The impact of hearing aids on cognitive function and quality of life in patients with hearing impairment: A cross-sectional study.

OBJECTIVE: Age-related cognitive decline involves a complex set of factors. Among these factors, hearing loss is considered to have a significant impact, but the effect of hearing aid use remains unresolved. The purpose of this study was to evaluate the effects of hearing aid use by simultaneously assessing various factors not only cognitive function but also frailty, anxiety, depression, and quality of life (QOL) in patients with hearing loss. METHODS: The cross-sectional study at the Hearing Aid (HA) Center was conducted between 2020 and 2021. Initially, associations with cognitive function, QOL, frailty, and mental state among patients with hearing loss were examined, irrespective of whether they wore a hearing aid or not. Next, these patients were divided into HA users (using HA for more than 1 year) and non-users (no prior use of HA) with 42 patients in each group. The average age and 6-frequency pure tone audiometry (PTA) was 74.5 ± 6.5 years and 50.6 ± 12.1 dB, respectively. All participants filled out the questionnaire about their life style, medical condition. Mini-Mental State Examination (MMSE) for cognitive function, Hospital Anxiety and Depression Scale for mental state, Short Form 36 version 2 (SF-36v2) for QOL, and Kihon Checklist for frailty were compared between HA users and non-users and correlated with the auditory data (PTA and speech discrimination). RESULTS: Among 84 patients, 40 had an MMSE score ≦26. All eight scores and three components of SF-36v2 were lower than those of the control group. The patients with hypertension were significantly more in HA user than in non-HA user, whereas there was no difference in diabetes, heart attack, stroke and education. Although HA users were older and showed hypertension more their PTA was worse than that of non-users, MMSE scores were not different between the groups. MMSE scores correlated with both PTA and speech discrimination in non-users but not in HA users. However, a multivariate analysis of the effect of HA use on MMSE scores adjusting for age, hypertension, and hearing loss, could not be revealed. The vitality and mental component summary of the SF-36v2 was better in HA users than in non-users. CONCLUSION: Elderly patients with hearing loss were cognitively impaired and had low QOL. HA users showed better QOL score than non-HA user, especially about the mental condition. The absence of a correlation between MMSE scores and hearing loss in HA users suggests the potential use of HA in preventing cognitive decline.

Otic Capsule Demineralization and Hearing Outcome of Stapes Surgery for Osteogenesis Imperfecta in Comparison With Otosclerosis.

OBJECTIVE: To assess the location/number of otic capsule demineralization and hearing outcomes of stapes surgery (SS) for osteogenesis imperfecta (OI) compared with otosclerosis (OS). PATIENTS: This study included 11 and 181 consecutive ears from 6 and 152 patients with OI and OS, respectively. INTERVENTIONS: Demineralization loci observed as hypodense area of the otic capsule were examined using high-resolution computed tomography. All patients underwent SS. MAIN OUTCOME MEASURES: Locations of the hypodense areas were classified into the anterior oval window, anterior internal auditory canal, and pericochlear area. The location/number of hypodense areas and preoperative/postoperative hearing parameters were correlated. Postoperative hearing outcome was evaluated 12 months after surgery. RESULTS: Hypodense area was more frequently observed in OI (9 of 11 ears [81.8%]) than in OS (96 of 181 ears [53.0%]), with significant differences. Multiple sites were involved in 81.8% OI and 18.8% OS patients, showing significant differences. Preoperative air conduction (AC), bone conduction, and air-bone gap (ABG) were 48.9 ± 17.8, 28.0 ± 11.3, and 20.7 ± 8.4 dB, respectively, in OI and 56.2 ± 13.5, 30.5 ± 9.9, and 26.4 ± 9.7 dB, respectively, in OS, demonstrating greater AC and ABG in OS than in OI. Postoperative AC (31.3 ± 20.5 dB), ABG (10.6 ± 10.0 dB), and closure of ABG (12.1 ± 4.7 dB), that is, preoperative ABG minus postoperative ABG of OI, were comparable to those of OS (AC, 30.9 ± 13.3 dB; ABG, 7.0 ± 7.4 dB; closure of ABG, 20.1 ± 11.6 dB). CONCLUSION: OI ears showed more severe demineralization of otic capsule than OS ears. However, favorable hearing outcomes could be obtained through SS for OI and OS ears.

