Herpesviruses, polyomaviruses, parvoviruses, papillomaviruses, and anelloviruses in vestibular schwannoma.

Etiology of vestibular schwannoma (VS) is unknown. Viruses can infect and reside in neural tissues for decades, and new viruses with unknown tumorigenic potential have been discovered. The presence of herpesvirus, polyomavirus, parvovirus, and anellovirus DNA was analyzed by quantitative PCR in 46 formalin-fixed paraffin-embedded VS samples. Five samples were analyzed by targeted next-generation sequencing. Viral DNA was detected altogether in 24/46 (52%) tumor samples, mostly representing anelloviruses (46%). Our findings show frequent persistence of anelloviruses, considered normal virome, in VS. None of the other viruses showed an extensive presence, thereby suggesting insignificant role in VS.

Inner ear barotrauma and inner ear decompression sickness: a systematic review on differential diagnostics.

INTRODUCTION: Inner ear barotrauma (IEBt) and inner ear decompression sickness (IEDCS) are the two dysbaric inner ear injuries associated with diving. Both conditions manifest as cochleovestibular symptoms, causing difficulties in differential diagnosis and possibly delaying (or leading to inappropriate) treatment. METHODS: This was a systematic review of IEBt and IEDCS cases aiming to define diving and clinical variables that help differentiate these conditions. The search strategy consisted of a preliminary search, followed by a systematic search covering three databases (PubMed, Medline, Scopus). Studies were included when published in English and adequately reporting one or more IEBt or IEDCS patients in diving. Concerns regarding missing and duplicate data were minimised by contacting original authors when necessary. RESULTS: In total, 25 studies with IEBt patients (n = 183) and 18 studies with IEDCS patients (n = 397) were included. Variables most useful in differentiating between IEBt and IEDCS were dive type (free diving versus scuba diving), dive gas (compressed air versus mixed gas), dive profile (mean depth 13 versus 43 metres of seawater), symptom onset (when descending versus when ascending or surfacing), distribution of cochleovestibular symptoms (vestibular versus cochlear) and absence or presence of other DCS symptoms. Symptoms of difficult middle ear equalisation or findings consistent with middle ear barotrauma could not be reliably assessed in this context, being insufficiently reported in the IEDCS literature. CONCLUSIONS: There are multiple useful variables to help distinguish IEBt from IEDCS. Symptoms of difficult middle ear equalisation or findings consistent with middle ear barotrauma require further study as means of distinguishing IEBt and IEDCS.

Inner ear decompression sickness in Finland: a retrospective 20-year multicenter study.

Introduction: Inner ear decompression sickness (IEDCS) is a condition from which only a minority of patients recover completely, the majority ending up with mild to moderate residual symptoms. IEDCS has been reported after deep technical dives using mixed breathing gases, and moderate recreational dives with compressed air as the breathing gas. Considering this and the high proportion of technical diving in Finland, a comparison between IEDCS cases resulting from technical and recreational dives is warranted. Methods: This is a retrospective examination of IEDCS patients treated at Hyperbaric Center Medioxygen or National Hyperbaric Centre of Turku University Hospital from 1999 to 2018. Patients were included if presenting with hearing loss, tinnitus, or vertigo and excluded if presenting only with symptoms of middle ear or cerebellar involvement. Patients were divided into technical and recreational divers, based on incident dive. Results: A total of 89 (15.6%) of all DCS patients presented with IEDCS, two-thirds treated during the latter decade. The most common predisposing factors were consecutive days of diving (47.2%), multiple dives per day (53.9%), and factors related to an increase in intrathoracic pressure (27.0%). The symptoms were cochlear in 19.1% and vestibular in 93.3% of cases, symptoms being more common and severe in technical divers. Complete recovery was achieved in 64.5% of technical and 71.4% of recreational divers. Conclusion: The incidence of IEDCS in Finland is increasing, most likely due to changing diving practices. A comprehensive examination should be carried out after an incident of IEDCS in all cases, irrespective of clinical recovery.

Dizzy triathlete-evidence supporting vestibular etiology.

