Validation of the Norwegian Version of the Speech, Spatial and Qualities of Hearing Scale (SSQ).

INTRODUCTION: The main objective of the study was to validate the Norwegian translation of the Speech, Spatial and Qualities of Hearing Scale (SSQ) and investigate the SSQ disability profiles in a cochlear implant (CI) user population. METHODS: The study involved 152 adult CI users. The mean age at implantation was 55 (standard deviation [SD] = 16), and the mean CI experience was 5 years (SD = 4.8). The cohort was split into three groups depending on the hearing modality: bilateral CIs (BCIs), a unilateral CI (UCI), and bimodal (CI plus contralateral hearing aid; HCI). The SSQ disability profiles of each group were compared with those observed in similar studies using the English version and other translations of the SSQ. Standard values, internal consistency, sensitivity, and floor and ceiling effects were investigated, and the missing-response rates to specific questions were calculated. Relationships to speech perception were measured using monosyllabic word scores and the Norwegian Hearing in Noise Test scores. RESULTS: In the BCI group, the average scores were around 5.0 for the speech and spatial sections and 7.0 for the qualities section (SD ∼2). The average scores of the UCI and HCI groups were about one point lower than those of the BCI group. The SSQ disability profiles were comparable to the profiles in similar studies. The slopes of the linear regression lines measuring the relationships between the SSQ speech and monosyllabic word scores were 0.8 per 10% increase in the monosyllabic word score for the BCI group (explaining 35% of the variation) and 0.4 for the UCI and HCI groups (explaining 22-23% of the variation). CONCLUSION: The Norwegian version of the SSQ measures hearing disability similar to the original English version, and the internal consistency is good. Differences in the recipients' pre-implantation variables could explain some variations we observed in the SSQ responses, and such predictors should be investigated. Data aggregation will be possible using the SSQ as a routine clinical assessment in global CI populations. Moreover, pre-implantation variables should be systematically registered so that they can be used in mixed-effects models.

Effect of Proximity to the Modiolus for the Cochlear CI532 Slim Modiolar Electrode Array on Evoked Compound Action Potentials and Programming Levels.

BACKGROUND: The first surgeries with CI532 showed an effect of the proximity of the electrode to the modiolus on the Evoked Compound Action Potentials (ECAPs). OBJECTIVES: Objectives of the study were to investigate the effect of the "pullback" procedure on intraoperative ECAP responses in three different electrode array positions and additionally to compare behavioral thresholds with the thresholds obtained in a group of patients using the standard insertion. The hypothesis of this study is that pullback will cause lower ECAPs and behavioral thresholds. PATIENTS: The study included 40 patients, 20 in the pullback insertion group and 20 in the standard insertion group (without pullback). METHOD: During insertion of the CI532 electrode array, ECAP was performed in three different positions for the pullback group: at initial insertion, at over-insertion, and after pullback. Insertion was monitored by fluoroscopy. In the standard group, ECAP was performed at the initial position, which is also the final position. ECAP thresholds (T-ECAPs) were compared within subjects at the initial and the final position in the pullback group and between groups in the final positions of the pullback and standard groups. Programming levels (C- and T-levels) were compared between the two groups 1 year after switch-on. RESULTS: Intraoperative measurements pullback shows lower average T-ECAPs after pullback compared to thresholds in initial position. Comparison of intraoperative T-ECAPs at the final positions showed no statistically significant difference between the pullback group and the standard insertion group. Furthermore, 1 year after switch-on there was no statistically significant difference in C- and T-levels between the two groups. CONCLUSION: The pullback maneuver of the CI532 electrode array after an over-insertion gave significantly lower T-ECAPs compared to the thresholds at the initial position. However, the between-groups analysis of pullback and standard insertion showed neither significantly different T-ECAPs nor different programming levels. Because T-ECAPs and programming levels vary considerably between subjects, large groups are required to detect differences between groups. Additionally, the effect pullback technique to preserving the residual hearing is not known yet.

Insertion Depth in Cochlear Implantation and Outcome in Residual Hearing and Vestibular Function.

