The journal of international advanced otology最新文献

Background: Idiopathic sudden sensorineural hearing loss is a disabling condition that lowers the quality of life specifically in older adults living alone. It is crucial to determine the outcome of the disease and to offer early treatment to prevent isolation caused by hearing impairment in this population. The objective of our study was to investigate whether the initial cochlear nerve thickness may predict the outcome of hearing recovery in older adults with idiopathic sudden sensorineural hearing loss.

Methods: The study population was composed of older adults that were referred with idiopathic sudden sensorineural hearing loss in 1 ear. Long-term audiological data of the cohort were analyzed according to Siegel's criteria on hearing recovery and were grouped according to complete recovery or treatment failure. Cochlear nerve diameters of the diseased and safe ears of each group, measured on reformatted images on magnetic resonance imaging, at the fundus, in the mid-internal acoustic canal, and at the entry point into the Pons were compared in each group and between groups.

Results: Mean cochlear nerve diameter was significantly larger in the recovered older adults (1.11 ± 0.27 mm) than in the non-recovered adults (0.94 ± 0.21 mm) at the mid-internal acoustic canal (Student's t-test, P < .05). Cochlear nerve thickness at mid-internal acoustic canal (≤0.8 mm) sensitivity for recovery failure was 89% and displayed an odds ratio 5.333, 95% CI (1.000-28.435).

Conclusion: Cochlear nerve thickness in mid-internal acoustic canal in non-recovered older adults with idiopathic sudden sensorineural hearing loss is significantly thinner than the completely recovered group. Older adults with mid-internal acoustic canal cochlear nerve greatest diameter cutoff level of ≤0.8 mm are 5.33 times more exposed to recovery failure.

Background: Temporary conductive hearing loss due to vernix accumulation in the external ear canal may lead to a false-positive result in newborn hearing screening tests. The aim of this study was to evaluate whether ear examination and intervention may reduce the false-positive rate prior to hospital discharge.

Methods: A case series of 42 newborns who failed initial otoacoustic emissions screening were studied in our institution between May and December 2020.

Results: During the study period, a total of 735 neonates (1470 ears) were screened by otoacoustic emissions in our hospital. Forty-two newborns who failed otoacoustic emissions were included in our study. They constituted 3.9% (n=58 ears) of the total number of ears screened. Forty-four ears (75.9%) passed and 14 ears (24.1%) failed otoacoustic emissions rescreening performed shortly following vernix cleaning. Twelve of the remaining 14 ears passed at 10-day rescreening. The remaining 2 ears presented true bilateral hearing loss. During the study period, the general false-positive rate decreased from 56/735 (7.61%) to 12/735(1.63%) (P < .00001).

Conclusion: Cleaning the vernix of infants who failed otoacoustic emissions prior to hospital discharge lowers the false-positive rate of universal neonatal hearing screening. We may assume that vernix cleaning will reduce significant healthcare workload, costs of unnecessary investigations, as well as parental anxiety.

Background: Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) presents an unpredictable and uneven clinical development of cerebellar ataxia, neuropathy, and vestibular areflexia. The aim of this study is to report the variability of vestibular test results in genetically confirmed patients with cerebellar ataxia, neuropathy, and vestibular areflexia syndrome.

Methods: Caloric testing, video head impulse test (vHIT), and rotatory chair testing were performed in 7 patients who presented pathogenic repeat expansions in the replication factor complex unit 1 gene related to cerebellar ataxia, neuropathy, and vestibular areflexia syndrome.

Results: Reduced vestibulo-ocular reflex (VOR) gain was observed in 100% of the patients in rotatory chair testing. Three of them had bilateral areflexia in caloric testing while 2 showed unilateral hypofunction and 2 had no alterations in the test. Only 1 patient had bilateral abnormal vHIT with gains under 0.6 in both ears.

Conclusion: Genetic testing allows an early diagnosis of cerebellar ataxia, neuropathy, and vestibular areflexia syndrome, whereby the vestibular system may be affected to different degrees. Rotatory chair testing has a higher sensitivity for the detection of vestibular hypofunction in these patients. Caloric testing can provide additional information. vHIT might underdiagnose patients with mild-to-moderate vestibulopathy.

