[Cochlear implantation with cochlear otosclerosis with bilateral deafness. Case from practice]. / Kokhlearnaia implantatsiia u bol'noi s otosklerozom i dvustoronnei glukhotoi. Sluchai iz praktiki.

Rehabilitation of patients with sensorineural hearing loss is an urgent task of otorhinolaryngology. One of the diseases leading to a pronounced hearing loss is the cochlear form of otosclerosis. The article describes a clinical case of rehabilitation of a patient with this pathology by means of cochlear implantation. The classification of otosclerosis based on the interpretation of computer tomography of temporal bones is presented.

Cochlear Implant Users with Otosclerosis: Are Hearing and Quality of Life Outcomes Worse than in Cochlear Implant Users without Otosclerosis?

BACKGROUND: The otosclerotic process may influence the performance of the cochlear implant (CI). Difficulty in inserting the electrode array due to potential ossification of the cochlea, facial nerve stimulation, and instability of the results are potential challenges for the CI team. OBJECTIVES: To evaluate hearing results and subjective outcomes of CI users with otosclerosis and to compare them with those of CI users without otosclerosis. METHOD: Retrospective review of 239 adults with bilateral profound postlingual deafness who underwent unilateral cochlear implantation between 1992 and 2017. Hearing and speech understanding were assessed via pure-tone audiometry and speech perception tests. Subjective outcomes were assessed via the Nijmegen Cochlear Implant Questionnaire (NCIQ), the Glasgow Benefit Inventory (GBI), and the Hearing Implant Sound Quality Index (HISQUI19) at 6 months, 12 months, and at the last follow-up. RESULTS: Subjects were 22 CI users with otosclerosis and 217 without otosclerosis. Both groups had a similar duration of deafness and age at CI implantation. Results did not significantly differ according to group: no significant intergroup difference was found regarding the frequency of complete electrode insertion, facial stimulation, reimplantation, or PTA4 scores at the last follow-up. Regarding speech perception, no significant intergroup difference was found on any test or at any interval. Further, subjective outcomes, as measured by the GBI, NCIQ, and HISQUI19, did not significantly differ between groups. CONCLUSIONS: Adults with otosclerosis and profound hearing loss derive significant benefit from CI use. Audiological and self-reported outcomes are not significantly different from that of other CI users with postlingual deafness.

Cochlear implants and magnetic resonance scans: A case report and review.

OBJECTIVE AND IMPORTANCE: Traditionally, magnetic resonance imaging (MRI) was contraindicated for patients with cochlear implants (CIs), due to concern about device displacement, overheating of the device or tissues, or direct damage to the device electrode. In addition, image artifact from the device magnet gave poor imaging information in the cranial and upper cervical spine region. Today, MRI is increasingly required in patients who have undergone cochlear implantation, and CI design and MRI protocols have therefore changed to allow implanted patients to safely enter the MRI field, in some cases without removal of the CI internal magnet. CLINICAL PRESENTATION: We present a patient with bilateral CIs who required MRI to investigate new neurological symptoms. Despite tight head bandaging applied according to our protocol, MRI at 1.5 T led to bilateral skin reactions and displacement of the magnet on the left. Both magnets were subsequently removed to allow the skin reactions to settle and for further imaging without artifact. The functioning of the patient's implants was not affected. CONCLUSION: The final decision to recommend that a patient with a CI undergoes MRI, with or without removal of the internal magnet, requires close cooperation between the CI team, the physician requiring the scan, and the radiology team involved in the patient's care. The case study we present highlights the need for patients to be fully informed of the risks involved.

Malleus neck-anchoring malleostapedotomy: preliminary results.

