Susan Arndt

Comparison of pseudobinaural hearing to real binaural hearing rehabilitation after cochlear implantation in patients with unilateral deafness and tinnitus.

Objective: Up to now, treatment modalities of unilateral deafness consist of no treatment, conventional contralateral routing of signal (CROS), or Bone-Anchored Hearing Aid (BAHA) hearing aid. Cochlear implantation makes a new treatment modality available for patients with single-sided deafness. The aim of this study is to evaluate the use of unilateral electrical stimulation with normal hearing on the contralateral side after a period of 6 months compared with the preoperative unaided situation, conventional CROS, or BAHA hearing aids. Study Design: Prospective design. Setting: Tertiary referral center; cochlear implant (CI) program. Patients: Eleven adult subjects with unilateral deafness of various causes were enrolled in the study. Only those patients were included in whom therapy with CROS hearing aid or BAHA was not successful and in whom the auditory nerve was found to be intact and the cochlea patent for cochlear implantation. Intervention: All subjects were fitted in random order with a BAHA Intenso mounted on the softband/tension clamp or with a CROS hearing aid. After test periods with both devices, the subjects received a CI. Main Outcome Measures: The Hochmair-Schulz-Moser sentence test and the Oldenburg sentence test were used to test speech comprehension in 3 presentation configurations in the unaided situation, with conventional CROS and BAHA hearing aids before cochlear implantation as well as after 6 months with CI. Localization was assessed using an array of 7 speakers at head level in a frontal semicircle. Subjective improvement in daily life was evaluated using the Speech, Spatial and Qualities of Hearing Scale, the Health Utilities Index 3 and the International Outcome Inventory for Hearing Aids questionnaires. Tinnitus distress was measured with a tinnitus scale before and after CI implantation. Results: The results show significant improvement in localization ability as well as in speech comprehension in most presentation configurations with the CI. Especially, there is no negative effect on speech comprehension if the noise is presented to the CI ear and speech to the normal hearing ear. With the CI, the summation and squelch effects are not significant, but a significant combined head shadow effect is seen. Speech, Spatial and Qualities of Hearing results show an overall benefit of wearing the CI compared with the other treatment options. The tinnitus scale revealed a positive effect of CI stimulation in cases of preoperative tinnitus. Conclusion: The results in these patients suggest that cochlear implantation improves hearing abilities in people with single-sided deafness and is superior to the alternative treatment options. The use of the CI does not interfere with speech understanding in the normal hearing ear. Our data suggest that the binaural integration of electric and acoustic stimulation is possible even with unilateral normal hearing.

Unilateral deafness in children: audiologic and subjective assessment of hearing ability after cochlear implantation.

Objectives Recently, several studies have shown that a cochlear implant is a suitable treatment for hearing rehabilitation of adults with unilateral sensorineural hearing loss (UHL), and benefits for speech comprehension in noise and localization have been demonstrated. The aim of this clinical study was to evaluate the benefit of cochlear implantation in children with UHL in comparison to their unaided listening situation. Study design Prospective repeated measures single subject design. Setting Tertiary referral center; cochlear implant (CI) program Patients Three children (4, 10, and 11 yr) with noncongenital UHL resulting from different causes were enrolled in the study. Intervention After extensive consultation with each family and confirming CI-candidacy, each child received a cochlear implant. Main Outcome Measures Open set speech recognition measures in competing background noise and tests of sound localization were performed for unaided preoperative and CI-aided postimplant intervals after 6 and 12 months. The parent and child versions of the Speech, Spatial and Qualities scale (SSQ) were used for subjective evaluation of hearing at preimplant and 12-month postimplant intervals. Conclusion All children consistently use their CI 1 year postimplant. Our preliminary results suggest binaural hearing benefits for speech understanding in noise, localization ability and subjectively perceived hearing ability for school-aged children with UHL. Trends for CI-benefits in younger preschoolaged children are confirmed through subjective assessment reported by the parents. The benefits in hearing performance suggest binaural integration and processing of electric and acoustic stimulation arriving at 2 different ears is possible for children with UHL.

Dynamic magnetic resonance imaging of swallowing and laryngeal motion using parallel imaging at 3 T

OBJECT To evaluate the feasibility of an optimized MRI protocol based on high field imaging at 3 T in combination with accelerated data acquisition by parallel imaging for the analysis of oropharyngeal and laryngeal function. MATERIALS AND METHODS Fast 2D gradient echo (GRE) MRI with different spatial resolutions (1.7x2.7 and 1.1x1.5 mm2) and image update rates (4 and 10 frames per second) was employed to assess pharyngeal movements and visualize swallowing via tracking of an oral contrast bolus (blueberry juice). In a study with 10 normal volunteers, image quality was semi-quantitatively graded by three independent observers with respect to the delineation of anatomical detail and depiction of oropharynx and larynx function. Additionally, the feasibility of the technique for the visualization of pathological pre- and post-surgical oropharynx and larynx function was evaluated in a patient with inspiratory stridor. RESULTS Image grading demonstrated the feasibility of dynamic MRI for the assessment of normal oropharynx and larynx anatomy and function. Superior image quality (P<.05) was found for data acquisition with four frames per second and higher spatial resolution. In the patient, dynamic MRI detected pathological hypermobility of the epiglottis resulting in airway obstruction. Additional post-surgical MRI for one clinical case revealed morphological changes of the epiglottis and improved function, i.e., absence of airway obstruction and normal swallowing. CONCLUSION Results of the volunteer study demonstrated the feasibility of dynamic MRI at 3 T for the visualization of the oropharynx and larynx function during breathing, movements of the tongue and swallowing. Future studies are necessary to evaluate its clinical value compared to existing modalities based on endoscopy or radiographic techniques.