OBJECTIVES: It has long been known that cochlear implantation may cause loss of residual hearing and vestibular function. Different insertion depths may cause varying degrees of intracochlear trauma in the apical region of the cochlea. The present study investigated the correlation between the insertion depth and postoperative loss of residual hearing and vestibular function. DESIGN: Thirty-nine adults underwent unilateral cochlear implantation. One group received a Med-El +Flex electrode array (24 mm; n = 4), 1 group received a Med-El +Flex electrode array (28 mm; n = 18), and 1 group received a Med-El +Flex electrode array (31.5 mm; n = 17). Residual hearing, cervical vestibular-evoked myogenic potentials, videonystagmography, and subjective visual vertical/horizontal were explored before and after surgery. The electrode insertion depth and scalar position were examined with high-resolution rotational tomography after implantation in 29 subjects. RESULTS: There was no observed relationship between the angular insertion depth (405° to 708°) and loss of low-frequency pure-tone average. Frequency-specific analysis revealed a weak relationship between the angular insertion depth and loss of hearing at 250 Hz (R= 0.20; p = 0.02). There was no statistically significant difference in the residual hearing and vestibular function between the +Flex and the +Flex electrode array. Eight percent of the cases had vertigo after surgery. The electrode arrays were positioned inside the scala tympani and not scala vestibuli in all subjects. In 18% of the cases, the +Flex electrode array was not fully inserted. CONCLUSIONS: The final outcome in residual hearing correlates very weakly with the angular insertion depth for depths above 405°. Postoperative loss of vestibular function did not correlate with the angular insertion depth or age at implantation. The surgical protocol used in this study seems to minimize the risk of postoperative vertigo symptoms.

Deep round window insertion versus standard approach in cochlear implant surgery.

The aim of this study was to compare the outcomes of vestibular tests and the residual hearing of patients who have undergone full insertion cochlear implant surgery using the round window approach with a hearing preservation protocol (RW-HP) or the standard cochleostomy approach (SCA) without hearing preservation. A prospective study of 34 adults who underwent unilateral cochlear implantation was carried out. One group was operated using the RW-HP (n = 17) approach with Med-El +Flex(SOFT) electrode array with full insertion, while the control group underwent a more conventional SCA surgery (n = 17) with shorter perimodiolar electrodes. Assessments of residual hearing, cervical vestibular-evoked myogenic potentials (cVEMP), videonystagmography, subjective visual vertical/horizontal (SVH/SVV) were performed before and after surgery. There was a significantly (p < 0.05) greater number of subjects who exhibited complete or partial hearing preservation in the deep insertion RW-HP group (9/17) compared to the SCA group (2/15). A higher degree of vestibular loss but a lower degree of vertigo symptoms could be seen in the RW-HP group, but the differences were not statistically significant. It is possible to preserve residual hearing to a certain extent also with deep insertion. Full insertion with hearing preservation was less harmful to residual hearing particularly at 125 Hz (p < 0.05), than was the standard cochleostomy approach.

Scalar position in cochlear implant surgery and outcome in residual hearing and the vestibular system.

OBJECTIVE: To evaluate the effect of the intracochlear electrode position on the residual hearing and VNG- and cVEMP responses. DESIGN: Prospective pilot study. STUDY SAMPLE: Thirteen adult patients who underwent unilateral cochlear implant surgery were examined with high-resolution rotational tomography after cochlear implantation. All subjects were also tested with VNG, and 12 of the subjects were tested with cVEMP and audiometry before and after surgery. RESULTS: We found that although the electrode was originally planned to be positioned inside the scala tympani, only 8 of 13 had full insertion into the scala tympani. Loss of cVEMP response occurred to the same extent in the group with full scala tympani positioning and the group with scala vestibuli involvement. There was a non-significant difference in the loss of caloric response and residual hearing between the two groups. Interscalar dislocation of the electrode inside the cochlea was observed in two patients. A higher loss of residual hearing could be seen in the group with electrode dislocation between the scalae. CONCLUSIONS: Our findings indicate that intracochlear electrode dislocation is a possible cause to loss of residual hearing during cochlear implantation but cannot be the sole cause of postoperative vestibular loss.