Locations and Predictive Factors of Hypertrophic Pachymeningitis in Otitis Media With Antineutrophil Cytoplasmic Antigen-Associated Vasculitis.

OBJECTIVE: To determine the locations and predictive factors of hypertrophic pachymeningitis (HP), a serious central complication of otitis media with antineutrophil cytoplasmic antigen-associated vasculitis (OMAAV). STUDY DESIGN: Retrospective study. SETTING: University hospital. PATIENTS: Thirty-six patients with OMAAV underwent contrast-enhanced magnetic resonance imaging (CE-MRI) of the brain, of whom 18 and 18 had or did not have HP, respectively. MAIN OUTCOME MEASURES: The location of HP lesions, which were detected as dural thickening on CE-MRI, was reviewed. Clinical characteristics, laboratory data, and audiometric data before treatment were correlated with the occurrence of HP. RESULTS: HP lesions were most frequently observed in the middle cranial fossa, followed by the internal auditory meatus, tentorium cerebelli, and posterior cranial fossa, which were adjacent to the middle ear primary lesion. Headache was more frequently observed in patients with HP than without HP. The neutrophil-to-lymphocyte ratio (NLR) of the HP (+) group (median, 3.91; interquartile range, 2.50-6.06) was higher than that of the HP (-) group (median, 2.40; interquartile range, 2.01-3.03). The area under the receiver operating characteristic curve for the NLR was 0.741. An NLR of 3.46 had the highest sensitivity (61.1%) and specificity (94.4%) for predicting HP. Other markers of systemic inflammation and comorbidities of systemic diseases were not different between the groups. CONCLUSIONS: HP may not be a systemic manifestation of OMAAV but may be related to local lesions in the adjacent brain. A high NLR may be a predictive factor for comorbid HP. Therefore, CE-MRI should be considered for patients with a high NLR.

Optimal Diagnostic Criteria and a Staging System for Otogenic Skull Base Osteomyelitis.

Objective Diagnostic criteria for otogenic skull base osteomyelitis (SBO) have been conflicting among researchers. We aimed to propose clinically useful diagnostic criteria and a staging system for otogenic SBO that is associated with infection control and mortality. Design The present study is designed as a retrospective one. Setting This study was conducted at the University Hospital. Participants Thirteen patients with otogenic SBO who met the novel rigorous diagnostic criteria consisted of symptomatic and radiological signs on high-resolution computed tomography (HRCT) and magnetic resonance imaging (MRI). Simple refractory external otitis was not included. A staging system according to disease extent revealed by HRCT and MRI is proposed: lesions limited to the temporal bone (stage 1), extending to less than half (stage 2), exceeding the midline (stage 3), and extending to the whole of the clivus (stage 4). All patients received long-term antibiotic therapy. Patients were divided into infection-uncontrolled or -controlled groups based on symptoms, otoscopic findings, and C-reactive protein level at the last follow-up. The mean follow-up period was 27.7 months. Main Outcome Measures Possible prognostic factors, such as immunocompromised status and symptoms, including cranial nerve palsy, pretreatment laboratory data, and treatments, were compared between the infection-uncontrolled and -controlled groups. Disease stages were correlated with infection control and mortality. Results The infection-uncontrolled rate and mortality rate were 38.5 and 23.1%, respectively. There were no significant differences in possible prognostic factors between the infection-uncontrolled and -controlled groups. HRCT-based stages significantly correlated with infection control and mortality. Conclusion We proposed here the clinically useful diagnostic criteria and staging systems that can predict infection control and prognosis of otogenic SBO.

Test Batteries and the Diagnostic Algorithm for Chronic Vestibular Syndromes.