Vocal tract and register changes analysed by real-time MRI in male professional singers—a pilot study

Changes of vocal tract shape accompanying changes of vocal register and pitch in singing have remained an unclear field. Dynamic real-time magnetic resonance imaging (MRI) was applied to two professional classical singers (a tenor and a baritone) in this pilot study. The singers sang ascending scales from B3 to G#4 on the vowel /a/, keeping the modal register throughout or shifting to falsetto register for the highest pitches. The results show that these singers made few and minor modifications of vocal tract shape when they changed from modal to falsetto and some clear modifications when they kept the register. In this case the baritone increased his tongue dorsum height, widened his jaw opening, and decreased his jaw protrusion, while the tenor merely lifted his uvula. The method used seems promising and should be applied to a greater number of singer subjects in the future.

High-speed imaging and electroglottography measurements of the open quotient in untrained male voices' register transitions.

Vocal fold oscillation patterns in vocal register transitions are still unclarified. The vocal fold oscillations and the open quotient were analyzed with high-speed digital imaging (HSDI) and electroglottography (EGG) in 18 male untrained subjects singing a glissando from modal to the falsetto register. Results reveal that the open quotient changed with register in both HSDI and EGG. The in-class correlations for different HSDI and EGG determinations of the open quotient were high. However, we found only weak interclass correlations between both methods. In 10 subjects, irregularities of vocal fold vibration occurred during the register transition. Our results confirm previous observations that falsetto register is associated with a higher open quotient compared with modal register. These data suggest furthermore that irregularities typically observed in audio and electroglottographic signals during register transitions are caused by irregularities in vocal fold vibration.

Rhino-orbital-cerebral mucormycosis and aspergillosis: differential diagnosis and treatment

In immunocompromised patients, symptoms and the pathogen spectrum of sinusitis are frequently atypical. If progressive loss of vision occurs, an infection of the anterior skull base or nasal sinuses should be considered. We report on four patients with orbit-associated symptoms. CT-imaging revealed bony defects in sinus borders to orbits or endocranium. In all the cases immediate surgical drainage was performed because complications following sinusitis were suspected. Histopathological diagnosis revealed two cases of aspergillosis and mucormycosis. The possibility of opportunistic infections by saprophytic fungi must be taken into account in immunocompromised patients, as they may endanger both vision and survival. Immediate diagnosis and therapy are essential. Nowadays, therapeutic success can be achieved due to advances in antimicrobial therapy, hyperbaric oxygen therapy and treatment of the underlying disease. Radical procedures like orbital exenteration must be considered in all cases. The current state of diagnostics, therapy and prognosis is discussed based on these case reports and the recent literature.

Cochlear Implantation in Children with Single-Sided Deafness: Does Aetiology and Duration of Deafness Matter?

For adult patients with single-sided deafness (SSD), treatment with a cochlear implant (CI) is well established as an acceptable and beneficial hearing rehabilitation method administered routinely in clinical practice. In contrast, for children with SSD, CI has been applied less often to date, with the rationale to decide either on a case-by-case basis or under the realm of clinical research. The aim of our clinical study was to evaluate the longitudinal benefits of CI for a group of children diagnosed with SSD and to compare their outcomes with respect to patient characteristics. Evaluating a pool of paediatric SSD patients presenting for possible CI surgery revealed that the primary aetiology of deafness was congenital cochlear nerve deficiency. A subgroup of children meeting the CI candidacy criteria for the affected ear (the majority with acquired hearing loss) were enrolled in the study. Preliminary group results suggest substantial improvements in speech comprehension in noise and in the ability to localise sound, which was demonstrated through objective and subjective assessments after CI treatment for the group, with results varying from patient to patient. Our study shows a trend towards superior outcomes for children with acquired hearing loss and a shorter duration of hearing loss compared to congenitally deafened children who had a longer duration of SSD. This indicates an interactive influence of the age at onset, aetiology and duration of deafness upon the restoration of binaural integration and the overall benefits of sound stimulation to two ears after CI treatment. Continued longitudinal investigation of these children and further studies in larger groups may provide more guidance on the optimal timing of treatment for paediatric patients with acquired and congenital SSD.

The new mid-scala electrode array: a radiologic and histologic study in human temporal bones.