OBJECTIVE: To compare the hearing outcomes between 2 malleostapedotomy (MS) procedures, handle-MS, connecting the prosthesis with the malleus handle and neck-MS, connecting the prosthesis with the malleus neck. PATIENTS: Fourteen individuals having undergone MS in the setting of otosclerosis or congenital ossicular fixation from January 1983 through December 2009. INTERVENTION: Review of preoperative and postoperative audiometric data, ossicular abnormalities, and postoperative complications. MAIN OUTCOME MEASURES: Postoperative air-bone gap (ABG), closure of ABG, and postoperative changes in bone conduction thresholds. RESULTS: Of 14 patients, 7 underwent handle-MS, and 7 underwent neck-MS. Morphologic or functional abnormalities of the incus were identified in all cases. There was no significant sensorineural hearing loss. The mean postoperative ABGs were 19.8 ± 11.9 dB in the handle-MS group and 14.7 ± 5.5 dB in the neck-MS group. The postoperative ABGs for single frequencies revealed better results for neck-MS at all frequencies (0.25, 0.5, 1, 2, 3, and 4 kHz) without statistical significance. The functional success rate (ABG closure, ≤ 10 dB) was 28.6% for the handle-MS group and 42.9% for the neck-MS group (p > 0.05). CONCLUSION: Inasmuch as neck-MS is easy to perform and yields comparable results to those of handle-MS, it may be an alternative procedure of use in selected cases of otosclerosis or stapes fixation with incus anomaly.

Cirugía de la otoesclerosis: estapedectomía y estapedotomía / Surgery of otosclerosis: stapedectomy and stapedotomy

Objetivo: Caracterización de la experiencia quirúrgica reciente en el tratamiento de la otoesclerosis.Materiales y método: Revisión sistemática de la historia clínica de pacientes operados por el autordurante el período enero 2005 a diciembre 2009. Resultados: Desde enero de 2005 a diciembre de 2009 se realizaron 246 cirugías de estribo en 201pacientes con diagnóstico de otoesclerosis en la Clínica Rivas de Bogotá, Colombia. 230 fueronprocedimientos de primera intervención y 16 de revisión. Se realizaron 213 estapedotomías y 19 estapedectomías. Se reportan en 50 intervenciones (20,3%) complicación perioperatoria siendo en su mayor proporción las relacionadas con desgarro de la membrana timpánica o piel del conductoauditivo externo (CAE) (56%), luxación de la platina (26%) y otras causas 18%.Conclusiones: Se corrobora la tendencia de incidencia de otosclerosis en la población de mujeres y es aún mayor en los casos operados bilateralmente. La estapedotomía es el procedimiento de elección para la cirugía de la otosclerosis en nuestros pacientes. La estapedectomía total o parcial se realizasólo en casos excepcionales. Importancia clínica: Consideraciones sobre la técnica quirúrgica. Accesibilidad a base de datos internacional de otología.

Objective: To characterize the recent surgical experience to treat otosclerosis.Materials and methods: systematic review of the surgical record of patients that have been operated on by the author during the period of January, 2005 to December, 2009. Results: From January, 2005 to December, 2009; 246 stapes surgeries were performed in 201 patients who had been diagnosed with otosclerosis at Clínica Rivas from Bogotá, Colombia. 230 of these werefirst time interventions and 16 of them were revisions. 213 stapedotomies were performed and 19 stapedectomies. Perioperatory complications were reported in 50 of the surgeries (20.3%). Out of those 50 surgeries the ones with the highest proportion were the ones related to tears of the tympanicmembrane or of the skin of the external auditory canal (EAC), (56%), dislocation of the foot plate (26%) and other causes 18%. Conclusions: A tendency for the incidence of otosclerosis is corroborated in the women populationand is even higher in those cases that have been bilaterally operated on. Stapedotomy is the procedure of choice to surgically treat our patients suffering from otosclerosis. Total or partial stapedectomy is performed under in exceptional cases. Clinical interest: Considerations on the surgical technique. Accessibility to an International Databaseof Otology.

Speech perception after cochlear implantation in 53 patients with otosclerosis: multicentre results.