Objective: To develop a diagnostic algorithm for chronic vestibular syndromes by determining significant items that differ among diagnoses. Methods: Two hundred thirty-one patients with chronic vestibular syndromes lasting for >3 months were included. Full vestibular tests and questionnaire surveys were performed: bithermal caloric test, cervical and ocular vestibular-evoked myogenic potential assessment, video head impulse test (vHIT), posturography, rotatory chair test, dizziness handicap inventory, hospital anxiety and depression scale (HADS), and Niigata persistent postural-perceptual dizziness (PPPD) questionnaire (NPQ). Differences in each item of the vestibular tests/questionnaires/demographic data were tested among the diagnoses. A receiver operating characteristic (ROC) curve was created for the significant items. The value that provided the best combination of sensitivity/specificity on the ROC curve was adopted as a threshold for diagnosing the targeted disease. Multiple diagnostic algorithms were proposed, and their diagnostic accuracy was calculated. Results: There were 92 patients with PPPD, 44 with chronic dizziness due to anxiety (CDA), 31 with unilateral vestibular hypofunction (UVH), 37 with undifferentiated dizziness (UD), and 27 with other conditions. The top four diagnoses accounted for 88% of all chronic vestibular syndromes. Five significant items that differed among the four diseases were identified. The visual stimulation and total NPQ scores were significantly higher in the patients with PPPD than in those with UVH and UD. The percentage of canal paresis (CP %) was significantly higher in the patients with UVH than in those with PPPD, CDA, and UD. The patients with CDA were significantly younger and had higher anxiety scores on the HADS (HADS-A) than those with UVH and UD. Moreover, catch-up saccades (CUSs) in the vHIT were more frequently seen in the patients with UVH than in those with PPPD. The most useful algorithm that tested the total and visual stimulation NPQ scores for PPPD followed by the CP%/CUSs for UVH and HADS-A score/age for CDA showed an overall diagnostic accuracy of 72.8%. Conclusions: Among the full vestibular tests and questionnaires, the items useful for differentiating chronic vestibular syndromes were identified. We proposed a diagnostic algorithm for chronic vestibular syndromes composed of these items, which could be useful in clinical settings.

Head Roll-Tilt Subjective Visual Vertical Test in the Diagnosis of Persistent Postural-Perceptual Dizziness.

OBJECTIVE: To examine the validity of head roll-tilt subjective visual vertical (HT-SVV) in diagnosing persistent postural-perceptual dizziness (PPPD). STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: Sixty-one patients with PPPD, 10 with unilateral vestibular hypofunction (UVH), and 11 with psychogenic dizziness (PD), showing chronic vestibular symptoms for >3 months. INTERVENTIONS: Head-tilt perception gain (HTPG, i.e., mean perceptual gain [perceived/actual tilt angle]) during right or left head tilt of approximately 30° (HT-SVV) and conventional head-upright SVV (UP-SVV) were measured. Bithermal caloric testing, cervical and ocular vestibular-evoked myogenic potentials (cVEMP and oVEMP), and posturography were conducted. MAIN OUTCOME MEASURES: Multiple comparisons were performed for the HT-SVV and other vestibular tests among the disease groups. A receiver operating characteristic curve was created to predict PPPD using HTPG. RESULTS: HTPG was significantly greater in the PPPD group than in the UVH and PD groups. There were no significant differences in UP-SVV, cVEMP, oVEMP, and posturography (foam ratio and Romberg ratio on foam) among the disease groups, while the UVH group had the highest canal paresis compared to the other two groups. The area under the curve of the receiver operating characteristic curve for predicting PPPD was 0.764, and the HTPG value of 1.202 had a specificity of 95.2% for diagnosing PPPD. CONCLUSIONS: While conventional vestibular tests including UP-SVV, VEMPs, and posturography did not show abnormalities in PPPD, high HTPG in the HT-SVV test, an excessive perception of head tilt, can be a specific marker for discriminating PPPD from other chronic vestibular diseases.

Synbindin Downregulation Participates in Slit Diaphragm Dysfunction.

