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Perilymphatic fistula (PLF) is a challenging inner ear condition, characterized by the abnormal connection between inner and middle ear and subsequent leakage of inner ear liquid (perilymph) into the middle ear. Early surgical intervention for PLF might lead to favorable audiological and vestibular outcomes. The conventional reference standard of PLF detection is the intraoperative visualization of perilymph leakage during explorative tympanotomy or endoscopic inspection; the specificity and sensitivity of this method is unknown. Systematic reviews assessing the accuracy of diagnostic tests for PLF are not available. Objective: To systematically review the literature for qualitative evidence exploring the diagnostic tests for the PLF diagnosis. The proposed systematic review will answer the following question: What is the accuracy of diagnostic tests in detecting perilymphatic fistula in humans? Methods: Eligibility criteria: original peer-reviewed articles regarding studies on humans of any age containing data with diagnostic test accuracy estimation (sensitivity and specificity) for PLF diagnosis or in which diagnostic test accuracy could be calculated based on data provided, without language, study design or study date limits. MEDLINE, EMBASE, the Cochrane Central Register of Controlled Trials, Scopus, and Web of Science will be searched for eligible articles. Additional manual searches will be performed. Covidence software will be used for title and abstract screening, full text review and data extraction. The risk of bias assessment will be conducted using the Quality Assessment of Diagnostic Test Accuracy 2 (QUADAS-2) tool. If two or more high-quality articles evaluating the same diagnostic test will be identified, their findings will be quantitatively synthesized; if a quantitative synthesis is not feasible, the results will be described in a narrative summary. Grading of Recommendations, Assessment, Development, and Evaluations (GRADE) framework will be used to evaluate evidence strength. Study funded by Oslo University Hospital. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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 [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]