OBJECTIVES: To analyse the speech perception performance of 53 cochlear implant recipients with otosclerosis and to evaluate which factors influenced patient performance in this group. The factors included disease-related data such as demographics, pre-operative audiological characteristics, the results of CT scanning and device-related factors. METHODS: Data were reviewed on 53 patients with otosclerosis from 4 cochlear implant centres in the United Kingdom and the Netherlands. Comparison of demographics, pre-operative CT scans and audiological data revealed that the patients from the 4 different centres could be considered as one group. Speech perception scores had been obtained with the English AB monosyllable tests and Dutch NVA monosyllable tests. Based on the speech perception scores, the patients were classified as poor or good performers. The characteristics of these subgroups were compared. RESULTS: There was wide variability in the speech perception results. Similar patterns were seen in the phoneme scores and BKB sentence scores between the poor and good performers. The two groups did not differ in age at onset of hearing loss, duration of hearing loss, progression, age at onset of deafness, or duration of deafness. CONCLUSIONS: The clinical presentation of the otosclerosis (rapid or slow progression) did not influence speech perception. Better performance was related to less severe signs of otosclerosis on CT scan, full insertion of the electrode array, little or no facial nerve stimulation and little or no need to switch off electrodes.

Hearing rehabilitation counseling for patients with otosclerosis-related hearing loss.

OBJECTIVE: To standardize preoperative counseling for stapedectomy candidates. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Preoperative and postoperative hearing thresholds of 54 patients (55 stapedectomies) were retrospectively recorded. Patients (age range, 17-67 yr) were divided into 3 groups that differed significantly in their mean preoperative air conduction (AC) pure-tone averages (PTA) (up to 50, 50-70, and 70-90 dB). INTERVENTION: Comparison of the gap between the mean hearing thresholds achieved postoperatively and the target threshold (normal hearing) in the 3 groups. MAIN OUTCOME MEASURES: Preoperative and postoperative AC thresholds, bone-conduction (BC) thresholds, and air-bone gap at 0.5, 1.0, 2.0, and 4.0 Hz. RESULTS: Patients in the group with a mean preoperative AC below 50 dB had excellent postoperative results and achieved normal hearing thresholds. In the group with a mean preoperative AC of 50 to 70 dB, the postoperative results were good, but normal hearing thresholds were not achieved. Postoperative results in the third group were within the range of moderate hearing loss, allowing these patients to use hearing aids much more successfully than preoperatively. In each of the 3 groups, mean group differences between the preoperative and the postoperative values of AC-PTA thresholds, BC-PTA thresholds, and air-bone gap were statistically significant. Comparisons between each pairing of the stratified groups also yielded statistically significant differences. CONCLUSION: The preoperative AC-PTA threshold value can be viewed as a convenient, valid, and standardized basis for better informed and more comprehensive counseling of stapedectomy candidates with regard to options for hearing rehabilitation.

[Treatment of tympanosclerosis]. / Die Therapie der Tympanosklerose.

Tympanosclerosis is a scarring process with a remarkable variability in its localization within the middle ear. It can lead to conductive hearing loss in many cases. It is usually caused by recurrent chronic inflammation of the middle ear. It is generally accepted that functionally relevant tympanosclerotic findings of the middle ear structures should be treated by surgery. Depending on the extent of the surgical resection, the tympanic membrane and the ossicular chain must be reconstructed individually. In cases of isolated myringosclerosis with no hearing loss, no surgery is required. When the ossicular chain is affected by the sclerosis, sound conduction can often be restored only by interposition of grafts. Stapes footplate fixation can be treated by a stapesplasty. Tympanosclerosis of the oval window combined with other chronic inflammation usually requires two-stage surgery. Tympanosclerotic findings without any clinical symptoms should not be removed. The fitting of conventional or implantable hearing aids is the only treatment if chain reconstruction fails or is impossible.

A novel implantable hearing system with direct acoustic cochlear stimulation.