Dizziness during or after the swimming leg is a common complaint among triathletes. We hypothesized that the dizziness is caused by asymmetrical cooling of the vestibular organ. This caloric response is characterized by involuntary eye movements called nystagmus. Altogether, 125 triathletes completed an electronic questionnaire. Fifteen triathletes who had frequently experienced dizziness during the swimming leg agreed to take part in a cold water swimming test. The test comprised two cold water swimming legs, first without earplugs and then with earplugs to prevent a potential caloric response. Eye movements and possible nystagmus were recorded immediately after the swimming legs. A majority (87%, 109/125) of athletes had experienced dizziness during triathlon races or training. Of these, almost all (97%, 106/109) experienced it during or after swimming. Dizziness affected the triathlon performance in half of the athletes with dizziness (50%, 51/102). Fifteen athletes participated in a cold water swimming test. During the first leg (without earplugs), 11/15 athletes (73%) experienced dizziness. Of these, six had nystagmus (55%), four had uncertain nystagmus (36%), and one did not have nystagmus (9%). Only one of these athletes experienced dizziness during the second leg with earplugs. The prevalence of dizziness among triathletes is notable. A large part of the dizziness is likely to be caused by caloric reaction of the vestibular organ. We recommend earplug usage for triathletes suffering from dizziness during the swimming leg.

Development of otology specific outcome measure: Ear Outcome Survey-16 (EOS-16).

PURPOSE: An important outcome measure of patient care is the impact on the patient's health-related quality of life (HRQoL). Current ear-specific HRQoL instruments are designed for one diagnosis and emphasize different subdivisions such as symptoms, hearing problems, psychosocial impact, and the need for care. The optimal length of the recall period has not been studied. For these reasons, a new survey is needed that would cover most chronic ear diseases. METHODS: A preliminary 24-item survey (EOS-24) was created. Untreated adult patients (included n = 186) with one of seven different chronic otologic conditions from all university hospitals in Finland were recruited to respond to EOS-24 and the 15D general HRQoL instrument. The recruiting otologists evaluated the severity of the disease and the disability caused by it. A control group was recruited. Based on the patients' responses in different diagnosis groups, the items were reduced according to pre-defined criteria. The resulting survey was validated using a thorough statistical analysis. RESULTS: The relevance and necessity of the original 24 items were thoroughly investigated, leading to the exclusion of 8 items and the modification of 1. The remaining 16 items were well-balanced between subdivisions and were useful in all seven diagnosis groups, thus constituting the final instrument, EOS-16. The most suitable recall period was three months. CONCLUSIONS: EOS-16 has been created according to the HRQoL survey guidelines with a versatile nationwide patient population. The survey has been validated and can be used for a wide range of chronic ear diseases as a HRQoL instrument.

Intraoperative transimpedance and spread of excitation profile correlations with a lateral-wall cochlear implant electrode array.

A limiting factor of cochlear implant technology is the spread of electrode-generated intracochlear electrical field (EF) leading to spread of neural excitation (SOE). In this study, we investigated the relation of the spread of the intracochlear EF, assessed via transimpedance matrix (TIM), and SOE. A total of 43 consecutive patients (ages 0.7-82 years; 31.0 ± 25.7 years, mean ± SD) implanted with a Cochlear Nucleus CI522 or CI622 cochlear implant with Slim Straight electrode array (altogether 51 ears) were included in the study. Cochlear nerve was visualized for all patients in preoperative imaging and there were no cochlear anomalies in the study sample. The stimulated electrodes were in the basal, middle, and apical parts of the electrode array (electrode numbers 6, 11, and 19, respectively). The stimulation level was 210 CL on average for the TIM measurement and always 230 CL for the SOE measurement. Approximately 90% of the individual TIM and SOE profiles correlated with each other (p < .05; r = 0.61-0.99). Also, the widths of the TIM and SOE peaks, computed at 50% of the maximum height, exhibited a weak correlation (r = 0.39, p = .007). The 50% widths of TIM and SOE were the same only in the apical part of the electrode array; in the basal part SOE was wider than TIM, and in the middle part TIM was wider than SOE (p < .01 and p = .048, respectively). Within each measurement, TIM 50% widths were different between all three parts of the electrode array, while for SOE, only the basal electrode differed from the middle electrode. Finally, the size of the cochlea and the 50% widths of TIM and SOE had the strongest correlation in the middle part of the electrode array (r = -0.63, and -0.37, respectively). Our results suggest that there is a correlation between the spread of intracochlear EF and neural SOE at least in the apical part of the electrode array used in this study, and that larger cochleae are associated with more focused TIM and SOE.

