Protocol for a validation study of the translated stapesplasty outcome test 25 for measurement of disease-specific quality of life in Dutch patients with otosclerosis.

INTRODUCTION: Otosclerosis is a common cause of acquired conductive hearing loss and can be treated using hearing aids or surgically. Surgical success rates or surgical results are usually reported using pure-tone audiometric thresholds and/or speech discrimination scores. Audiometric results and patient-reported quality of life after stapes surgery do not seem to correlate well. It is therefore our opinion that health-related quality of life measurements should be implemented as an additional outcome measure after stapes surgery. So far, there is a lack of a valid, reliable and clinically feasible measuring tool for determining health-related quality of life in Dutch patients with otosclerosis who undergo stapes surgery. METHODS AND ANALYSIS: A prospective validation study was designed to translate and validate the disease-specific Stapesplasty Outcome Test 25 (SPOT-25) in a population of Dutch patients with otosclerosis who undergo primary stapes surgery. A total of 125 patients with otosclerosis who will be undergoing primary stapes surgery and 50 healthy controls will be included. The patients with otosclerosis will fulfil several questionnaires preoperatively, 6 to 8 weeks postoperatively and 8 to 10 weeks postoperatively with a 2-week interval between the postoperative administrations. The patients' audiometric results, which are measured routinely before and after undergoing primary stapes surgery, will also be used. The healthy controls will fulfil the translated SPOT-25 once. First, the original SPOT-25 will be translated from German to Dutch in a six-step process. Second, the translated SPOT-25 will be pilot-tested in a subset of patients. Lastly, validity, reliability and responsiveness of the translated SPOT-25 will be analysed. ETHICS AND DISSEMINATION: The research protocol was approved by the Institutional Review Board of the University Medical Center Utrecht (protocol 18-768/C; V.1, November 2018). Initially, we planned to include 50 patients. At a later stage we decided to increase the sample size to 100 patients. We notified the Institutional Review Board of this change to the protocol. The trial results will be disseminated through peer-reviewed medical journals and presented at scientific conferences. TRIAL REGISTRATION NUMBER: NL7586.

Stapes Surgery for Profound Hearing Loss Secondary to Otosclerosis.

The objective of this study is to evaluate stapes surgery in patients with otosclerosis and "profound" hearing loss. This means they meet hearing threshold criteria for cochlear implantation (CI). We performed a retrospective study and patient questionnaire. The results from 33 patients (35 ears) were recorded (mean age: 63.6, range: 40-85). The primary outcome measure was hearing thresholds recorded before and after surgery at 0.5, 1, 2, 3, and 4 kHz. Hearing thresholds at 2 and 4 kHz were also analyzed. Glasgow Benefit Inventory (GBI) was used in 21 patients to assess life quality changes. Hearing thresholds improved in 80% of ears (mean improvement, 26.3 dB), were unchanged in 11.4%, and worsened in 8.6%. Mean GBI score was +20.7. Hearing aid use decreased in 23.8% and ceased in 28.6%. One patient subsequently underwent CI. For patients with profound otosclerosis, stapes surgery provides a quantitative improvement in hearing thresholds and improvement in quality of life, with reduced reliance on hearing aids. This avoids CI, auditory rehabilitation, and a change in quality and tonality of sound.

Surgical treatment in children with otosclerosis and congenital stapes fixation: our experience and outcome.

AIM: To evaluate outcomes of stapes surgery in children with congenital stapes fixation and juvenile otosclerosis. METHODS: A retrospective chart review was performed from 1987 to 2013 to identify patients under 18 years old who underwent a stapes surgery. Patients' age, gender, pre- and postoperative audiograms, intraoperative findings including aetiology of stapes fixation, prosthesis type, and complications were analysed. RESULTS: 18 children (6 - 17 years old), all with bilateral conductive hearing loss were identified and 34 stapes surgeries were performed (two patients underwent surgery only on one side). The cause of fixation included juvenile otosclerosis in 88% and congenital stapes fixation in 12%. The mean pre-operative air-bone gap (ABG) was 36,24 dB (SD: 10,86) compared to a postoperative mean ABG of 7,74 (SD: 3,3) (p < 0.000). The profound sensorineural hearing loss was not observed in long-term follow-up. CONCLUSIONS: Paediatric stapes surgery has comparable results to stapedectomy in adults regardless of the cause of stapes fixation; however, the better hearing outcome was observed for cases of juvenile otosclerosis rather than congenital stapes fixation.

