Cochlear Implantation: Concept, Results Outcomes and Quality of Life. / Cochlea-Implantation: Konzept, Therapieergebnisse und Lebensqualität.

Cochlear implant today are an essential method of auditory rehabilitation in patients with severe to profound hearing loss. Due to the rapid development of implant technology the results have been markedly improved. Today about 80 % of patients can use the telephone and children achieve near to normal hearing and speech development. In consequence, more patients are candidates for a cochlear implant today including those with high frequency deafness and single sided deafness. However, today only 60,000 out of 1 Million CI-candidates in Germany have been implanted so far. In future multi modal universal auditory implants will provide combined electric-mechanical stimulation to make best use of the residual auditory hearing and the electrical stimulation of the auditory nerve. They allow a continuous adaptation of the stimulation strategy onto the given functional status of haircells and auditory nerve fibers especially in cases of progressive hearing loss. Brain computer interfaces will allow the automated fitting and adaptation to the acoustic scene by optimizing the signal processing for best possible auditory performance. Binaural hearing systems will improve directional hearing and speech perception in noise. Advanced implants are composed of individualized electrodes by additive manufacturing which can be inserted atraumaticly by a computer and robot assisted surgery. After insertion they automatically adept to the anatomy of the individual cochlear. These advanced implants are composed with additional integrated biological components for the preservation of residual hearing and regeneration of neural elements to improve the electrode nerve interface. This will allow to increase the number of electrical contacts as a major step towards the bionic ear. This will allow overcoming the principal limits of today's cochlear implant technology. Advanced care models will allow an easy way for the patient towards hearing preservation cochlear implantation under local anesthesia using minimal invasive high precision cochlear implant surgery. These implant systems will become a personal communicator with improved connectivity. Remote care and self-fitting will empower the patient to optimize his own hearing.

Influence of single-sided deafness on the auditory capacity of the better ear.

BACKGROUND: Patients with single-sided deafness (SSD) are limited by their asymmetric hearing in various areas of everyday life. OBJECTIVE: The aim of this investigation was to perform an age-correlated comparison of the hearing threshold of the better ear of SSD patients with a normal-hearing (NH) reference cohort. In addition, the potential influence of etiology, duration of deafness, and cochlear implantation (CI) of the poorer ear on the peripheral hearing ability of the better ear was investigated. MATERIALS AND METHODS: In a multicenter study, the mean bone conduction hearing threshold of the better ear of 413 adult SSD patients was compared with that of an NH cohort drawn from ISO 7029:2017 for the frequencies 0.5, 1, 2, and 4 kHz. RESULTS: SSD patients showed significantly poorer hearing in the better ear compared to the age-correlated group of NH subjects. CI, duration of deafness, and etiology had no significant effect on the hearing ability of the better ear. CONCLUSION: The origin of the poorer hearing of the better-hearing ear of SSD patients compared to an age-correlated NH cohort is still unclear. It is most likely a combination of different anatomical, immunological, etiological, and microcirculatory causes, which lead to poorer hearing of the better-hearing ear in SSD patients.

[Influence of single-sided deafness on the auditory capacity of the better ear. German version]. / Einfluss einseitiger Taubheit auf das Hörvermögen des besseren Ohrs.

BACKGROUND: Patients with single-sided deafness (SSD) are limited by their asymmetric hearing in various areas of everyday life. OBJECTIVE: The aim of this investigation was to perform an age-correlated comparison of the hearing threshold of the better ear of SSD patients with a normal-hearing (NH) reference cohort. In addition, the potential influence of etiology, duration of deafness, and cochlear implantation (CI) of the poorer ear on the peripheral hearing ability of the better ear was investigated. MATERIALS AND METHODS: In a multicenter study, the mean bone conduction hearing threshold of the better ear of 413 adult SSD patients was compared with that of an NH cohort drawn from ISO 7029:2017 for the frequencies 0.5, 1, 2, and 4 kHz. RESULTS: SSD patients showed significantly poorer hearing in the better ear compared to the age-correlated group of NH subjects. CI, duration of deafness, and etiology had no significant effect on the hearing ability of the better ear. CONCLUSION: The origin of the poorer hearing of the better-hearing ear of SSD patients compared to an age-correlated NH cohort is still unclear. It is most likely a combination of different anatomical, immunological, etiological, and microcirculatory causes, which lead to poorer hearing of the better-hearing ear in SSD patients.

[Rehabilitation in children and adults : An overview]. / Rehabilitation bei Kindern und Erwachsenen : Ein Überblick.