Intraoperative Measured Electrocochleography and Fluoroscopy Video to Detect Cochlea Trauma.

HYPOTHESIS: Gross electrode movements detected with intraoperative, real-time X-ray fluoroscopy will correlate with fluctuations in cochlear output, as measured with intraoperative electrocochleography (ECochG). BACKGROUND: Indications for cochlear implantation (CI) are expanding to include patients with residual hearing; however, implant recipients often lose residual hearing after CI. The objective of this study was to identify probable traumatic events during implantation by combining electrophysiological monitoring of cochlear function with simultaneous X-ray monitoring. The surgical timing of these apparently traumatic events was then investigated. METHODS: For 19 adult patients (21 surgeries, 2 bilateral), the ECochG responses were measured during implantation of a cochlear nucleus slim modiolar electrode (CI532/CI632, Cochlear Ltd Australia Nucleus slim modiolar). Simultaneous fluoroscopy was performed, as well as a postoperative cone-beam computed tomography (CT) scan. For all patients, pre- and postoperative audiograms were recorded up to 1 year after surgery to record the loss of residual hearing. RESULTS: Electrode insertions for 21 surgeries were successfully monitored. A drop in ECochG response was significantly correlated with reduced hearing preservation compared with patients with preserved responses throughout. Drops in the ECochG response were measured to occur during insertion, because of movement of the array after insertion was complete, including while sealing of the electrode array at the round window or coiling of the array lead within the mastoid cavity. In some patients, a reduction in cochlear output, resulting in poor ECochG response, was inferred to occur before the beginning of implantation. CONCLUSION: The combination of perioperative ECochG measurements, microscope video, fluoroscopy, and postoperative CT scan may inform on what causes the loss of residual hearing after implantation. These findings will be used to improve the surgical procedure in future.

Classification of Acoustic Hearing Preservation After Cochlear Implantation Using Electrocochleography.

The objective to preserve residual hearing during cochlear implantation has recently led to the use of intracochlear electrocochleography (ECochG) as an intraoperative monitoring tool. Currently, a decrease in the amplitude of the difference between responses to alternating-polarity stimuli (DIF response), predominantly reflecting the hair cell response, is used for providing feedback. Including other ECochG response components, such as phase changes and harmonic distortions, could improve the accuracy of surgical feedback. The objectives of the present study were (1) to compare simultaneously recorded stepwise intracochlear and extracochlear ECochG responses to 500 Hz tone bursts, (2) to explore patterns in features extracted from the intracochlear ECochG recordings relating to hearing preservation or hearing loss, and (3) to design support vector machine (SVM) and random forest (RF) classifiers of acoustic hearing preservation that treat each subject as a sample and use all intracochlear ECochG recordings made during electrode array insertion for classification. Forty subjects undergoing cochlear implant (CI) surgery at the Oslo University Hospital, St. Thomas' Hearing Implant Centre, or the University Hospital of Zurich were prospectively enrolled. In this cohort, DIF response amplitude decreases did not relate to postoperative acoustic hearing preservation. Exploratory analysis of the feature set extracted from the ECochG responses and preoperative audiogram showed that the features were not discriminative between outcome classes. The SVM and RF classifiers that were trained on these features could not distinguish cases with hearing loss and hearing preservation. These findings suggest that hearing loss following CI surgery is not always reflected in intraoperative ECochG recordings.

The Risk of Benign Paroxysmal Positional Vertigo After Head Trauma.