Simultaneous bilateral stapes surgery after follow-up of 13 years.

BACKGROUND: Eighteen patients underwent simultaneous bilateral stapes surgery in 2003-2006. OBJECTIVES: We evaluated the long-term outcomes in this patient group, and assessed their hearing in noise and binaural hearing. MATERIAL AND METHODS: Fifteen patients returned questionnaires concerning their hearing, taste function, and balance. Thirteen patients underwent pure-tone and speech audiogram, Finnish matrix sentence test, video head impulse test, and clinical examination on average 13 years after surgery. RESULTS: We found no significant difference in air- and bone conduction pure-tone average, speech audiometry, and the air-bone gap between the 1-year and the late postoperative visits. One patient had bilaterally a partial loss of the vestibulo-ocular reflex of unknown cause. CONCLUSIONS AND SIGNIFICANCE: The hearing results 13 years after simultaneous bilateral stapes surgery remained good without any significant delayed complications. Simultaneous bilateral stapes surgery is a viable treatment option in selected patients with otosclerosis.

Bilateral Vestibular Hypofunction in Quantitative Head Impulse Test: Clinical Characteristics in 23 Patients.

OBJECTIVE: To explore clinical features of patients with bilateral vestibular hypofunction (BVH) verified in motorized head impulse test (MHIT). MATERIALS AND METHODS: We examined clinical records of 23 adult patients (10 males and 13 females), whose gain of the vestibulo-ocular reflex in the MHIT was bilaterally lowered. Fifteen of 62 unilateral cochlear implant (CI) recipients routinely tested both pre- and postoperatively with the MHIT had BVH. Eight of 198 vestibular outpatients selected to the MHIT due to clinical causes had BVH. Clinical characteristics and a questionnaire regarding current sensations were analyzed. RESULTS: The mean gain±SD in the MHIT was 0.26±0.17 on the right and 0.26±0.14 on the left side. The mean gain in the CI recipients did not differ from that of vestibular outpatients (p>0.05). All outpatients with BVH suffered from oscillopsia, whereas only 46% of CI recipients experienced oscillopsia (p=0.048). Instability was more prominent (p=0.004) and quality of life further decreased (p=0.012) among vestibular outpatients compared with CI patients. Most common etiology for the BVH was meningitis. Other causes were either sudden or progressive loss of labyrinthine function, bilateral Meniére's disease, and ototoxicity. CONCLUSION: BVH is rare even in a specialized clinic. Vestibular outpatients were more disabled than CI recipients with the BVH.

"Wait and scan" management of patients with vestibular schwannoma and the relevance of non-contrast MRI in the follow-up.

Vestibular schwannoma (VS) is a slow-growing benign neoplasm. There has been an evolution in the management of VS from active treatments (microsurgery and stereotactic radiotherapy) to conservative management (wait and scan). Regular MRI scanning is necessary to monitor tumor progression. Conservative management causes significantly less complications and offers a higher quality of life compared with active treatments. The mean growth rate of VS varies from 0.4 to 2.9 mm/year, and spontaneous shrinkage is observed in 3.8 percent of tumors during observation. If significant growth occurs, active treatment is considered. Significant growth is defined as an increase of at least 3 mm in the largest extrameatal diameter in any plane between the first and last available scans. The vestibulocochlear nerve is surrounded by cerebrospinal fluid, which provides natural contrast for MRI; thus, gadolinium may not be needed to detect VS. Specific sequences have high sensitivity, specificity, and accuracy for detection of progression. Hypointense signal in the ipsilateral inner ear fluid might be a useful sign to distinguish VS from meningioma. In this paper, we summarize the current status of research on conservative management and non-contrast MRI for the detection of VS.