BACKGROUND: The rehabilitation of patients with hearing loss includes technical, medical, and therapeutic education interventions. The success of cochlear implant (CI) treatment depends on joint coordination and execution of all measures. During the history of CI, expansion of the spectrum of indication criteria has led to evolution of auditory-verbal therapy, speech therapy, and auditory training. MATERIALS AND METHODS: This paper describes the current status of therapeutic knowledge and experience of hearing and speech therapeutic rehabilitation in children and adults. Current treatments and quality assurance measures are presented. RESULTS: Effective auditory-verbal rehabilitation of children and auditory training in adults depends on individual objectives. In addition to subjective patient reports, the success of CI fitting is regularly monitored using specific tests. Therapeutic approaches that enhance the communication skills of children and adults are described in a structured manner. Established tests and their application in treatment, educational diagnostics, aftercare, and quality assurance complement the description. CONCLUSION: Within an interdisciplinary team, structured auditory-verbal therapeutic interventions and reliable testing are crucial for documenting individual outcomes, discussing these compared to the expected result, and counselling the patient in terms of further therapeutic measures.

[Paediatric cochlear implantation in the first year of life: preliminary results]. / Kochleaimplantation bei Kindern im 1. Lebensjahr: Vorläufige Ergebnisse.

BACKGROUND: The success of cochlear implants in children was followed by a stepwise reduction in age at time of surgery. As a result of newborn hearing-screening (NHS) and the reliable audiologic diagnostic procedure, the question is raised as to whether an implantation before the age of 1 year is effective and safe in terms of surgery and rehabilitation. METHOD AND PATIENTS: This retrospective study included 27 children implanted before the age of 1 year (Gr. 1) and 89 children implanted between the age of 1 and 2 years (Gr. 2). Patient related data were analysed for individual history, surgery, rehabilitation and speech understanding. RESULTS: The incidence of complications was not increased in Gr. 1. The fitting of a speech processor was effective and uneventful in all children. The development of hearing and speech understanding showed better results after 2 years in Gr. 1. This development is more obvious for absolute age and not to rehabilitation time. CONCLUSION: In order to achieve an optimal timing for the development of speech understanding, cochlear implantation should be performed before the age of 2 years. This study revealed no additional risks for children in Gr. 1, but the development of speech understanding was better. As a consequence, cochlear implantation should be considered for very young children with an identified bilateral profound hearing loss.

Paediatric cochlear implantation in the first and in the second year of life: a comparative study.

BACKGROUND: Successful outcomes of cochlear implantation in children have led to a gradual reduction in the age at which implantation is performed. Now that newborn hearing screening and a reliable audiological diagnostic procedure are well established, the question has been raised as to whether implantation before the age of 1 year is effective and safe. MATERIAL AND METHOD: The study included 27 children implanted before the age of 1 year (group 1) and 89 children implanted between the ages of 1 and 2 years (group 2). Patient-related data were analysed with respect to individual anamnesis, implantation, rehabilitation and speech understanding. RESULTS: Irrespective of the children's age, the incidence of surgical or anaesthesiological complications did not increase. After two years, group 1 demonstrated better results in terms of development of hearing and speech understanding. These results correlated more closely with the children's actual age than with the length of time in rehabilitation. CONCLUSION: This study revealed that children implanted before the age of 1 year were subjected to no additional risks and showed superior development of speech understanding. Cochlear implantation should therefore be performed in very young children identified as suffering from profound bilateral hearing loss.

[Pediatric cost-benefit analysis]. / Kosten-Nutzen-Analyse der Cochlea-Implantation bei Kindern.

BACKGROUND: The aim of this study was to explore the cost-benefit-ratio of pediatric cochlear implantation for congenitally deaf and prelingually deafened children compared to children with hearing aids. The payers' perspective was chosen as this is the most relevant for cost discussions. The study should verify the hypothesis that educational and associated full costs increase with the age at implantation and that these can be below costs for children with hearing aids. METHODS: Children implanted at different ages (group 1: 0 - 1.9 yr., group 2: 2 - 3.9 yr., group 3: 4 - 6.9 yr.) were compared with deaf children using hearing aids (group 4). Payers were sick funds and public authorities, the first paying for medical and indirect costs, the latter paying for education. Educational settings were used as measure for benefit. All costs related to the hearing deficiency were included up to the age of 16 years (end of primary education) based on 1999 costs. RESULTS: Discounted medical and indirect costs for a pediatric cochlear implant user varied between DM 112,000 (53,300 US dollars) and DM 91,000 (43,300 US dollars) depending on the age at implantation. Costs for a hearing aid user added up to DM 36,000 (17,100 US dollars). These costs were paid by the sick funds. Costs for education varied between DM 159,000 (75,700 US dollars) for group 1 and DM 257,000 (122,400 US dollars) for group 3 compared to DM 277,000 (131,900 US dollars) for hearing aid users. These differences are mainly based on the use of mainstream schools. Total costs for sick funds and public authorities ad up to DM 271,000 (129,000 US dollars), DM 334,000 (159,000 US dollars) and DM 348,000 (165,700 US dollars), respectively, for the three age groups of implanted children compared to DM 313,000 (148,600 US dollars) for hearing aid users. CONCLUSION: This study supports the view that pediatric cochlear implantation provides positive cost-benefit ratios compared to hearing aid users depending on the age at implantation. From a societal/payer perspective implantation of prelingually deafened children is especially recommended for children under the age of 2 years. Implantation between ages 2 and 3,9 can be recommended from an educational perspective. Implantation at ages >7 years must be based on individual decisions considering psychosocial environment, speech and language status and type of communication.