OBJECTIVES: Head trauma may cause dislodgement of otoconia and development of benign paroxysmal positional vertigo (BPPV). The risk of developing BPPV is expected to be highest shortly after the trauma, then decrease and approach the risk seen in the general population. The aim of this study was to estimate the risk-time curve of BPPV development after head trauma. STUDY DESIGN: Prospective observational study. METHODS: Patients with minimal, mild, or moderate head trauma treated at the Department of Neurosurgery or the Department of Orthopedic Emergency at Oslo University Hospital, were interviewed and examined for BPPV using the Dix-Hallpike and supine roll maneuvers. BPPV was diagnosed according to the International diagnostic criteria of the Bárány Society. Telephone interviews were conducted at 2, 6, and 12 weeks after the first examination. RESULTS: Out of 117 patients, 21% developed traumatic BPPV within 3 months after the trauma. The corresponding numbers were 12% with minimal trauma, 24% with mild, and 40% with moderate trauma. The difference in prevalence between the groups was significant (P = .018). During the first 4 weeks after the trauma, it was observed 20, 3, 0, and 1 BPPV onsets, respectively. No BPPV cases were seen for the remainder of the 3-month follow-up. CONCLUSION: The risk of developing BPPV after minimal-to-moderate head trauma is considerable and related to trauma severity. Most cases occur within few days after the trauma, but any BPPV occurring within the first 2 weeks after head trauma are likely due to the traumatic event. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:443-448, 2022.

Suitable Electrode Choice for Robotic-Assisted Cochlear Implant Surgery: A Systematic Literature Review of Manual Electrode Insertion Adverse Events.

Background and Objective: The cochlear implant (CI) electrode insertion process is a key step in CI surgery. One of the aims of advances in robotic-assisted CI surgery (RACIS) is to realize better cochlear structure preservation and to precisely control insertion. The aim of this literature review is to gain insight into electrode selection for RACIS by acquiring a thorough knowledge of electrode insertion and related complications from classic CI surgery involving a manual electrode insertion process. Methods: A systematic electronic search of the literature was carried out using PubMed, Scopus, Cochrane, and Web of Science to find relevant literature on electrode tip fold over (ETFO), electrode scalar deviation (ESD), and electrode migration (EM) from both pre-shaped and straight electrode types. Results: A total of 82 studies that include 8,603 ears implanted with a CI, i.e., pre-shaped (4,869) and straight electrodes (3,734), were evaluated. The rate of ETFO (25 studies, 2,335 ears), ESD (39 studies, 3,073 ears), and EM (18 studies, 3,195 ears) was determined. An incidence rate (±95% CI) of 5.38% (4.4-6.6%) of ETFO, 28.6% (26.6-30.6%) of ESD, and 0.53% (0.2-1.1%) of EM is associated with pre-shaped electrodes, whereas with straight electrodes it was 0.51% (0.1-1.3%), 11% (9.2-13.0%), and 3.2% (2.5-3.95%), respectively. The differences between the pre-shaped and straight electrode types are highly significant (p < 0.001). Laboratory experiments show evidence that robotic insertions of electrodes are less traumatic than manual insertions. The influence of round window (RW) vs. cochleostomy (Coch) was not assessed. Conclusion: Considering the current electrode designs available and the reported incidence of insertion complications, the use of straight electrodes in RACIS and conventional CI surgery (and manual insertion) appears to be less traumatic to intracochlear structures compared with pre-shaped electrodes. However, EM of straight electrodes should be anticipated. RACIS has the potential to reduce these complications.

Fusion of Technology in Cochlear Implantation Surgery: Investigation of Fluoroscopically Assisted Robotic Electrode Insertion.

The HEARO cochlear implantation surgery aims to replace the conventional wide mastoidectomy approach with a minimally invasive direct cochlear access. The main advantage of the HEARO access would be that the trajectory accommodates the optimal and individualized insertion parameters such as type of cochlear access and trajectory angles into the cochlea. To investigate the quality of electrode insertion with the HEARO procedure, the insertion process was inspected under fluoroscopy in 16 human cadaver temporal bones. Prior to the insertion, the robotic middle and inner ear access were performed through the HEARO procedures. The status of the insertion was analyzed on the post-operative image with Siemens Artis Pheno (Siemens AG, Munich, Germany). The completion of the full HEARO procedure, including the robotic inner ear access and fluoroscopy electrode insertion, was possible in all 16 cases. It was possible to insert the electrode in all 16 cases through the drilled tunnel. However, one case in which the full cochlea was not visible on the post-operative image for analysis was excluded. The post-operative analysis of the electrode insertion showed an average insertion angle of 507°, which is equivalent to 1.4 turns of the cochlea, and minimal and maximal insertion angles were recorded as 373° (1 cochlear turn) and 645° (1.8 cochlear turn), respectively. The fluoroscopy inspection indicated no sign of complications during the insertion.