INTRODUCTION: Synbindin, originally identified as a neuronal cytoplasmic molecule, was found in glomeruli. The cDNA subtractive hybridization technique showed the mRNA expression of synbindin in glomeruli was downregulated in puromycin aminonucleoside (PAN) nephropathy, a mimic of minimal-change nephrotic syndrome. METHODS: The expression of synbindin in podocytes was analyzed in normal rats and 2 types of rat nephrotic models, anti-nephrin antibody-induced nephropathy, a pure slit diaphragm injury model, and PAN nephropathy, by immunohistochemical analysis and RT-PCR techniques. To elucidate the function of synbindin, a gene silencing study with human cultured podocytes was performed. RESULTS: Synbindin was mainly expressed at the slit diaphragm area of glomerular epithelial cells (podocytes). In both nephrotic models, decreased mRNA expression and the altered staining of synbindin were already detected at the early phase when proteinuria and the altered staining of nephrin, a key molecule of slit diaphragm, were not detected yet. Synbindin staining was clearly reduced when severe proteinuria was observed. When the cultured podocytes were treated with siRNA for synbindin, the cell changed to a round shape, and filamentous actin structure was clearly altered. The expression of ephrin-B1, a transmembrane protein at slit diaphragm, was clearly lowered, and synaptic vesicle-associated protein 2B (SV2B) was upregulated in the synbindin knockdown cells. CONCLUSION: Synbindin participates in maintaining foot processes and slit diaphragm as a downstream molecule of SV2B-mediated vesicle transport. Synbindin downregulation participates in slit diaphragm dysfunction. Synbindin can be an early marker to detect podocyte injury.

Subtypes of Persistent Postural-Perceptual Dizziness.

Background: Persistent postural-perceptual dizziness (PPPD) is a persistent chronic vestibular syndrome exacerbated by upright posture/walking, active or passive motion, and exposure to moving or complex visual stimuli. PPPD has four precursors: phobic postural vertigo, space-motion discomfort, visual vertigo, and chronic subjective dizziness. These four diseases share clinical features that form the basis of the diagnostic criteria for PPPD. Semiological similarities do not necessarily mean that PPPD is a single entity. However, if PPPD is not a single disorder but just a composite of four precursors, it may be subdivided according to the characteristics of each precursor. Objective: To test whether PPPD is a single disorder, we attempted a subtyping of PPPD. Methods: One-hundred-eight untreated patients with PPPD were enrolled in the study, who filled out the Niigata PPPD Questionnaire (NPQ) that consists of 12 questions on exacerbating factors for PPPD. A factor analysis of the patients' answers to the NPQ and a subsequent cluster analysis of the patients with PPPD using factors revealed by the factor analysis were performed. To validate our cluster classification, cluster differences were assessed using analysis of variance. Multiple comparison analyses were performed on demographical data, precipitating diseases, the Dizziness Handicap Inventory, the Hospital Anxiety and Depression Scale, and several vestibular tests to characterize each cluster. Results: Factor analysis revealed three underlying factors among the exacerbating factors in the NPQ. Exacerbation by visual stimuli (visual factor) accounted for 47.4% of total variance in the questionnaire. Exacerbation by walking/active motion (active-motion factor) and by passive motion/standing (passive-motion/standing factor) accounted for 12.0 and 7.67% of variance, respectively. Cluster analysis revealed three clusters: the visual-dominant subtype (n = 49); the active motion-dominant subtype (n = 20); and the mixed subtype (n = 39). The patients in the active motion-dominant subtype were significantly older than those in the visual-dominant subtype. There were no significant differences among the subtypes in other demographical data or conventional vestibular tests. Conclusions: The most common main exacerbating factor of PPPD was the visual factor. PPPD may be categorized into three subtypes. Conventional vestibular tests failed to point the characteristics of each subtype.

Patient-specific 3D-printed Model-assisted Supracochlear Approach to the Petrous Apex.