Living with otosclerosis: disease-specific health-related quality-of-life measurement in patients undergoing stapes surgery.

INTRODUCTION: Otosclerosis is associated with hearing disability, leading to communication deficits and social and psychological restrictions. The objective of our study was to analyze disease-specific health-related quality of life (HRQOL) after stapes surgery and compare the outcome of HRQOL with audiometric parameters. SUBJECTS AND METHODS: Our clinical case study was conducted at two tertiary referral centers. All the 37 patients who had undergone stapes surgery were analyzed clinically and by audiometric testing (pure tone and speech audiometry) in the pre- and postoperative settings. Disease-specific HRQOL was assessed by the validated Stapesplasty Outcome Test 25 (SPOT-25) pre- and postoperatively. The subjective hearing disability was evaluated by the hearing handicap inventory for adults (HHIA). The postinterventional benefit was measured by the Glasgow Benefit Inventory (GBI). RESULTS: Disease-specific HRQOL improved significantly after stapes surgery in all scales of the SPOT-25. Postoperatively, the total score and the subscore "hearing function" correlated well with the audiometric data. The subscores "tinnitus", "social restrictions", and "mental condition" did not show significant association with audiometric parameters. The comparison of pre- and postoperative HHIA offered a significant improvement in the hearing disability. The scores of the HHIA correlated very well with the audiometric data. The GBI showed a postoperative benefit for each individual patient. CONCLUSION: Stapes surgery leads to a significant improvement in the hearing handicap and of disease-specific HRQOL. The audiometric parameters were shown as not being a sufficient indicator of social and mental well-being. HRQOL outcome measuring instruments should be used routinely in clinical practice to provide an individualized postoperative assessment.

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.

Obsessive-compulsive disorder presenting with musical obsessions in otosclerosis: a case report.

INTRODUCTION: Musical obsessions consist of intrusive recollections of music fragments that are experienced as unwanted. Otosclerosis is caused by an abnormal bone homeostasis of the otic capsule and represents a frequent cause of hearing impairment. Many conditions causing hearing loss have been associated with musical hallucinations, but the association between musical obsessions and hearing loss is frequently overlooked. CASE PRESENTATION: We present the case of a 51-year-old Caucasian woman with a history of obsessive-compulsive disorder who developed musical obsessions soon after being diagnosed with otosclerosis. She was referred to our obsessive-compulsive disorder outpatient unit by her general psychiatrist. At the time of our first evaluation, she had severe musical obsessions that interfered with her social functioning and made her unable to follow conversations. She was started on 40mg of paroxetine and 2.5mg of aripiprazole, which led to significant improvement of her symptoms and of her social and work functioning. CONCLUSIONS: To the best of our knowledge, this is the first report of musical obsessions in a patient with hearing loss due to otosclerosis and a history of obsessive-compulsive disorder. This case suggests that a differential diagnosis of obsessive-compulsive disorder should be carefully considered in patients with hearing impairment who complain of involuntary musical imagery, especially in those patients who have a previous history of obsessive-compulsive disorder.

Quality of perceived sound after stapedotomy.

OBJECTIVE: To evaluate the quality of perceived sound in relation to the audiometric result after stapedotomy. STUDY DESIGN: Ninety-eight patients with otosclerosis, who underwent stapedotomy between 2004 and 2005, participated in this retrospective study. Audiometric data were obtained before and after stapedotomy. Patients filled out two questionnaires: the Amsterdam Post Operative Sound Evaluation and the Operation Benefit Profile, which is based on the Glasgow Hearing Aid Benefit Profile. RESULTS: There were 83 responders and 15 nonresponders; 83% indicated that their hearing was now better compared to preoperatively. The sounds that gave the highest percentage of distortion were "high pitched sound" (15%) and "loud sound" (13%). The groups with an air-bone gap of 0 to 10 dB and 10 to 20 dB report a relatively high number of patients who experience distortion. The audiometric data correlate well with the Operation Benefit Score. CONCLUSIONS: Audiometric improvement does not necessarily mean an improvement in perceived sound and vice versa. But the audiometric outcome is significantly related to the patient's experienced handicap, benefit of the operation, residual difficulty, and overall satisfaction.

