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.

Effects of sound source localization of masking sound on perception level of simulated tinnitus.

Tinnitus therapies have been combined with the use of varieties of sound/noise. For masking external sounds, location of the masker in space is important; however, effects of the spatial location of the masker on tinnitus are less understood. We aimed to test whether a masking sound location would affect the perception level of simulated tinnitus. The 4 kHz simulated tinnitus was induced in the right ear of healthy volunteers through an open-type earphone. White noise was presented to the right ear using a single-sided headphone or a speaker positioned on the right side at a distance of 1.8 m for masking the simulated tinnitus. In other sessions, monaurally recorded noise localized within the head (inside-head noise) or binaurally recorded noise localized outside the head (outside-head noise) was separately presented from a dual-sided headphone. The noise presented from a distant speaker and the outside-head noise masked the simulated tinnitus in 71.1% and 77.1% of measurements at a lower intensity compared to the noise beside the ear and the inside-head noise, respectively. In conclusion, spatial information regarding the masking noise may play a role in reducing the perception level of simulated tinnitus. Binaurally recorded sounds may be beneficial for an acoustic therapy of tinnitus.

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.

Traumatic Nonmissile Penetrating Transnasal Anterior Skull Base Fracture and Brain Injury with Cerebrospinal Fluid Leak: Intraoperative Leak Detection and an Effective Reconstruction Procedure for a Localized Skull Base Defect Especially After Coronavirus Disease 2019 Outbreak.

BACKGROUND: Cerebrospinal fluid (CSF) leakage after penetrating skull base injury is relatively rare compared with close head injuries involving skull base fractures. CASE DESCRIPTION: We report the case of a 65-year-old man who had presented with epistaxis and serous rhinorrhea. When he had fallen to the ground near his bee boxes, a garden pole had poked into his right nostril. He had instantly removed the pole from his nostril himself. However, immediately after removal of the pole, he had developed nasal bleeding and serous rhinorrhea. He then drove to our emergency room. Computed tomography showed pneumocephalus with a minor cerebral contusion in the left frontal lobe and a penetrating injury in the left anterior skull base. His CSF leakage had not resolve spontaneously within 1 week after the injury with strict bed rest. We repaired the CSF leakage using a fat (adipose tissue)-on-fascia autograft plug and caulked the defect in the anterior skull base with the fat-on-fascia graft (FFG) plug through the left nostril with endoscopic guidance. The CSF rhinorrhea was successfully controlled. Intranasal local application of fluorescein aided in the detection of the direction of flow of the CSF leakage. CONCLUSIONS: Endonasal endoscopic caulking of a skull base defect using an FFG plug can be useful to treat CSF leakage due to the localized skull base defect, especially in the coronavirus disease 2019 pandemic. It is simple, inexpensive, and timesaving. It requires no special skills nor sophisticated instruments that can cause aerosolization, reducing the risk of infection during the surgery.

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.

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.

Characterisation of N-glycans in the epithelial-like tissue of the rat cochlea.

Membrane proteins (such as ion channels, transporters, and receptors) and secreted proteins are essential for cellular activities. N-linked glycosylation is involved in stability and function of these proteins and occurs at Asn residues. In several organs, profiles of N-glycans have been determined by comprehensive analyses. Nevertheless, the cochlea of the mammalian inner ear, a tiny organ mediating hearing, has yet to be examined. Here, we focused on the stria vascularis, an epithelial-like tissue in the cochlea, and characterised N-glycans by liquid chromatography with mass spectrometry. This hypervascular tissue not only expresses several ion transporters and channels to control the electrochemical balance in the cochlea but also harbours different transporters and receptors that maintain structure and activity of the organ. Seventy-nine N-linked glycans were identified in the rat stria vascularis. Among these, in 55 glycans, the complete structures were determined; in the other 24 species, partial glycosidic linkage patterns and full profiles of the monosaccharide composition were identified. In the process of characterisation, several sialylated glycans were subjected sequentially to two different alkylamidation reactions; this derivatisation helped to distinguish α2,3-linkage and α2,6-linkage sialyl isomers with mass spectrometry. These data should accelerate elucidation of the molecular architecture of the cochlea.