OBJECTIVE: To present a case of pediatric cholesteatoma that invaded the petrous apex (PA) and discuss the usefulness of preoperative three-dimensional (3D) surgical simulation on a personal computer (PC) and patient-specific 3D printed model-assisted surgery. PATIENT: A 5-year-old boy with congenital cholesteatoma underwent a planned two-stage canal wall up mastoidectomy. The cholesteatoma had invaded the PA from a small space anterior to the superior semicircular canal (SSCC). During the removal of this lesion in the first surgery, the tip of a 1-mm round knife broke off and fell into the PA. The surgeon could not remove it, as it was thought that opening the space might damage the SSCC and the facial nerve (FN). INTERVENTION: Before the second surgery, a preoperative 3D surgical simulation on a PC was performed, and an approach to the PA via the triangle surrounded by the SSCC, FN, and middle cranial fossa, namely, the supracochlear approach, was discovered. A patient-specific 3D-printed model, which had been drilled to make each surface of the triangle including the SSCC, FN, and middle cranial fossa visible in the PC simulation surgery, was then created and a 3D-printed model-assisted surgery was planned. RESULTS: By placing the sterilized patient-specific 3D model close to the surgical field, the cholesteatoma and iatrogenic foreign body could be successfully removed from the PA without damaging the important surrounding structures. CONCLUSIONS: Preoperative 3D surgical simulations and intraoperative patient-specific 3D-printed model-assisted surgeries are new, powerful tools that aid in performing challenging surgeries on temporal bones.

Tympanic membrane findings of otitis media with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV).

OBJECTIVE: Otitis media with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) is characterized by adult otitis media refractory to conventional treatments. OMAAV is either an aural manifestation of existing ANCA-associated vasculitis (AAV) or an initial aural manifestation of AAV. OMAAV occasionally causes an irreversible profound sensorineural hearing loss that may require a cochlear implant even in the latter case. In such a case, prompt diagnosis of OMAAV is important but sometimes difficult. When diagnosing OMAAV, repetitive otitis media with effusion (OME) in adults is the most difficult differential diagnosis. Precise evaluation of tympanic membrane (TM) findings would help to achieve a prompt diagnosis. The objective of this study was to discriminate OMAAV from adult OME based on tympanic TM findings. METHODS: 10 with OMAAV and 10 with adult OME were included. We established a scoring system of OMAAV tympanic membrane (SCOT) to evaluate TM findings of OMAAV consisted of following three characteristic findings: thickening of pars tensa, vasodilation of pars tensa, and posterior wall swelling. Each TM finding in OMAAV and OME was scored from 0 to 3 by 20 otolaryngologists who never knew the diagnosis. Reliability of the scoring system in terms of consistency between examiners was evaluated by intraclass correlation coefficients (ICC). Validity was tested by comparing the TM scores between OMAAV and OME and by the area under the curve (AUC) of receiver operating characteristic (ROC) curve to discriminate OMAAV from OME. Correlations between the TM scores and various systemic markers of OMAAV including white blood cell count, C-reactive protein, myeloperoxidase-anti-neutrophil cytoplasmic antibody, and Birmingham Vasculitis Activity Score were examined. RESULTS: The ICC of each score was over 0.95. Each of and the total TM scores were significantly higher in OMAAV than in OME. AUC of ROC curve was 0.9134. The cut-off value set at 2 points had the best combination of sensitivity (93.0%) and specificity (74.0%) to distinguish OMAAV from OME. No significant correlations were found between the total score of SCOT and systemic markers. However, the total score of SCOT significantly correlated with the average hearing level of both air (p = 0.021) and bone conductions (p = 0.032). CONCLUSION: Reliability and validity of SCOT in discriminating OMAAV from adult OME, the most difficult differential diagnosis, were demonstrated, suggesting that SCOT would be useful to make an early diagnosis of OMAAV. Correlation of SCOT with hearing level suggests that SCOT is also useful to evaluate disease status of OMAAV.

Nerve Integrity Monitor Responses to Direct Facial Nerve Stimulation During Facial Nerve Decompression Surgery Can Predict Postoperative Outcomes.