A new implantable hearing system, the direct acoustic cochlear stimulator (DACS) is presented. This system is based on the principle of a power-driven stapes prosthesis and intended for the treatment of severe mixed hearing loss due to advanced otosclerosis. It consists of an implantable electromagnetic transducer, which transfers acoustic energy directly to the inner ear, and an audio processor worn externally behind the implanted ear. The device is implanted using a specially developed retromeatal microsurgical approach. After removal of the stapes, a conventional stapes prosthesis is attached to the transducer and placed in the oval window to allow direct acoustical coupling to the perilymph of the inner ear. In order to restore the natural sound transmission of the ossicular chain, a second stapes prosthesis is placed in parallel to the first one into the oval window and attached to the patient's own incus, as in a conventional stapedectomy. Four patients were implanted with an investigational DACS device. The hearing threshold of the implanted ears before implantation ranged from 78 to 101 dB (air conduction, pure tone average, 0.5-4 kHz) with air-bone gaps of 33-44 dB in the same frequency range. Postoperatively, substantial improvements in sound field thresholds, speech intelligibility as well as in the subjective assessment of everyday situations were found in all patients. Two years after the implantations, monosyllabic word recognition scores in quiet at 75 dB improved by 45-100 percent points when using the DACS. Furthermore, hearing thresholds were already improved by the second stapes prosthesis alone by 14-28 dB (pure tone average 0.5-4 kHz, DACS switched off). No device-related serious medical complications occurred and all patients have continued to use their device on a daily basis for over 2 years.

Otosclerosis: mid-term results of cochlear implantation.

INTRODUCTION: Constant histological changes in otosclerosis lead to progressive hearing loss which may end up in a profound hearing loss and then be treated by means of cochlear implants. These progressive changes could be followed by changes in cochlear implants fitting and speech discrimination results over the years. OBJECTIVES: The aim of the study is to correlate the progressive histological changes to the cochlear implant clinical outcomes (fitting and speech discrimination results). Also main complications (facial nerve stimulation and difficulties at insertion) and new complications will be discussed. DESIGN: A 5-year prospective case-control study was performed in order to compare cochlear implant results in otosclerosis patients to those in a matched-pair control group. MATERIALS AND METHODS: Fifteen otosclerosis patients were followed throughout the study. Preoperatively temporal bone high-resolution computed tomography, electrically evoked auditory brainstem responses and speech discrimination tests were performed in order to select the patients to be implanted. RESULTS: Not only difficulties with electrode guide insertion were reported, but also difficulties with fitting over the years, due to increasing difficulties to spread the electrical stimuli, which provokes increasing thresholds, maximum comfort levels and charges needed to stimulate hearing cells in basal and medial turn electrodes (p < 0.05), which required deactivating some basal and medial turn electrodes in order to improve cochlear implant effectiveness. The results demonstrated no statistical differences in speech discrimination in otosclerosis patients compared to the control group (p > 0.05). Several complications were reported: facial nerve stimulation (7.14%) and sudden episodes of tinnitus and headaches (14.28%). CONCLUSIONS: Although progressive histological changes in otosclerosis lead to increasing thresholds, maximum comfort levels and charges needed to stimulate hearing cells, speech discrimination results support the cochlear implantation in otosclerosis.

Cochlear implantation and far-advanced otosclerosis.

OBJECTIVE: To evaluate results of cochlear implantation in patients with far-advanced otosclerosis. METHODS: Sixteen patients with far-advanced otosclerosis had undergone unilateral (n = 13) or bilateral (n = 3) cochlear implantation. Surgical difficulties, incidence of complications and postoperative benefit were analyzed. RESULTS: A full electrode insertion was achieved in all patients without surgical difficulties. All patients demonstrated excellent benefit of cochlear implantation. Binaural implantation still improves speech performances, compared to unilateral implantation. In case of residual cochlear function of one nonoperated side, a stapes surgery, performed during the same surgical time as cochlear implantation, can improve speech scores and restore bilateral hearing. Facial nerve stimulation occurred only in 1 patient. CONCLUSION: Cochlear implantation is the method of choice for rehabilitation of patients with otosclerosis, presenting profound or total hearing loss. Patients obtain excellent benefit with a low rate of complications.