[Test set for the evaluation of hearing and speech development after cochlear implantation in children]. / Evaluationsset zur Hör-/Sprachentwicklung nach Cochlea-Implantation bei Kindern.

BACKGROUND: Since autumn 1998 the multicenter interdisciplinary study group "Test Materials for CI Children" has been compiling a uniform examination tool for evaluation of speech and hearing development after cochlear implantation in childhood. METHODS USED: After studying the relevant literature, suitable materials were checked for practical applicability, modified and provided with criteria for execution and break-off. For data acquisition, observation forms for preparation of a PC-version were developed. RESULTS: The evaluation set contains forms for master data with supplements relating to postoperative processes. The hearing tests check supra-threshold hearing with loudness scaling for children, speech comprehension in silence (Mainz and Göttingen Test for Speech Comprehension in Childhood) and phonemic differentiation (Oldenburg Rhyme Test for Children), the central auditory processes of detection, discrimination, identification and recognition (modification of the "Frankfurt Functional Hearing Test for Children") and audiovisual speech perception (Open Paragraph Tracking, Kiel Speech Track Program). The materials for speech and language development comprise phonetics-phonology, lexicon and semantics (LOGO Pronunciation Test), syntax and morphology (analysis of spontaneous speech), language comprehension (Reynell Scales), communication and pragmatics (observation forms). The MAIS and MUSS modified questionnaires are integrated. CONCLUSIONS: The evaluation set serves quality assurance and permits factor analysis as well as controls for regularity through the multicenter comparison of long-term developmental trends after cochlear implantation.

Cost-benefit analysis of pediatric cochlear implantation: German experience.

HYPOTHESIS: To explore, from the payers' perspective, the cost-benefit ratio of pediatric cochlear implantation for congenitally deaf and prelingually deafened children compared with children with hearing aids. The study should verify the hypothesis that educational and associated costs increase with age at implantation and that these can be below costs for children with hearing aids. METHODS: Children who received implants at the Medical University of Hanover at different ages (Group 1, 0-1.9 yr; Group 2, 2-3.9 yr; Group 3, 4-6.9 yr) were compared with deaf children using hearing aids (Group 4). Sick funds were the payers for direct and indirect costs, and public authorities were the payers for educational costs. Educational settings were used to measure for benefits. All costs related to the hearing deficiency were included up to the age of 16 years on the basis of 1999 currency values. A scenario analysis was used to explore the impact of variation of costs and discount rates. RESULTS: Total costs for the three age groups of children with implants were from euro 138,000 (approximately US$113,100) to euro 177,000 (approximately US$152,700), compared with euro 160,000 (approximately US$138,000) for hearing aid users. CONCLUSION: This study supports the view that pediatric cochlear implantation provides positive cost-benefit ratios compared with hearing aid users, depending on the age at implantation. Implantation is strongly recommended from the payers' perspective for children implanted before the age of 2 years.

Auditory brainstem implant: part I. Auditory performance and its evolution over time.

OBJECTIVE: Evaluation of auditory performance and its evolution over time in patients with the auditory brainstem implant. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS AND METHODS: Between May 1996 and April 2000, 14 patients with neurofibromatosis type 2 underwent implantation with a multichannel auditory brainstem implant. Auditory performance data were obtained in 13 patients who had used their device on a regular daily basis for 1 to 41 months (average 19 months). Hearing evaluation was based on the results of four tests (vowel confusion, consonant confusion, Freiburger numbers, and speech-tracking test), which were performed with and without lip-reading at regular intervals after device activation. RESULTS: 12 patients received auditory sensation through the auditory brainstem implant immediately after device activation. In one patient, because of postoperative electrode migration, device activation was not successful. In this case, after the electrode array was repositioned, activation was successful. The results of the audiovisual mode 2 weeks after device activation revealed a lip-reading enhancement above the chance level in about 50% of the patients in the vowel confusion and speech-tracking tests and in 70% of the patients in the consonant confusion test. Lip-reading enhancement improved within the first 6 months and then entered a plateau phase, which was more prominent in the monosyllabic vowel and consonant tests. In the auditory alone mode, more than half of the patients showed their first positive result in the vowel test 3 months after device activation, but it took about 6 months until half of the patients revealed a result above the chance level in the consonant and Freiburger numbers tests. Open set speech recognition in the auditory alone mode (in the speech-tracking test) was not common and happened relatively late (within 1 year or later). DISCUSSION AND CONCLUSION: Although auditory sensation appeared immediately after device activation, a period of 6 months was necessary for relearning and adaptation of the central auditory system to the altered form of auditory information presented by the auditory brainstem implant.