Patients' lives following stapedectomy complications.

Nowadays, it is widely accepted that patients must be informed about the risks associated with any type of surgical procedure. Usually, the information provided consists of quoting a list of risks and their probability of occurrence based on data from the literature, and, more appropriately, the figures reflecting the surgeon's personal experience. As a rule, such data are sufficient to hold up in court in the event of a lawsuit, but may be insufficient to help patients make the most appropriate choice. We report two cases that presented complications following stapedotomy. Both cases had a sensorineural hearing loss and a vestibular deficit. This paper aims at describing the important psychological and social consequences of these complications. The potential impact of such complications on the quality of life and the projects for the future of these young patients was predictable. In order to better assist patients in choosing amongst options, we have taken action and modified our approach in advising our patients eligible for stapedotomy procedures.

Patient's quality of life and hearing outcomes after stapes surgery.

OBJECTIVES: To determine the quality of life (QOL) after stapes surgery and whether audiological parameters for hearing correlate with specific QOL factors. DESIGN: A retrospective cross-sectional study. SETTING: A tertiary referral centre. PARTICIPANTS: A series of 35 patients who underwent stapes surgery of which three were excluded because they were <18 years of age, chronically or mentally ill, or in a dependant relationship. Response rate was 93% (30/32). Nine were further excluded because they had revision or bilateral surgery, or missing data. Twenty-one patients were included in this study. MAIN OUTCOME MEASURES: The Glasgow Benefit Inventory (GBI) was used to evaluate general QOL and the Hearing Disability and Handicap Scale (HDHS) was used as a disease-specific measure. The Belfast Rule of Thumb and Glasgow Benefit Plot assessed hearing outcomes. RESULTS: Operative success was 86% using the Belfast Rule of Thumb and 95% had closure of the air-bone gap to within 20 dB. 81.8% of patients reported a better overall QOL as surgery. Glasgow Benefit Inventory Social and GBI Physical scores correlated positively with the HDHS speech component (P < 0.05). The duration of hearing loss correlated inversely with the average HDHS score (P < 0.05). CONCLUSION: The majority of patients report a better QOL as undergoing stapes surgery. Speech impacts on people's physical and social QOL of patients. Quality of life tools, in addition to objective audiologic measurements can provide clinicians with patients' subjective perspective that helps guide clinical decision-making and counselling.

[Demographic and audiological factors as predictors of hearing handicap].

INTRODUCTION: Currently available evidence reveals comparatively few studies of psychological effects of hearing impairments, in spite of the fact that clinicians have for a long time been aware of a connection between the acquired hearing impairment and mental disorders. They are focused on the investigation of dysfunction in general. Thus, three domains of the auditory imbalance may be distinguished: disorder, disability and handicap. 'Handicap', according to the definition of the World Health Organization, is a hindrance in an individual that results from an impairment or disability and represents psychological response of the individual to the impairment. OBJECTIVE: Validation of acquired hearing impairment as a risk factor of psychical disorders as well as an analysis of relation of some demographic factors (sex, age, education) and audiological factors (degree and duration of the impairment) with the frequency of hearing handicap. METHOD: MMPI-201 has been applied in 60 subjects affected with otosclerosis, potential candidates for stapedectomy, before and after the surgery. RESULTS: Individuals with acquired hearing impairment manifest more frequent disorders of psychical functioning in comparison with general population, while demographic and audiometric parameters did not correlate with acquired hearing handicap. CONCLUSION: It may be assumed that the very recognition of demographic and audiological factors can not help much in the understanding of the psychological stress associated with hearing impairment.

Psychosocial aspects of acquired hearing impairment in the patients with otosclerosis.