Cochlear implantation in otosclerotic deafness.

The otosclerotic process commonly involves the otic capsule and may cause quite widespread demineralisation which leads to a progressive and often profound bilateral sensorineural hearing loss. In this situation cochlear implantation may be the only effective treatment. This chapter considers the pathology of this hearing loss, the effects of cochlear obliteration on implantation, and the effects of demineralisation of the otic capsule on placement of the electrode and nonauditory stimulation. A study is reported from 4 cochlear implant centres in the UK and the Netherlands of 53 patients with cochlear otosclerosis who received cochlear implantation. The CT features of their petrous bones are presented and a classification of the radiological features suggested. 38% of patients experienced facial nerve stimulation presumably due to spread of current through an otic capsule with lower than usual electrical impedance. The most common rogue electrodes were in the proximity of the geniculate ganglion. These could usually be successfully programmed out of the MAP.

[Surgical treatment of otosclerosis]. / Place de la chirurgie dans le traitement de l'otospongiose.

A significant step forward in otosclerosis surgery was made in 1956 with the advent of stapedectomy. This led to a significant reduction in surgical complications and to a high level of patient satisfaction. Hearing aids are the alternative to surgery, and have themselves undergone considerable technical improvements. In advanced otosclerosis, cochlear implants can improve hearing when stapes surgery and a conventional hearing aid are inadequate. These advances are modifying the surgical indications.

Selected complex auditory disorders.

This article provides an overview of four auditory disorders relevant generally to adults and especially to veterans. The disorders are noise-induced hearing loss, idiopathic sudden sensorineural hearing loss, otosclerosis, and Méniàre's disease. Sensorineural hearing loss characterizes each, but additional aspects vary with each of the four conditions. This article describes the conditions, discusses their diagnoses and treatments, and outlines current and suggested rehabilitation. The emphasis is on recent advances, some of which await confirmation prior to possible acceptance as standard practice.

[Results with the Contour cochlear implant in patients with cochlear otosclerosis]. / Ergebnisse mit dem Contour-Cochlear-Implant bei Kapselotosklerose.

BACKGROUND: Results after cochlear implant surgery may be complicated by postoperative facial nerve stimulation. Aim of the study presented was to evaluate postoperative results in implanting the straight Nucleus electrode array and the preformed Contour array in patients with deafness due to cochlear otosclerosis. METHODS: A retrospective analysis of intra- and postoperative reports of all patients with cochlear otosclerosis was carried out. Results with the Nucleus straight electrode array and the Contour array were compared with regard to postoperative facial nerve stimulation. RESULTS AND CONCLUSION: None of the Contour patients (n = 7) presented with postoperative facial nerve stimulation. This is in contrast to the majority (4 of 6) of patients being implanted with the straight electrode array. Our results indicate that the use of the Contour array is advantageous in patients being at risk for facial nerve stimulation. In addition intraoperative reports suggest a more reliable insertion of the Contour electrode array in cochlear otosclerosis with partial obliteration.

Bone-anchored hearing aid quality of life assessed by Glasgow Benefit Inventory.

OBJECTIVE/HYPOTHESIS: The bone-anchored hearing aid (BAHA) is a well established mode of treatment and many studies show the audiological benefit, but none has assessed the benefit to the quality of life of patients. This study uses the validated Glasgow Benefit Inventory to quantify the changes in quality of life. STUDY DESIGN: Retrospective questionnaire study. METHODS: Sixty consecutive patients receiving treatment with BAHA were enrolled in the study. The male/female ratio was 1.26 to 1; mean patient age was 45 years. The most common indication was hearing loss secondary to mastoid disease and surgery followed by congenital atresia and chronic discharge from the ear. RESULTS: The response rate was 85%, which is high and adds weight to the results. The general benefit score was +34 (range, +27-+48), which is comparable to middle ear surgery but just below benefit from cochlear implantation. The social benefit was +21 (range, +12-+37) with only +10 (range, +2-+26) for the physical score. This pattern mirrors that reported for other ear interventions. Maximum benefit was noted in patients with congenital atresias followed by discharging mastoid cavities. CONCLUSION: This study is the first to demonstrate significant quality of life benefit from BAHA surgical intervention as measured by the Glasgow Benefit Inventory.