Carbon monoxide (CO) can cause "irreversible" severe-to-profound sensorineural hearing loss. However, there are few reports of detailed hearing test results. Here, we report a case of acute sensorineural hearing loss caused by acute CO poisoning with partial hearing recovery, evaluated by a detailed hearing examination. A 25-year-old woman was brought to the emergency department for attempted suicide. On admission, her consciousness was impaired, and she was treated for severe CO poisoning, including using hyperbaric-oxygen therapy. After regaining consciousness, symptoms of hearing loss and tinnitus were discovered, and a detailed audiological examination revealed bilateral hearing loss, suggesting cochlear damage. Steroids were systemically administered, and her hearing impairment was partially resolved. Sensorineural hearing loss caused by acute CO poisoning includes cochlear pathology and may be partially treatable. The early evaluation of hearing in patients with severe CO poisoning is advisable for early treatment.

Recent breakthroughs in our understanding of sensorineural hearing loss etiology have encouraged the identification of novel hearing therapeutics, paving the way for precision hearing medicine. Critical to this field is the curation of health resources on hearing data. A systematic review of the literature was conducted to map existing (inter)national and regional datasets that include hearing data to inform the development of future hearing repositories. Systematic literature review was performed adhering to Preferred Reporting Items for Systematic Review and MetaAnalysis recommendations. Databases, including those from gray literature, were searched to identify publications reporting on phenotypic and/ or genotypic hearing data in May 2019. The databases reviewed were Medline, PubMed, Embase databases, and Google Scholar. Publications on local datasets were excluded. All hearing datasets identified in the screening process were noted. For each dataset, geography, context, objective, period of time run, numbers and demographics of participants, genomic data, hearing measures and instruments used were extracted and cataloged. One hundred and eighty-eight datasets were identified, containing hearing data on populations ranging from 100 to 1.39 million individuals, and all extracted data have been cataloged. This searchable resource has been made accessible online. This unique catalog provides an overview of existing datasets that contain valuable information on hearing. This can be used to inform the development of national and international patient data repositories for hearing loss and guide strategic collaboration between key stakeholder groups, pivotal to the delivery and development of sensorineural hearing loss precision diagnostics and treatments.

Background: The goal of this study was to compare video head impulse test, video-oculography, and clinical balance test changes induced by ethanol consumption, in order to acquire a model for acute bilateral vestibular syndrome.

Methods: Four healthy adult men and 5 healthy adult women were recruited as volunteers in the study. Initial video head impulse test, videooculography, and clinical balance test examinations were made. Participants proceeded to drink standard alcohol doses until a maximum of 1.2‰ breath alcohol concentration was reached. Video head impulse test and clinical balance tests were repeated at every 0.2‰ breath alcohol concentration interval and at the final 1.0-1.2‰ breath alcohol concentration range. Video-oculography examinations were repeated at 1.0- 1.2‰ breath alcohol concentration.

Results: Decrease in mean vestibulo-ocular gain at 60 ms between the 0‰ and 1.0-1.2‰ was 0.16 on the left side (P < .05) and 0.16 on the right side (P < .05). A borderline abnormality (mean 0.79/0.82) (left/right) was observed in vestibulo-ocular gain at the highest breath alcohol concentration. Corrective saccades increased significantly in amplitude and latency. There was a statistically significant, symmetrical decrease in video-oculography smooth pursuit gain. Saccade latency increased but statistically significantly only with right-sided cycles. Saccade accuracy remained constant. Optokinetic reflex gain showed significant decrease. Romberg's test was performed with normal results initially and at 1.0-1- 2‰ breath alcohol concentration.

Conclusion: Ethanol produces a symmetrical loss in vestibulo-ocular gain measured by video head impulse test. Ethanol also decreases smooth eye pursuit gain and increases pro-saccade latency. Similar findings can be made in vestibular disorders as well as in cerebellar dysfunction. Central pathology should be ruled out in acute bilateral vestibular syndrome.