OBJECTIVE: To test whether the threshold of nerve integrity monitor (NIM) responses during facial nerve decompression surgery can predict the postoperative outcome. STUDY DESIGN: Retrospective study. SETTING: University hospital. PATIENTS: Twenty peripheral facial palsy patients who underwent transmastoid decompression surgery. INTERVENTION: During decompression surgery, thresholds of NIM responses were measured via direct facial nerve stimulation at three sites: the geniculate ganglion (GG), the second genu (2 G), and the stylomastoid foramen. MAIN OUTCOME MEASURES: Facial nerve function was evaluated before and 6 months after surgery using the Yanagihara grading score (maximum score = 40 points). Complete recovery was defined as an improvement of the grading score to ≥ 36 points without synkinesis. Variables including age, sex, disease (Bell's palsy or Ramsay Hunt syndrome), time after onset, Yanagihara grading score, and electroneurography before surgery, and the thresholds of NIM responses during surgery were compared in the complete and incomplete recovery groups. NIM responders were defined as those exhibiting a NIM response of < 1.5 mA at any site. Postoperative Yanagihara grading scores in NIM responders and NIM nonresponders were compared. RESULTS: No variables differed significantly in the complete and incomplete recovery groups before surgery. NIM response thresholds in the complete recovery group at the GG and the 2nd G were significantly lower than the corresponding thresholds in the incomplete recovery group. The postoperative Yanagihara grading scores of NIM responders were significantly better than those of NIM nonresponders. CONCLUSION: NIM responses to intraoperative direct facial nerve stimulation were useful for predicting outcomes after decompression surgery.

Is Vestibular Meniere's Disease Associated With Endolymphatic Hydrops?

Background: Vestibular Meniere's disease (American Academy of Ophthalmology and Otolaryngology, 1972) also known as possible Meniere's disease (American Academy of Otolaryngology Head and Neck Surgery, 1995) or vestibular type of atypical Meniere's disease (V-AMD) (Japan Society for Equilibrium Research, 2017) is characterized by an episodic vertigo without hearing loss. Though named as Meniere's disease (MD), this entity may not be caused solely by endolymphatic hydrops (EH). Objective: To estimate the role of EH in vestibular Meniere's disease in comparison with definite Meniere's disease. Methods: Thirty patients with unilateral definite MD and 16 patients with vestibular Meniere's disease were included. Those who met the criteria for definite or probable vestibular migraine were excluded. All patients underwent vestibular assessments including inner ear MRI 4 h after intravenous gadolinium injection, bithermal caloric testing, directional preponderance of vestibulo-ocular reflex in rotatory chair test, cervical- and ocular-vestibular evoked myogenic potential, stepping test, dizziness handicap inventory (DHI), and hospital anxiety and depression scale (HADS). All above tests and frequency/duration of vertigo spells were compared between vestibular Meniere's disease and MD. Results: Even in unilateral MD, cochlear and vestibular endolymphatic hydrops (c-, v-EH) were demonstrated not only in the affected side but also in the healthy side in more than half of patients. Positive rate of v-EH in vestibular Meniere's disease (68.8%) was as high as that of MD (80%). In vestibular Meniere's disease, the number of bilateral EH was higher in the vestibule (56.3%) than that in the cochlea (25.0%). There were no differences in vestibular tests and DHI between vestibular Meniere's disease and MD; however, the frequency of vertigo spells was lower in vestibular Meniere's disease (p = 0.001). The total HADS score in the MD group was significantly higher than that in the vestibular Meniere's disease group. Conclusions: MD is a systemic disease with bilateral involvement of inner ears. V-EH is a major pathophysiology of vestibular Meniere's disease, which would precede c-EH in the development of vestibular Meniere's disease, a milder subtype of MD. MRI is useful for differentiating MD from other vertigo attacks caused by different pathologies in bringing EH into evidence.

Age-related Hearing Loss Is Strongly Associated With Cognitive Decline Regardless of the APOE4 Polymorphism.