Label-free visualization of cholesteatoma in the mastoid and tympanic membrane using CARS microscopy.

OBJECTIVE: The present study aimed to evaluate the possibility of using coherent anti-Stokes Raman spectroscopy (CARS) microscopy to determine the specific molecular morphology of cholesteatoma by detecting the natural vibrational contrast of the chemical bonds without any staining. MATERIALS AND METHODS: Specimens from the mastoid and tympanic membrane with and without cholesteatoma were analyzed using CARS microscopy, two-photon excited fluorescence (TPEF) microscopy, and the second harmonic generation (SHG) microscopy. RESULTS: In cholesteatoma tissues from the mastoid, a strong resonant signal at 2845 cm-1 was observed by CARS, which indicated the detection of the CH2 hydro-carbon lipid bonds that do not generate visible signals at 2940 cm-1 suggestive of CH3 bonds in amino acids. A strong resonant signal at 2940 cm-1 appeared in an area of the same specimen, which also generated abundant signals by TPEF and SHG microscopy at 817 nm, which was suggestive of collagen. In the tympanic membrane specimen with cholesteatoma, a strong resonant signal with corrugated morphology was detected, which indicated the presence of lipids. A strong signal was detected in the tympanic membrane with chronic otitis media using TPEF/SHG at 817 nm, which indicated collagen enrichment. The CARS and TPEF/SHG images were in accordance with the histology results. CONCLUSION: These results suggest the need to develop a novel CARS microendoscope that can be used in combination with TPEF/SHG to distinguish cholesteatoma from inflammatory tissues.

Two-dimensional signal analysis reduced from three-dimensional nystagmus eye movements.

Earlier we developed signal analysis for nystagmus measured from otoneurological patients suffering from vertigo and dizziness. It was based on three rotation directions of the eye: horizontal, vertical and torsional. However, nystagmus frequently appears only in two of the former directions. In order to enable two-dimensional analysis approach on the basis of various pairs from the original three, we designed and implemented an advanced method from our earlier one. These signals can be used to investigate and model the dysfunction of semicircular canals in the inner ear. Nystagmus has to be stimulated for healthy subjects, but in patients it can also be spontaneous. The method developed was tested with the signals of 30 otoneurological patients.

Head tilt is pronounced after an ipsilateral head roll in patients with vestibular schwannoma.

The study aimed to measure utricular function by directly quantifying head tilt in vestibular schwannoma (VS) patients using regular video-oculography (VOG) equipment with integrated head-position sensor, and to correlate the results with patients' symptoms and signs. We recorded head tilting after exclusion of visual cues (static head tilt), and after returning to the centre following lateral head rolls towards each side [subjective head vertical (SHV)]. Head tilt in 43 patients was measured preoperatively and approximately 4 months postoperatively, and compared to that of 20 healthy subjects. Symptoms were assessed with a structured questionnaire. Static head tilt in patients was significantly greater than in controls (1.0° ± 0.9°) preoperatively (1.6° ± 1.5°, p = 0.04) and postoperatively (1.7° ± 1.5°, p = 0.01). Mean SHV in patients was significantly greater than in controls (1.2° ± 1.0°) preoperatively (2.0° ± 1.9°, p = 0.03) and postoperatively (2.5° ± 1.8°, p = 0.001), increasing non-significantly after surgery (p = 0.3). Side-specific SHV after ipsilateral head rolls was significantly greater than after contralateral head rolls preoperatively (2.8° ± 3.3° vs. -0.5° ± 3.0°, p = 0.001) and postoperatively (3.3° ± 3.0° vs. 0.6° ± 3.2°, p < 0.001). The intensity of dizziness increased postoperatively (p = 0.04), but its effect on quality of life remained unchanged. In conclusion, commercial VOG equipment including a head-position sensor allows direct evaluation of head tilt in VS patients. The slight head tilt towards the ipsilateral side becomes most evident after returning from an ipsilateral head roll.

[Bilateral failure of the vestibular system of the inner ear underlying an equilibrium disturbance]. / Molempien sisäkorvan tasapainoelinten vajaatoiminta tasapainohäiriön taustalla.

