Children's Tinnitus Questionnaire - A novel tool for assessing the impact of tinnitus on a child's everyday life.

OBJECTIVE: Self-report instruments are commonly used in tinnitus clinics, but they are presently available only for adults. There is a lack of a validated multi-item instrument to capture tinnitus-related problems in children and their impact on everyday life. This study has developed and validated a specifically child-centered questionnaire to assess the impact of tinnitus. METHODS: Development of the tool consisted of several stages. Following a pilot study on 12 children with tinnitus, a validation study was done on a further 192 children with tinnitus aged between 11 and 14 years. The children had an audiological examination, completed a Visual Analogue Scale (VAS) and the newly framed questionnaire. RESULTS: The development and validation process resulted in the new 11-item Children's Tinnitus Questionnaire (CTQ). It includes items concerning the impact of tinnitus on functional, cognitive, emotional and social domains. The validity of the new tool has been established by finding significant correlations between it and VAS loudness (r = 0.42), VAS annoyance (r = 0.67), and VAS coping (r = -0.41). Validity has also been confirmed by measuring differences in CTQ scores and 4 groups of children having graded incidences of tinnitus. The internal consistency assessed with Cronbach's alpha was high (α = 0.82). CONCLUSION: The Children's Tinnitus Questionnaire (CTQ) is the first fully validated multi-item instrument designed specifically for children. The tool has the potential to become a valuable new instrument for use in clinical practice and research; it might be useful for assessing the impact of tinnitus on those children who find that the condition creates problems in their everyday life.

Outcomes of Cochlear Implantation Using FLEX26 Electrode: Audiological Results and Quality of Life after 12 Months.

INTRODUCTION: The electrode length is one of the many factors impacted on results of cochlear implantation. Among lateral wall flexible electrode arrays the latest one is FLEX26 (MED-EL GmbH, Innsbruck, Austria). The main aim of the study was to evaluate the preservation of residual hearing, the level of speech understanding, and quality of life after cochlear implantation with FLEX26 electrode array. METHODS: The study was conducted in a tertiary referral centre. Fifty-two patients implanted unilaterally with FLEX26, including 10 EAS patients (electric acoustic stimulation) and 42 ES patients (electric stimulation). The intervention was minimally invasive cochlear implantation via the round window. Pure-tone audiometry (0.125-8 kHz) was performed preoperatively and at 1, 6, and 12 months postoperatively. Twelve-month hearing preservation was established using HEARRING group formula. Quality of life was measured with AQoL-8D (Assessment of Quality of Life-8 Dimensions) pre- and postoperatively. RESULTS: Residual hearing was preserved in 88.8% EAS patients. Quality of life was significantly better postoperatively in comparison to preoperative period (the effect size for overall quality of life was 0.49). Especially, it increased in relationships and senses dimensions (the effect sizes 0.47 and 0.44, respectively). CONCLUSION: Preservation of residual hearing can be achieved in the majority of patients implanted with FLEX26. Improvement of quality of life was also documented. FLEX26 seems to be an option for surgeons who seek an electrode providing sufficient cochlear coverage.

Stapedotomy in Congenital Stapes Ankylosis with Mobile Footplate: A Case Report.

BACKGROUND This paper presents a case of a 15-year-old child with a rare congenital anomaly of the middle ear in which the stapes was fused to the medial wall of the tympanic cavity. CASE REPORT This defect coexisted with partial malleus fixation in the attic and caused conductive hearing loss at an average level of 35-40 dB. Two exploratory tympanotomies were performed, where excess bone between the stapes and promontory was removed and the head of the malleus was released in the attic. The good effect of these procedures was unstable, mainly due to re-attachment of the stapes to the medial wall of the tympanic cavity. At the next surgery it was decided to perform stapedotomy, despite the mobile stapes footplate. The operation was performed with a small-fenestra stapedotomy technique. Perforation of the footplate was done using a microdrill with a balanced speed. A KURZ prosthesis with a diameter of 0.5 mm was used. The postoperative period passed without any complications. Three and 6 months after the surgery, control pure tone audiometry was performed and showed significant improvement in hearing thresholds. During the follow-up period of more than 6 months, hearing improvement remained stable. CONCLUSIONS We concluded that it was safe to perform stapedotomy in the presence of a mobile stapes footplate when congenital anomaly of the stapes superstructure caused its severe fixation in the middle ear. In our opinion, in a child with congenital ear anomaly, consideration should be given to the multifocal origin of the hearing loss.