Dystonia-deafness syndrome caused by ACTB p.Arg183Trp heterozygosity shows striatal dopaminergic dysfunction and response to pallidal stimulation.

BACKGROUND: Dystonia-deafness syndrome is a well-known clinical entity, with sensorineural deafness typically manifesting earlier than dystonia. ACTB p.Arg183Trp heterozygosity has been reported in six patients to cause combined infant-onset deafness and dystonia manifesting in adolescence or young adulthood. Three of these have received beneficial pallidal stimulation. Brain imaging to assess striatal function has not been reported previously, however. Nor has a comprehensive hypothesis been presented for how the pleiotropic manifestations of this specific beta-actin gene mutation originate developmentally. CASE PRESENTATION: A 19-year-old girl with congenital mild dysmorphic facial features, cochlear implants for infant-onset deafness, and mild cognitive and emotional disability, presented with an adolescent-onset, severe generalized dystonia. Brain MRI and multiple single gene sequencing were inconclusive. Due to life-threatening dystonia, we implanted a neurostimulation device, targeting the postero-ventral internal pallidum bilaterally. The Burke-Fahn-Marsden Dystonia Rating Scale motor/disability scores improved from 87/25 to 21/13 at 2.5 months postoperatively, 26/14 at 3 years, and 30/14 at 4 years. Subsequent whole exome sequencing identified heterozygosity for the ACTB p.Arg183Trp variant. Brain imaging included 123I-ioflupane single photon emission computed tomography (Dopamine Transporter-SPECT), SPECT with 123I-epidepride (binds to dopamine type 2-receptors) and 18 Fluoro-Deoxy-Glucose (FDG)-PET. Both Epidepride-SPECT and FDG-PET showed reduced tracer uptake in the striatum bilaterally, particularly in the putamen. DaT-SPECT was slightly abnormal. CONCLUSIONS: In this patient with dystonia-deafness syndrome caused by ACTB p.Arg183Trp heterozygosity, unprecedented brain imaging findings strongly indicate striatal neuronal/dopaminergic dysfunction as the underlying cause of the dystonia. Pallidal stimulation provided a substantial improvement of the severe generalized dystonia, which is largely sustained at 4-year follow-up, and we advise this treatment to be considered in such patients. We hypothesize that the pleiotropic manifestations of the dystonia-deafness syndrome caused by this mutation derive from diverse developmental functions of beta-actin in neural crest migration and proliferation (facial dysmorphogenesis), hair cell stereocilia function (infant-onset deafness), and altered synaptic activity patterns associated with pubertal changes in striatal function (adolescent-onset dystonia). The temporal differences in developmental onset are likely due to varying degrees of susceptibility and of compensatory upregulation of other actin variants in the affected structures.

Cochlear implantees: Analysis of behavioral and objective measures for a clinical population of various age groups.