Bilateral vestibular failure is a fairly rare, though possible cause underlying the symptoms of a dizzy patient. A recognizable oscillopsia symptom makes the diagnosis easier, though confirmation of the diagnosis requires a more precise measurement. The prognosis for recovery is not good, and curative treatment is not available. Diagnosis is nevertheless important with regard to rehabilitation and maintaining of functional capacity.

Recovery of bilateral vestibular loss in Cogan's syndrome--a case report.

OBJECTIVE: To show that both hearing and vestibular loss can be reversible and quantified in Cogan's syndrome. PATIENT AND METHODS: Immunosuppressive medication was continued for more than 6 years in a young woman with Cogan's syndrome. Standard pure tone audiometry (PTA) and speech discrimination score (SDS) for hearing, motorized head impulse test (MHIT) for horizontal, angular vestibulo-ocular reflex, and erythrocyte sedimentation rate were followed serially. RESULTS: The PTA before medication was 59/74 dB, and it stabilized to 13/46 dB in 2 years. The initial SDS of 92/72% improved later to 100/100%, respectively. The gain of the vestibulo-ocular reflex was asymmetric (32%) and decreased to 0.48/0.25 at the beginning. Two years after the treatment, both the asymmetry (7%) and the gain (0.95/0.82) were recovered within normal limits. Erythrocyte sedimentation rate improved from 40 to 5 mm/h. CONCLUSION: Our case report of Cogan's syndrome demonstrates objective, simultaneous deterioration of hearing and vestibular function, which improved and stabilized after the introduction of immunosuppressive medication. The efficacy of immunosuppressive medication on vestibular function may be followed repetitively using MHIT in patients with Cogan's syndrome.

[Nystagmus]. / Nystagmus.

Physiological nystagmus stabilizes gaze during head movements and pathological nystagmus reflects a disorder of the vestibulo-ocular reflex (VOR). Pathological nystagmus appears or strengthens usually during change in head position. Therefore, dizziness or nystagmus associated with head movements is not specific to benign paroxysmal positional vertigo unless it is verified in specific positional test. Peripheral nystagmus decelerates during visual fixation, accelerates when gaze is turned towards the fast phase, does not change direction, and is usually composed of several directional components unlike central nystagmus. The velocity and frequency of the slow phase of nystagmus can be measured with electronystagmography or video-oculography.

Immediate postoperative nystagmus and vestibular symptoms after stapes surgery.

CONCLUSION: Vestibular disturbance is frequent, but mild even immediately after stapes surgery. Vestibular symptoms improved or disappeared quickly, and they did not correlate with nystagmus. Outpatient stapes surgery performed under local anaesthesia is a feasible approach. OBJECTIVE: Vestibular symptoms are common and may prevent outpatient surgery. The time course of vestibular disturbance is unclear, and we aimed to evaluate it immediately after the operation in the recovery room. METHODS: Twenty patients with otosclerosis undergoing stapedotomy were prospectively included in the study. Postoperative symptoms were collected and nystagmus was recorded with video-oculography (VOG) on average 29 min after the surgery. RESULTS: None of the patients had spontaneous nystagmus with gaze fixation. Nine patients (45%) had slow spontaneous horizontal nystagmus (mean slow phase velocity of 1.1°/s) in the primary position without gaze fixation. In seven of these, the nystagmus obeyed Alexander's law. Nine patients (45%) had vestibular symptoms at the end of the surgery, and four patients at the time of VOG recording. Vertigo was experienced immediately after the operation in five, floating sensation in two, and unspecific dizziness in two patients. Vestibular symptoms were mild or moderate in most patients. The occurrence of nystagmus did not correlate with vestibular symptoms (p > 0.05).

Cochlear implantation rarely alters horizontal vestibulo-ocular reflex in motorized head impulse test.