Mastoid Obliteration with S53P4 Bioactive Glass Can Make Bonebridge Implantation Feasible: A Case Report.

BACKGROUND Obliteration of the mastoid cavity with S53P4 bioactive glass is becoming a popular method of treatment, allowing most of the problems with the postoperative cavity to be eliminated. In the case of a hearing aid, reconstruction of the posterior wall of the auditory canal is an extremely beneficial procedure and, in the case of the Bonebridge implant, is necessary. After reconstruction, the FMT transducer is covered by bone and bioactive glass and has no contact with the postoperative cavity. The aim of this article is to present a case report. CASE REPORT A 41-year-old male patient with a history of bilateral otitis media with cholesteatoma since childhood had undergone many ear operations since 2001, including radical modified operations and postoperative revisions. There had been ossiculoplasty using own materials and a Kurz TORP prosthesis which gave a short-term hearing improvement for 3 months. The patient underwent tests for implantable devices, which showed a potential significant improvement in hearing and understanding speech. The patient met the audiological criteria qualifying him for the use of an implantable bone conduction device. However, a CT scan of the temporal bone showed that the Bonebridge implant could not be implanted due to insufficient mastoid volume. In order to safely implant the Bonebridge device, it was necessary to first rebuild the posterior wall of the left ear canal. The absolute condition was no inflammation of the ear or leaks for several months. CONCLUSIONS The two-stage surgical procedure as described in this case report can allow the Bonebridge implant to be used in a wider group of patients with previous anatomical limitations.

Cochlear implantation with the nucleus slim straight electrode in subjects with residual low-frequency hearing.

OBJECTIVES: To measure benefit in terms of speech recognition in quiet and in noise, and conservation of residual hearing in three groups of subjects implanted with the Nucleus Straight Research Array cochlear implant. This device incorporates the Nucleus Slim Straight electrode carrier designed to be easier to insert into the cochlea via the round window while potentially minimizing insertion trauma. DESIGN: The study was prospective, with sequential enrolment and within-subject repeated measures; 35 subjects were 15 to 84 years of age with varying levels of bilateral high-frequency HL. Subjects were divided into three groups (A, B, and C) according to preoperative air conduction hearing thresholds in the ear to implant at 500 Hz; A ≤ 50 (n = 11), 50 < B < 80 (n = 13), and C ≥ 80 (n = 11) dB HL. Speech recognition was assessed preoperatively and at intervals up to 1 year postimplantation. Hearing thresholds were monitored over time and CT scans were used to estimate electrode positions. RESULTS: Preoperative mean word recognition score was significantly greater for group A compared with group C in quiet (diff. 26.6%pts, p < 0.05), but not so in noise (diff. 7.9%pts, p = 0.72). However, a greater proportion of subjects in group A (81%) achieved a "worthwhile" gain in speech recognition score (>20%pts) in quiet compared with group C (63%). More importantly, for speech recognition in noise, all subjects in groups A and B achieved a >20%pts gain compared with only 73% in group C. Hearing in implanted ears was well conserved for low frequencies, both initially and up to 12 months postoperatively (15 dB median increase in thresholds 250 to 500 Hz). Only 3 of 35 (9%) cases lost all residual hearing in the implanted ear by 12 months. Where characteristic frequency corresponded to a position occupied by the electrode array, threshold increase was correlated with the preoperative hearing threshold (r = 0.7; p < 0.001) and closely approximated reported estimates of residual outer hair cell gain. For characteristic frequencies at positions apical to the electrode tip, the relation between threshold increase and residual hearing decreased in amplitude at 45 to 135 degrees (r = 0.42; p < 0.05), and disappeared at >135 degrees (r = 0.05; p > 0.05). CONCLUSION: Gains in speech recognition scores for subjects with better residual low-frequency hearing were greater or equal to those obtained by subjects with poorer residual hearing. Residual hearing after cochlear implantation with the Nucleus Slim Straight electrode array was well conserved across all three groups. It appears that the gain provided by outer hair cell function may be completely suppressed when an electrode array is in close proximity to the organ of Corti.

Direct round window stimulation with the Med-El Vibrant Soundbridge: 5 years of experience using a technique without interposed fascia.

