Auditory performance in a group of elderly patients after cochlear implantation.

PURPOSE: The retrospective case review investigated the effect of cochlear implantation in subjects aged 61 years or older with respect to their auditory performance. The study also analysed the effect of age on the performance, and it drew a comparison between the outcomes of older and younger adults. METHODS: The outcome in a group of 446 patients aged 61 to 89 years at the time of unilateral cochlear implantation was compared with the outcome in a group of 110 patients aged 17 to 42 years. Auditory performance was measured with open-set monosyllabic word testing and sentences in quiet and in noise. RESULTS: In the monosyllabic word recognition test, the group of older adults performed significantly better after cochlear implantation compared with their scores prior to implantation (p < 0.001; r = 0.59). Their auditory performance correlated negatively with their age. However, the correlation was of small strength. Significant differences in auditory performance were detected between sexagenarians and octogenarians (p < 0.001; r = 0.27). Additionally, a statistically significant difference was revealed between the groups of older and younger adults in the monosyllabic word test (p = 0.001; r = 0.15). CONCLUSION: Elderly cochlear implant recipients can benefit significantly from cochlear implantation. Although higher age correlates negatively with auditory performance, its influence in the presented sample is small.

First results of electrode reimplantation and its hypothetical dependence from artificial brain maturation.

BACKGROUND: After introducing the first Cochlear Implants also in children theses are grown with electrical intracochlear stimulation and subsequent auditory cortical development. Over the meantime the positioning of the electrode was changed orientated on the development of electrode design, ability to insert atraumatic and on the widening of the indications towards highfrequency deafness. METHODS: In this pilot study we analysed five prelingually deafened patients implanted as child in the late 90's and had a reimplantation 2016 or later. We compared CT and DVT (cone beam CT) scans of the temporal bone and measured the insertion angle, the cochlear coverage, the total length of the electrode in the cochlea and the distance of the first active electrode to the round window. Moreover, we compared their speech understanding before and after reimplantation. RESULTS: The results show a lowering in the insertion angle, the cochlear coverage, the total length of the electrode in the cochlea, in the distance of the first active electrode to the round window and in the speech understanding after reimplantation. CONCLUSION: These results show a difference in the depth of insertion while the speech understanding is not significantly improving in this group-although the technology is advanced. The influence of auditory maturation with CI in these patients will be discussed.

Does severity of cerebral MRI lesions in congenital CMV infection correlates with the outcome of cochlear implantation?

The objective is to investigate whether there is a correlation between the severity of typical brain lesions in congenital cytomegalovirus (cCMV) infection and cochlear implant (CI) outcome. The design of the study is a retrospective single-institutional chart review (2005-2015), performed in a tertiary academic referral center. 23 children with typical signs of cCMV infection on cerebral magnetic resonance imaging (MRI) and bilateral severe-to-profound sensorineural hearing loss were retrospectively evaluated. They were graded in three groups according to the severity of brain involvement. The average implantation age of the first CI is 1.8 years (range 0.6-5.8). Five patients were implanted unilaterally, 18 bilaterally. The average follow-up time after implantation was 3.3 years (range 0.6-6.9). Hearing performance was assessed using the Categories of Auditory Performance (CAP), and speech development was assessed using Speech Intelligibility Rating (SIR). The outcome in each group showed great variation. The majority of children achieved moderate-to-good auditory and speech rehabilitation. The children with severe MRI changes had comparatively better auditory than speech scores. There were children with good auditory performance (CAP ≥6) both in grades II and III, while poor performers (CAP ≤3) were encountered in each group. The severity of brain lesions on its own does not directly correlate with the outcome of cochlear implantation. Despite good retrospective diagnostic evidence of cCMV infection through MRI patterns, this has no predictive role for future hearing and speech rehabilitation.

Cochlear implantation in patients with definite Meniere's disease.

The exact pathomechanism of deafening in Meniere's disease (MD) is still unknown; intoxication of hair cells and neural damage from endolymphatic hydrops is discussed. In the literature, there are only a few reports on hearing outcome of MD patients after treatment with cochlear implantation (CI) whereby especially the comparison of MD vs. non-MD patients with CI differs. In this retrospective study, results in speech understanding [Freiburger Einsilber (FES65) and Hochmair-Schulz-Moser test in quiet (HSM) and in noise (HSM + 10 dB)] of 27 implanted MD patients were collected and compared to a matched standard CI cohort. Alternative diagnoses were excluded as far as possible by re-analyzing neuroradiologic imaging. After first fitting, MD patients showed significantly better results in FES and HSM testing compared to controls. At 1-year refitting, this effect could not be seen anymore. To conclude, cochlear implantation is a safe and effective treatment for deafness in MD patients. Results in speech understanding are at least equal compared to general CI recipients. To the best of our knowledge, this retrospective study examined the largest collective of CI users deafened by MD so far.

Mental health problems in adolescents with cochlear implants: peer problems persist after controlling for additional handicaps.