OBJECTIVE: Determine the change in vestibular function in patients receiving a unilateral cochlear implant, and to compare these results with other signs and symptoms. STUDY DESIGN: Prospective cohort study. SETTING: Academic tertiary referral center. PATIENTS: Forty-four adults (mean age, 55 yr; range, 30-76 yr) receiving their first cochlear implant. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Horizontal high-frequency vestibulo-ocular reflex (VOR) was measured using the motorized head impulse rotator preoperatively and twice (on average two and 19 months) postoperatively. VOR gain and asymmetry were calculated (mean ± standard deviation). Symptoms were assessed with a structured questionnaire. RESULTS: Gain on the operated side was 0.77 ± 0.26 preoperatively, 0.75 ± 0.30 in the early and 0.73 ± 0.33 in the late postoperative control, and did not change significantly. Mean asymmetry remained within 9% to 10% in all test occasions. Dizziness symptom score or dizziness-related quality of life score did not change significantly. General quality-of-life score improved significantly from that of preoperative 3.5 ± 1.2 to that of 2.6 ± 1.1 postoperatively (p = 0.01). Subjective hearing scores improved significantly from 4.9 ± 0.3 to 2.4 ± 1.0, respectively (p = 0.0000). Gain was decreased significantly in 4 patients (10%) in the early and in 2 patients (7%) in the late postoperative control. CONCLUSION: Late high-frequency loss of vestibular function or vestibular symptoms is rare but possible after cochlear implantation surgery. This should be taken into account in patient counseling especially when considering bilateral cochlear implant surgery.

Recovery of the horizontal vestibulo-ocular reflex in motorized head impulse test is common after vestibular loss.

CONCLUSION: Decreased horizontal vestibulo-ocular reflex (VOR) gain measured with the motorized head impulse rotator usually recovers at least partially within a few months after sudden unilateral vestibular loss. In addition to traditional evaluation of nystagmus, head impulse test responses provide valuable information on the severity and recovery of vestibular loss. OBJECTIVES: To quantify recovery of vestibular function with the motorized head impulse test in patients with acute unilateral peripheral vestibular loss, and to compare these results with other signs and symptoms. METHODS: We recorded prospectively the horizontal VOR with the motorized head impulse rotator in 30 patients with sudden unilateral vestibular deficit on average 3 days after the onset (early). Twenty patients were measured sequentially on average 3 months later (late). We calculated VOR gain and asymmetry (mean ± standard deviation). RESULTS: The early ipsilesional gain of 0.49 ± 0.21 improved highly significantly to the late gain of 0.79 ± 0.23 (p = 0.0000). The respective asymmetry improved highly significantly from 32 ± 18% to 12 ± 14% (p = 0.0002). Gain or asymmetry recovered at least partially in 80% of the patients. The late high symptom score correlated with low gain (p = 0.043) and high asymmetry (p = 0.018).

Suppression of spontaneous nystagmus during different visual fixation conditions.

Analysis of spontaneous nystagmus is important in the evaluation of dizzy patients. The aim was to measure how different visual conditions affect the properties of nystagmus using three-dimensional video-oculography (VOG). We compared prevalence, frequency and slow phase velocity (SPV) of the spontaneous nystagmus with gaze fixation allowed, with Frenzel's glasses, and in total darkness. Twenty-five patients (35 measurements) with the peripheral vestibular pathologies were included. The prevalence of nystagmus with the gaze fixation was 40%, and it increased significantly to 66% with Frenzel's glasses and regular room lights on (p < 0.01). The prevalence increased significantly to 83% when the regular room lights were switched off (p = 0.014), and further to 100% in total darkness (p = 0.025). The mean SPV of nystagmus with visual fixation allowed was 1.0°/s. It increased to 2.4°/s with Frenzel's glasses and room lights on, and additionally to 3.1°/s, when the regular room lights were switched off. The mean SPV in total darkness was 6.9°/s. The difference was highly significant between all test conditions (p < 0.01). The frequency of nystagmus was 0.7 beats/s with gaze fixation, 0.8 beats/s in both the test conditions with Frenzel's glasses on, and 1.2 beats/s in total darkness. The frequency in total darkness was significantly higher (p < 0.05) than with Frenzel's glasses, and more so than with visual fixation (p = 0.003). The VOG in total darkness is superior in detecting nystagmus, since Frenzel's glasses allow visual suppression to happen, and this effect is reinforced with gaze fixation allowed. Strict control of visual surroundings is essential in interpreting peripheral nystagmus.