The objective of this study was to present 5 years of surgical experience, and the extended results of hearing preservation (based on 3-year follow-up), with the Med-El Vibrant Soundbridge (VSB) in which the floating mass transducer (FMT) is placed directly against the round window membrane, and the fascia is used only as covering tissue to keep it in position. A retrospective survey of surgical and audiological data was conducted to evaluate the performance and stability of patient hearing, with audiometric measurements performed over fixed time intervals up to 36 months. 21 patients, aged 19-62 years (mean 48.4), with mixed or conductive, bilateral or unilateral hearing loss were included in this study. Surgical intervention involved monaural implantation of the Med-El VSB between 2006 and 2009. The results were assessed using pure tone audiometry. In 5 years of experience with the technique, no significant complications or device extrusion were observed except for two revision surgeries requiring FMT repositioning. In the 3-year follow-up, we observed stable hearing in the implanted ear. It is concluded that direct round window stimulation without interposed fascia is an alternative for patients with hearing impairment caused by chronic otitis media and/or lack of ossicles, especially after modified radical mastoidectomy. It allows good results in a selected group of patients, although further observation on a larger population is needed to confirm long-term validity and effectiveness.

Partial deafness treatment with the nucleus straight research array cochlear implant.

INTRODUCTION: The Nucleus Straight Research Array (SRA) cochlear implant has a new 25-mm electrode carrier designed to minimize insertion trauma, in particular allowing easy insertion via the round window. The aims of this study were to measure preoperative to postoperative benefit in terms of speech recognition in quiet and in noise in three groups of patients (electrical complement, EC; electrical stimulation, ES; electro-acoustic stimulation, EAS) with varying levels of low-frequency hearing, and to evaluate the preservation of residual hearing after implantation with the SRA cochlear implant. METHODS: The study design was prospective with sequential enrolment and within-subject comparisons: 23 adult cochlear implant candidates were divided into three groups according to their level of preoperative residual hearing at 500 Hz (EC ≤50 dB; 50 dB < EAS < 80 dB; ES ≥80 dB). Monosyllabic word recognition using the SRA cochlear implant in combination with residual low-frequency hearing was assessed at 4 and 13 months after implantation. Hearing threshold levels were also monitored over time. RESULTS: Subjects across all three groups had significant improvements in speech recognition scores (i.e. >20 percentage points) both for listening in quiet (71% of subjects) and in noise (100% of subjects). The average score at 4 months after operation for words presented in quiet was 61.7%, and in 10 dB SNR noise 46.5%, compared to 34.4 and 10.6% preoperatively (p < 0.001). All subjects retained measurable hearing at 500 Hz in the implanted ear at 4 months after the operation; mean increases were 19, 29 and 1 dB for the EC, EAS and ES groups (n = 21). Across frequencies of 125-1000 Hz, the median increase in thresholds was 15 dB up to 13 months postoperatively (n = 15). CONCLUSIONS: Speech recognition performance of subjects with various levels of residual low-frequency hearing was significantly improved with the SRA cochlear implant. A high level and rate of hearing preservation was achieved with the SRA implanted using a round window surgical technique. Subjects with preoperative low-frequency hearing levels between 50 and 80 dB HL (EAS group) tended to lose more hearing than those with either better or worse hearing.

Treatment of otological features of the oculoauriculovertebral dysplasia (Goldenhar syndrome).

The oculoauriculovertebral dysplasia is a rare congenital malformation that is characterized by a hemifacial microsomia with ocular abnormalities and coexisting disorders in the spinal column and other organs. Characteristic features of the disease were first described by Maurice Goldenhar in 1952. A broad spectrum of hearing impairment is a result of complex malformations of the external ear, the middle ear and in some cases-the inner ear. The degree of hearing loss can range from mild to moderate conductive type in cases of chronic otitis media with effusion and severe to profound sensorineural hearing impairment with malformations of the inner ear in various forms of cochlear hypoplasia. This study focuses on surgical methods of hearing improvement in patients with the oculoauriculovertebral dysplasia (OAVD). We present 11 patients diagnosed with OAVD who are under supervision of the Institute of Physiology and Pathology of Hearing in Warsaw. It is, to our knowledge, one of the largest groups of patients reported by a single ORL medical center. The degree of hearing impairment was thoroughly described in all patients and individual treatment was introduced. The aim of this report was to present algorithm for diagnostics and treatment of symptoms of Goldenhar syndrome (GS) based upon methodology used in modern otosurgery.