Hypothesis To analyze the quality of insertion of the newly developed midscala (MS) electrode, which targets a midscalar electrode position to reduce the risk of trauma to the lateral wall and the modiolus. Background Modern cochlear implant surgery aims for a safe intracochlear placement of electrode arrays with an ongoing debate regarding cochleostomy or round window (RW) insertion and the use of lateral wall or perimodiolar electrode placement. Intracochlear trauma after insertion of different electrodes depends on insertion mode and electrode design and may result in trauma to the delicate structures of the cochlear. Methods We performed a temporal bone (TB) trial with insertion of the MS electrode in n = 20 TB‘s after a mastoidectomy and posterior tympanotomy. Insertion was performed either via the RW or a cochleostomy. Electrode positioning, length of insertion, and angle of insertion were analyzed with rotational tomography (RT). TBs were histologically analyzed. Results of RT and histology were compared. Results Scala tympani (ST) insertion could be accomplished reliably by both RW and via a cochleostomy approach. In 20 TBs, 1 scala vestibuli insertion, 1 incomplete (ST), and 1 elevation of basilar membrane were depicted. No trauma was found in 94.7% of all ST insertions. RT allowed determination of the intracochlear electrode position, which was specified by histologic sectioning. Conclusion The new MS electrode seems to fulfill reliable atraumatic intracochlear placement via RW and cochleostomy approaches. RT is available for evaluation of intracochlear electrode position, serving as a potential quality control instrument in human implantation.

Revision surgery due to magnet dislocation in cochlear implant patients: an emerging complication.

Objectives To analyze the cause and effect of magnet dislocation in cochlear implant (CI) recipients requiring magnet revision surgery for treatment. Study Design Retrospective study. Setting Tertiary referral center. Interventions Case reports from 1,706 CI recipients consecutively implanted from January 2000 to December 2011 were reviewed. The number of cases requiring magnet revision surgery was assessed. Results Revision surgery involving magnet removal or replacement was indicated in 1.23% (21/1,706), of all CI recipients. Magnet dislocation occurring during magnetic resonance tomography (MRI), at 1.5 Tesla (T), with the magnet in place and with the application of compression bandaging around the head, was the main cause for revision surgery in 47.62% (10/21) of the affected cases. All 10 cases were implanted with Cochlear Nucleus cochlear implants. These events occurred, despite adherence to current recommendations of the manufacturer. Conclusion The present study underlines that MRI examination is the main cause of magnet dislocation. The use of compressive bandaging when using 1.5-T MRI does not eliminate the risk of magnet dislocation. Additional cautionary measures are for required for conditional MRI. We recommend X-ray examination after MRI to determine magnet dislocation and avoid major complications in all cases reporting pain during or after MRI. Additional research regarding silicon magnet pocket design for added retention is needed. Effective communication of guidelines for precautionary measures during MRI examination in CI patients is mandatory for all clinicians involved. MRI in CI recipients should be indicated with caution.

Spectrum of hearing disorders and their management in children with CHARGE syndrome.

Objective: The CHARGE syndrome is associated with ear anomalies and deafness in addition to other malformations. Deformations of the ossicles or aplasia of the semicircular canals, cochlear hypoplasia, hypoplasia or aplasia of the VIIIth cranial nerve and abnormal routing of the VIIth cranial nerve, sigmoid sinus, and emissaries are typical findings. The aim of this study is to explore the feasibility and procedure of cochlear implantation in patients with CHARGE syndrome and to assess the outcome. Study Design: Retrospective case review. Setting: Tertiary referral center; cochlear implant program. Patients: Ten patients with CHARGE syndrome and 3 patients with CHARGE-like syndrome treated in our center due to hearing impairment. Eleven patients were congenitally deaf, 1 patient had progressive hearing loss, and 1 patient had mixed hearing loss. Intervention: Computed tomography of temporal bones and magnetic resonance imaging of the brain; bone-anchored hearing aid surgery, cochlear implantation, rehabilitation results. Main Outcome Measures: Surgical suitability and hearing rehabilitation. Results: We illustrate the management of preoperative diagnostics, surgical planning, and hearing rehabilitation. One patient with mixed hearing loss underwent bilateral bone-anchored hearing aid surgery. Because 2 patients had bilateral aplasia of the auditory nerves, we recommended an auditory brainstem implant. The unilateral cochlear implantation was performed in 9 patients and bilateral in 1 patient. In selected cases, it was helpful to plan the operation using a simulator for temporal bone surgery. Complex malformations, such as in CHARGE syndrome, with an increased intraoperative risk for complications should be facilitated by using intraoperative digital volume tomography-assisted navigation and intraoperative digital volume tomography control of electrode position. The results after CI surgery vary due to the differing extent of additional disabilities such as developmental delay, intellectual delay, and visual impairment. Nine of our patients showed improved responsiveness with the cochlear implant. Open speech comprehension could not be observed in 8 patients, whereas the follow-up period was less than 1 year in 4 patients. The relatively high age of our patients at implantation might be an important factor. Conclusion: Careful planning of the treatment of CHARGE syndrome patients with sensorineural hearing loss can, to a limited extent, lead to auditory benefit without increasing surgical complications. Cochlear implantation is therefore indicated after critical assessment.