The aims of the present multi-center study were to investigate the extent of mental health problems in adolescents with a hearing loss and cochlear implants (CIs) in comparison to normal hearing (NH) peers and to investigate possible relations between the extent of mental health problems of young CI users and hearing variables, such as age at implantation, or functional gain of CI. The survey included 140 adolescents with CI (mean age = 14.7, SD = 1.5 years) and 140 NH adolescents (mean age = 14.8, SD = 1.4 years), their parents and teachers. Participants were matched by age, gender and social background. Within the CI group, 35 adolescents were identified as "risk cases" due to possible and manifest additional handicaps, and 11 adolescents were non-classifiable. Mental health problems were assessed with the Strengths and Difficulties Questionnaire (SDQ) in the versions "Self," "Parent," and "Teacher." The CI group showed significantly more "Peer Problems" than the NH group. When the CI group was split into a "risk-group" (35 "risk cases" and 11 non-classifiable persons) and a "non-risk group" (n = 94), increased peer problems were perceived in both CI subgroups by adolescents themselves. However, no further differences between the CI non-risk group and the NH group were observed in any rater. The CI risk-group showed significantly more hyperactivity compared to the NH group and more hyperactivity and conduct problems compared to the CI non-risk group. Cluster analyses confirmed that there were significantly more adolescents with high problems in the CI risk-group compared to the CI non-risk group and the NH group. Adolescents with CI, who were able to understand speech in noise had significantly less difficulties compared to constricted CI users. Parents, teachers, and clinicians should be aware that CI users with additionally special needs may have mental health problems. However, peer problems were also experienced by CI adolescents without additional handicaps.

Schooling Relates to Mental Health Problems in Adolescents with Cochlear Implants-Mediation by Hearing and Family Variables.

Aim of this multicenter study was to investigate whether schooling relates to mental health problems of adolescents with cochlear implants (CI) and how this relationship is mediated by hearing and family variables. One hundred and forty secondary school students with CI (mean age = 14.7 years, SD = 1.5), their hearing parents and teachers completed the Strengths and Difficulties Questionnaire (SDQ). Additional audiological tests (speech comprehension tests in quiet and noise) were performed. Students of special schools for hearing impaired persons (SSHIs) showed significantly more conduct problems (p < 0.05) and a significantly higher total difficulty score (TDS) (p < 0.05) compared to students of mainstream schools. Mental health problems did not differ between SSHI students with sign language education and SSHI students with oral education. Late implanted students and those with indication for additional handicaps were equally distributed among mainstream schools and SSHIs. However, students in SSHIs were more restricted to understand speech in noise, had a lower social background and were more likely to come from single-parent families. These factors were found to be partial mediators of the differences in mental health problems between the two school types. However, no variable could explain comprehensively, why students of SSHIs have more mental health problems than mainstream pupils.

Aplasia of the cochlea: radiologic assessment and options for hearing rehabilitation.

OBJECTIVE: Hearing rehabilitation of patients with severe inner ear malformations remains controversial. Our objective was to describe the radiologic findings of aplasia of the cochlea (AC) and evaluate the existing therapeutic options in such patients. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Pediatric and adult patients with AC evaluated at our institution from 1995 to 2010. INTERVENTIONS: The precise radiologic findings were identified using high-resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) scans of the inner ear. In cases of auditory implantation on the AC side, the achieved outcome was recorded using categories of auditory performance (CAP). Related surgical aspects were analyzed. RESULTS: Twenty-three patients (28 ears) with AC were found. In 5 patients AC was bilateral. The remaining unilateral cases had contralateral normal ears (2 patients), cochlea hypoplasia (5 patients), common cavity (6 patients), incomplete partition Type I (4 patients), and atresia of the internal auditory canal (1 patient). Four patients (3 bilateral, 1 unilateral ACs) were treated with cochlear implants in ears with AC, and 1 patient underwent auditory brainstem implantation. All implanted patients achieved speech perception with limited vocabulary (CAP scores between 4 and 5). CONCLUSION: AC is defined as the total absence of the cochlea, with a present, although malformed, vestibule. Although a distinct auditory nerve was not seen in these cases of AC, results following cochlear implantation suggest functional cochlear nerve fibers in the remaining dysplastic inner ear structures. In selected cases, cochlear implantation may be a reasonable option for the habilitation of deafness associated with AC.

Speech comprehension in children and adolescents after sequential bilateral cochlear implantation with long interimplant interval.