UNLABELLED: Currently available evidence reveals comparatively few studies of the psychological and social effects of acquired hearing impairment, in spite of the fact that clinicians have for a long time been aware of a connection between acquired hearing impairment and mental disorders. The existing data indicate that psychological disorders are significantly more frequent in a population with hearing impairment than in the average population and are accompanied by disharmony in interpersonal and social relations. AIM: Establishing the presence and the type of mental disorders in patients with hearing impairment due to otosclerosis, before and 6 months after corrective surgery, as well as finding the role of adaptation style (personal adjustment) and facing strategy (communication strategies) as mediating variables between the acquired hearing impairment and observed psychical changes. METHODOLOGY: in 60 subjects, potential candidates for stapedectomy, MMPI-201 has been applied before and after surgery, in order to evaluate the mental state. For assessments of personal adjustment to the impairment and communication strategies, a communication personal profile has been applied preoperatively. RESULTS: more psychiatric disorders are present in the preoperative group compared to the general population and the postoperative group. These disorders are of exclusively a neurotic nature; the most frequent are depressive, anxious depressive and anxious reactions. A connection was found between adaptation style and facing strategies whether in the presence or absence of psychological disorders. CONCLUSION: the findings indicate a need for professional psychiatric-psychological support as a part of a rehabilitation program designed to reduce psychological difficulties, i.e. to help individuals to become adapted to the acoustic environment and to prevent or at least minimize the negative consequences of hearing impairment by efficient communication strategies.

Quality of life following small fenestra stapedotomy.

OBJECTIVES: A cross-sectional observational study was designed to evaluate patient satisfaction after stapedotomy. METHODS: Two validated questionnaires, the Psychosocial Impact of Assistive Devices Scale (PIADS) and the Hearing Handicap Inventory (HHI), were used to assess patients for whom audiometric data were available. RESULTS: The response rate was 79%. The PIADS score showed that 96% of patients reported an improvement in quality of life. The HHI score, however, showed that 32% of patients still experienced a marked degree of handicap after surgery. The HHI scores correlated positively with postoperative audiometric data, and the PIADS scores correlated positively with the degree of change in audiometric data. CONCLUSIONS: The results of this study support the role of small fenestra stapedotomy as a primary treatment for otosclerosis. The study also identifies a certain subpopulation of patients with residual handicap who may require further aural rehabilitation.

Unilateral hallucinations and other psychotic symptoms due to otosclerosis.

We report the case of a 35-year-old man suffering from otosclerosis and unilateral auditory hallucinations as well as other psychotic symptoms that disappeared completely after surgery for otosclerosis. The patient experienced a change of his acoustic sensations: the tinnitus was transformed into music, and 4 months later the music changed into commenting and imperative voices. However, on both occasions the transformation from one form to another occurred during an alcohol withdrawal syndrome characterized mainly by vivid visual hallucinations. Some theoretical considerations on hallucinatory predisposition, development of hallucinations, and psychological factors determining psychotic symptoms will be discussed.

The subjective meaning of illness in severe otosclerosis: a descriptive study in three steps based on focus group interviews and written questionnaire.

The aim of this study of otosclerosis patients was to highlight the circumstances specific to an operable middle ear disease and to describe the psychologically unique aspects accompanying this disorder. In a first step five subjects participated in four focus group interviews, from which emanated a questionnaire answered in a second step by 28 randomly chosen subjects. Finally a second series of supplementary focus interviews were conducted with another group of subjects, after which the interview material was subjected to code-mapping, agreement and validation. The study results indicate that ear surgery was a very important occurrence in the lives of the patients and a unique aspect was that they were burdened by a sense of responsibility for deciding in favour of or against the ear surgery and in choosing the time for operation. An event as tangible as an operation impacts on the individual's psychological processing of the fact that one of his or her senses is damaged. The message to the person's surroundings is very clear: the operation makes others understand how serious the situation is, gives rise to a sense of sympathy. These factors together may promote adaptation to the handicap, or alternately, at least for some persons, may impede adaptation to the necessary hearing aids. To some individuals hearing aids were strongly associated with periods of deteriorating hearing and therefore had negative connotations. Thus the hearing aid was used while waiting for surgery or instead of surgery. The constant hope of regaining one's hearing through ear surgery, although not totally realistic in these cases of severe otosclerosis, always makes the hearing aid the second best solution. The overall conclusion from this study is that there are specific circumstances of an operable ear disease which have great impact upon quality of life, well-being and adaptation. The psychological situation is one of instability, feelings of responsibility and at times of anxiety. Patients with otosclerosis are sometimes told that they are better off than others with impaired hearing, because surgery can improve their hearing and because good amplification through hearing aids can be achieved in persons with conductive hearing losses. It is our hope that this study has helped to provide a fuller picture of the facts of life for persons with severe otosclerosis.

