remote programming experience.

CONCLUSION: Remote programming is safe and is well received by health-care professionals and cochlear implant (CI) users. It can be adopted into clinic routine as an alternative to face-to-face programming. OBJECTIVES: Telemedicine allows a patient to be treated anywhere in the world. Although it is a growing field, little research has been published on its application to CI programming. We examined hearing professionals' and CI users' subjective reactions to the remote programming experience, including the quality of the programming and the use of the relevant technology. METHODS: Remote CI programming was performed in Italy, Sweden, and Russia. Programming sessions had three participants: a CI user, a local host, and a remote expert. After the session, each CI user, local host, and remote expert each completed a questionnaire on their experience. RESULTS: In all, 33 remote programming sessions were carried out, resulting in 99 completed questionnaires. The overwhelming majority of study participants responded positively to all aspects of remote programming. CI users were satisfied with the results in 96.9% of the programming sessions; 100% of participants would use remote programming again. Although technical problems were encountered, they did not cause the sessions to be considerably longer than face-to-face sessions.

Endocochlear inflammation in cochlear implant users: case report and literature review.

OBJECTIVES: Cochlear implantation is a relatively safe procedure with a low complication rate. The overall rate of complications among cochlear implant patients ranges from 6% to 20%. Major complications are those that are life-threatening or require surgery, whereas minor complications are those that can be medically treated. Nonetheless, certain complications, even if highly rare, may require specific investigations and treatments. Among these rare complications are those with endocochlear involvement, such as cochleitis or labyrinthitis, with fibrosis or ossification that could lead to explantation. The aims of the present study were to report a particular case of post-operative cochleitis and to review the rate of complications after cochlear implantation, emphasising those conditions with proven endocochlear involvement. METHODS: We refer to the case of an eight-year-old Italian boy affected by the sudden onset of headache, ipsilateral otalgia and facial paresis, who presented to our clinic for inexplicable worsening of the performance of his implant and his residual hearing, six years after surgery. A complete investigation including (clinical history, routine, autoimmune and serological blood tests, electrophysiological measurements from the cochlear implant and neuroimaging) was performed and is herein described. Additionally, a comprehensive review of the literature was conducted using internet search engines; 274 papers were selected, 88 of which were best suited to our purposes. RESULTS: In our case, the progression of the symptoms and the performance decrement required explantation, followed by a complete recovery. Reviewing the literature revealed only three reports concerning cases of proven endocochlear phlogosis that required revision surgery. Wound swelling/infection and vertigo remain the two most common complications of cochlear implantation. Failure of the device is the third most frequent complication (10.06% of all complications and 1.53% of cochlear implantations). Other rare conditions (such as granulating labyrinthitis with cochlear fibrosis, ossification and erosion, silicone allergy and the formation of a biofilm around the internal device) are possible and unpredictable. Although rare (approximately 1%), such cases may require explantation. CONCLUSIONS: Despite efforts by both surgeons and manufacturers, device-related and surgical complications still occur. These and other rare conditions demand specific management, and their frequency may be underestimated. Further studies are needed to assess more realistic rates of complications and devise more efficient strategies for early diagnosis and treatment.

Multicentre investigation on electrically evoked compound action potential and stapedius reflex: how do these objective measures relate to implant programming parameters?

OBJECTIVES: The aims of this study were to collect data on electrically evoked compound action potential (eCAP) and electrically evoked stapedius reflex thresholds (eSRT) in HiResolution(TM) cochlear implant (CI) users, and to explore the relationships between these objective measures and behavioural measures of comfort levels (M-levels). METHODS: A prospective study on newly implanted subjects was designed. The eCAP was measured intra-operatively and at first fitting through neural response imaging (NRI), using the SoundWave(TM) fitting software. The eSRT was measured intra-operatively by visual monitoring of the stapes, using both single-electrode stimulation and speech bursts (four electrodes stimulated at the same time). Measures of M-levels were performed according to standard clinical practice and collected at first fitting, 3 and 6 months of CI use. RESULTS: One hundred seventeen subjects from 14 centres, all implanted unilaterally with a HiResolution CII Bionic Ear(®) or HiRes 90K(®), were included in the study. Speech burst stimulation elicited a significantly higher eSRT success rate than single-electrode stimulation, 84 vs. 64% respectively. The NRI success rate was 81% intra-operatively, significantly increasing to 96% after 6 months. Fitting guidelines were defined on the basis of a single NRI measurement. Correlations, analysis of variance, and multiple regression analysis were applied to generate a predictive model for the M-levels. DISCUSSION: Useful insights were produced into the behaviour of objective measures according to time, electrode location, and fitting parameters. They may usefully assist in programming the CI when no reliable feedback is obtained through standard behavioural procedures.