Medical, surgical and audiological complications of the first 100 adult cochlear implant patients in Birmingham.

Of the first 100 patients implanted on the Midland Cochlear Implant Programme the commonest aetiologies of deafness were idiopathic 31 per cent, meningitis 28 per cent and cochlear otosclerosis 16 per cent. The major complication rate was three per cent. The most severe was one individual who post-operatively developed a cerebral infarct and subsequently died. The minor complication rate was 39 per cent, all of which successfully resolved, and included 11 cases of wound infection, nine cases of vertigo, three transient facial palsies and two post-operative bleeds. Older patients and men were most likely to have a post-operative medical complication. Women were more likely to have an abnormal electrode insertion. Meningitis and otosclerosis were the most complicated aetiologies in terms of cochlear ossification and electrode insertion. A non-patient cochlea was associated with fewer active electrodes. In six cases which had been reported pre-operatively as showing patent cochleas, some form of obstructional ossification was encountered. Patients functioning with greater than 15 active electrodes performed better on auditory tests than patients with fewer than 15 active electrodes.

[Detection of inactivity of the auditory system in the beginning stage with the Freiburg masked speech test]. / Zur Problematik des Nachweises einer Inaktivität des auditorischen Systems im Anfangsstadium mit dem verhallten Freiburger Sprachtest.

Late-onset auditory deprivation or "inactivity" phenomenon has been reported in single cases only because it has not been possible to assemble a larger number of calculable cohorts for which all necessary details concerning individual histories are available. Subjects should not be older than 60 years of age and should have had asymmetric hearing for at least about 10 years or should have been wearers of monaurally fitted hearing aids. The development of late-onset auditory deprivation is presented in 6 single cases. All were assessed by the Freiburg speech discrimination test and the distorted Freiburg speech test, with the latter showing greater sensitivity and variability. It is of special interest that the quotient of distorted speech is reduced in subjects who have normal hearing in one ear and considerable hearing loss in the other ear (for example, in the case of unilateral microtia). This effect may be evidence for significant activation of hearing selectivity developing in the brainstem versus inactivation.

Far-advanced otosclerosis.

Profound deafness has received increasing attention in recent years, largely because of the availability of cochlear implants. Consequently, it is especially important for otolaryngologists to remember that a "blank" audiogram does not necessarily mean total or even profound deafness. Patients with far-advanced otosclerosis may have no measurable hearing with routine audiometric testing even in the presence of serviceable sensorineural hearing. Review of nine patients (10 ears) who underwent stapedectomy from 1980 to 1987 reveals that seven of the nine (78%), who had been unable to use a hearing aid preoperatively, obtained serviceable hearing with hearing aids following surgery. Otolaryngologists should depend on a good history and tuning fork examination to avoid being misled by the audiogram, and should not hesitate to offer stapes surgery to patients with far-advanced otosclerosis.

Hearing aids and otosclerosis.

The purpose of this article on the use of hearing aids in patients with otosclerosis is to emphasize that all otosclerosis patients cannot be assumed to be good users of hearing aid amplification. Patients with losses of 60 dB or less and with purely conductive lesions may be able to use aids well. Individuals with mixed-type impairments due to otosclerosis may have significant problems utilizing amplification. These cases demonstrate the use of hearing aid amplification in patients with mixed-type hearing impairment due to otosclerosis. This is a very different group of patients from the group with a pure conductive lesion, in which there is little problem in fitting a hearing aid, provided that the instrument has sufficient power and gain to override the loss. A special subgroup of patients who have far-advanced otosclerosis and have successful stapes surgery to correct the conductive component of their hearing loss present a unique problem. They may initially suffer from severe recruitment requiring patience, special training and compression-type hearing aids.