OBJECTIVE: Identify likely predictors for the outcome after contralateral cochlear implantation with a long interimplant delay. STUDY DESIGN: Retrospective case reviews. SETTING: Outpatient cochlear implant (CI) center. PATIENTS: Seventy-three children and adolescents who underwent sequential bilateral cochlear implantation with an interval between both implantations of 5 years or longer. The mean age of the patients at the first and second cochlear implantations was 2.72 ± 1.52 and 11.57 ± 2.9 years, respectively. The mean duration of experience with both implants was 4.01 ± 1.57 years. INTERVENTION: Rehabilitative. MAIN OUTCOME MEASURES: All 73 patients underwent periodic speech perception testing in quiet and noise. The most recent unilateral data for each side were statistically analyzed. The speech test results were evaluated by the age at first implantation, the interval between both implantations, the duration of hearing aid use in the second side, and the duration of the bilateral CI use. RESULTS: A statistically significant difference for speech test results was obtained between the early-implanted ears and the late-implanted ears for all children. These results were dependent on the interimplant interval. All age groups demonstrated significant differences (p > 0.05) for the second side between the speech test results and the interval between both cochlear implantations. In addition, statistically significant differences influenced by the duration of hearing aid use were found for speech test results for the second side. Experience was also a factor for the second CI, yielding significantly higher speech test scores with longer use. CONCLUSION: The development of hearing abilities in a second-implanted side depends on the interimplant interval, the hearing aid use, and the duration of the second CI use.

Radiological diagnosis of incomplete partition type I versus type II: significance for cochlear implantation.

OBJECTIVES: To investigate an extended cohort of patients with incomplete partition (IP) and examine the adequacy of the existing classifications based on radiological criteria and on their implications for cochlear implantation. METHODS: Patients with IP admitted to a tertiary referral centre during the period 2000-2010 were retrospectively examined. The subjects were initially classified into IP-I, IP-II and atypical cases. For cochlear implant recipients relevant aspects were analysed. RESULTS: Eighty-three ears (49 patients) with IP were found, 19 with IP-I, 54 with IP-II and 10 atypical. Thirty-three patients received a cochlear implant (11 with IP-I, 19 with IP-II and three atypical) achieving heterogeneous though mostly promising results. Cerebrospinal fluid gushing was the commonest surgical complication, particularly in cases of IP-I. In general, patients with IP-II performed better than those with IP-I. CONCLUSIONS: Cochlear implantation promises adequate hearing rehabilitation for most patients with IP. As we move from IP-I to IP-II better results and lower risk for gushing shall be expected. Based on radiological findings we suggest a modified classification into IP-I, atypical IP-I (with large vestibular aqueduct (LVA) and better partition), IP-II (Mondini deformity) and atypical IP-IIa (without LVA) and b (without LVA but with semicircular canal dysplasia). KEY POINTS: • Radiological (CT and MR) features are of crucial importance for cochlear implantation • Imaging can identify two types of incomplete cochlear partition and atypical cases • Detailed pre-operative radiological assessment can help predict complications and outcome • A more comprehensive radiological classification of these anomalies is proposed.

Outcomes and special considerations of cochlear implantation in waardenburg syndrome.

OBJECTIVES: The objective of this study was a state-of-the-art analysis of cochlear implantation in patients with Waardenburg syndrome (WS). PATIENTS: Twenty-five patients with WS treated with cochlear implants in our department from 1990 to 2010. INTERVENTIONS: The 25 patients with WS underwent 35 cochlear implantations. MAIN OUTCOME MEASURES: Hearing outcome was evaluated using HSM sentence test in 65 dB in quiet, Freiburg Monosyllabic Test, and categories of auditory performance for children and compared with that of a control group. Anatomic abnormalities of the inner ear were examined using magnetic resonance imaging and computed tomography of the temporal bones. RESULTS: The mean follow-up time was 8.3 years (range, 0.3-18.3 yr). The majority achieved favorable postimplantation performance with mean HSM scores of 75.3% (range, 22.6%-99%) and Freiburg Monosyllabic Test scores of 67.8% (range, 14%-95%). However, in 4 cases, the results were less satisfactory. The comparison with the control group did not reveal any statistical significance (p = 0.56). In 6 patients (24%), behavioral disorders caused temporary difficulties during the rehabilitation procedure. Except of isolated large vestibule in 1 patient, the radiological assessment of the 50 temporal bones did not reveal any temporal bone abnormalities. CONCLUSION: Most patients with WS performed well with cochlear implants. However, WS is related to behavioral disorders that may cause temporary rehabilitation difficulties. Finally, temporal bone malformations that could affect cochlear implantation are notcharacteristic of WS.

Long-term evaluation of Cochlear implantation in Cogan syndrome.

PURPOSE: The evaluation of long-term results of cochlear implantation in patients with typical Cogan syndrome. PROCEDURES: The medical records of approximately 3,000 patients who underwent cochlear implantation in a tertiary centre within the period 1992-2007 were retrospectively examined. RESULTS: Four cochlear implant recipients with Cogan syndrome (6 implantations) were identified. One cochlea was found to be partially obliterated; the electrode could, however, be completely inserted. Wound healing disorders and infections in a patient with systemic symptoms were the only postoperative complications. Hearing outcome was favorable, with average HSM and monosyllabic scores of 96.7 and 82.5%, respectively, in an average follow-up time of 9.25 years. CONCLUSIONS: Cochlear implantation is the appropriate hearing rehabilitation method in Cogan syndrome patients. Although the basic illness does not affect the long-term hearing outcome, skin-atrophy-related complications in cases with systemic symptoms may occur. Finally, the cochlear implant surgeon should be aware of the possible cochlea obliteration.