Erdheim-Chester disease: evidence for a disease entity different from Langerhans cell histiocytosis? Three cases with detailed radiological and immunohistochemical analysis.

Erdheim-Chester (EC) disease is a rare pathological entity with a highly specific and characteristic pattern of radiographic bone changes. Histologically it resembles Langerhans cell histiocytosis (LCH), and it is still a matter of discussion whether EC disease is a distinct entity or a type of LCH. In this study, 3 cases of Erdheim-Chester disease were followed up over years and examined in detail both radiologically and immunohistochemically. All 3 cases showed the pathognomonic skeletal features for EC disease as well as an identical immunohistochemical phenotype quite different from LCH. Macrophages and Touton cells reacted strongly positive with the histiocytic marker CD 68, whereas staining with S100 and CD1a, markers for Langerhans cells, were negative. Both the immunohistochemical phenotype and the bone changes were clearly distinct from LCH.

Auditory brainstem implant in auditory rehabilitation of patients with neurofibromatosis type 2: Hannover programme.

An auditory brainstem implant (ABI) is indicated for patients suffering from bilateral neural deafness. The most affected patients are those with neurofibromatosis type 2 (NF2). An implantation is possible either at the same time as, or after, surgical removal of an acoustic neuroma. This paper demonstrates the results of eight out of 11 patients with NF2, seven of whom received an ABI after tumour removal. Pre-operatively, all of them were deaf. Post-operatively, the first fitting served to determine the individual stimulation parameters for each electrode. The stimulation-dependent side-effects were eliminated by reducing the stimulus intensity without causing negative effects on the hearing with the ABI. Only in one case was an open set understanding achieved within the first year. However, all patients had a better speech understanding when they combined their hearing with the ABI and their lip-reading abilities. There is no correlation between the performance with ABI and the tumour size or the duration of deafness.

Cochlear implantation in children under the age of two: the MHH experience with the CLARION cochlear implant. Medizinische Hochschule Hannover.

This paper examines reports on the selection criteria, the surgical procedure, and the postoperative performance for children under the age of 2 implanted with the CLARION Multi-Strategy Cochlear Implant (1.2 device). Eighteen children have been implanted since 1996 with a mean age at implantation of 18 months (range 11 to 23 months). All children were selected by means of a standardized preoperative diagnostic protocol. The surgical procedure used in older children was modified depending on the head and mastoid size, skull thickness, and recurrent otitis media. Auditory perception was tested prior to as well as 3, 6, 12, and 18 months following implantation by means of a standardized age-adapted test protocol. The electrode array was inserted without difficulty in all cases, with no complications to date. On average, auditory performance improved over time up to 18 months after implantation. Closed-set test scores increased by 25% to 55% in 18 months. Open-set test scores began to show improvement between 6 and 12 months postoperatively. Overall, our experience indicates that cochlear implantation in children under the age of 2 is relatively safe and reliable. The Clarion 1.2 device surgery can be performed without complications. Auditory performance results support the effectiveness of early implantation.

Speech perception results for children implanted with the CLARION cochlear implant at the Medical University of Hannover.

The perception of speech of 167 children implanted with a CLARION Multi-Strategy Cochlear Implant (1.2 device) was evaluated preimplantation and at 3, 6, 12, 18, and 24 months postimplantation. The children were between 15 months and 15 years of age. The test materials consisted of 8 tests involving syllable structure, single- and 2-syllable words, differentiation of word pairs, and sentences. Two difficulty levels were used, depending on developmental age (<7, and 7 to 15 years). There was an improvement in test scores over time for both age groups. The younger children (particularly those under age 4) improved steadily over the first 2 years, while the older children tended to plateau between 12 and 18 months after implantation. These findings demonstrate that deaf children up to 15 years old benefit from cochlear implants. Children under 4 years of age may even have the ability to compensate for delays in speech development before they reach school age.