INTRODUCTION: As of 2014 more than 1200 patients have received a cochlear implant (CI) at Oslo University Hospital (OUS) and approximately half of them have been children. The data obtained from these patients have been used to develop a comprehensive database for a systematic analysis of several objective measurements and programming measurements. During the past 10 years, we have used an objective measurements protocol for our CI surgeries. Our intra-operative protocol includes: Evoked Compound Action Potentials (ECAP), visually observed Electrically evoked Stapedius Reflex Threshold (ESRT), and electrode impedances. Post-operative (Post-OP) programming sessions typically begin 4-6 weeks after surgery and continue on a scheduled basis. The initial programming data include threshold levels (T-levels) and comfortable levels (C-levels) for the different patient age groups. In this study, we compared initial stimulation levels and stimulation levels after at least 1 year of CI with objective measurements obtained intra-operatively. METHOD: This study focused on the development of a comprehensive database of detailed intra-operative objective measures and post-OP programming measurements from a group of 296 CI patients who received the same type of CI and electrode configuration (Cochlear Corporation CI with Contour electrode). This group included 92 bilateral CI patients. Measurements from 388 CI devices were studied. Patients were divided into 5 different age groups at the age of implantation: 0-2, 2-5, 5-10, 10-20, and above 20 years in order to investigate age-related differences in programming levels and objective measurements. For the comparison analysis we used T- and C-levels obtained after the last day of initial programming and also after at least 1 year implant use. These programming levels were then correlated with some of the intra-operative objective measurements. RESULTS: T-levels were found to be the lowest for the youngest patient group and increased with age. C-levels varied within age groups and frequency range. Patients above 20 years of age had the highest comfort levels in the low to mid-frequencies (electrodes 22-8) and the lowest comfort levels in the high-frequency range (electrodes 1-7). Correlation coefficients between intra-operative objective measurements and programming levels were found to be in the range of no correlation to moderate correlation. Adult patients had the most significant correlation coefficients between ECAP thresholds and T-levels in the low frequencies. The younger patients aged 10-20 years and 5-10 years had more significant correlations in the higher frequency channels compared to the other age groups. Intra-operative visually observed ESRTs and electrode impedances were not significantly correlated with initial or stable programming levels for the children or adults. CONCLUSION: Analyzing initial and follow-up mapping levels from previous patients is very important for a CI Center in terms of quality control. The mean T/C-levels reported in this study can provide guidance to our programming audiologists and help them determine the initial programming levels to be stored in the speech processor, especially for very young patients. Unfortunately intra-operative objective measures in our study, such as ECAP, ESRT, and electrode impedances did not provide statistically significant correlations that may help to predict the programming T- and C-levels for all patients. However, we have observed cases where the intra-operative objective measures of ESRT and TECAP profiles were very similar to an individual's MAP profile. It was not possible, however, to determine why some patients did not have an objective measures profile that was similar to their programming levels profile.

STUB1 mutations in autosomal recessive ataxias - evidence for mutation-specific clinical heterogeneity.

BACKGROUND: A subset of hereditary cerebellar ataxias is inherited as autosomal recessive traits (ARCAs). Classification of recessive ataxias due to phenotypic differences in the cerebellum and cerebellar structures is constantly evolving due to new identified disease genes. Recently, reports have linked mutations in genes involved in ubiquitination (RNF216, OTUD4, STUB1) to ARCA with hypogonadism. METHODS AND RESULTS: With a combination of homozygozity mapping and exome sequencing, we identified three mutations in STUB1 in two families with ARCA and cognitive impairment; a homozygous missense variant (c.194A > G, p.Asn65Ser) that segregated in three affected siblings, and a missense change (c.82G > A, p.Glu28Lys) which was inherited in trans with a nonsense mutation (c.430A > T, p.Lys144Ter) in another patient. STUB1 encodes CHIP (C-terminus of Heat shock protein 70 - Interacting Protein), a dual function protein with a role in ubiquitination as a co-chaperone with heat shock proteins, and as an E3 ligase. We show that the p.Asn65Ser substitution impairs CHIP's ability to ubiquitinate HSC70 in vitro, despite being able to self-ubiquitinate. These results are consistent with previous studies highlighting this as a critical residue for the interaction between CHIP and its co-chaperones. Furthermore, we show that the levels of CHIP are strongly reduced in vivo in patients' fibroblasts compared to controls. CONCLUSIONS: These results suggest that STUB1 mutations might cause disease by impacting not only the E3 ligase function, but also its protein interaction properties and protein amount. Whether the clinical heterogeneity seen in STUB1 ARCA can be related to the location of the mutations remains to be understood, but interestingly, all siblings with the p.Asn65Ser substitution showed a marked appearance of accelerated aging not previously described in STUB1 related ARCA, none display hormonal aberrations/clinical hypogonadism while some affected family members had diabetes, alopecia, uveitis and ulcerative colitis, further refining the spectrum of STUB1 related disease.