OBJECTIVE: To examine the association between hearing impairment and cognitive decline and to identify possible risk factors for presbycusis. STUDY DESIGN: Cross-sectional survey in prospective cohort study. SETTING: University hospital. PATIENTS: A total of 322 participants aged >60 years, for whom all the below data were available, were enrolled in the study. There were 168 females and 154 males with a median age of 71 years (range: 60-89 yrs). INTERVENTIONS: PROST (Project in Sado for Total Health), a medical database in Sado island Japan, was analyzed. MAIN OUTCOME MEASURES: Data on pure-tone audiometry, mini-mental state examination (MMSE), polymorphism of apolipoprotein E4 (ApoE4), diabetes mellitus, hypertension, smoking, and alcohol consumption were extracted. Hearing impairment was defined as an average frequency between 0.25 and 8 kHz that exceeded 30 dB. Multivariate analysis was used to identify which of the above factors could predict the hearing impairment. Hearing threshold of each Hz was compared between the ApoE4 (+/+), (+/-), and (-/-) groups. RESULTS: Among various factors, only low MMSE scores (<24) showed significant association with hearing impairment. There were no differences in the hearing threshold of all frequencies between ApoE status groups. CONCLUSIONS: Hearing impairment was associated with low MMSE sores, regardless of the ApoE4 status. If ApoE4 status would be a common upstream predictor for both the hearing and cognitive impairment, hearing threshold would be related to ApoE4 status. However, these results may suggest that hearing impairment may be causally related to the cognitive dysfunction, perhaps via the cognitive load mechanisms.

A Validated Questionnaire to Assess the Severity of Persistent Postural-Perceptual Dizziness (PPPD): The Niigata PPPD Questionnaire (NPQ).

OBJECTIVE: To establish a questionnaire to diagnose and assess the severity of persistent postural-perceptual dizziness (PPPD). STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Fifty PPPD patients and 50 consecutive control patients with other vestibular disorders. INTERVENTIONS: Patients answered questions on three exacerbating factors of PPPD (upright posture/walking, movement, and visual stimulation), and each factor was evaluated using four questions scoring the severity from 0 (none) to 6 (unbearable). Somatic and psychological distress was evaluated by the Visual Analog Scale (VAS) and the Hospital Anxiety and Depression Scale (HADS), respectively. MAIN OUTCOME MEASURES: The questionnaire's reliability was tested by Cronbach's alpha, and it was validated by examining the differences in the questionnaire's scores between PPPD patients and controls. The area under the curve (AUC) of the receiver operating characteristic curve for each factor was calculated. RESULTS: Cronbach's alpha coefficient was >0.8 for all factors, except the movement factor. There were no significant differences in the VAS and HADS scores between the two groups. However, the combined and individual questionnaire scores for each factor were higher in PPPD patients than in controls, indicating the questionnaire's high validity. The AUC was widest for the visual stimulation factor (0.830), and a score of 9 (full score 24) had the best sensitivity (82%) and specificity (74%) for discriminating PPPD patients from controls. CONCLUSIONS: We developed a questionnaire that exhibited high reliability and validity in evaluating PPPD severity. The visual stimulation factor may be the most characteristic among the three exacerbating factors.

Surgical treatment of enterovirus D68 brainstem encephalitis-induced dysphagia.

Cluster of acute flaccid paralysis and cranial nerve dysfunction was associated with a 2014 outbreak of enterovirus D68 (EV-D68) respiratory illness in US. We describe a 33 year-old male patient of refractory dysphagia due to EV-D68-induced brainstem encephalitis successfully treated by surgery. Following acute upper respiratory tract infection, he developed dysphagia and bilateral facial paralysis. A coughing reflex was readily produced when the laryngopharyngeal fiberscope touched the epiglottis, however, water infusion induced only very weak and slow swallowing reflex, suggesting that only motor component was impaired but sensory function was preserved during swallowing. Despite eight months-conservative rehabilitations, Food Intake Level Scale (FILS) remained level 4. Therefore, corrective surgeries including cricopharyngeal myotomy, laryngeal suspension, and pharyngeal flap were performed. Thirty-six days after surgery, FILS rapidly and dramatically improved to level 8. This is the first report describing a successful surgical intervention for EV-D68-induced refractory dysphagia. Surgical treatment was suitable for EV-D68-induced dysphagia, perhaps because sensory function was preserved and only motor disturbance was present during the pharyngeal stage of swallowing.