[The propagation pathways of an excitatory stimulus to the receptor apparatus during the initiation of auditory sensations using amplitude-modulated focused ultrasound]. / Puti provedeniia razdrazhaiushchego stimula k retseptornomu apparatu pri initsiatsii slukhovykh oshchushchenii s pomoshch'iu amplitudno-modulirovannogo fokusirovannogo ul'trazvuka.

Studies have been made on thresholds of perception of amplitude-modulated focused ultrasound (AMFU) during modulation by 125-8,000 Hz in 6 healthy subjects, 100 patients with otosclerosis before and after operation, and 13 subjects with other disturbances in hearing. Perception thresholds for AMFU were compared with bone and air audiograms in the same subjects. Changes in the thresholds for AMFU after operation were of the same pattern as those in the thresholds for air audiometry, indicating mainly aerial pathway of signal transmission for AMFU. Perception of AMFU in otosclerotic patients is characterized by "gaps"--a new index which allows to use AMFU for differential diagnostics of otosclerosis in the clinic.

The minimum detectable duration of auditory signals for normal and hearing-impaired listeners.

The minimum detectable duration of a brief burst of noise and a brief gap in a noise were measured by the two-alternative forced-choice method. For all listeners the minimum detectable duration of a burst was shorter than the minimum detectable duration of a gap at equal signal-to-noise ratios. For 12 listeners with normal hearing, the average minimum detectable duration of a burst with a 10-dB signal-to-noise ratio was 1.2 msec, whereas the average of a gap at the same signal-to-noise ratio was 4.2 msec. Nine of ten hearing impaired listeners required longer durations, whether for bursts or gaps, than the average of normal listeners. These nine impaired listeners had slower time constants than any of the normal listeners. Both the rise and decay of auditory sensation in the impaired listeners were therefore abnormally slow.

[Clinics and psychopathology of tinnitus: a psychiatric view (author's transl)]. / Klinik und Psychopathologie von Ohrgeräuschen aus psychiatrischer Sicht.

Unstructured and structured, elementary tinnitus or achoasms are regarded as symptoms of various origins and individual processing of experience even in psychiatric diseases. In the context of a multidimensional causal structure for disturbances of acoustic perception, the lesion and compensatory capacity of the sensory system, primary personality structure, strategy for psychodynamic conflict resolution and above all the constitutionally determined or acquired biological structure of the central nervous system decide on what becomes subjective experience from the objective reduction of auditory function and what is observed as clinically as tinnitus. Besides psychopathological and neuropsychological aspects, the clinics of tinnitus is touched on an acoustic hallucinosis of the hard of hearing is illustrated by reference to a case history and its pathogenesis is discussed.

Personality system and sudden deafness: a comparative psychological study.

The psychic structure of 30 patients suffering from sudden deafness and of 35 patients suffering from otosclerosis was tested by the FPI. Additionally the intelligence was tested by the MWT-B. It could be shown that deafness on one ear does not cause any significant change of personality. Patients with sudden deafness and additional organic diseases did not show any psychic changes or these changes are due to a basic disease. Patients suffering from idiopathic sudden deafness can be differentiated into two groups: the first group consists of patients with no psychic disease; these patients show a good recovering tendency from sudden deafness. The second group consists of patients with a slightly neurotic personality which is in full accordance with those of psychosomatic patients. In these case the organic therapy did not lead to recovery.

Temporal acuity in normal and hearing-impaired listeners.

Temporal acuity, defined as the minimum detectable gap in an otherwise continuous noise, was measured with an adaptive version of the two-alternative forced-choice task. For 6 normal listeners, the minimum duration detectable decreased from an average of 20.3 ms at a noise level of 30 dB SPL to 3.2 ms at 80 dB SPL. By comparison, the temporal acuity of 4 listeners with conductive hearing losses, 2 with otosclerosis, and 7 with sensorineural hearing losses, was poorer than that for normal listeners at equal sound pressure levels. At higher sound pressure levels, the acuity of those with conductive hearing losses or otosclerosis approached normal values, but the acuity for listeners with sensorineural hearing losses did not.

The cochlear component in operated otosclerosis after a mean period of 16 years. A follow-up study.

107 patients operated on for otosclerosis were studied after an average of 16 years after the operation. The mean age-corrected bone thresholds measured after the postoperative stabilization period and at the follow-up examination did not differ significantly. Our results support the opinion that, in cases of operated otosclerosis, the sensorineural component follows the clinical course of presbyacusis.