Background: Petrous temporal bone cone-beam computed tomography scans help aid diagnosis and accurate identification of key operative landmarks in temporal bone and mastoid surgery. Our primary objective was to determine the accuracy of using a deep learning convolutional neural network algorithm to augment identification of structures on petrous temporal bone cone-beam computed tomography. Our secondary objective was to compare the accuracy of convolutional neural network structure identification when trained by a senior versus junior clinician.

Methods: A total of 129 petrous temporal bone cone-beam computed tomography scans were obtained from an Australian public tertiary hospital. Key intraoperative landmarks were labeled in 68 scans using bounding boxes on axial and coronal slices at the level of the malleoincudal joint by an otolaryngology registrar and board-certified otolaryngologist. Automated structure identification was performed on axial and coronal slices of the remaining 61 scans using a convolutional neural network (Microsoft Custom Vision) trained using the labeled dataset. Convolutional neural network structure identification accuracy was manually verified by an otolaryngologist, and accuracy when trained by the registrar and otolaryngologist labeled datasets respectively was compared.

Results: The convolutional neural network was able to perform automated structure identification in petrous temporal bone cone-beam computed tomography scans with a high degree of accuracy in both axial (0.958) and coronal (0.924) slices (P < .001). Convolutional neural network accuracy was proportionate to the seniority of the training clinician in structures with features more difficult to distinguish on single slices such as the cochlea, vestibule, and carotid canal.

Conclusion: Convolutional neural networks can perform automated structure identification in petrous temporal bone cone-beam computed tomography scans with a high degree of accuracy, with the performance being proportionate to the seniority of the training clinician. Training of the convolutional neural network by the most senior clinician is desirable to maximize the accuracy of the results.

Background: This study aimed to discuss 3 cases of congenital cholesteatoma located posterior to the sigmoid sinus, with no/minimal involvement of mastoid, and compare them with cases presented in the literature to better define this rare entity.

Methods: Retrospective chart analysis of 3 congenital cholesteatomas located posterior to the sigmoid sinus treated surgically in 2 skull-base centers and literature review. Though congenital cholesteatoma can arise outside the middle ear, only a few cases presenting in the retrosigmoid occipital bone have been described earlier.

Results: In all 3 patients, there was a delay in the presentation, as symptoms were nonspecific or lacking, leading in 1 case to severe complications. Computed tomography and magnetic resonance imaging, especially diffusion-weighted imaging scans, allowed accurate diagnosis and surgical planning. Surgery happened to be challenging due to the tight adherence of the cholesteatoma to the thinned dural surface. Complete excision was achieved in all the cases.

Conclusion: Congenital cholesteatoma located posterior to the sigmoid sinus is a rare entity and is even more exceptional after a critical review of the literature. Complete excision is quintessential to prevent intradural extension or infection. The most important surgical issue is the management of the posterior fossa dura and the sigmoid sinus. We recommend meticulous dissection with slow peeling of the epithelial lining from the dura. Bipolar coagulation of the dura may help in avoiding recidivism. Moreover, cerebrospinal fluid (CSF) leak during dissection has to be avoided as long as possible, because the loss of tension of the already thinned dura makes its peeling particularly difficult.

Background: Through the clinical analysis of 4 clinically confirmed cases of delayed gadolinium enhancement of Ramsay Hunt syndrome 3D-Fluid Attenated Inversion Recovery'and 'T1volume interpolated body examination (3D-FLAIR and T1VIBE) sequences, the more suitable sequences and pathologically damaged tissue sites of deep tissues of Ramsay Hunt syndrome by magnetic resonance imaging gadolinium enhancement were preliminarily explored.

Methods: From October 2020 to March 2021, 4 clinically confirmed patients with Ramsay Hunt syndrome, 2 males and 2 females, aged 27-63, were continuously collected in the hospital otology clinic. Siemens Avento 1.5T magnetic resonance imaging 3D-FLAIR and T1VIBE sequencedelayed gadolinium enhancement scans and serological laboratory tests were performed, respectively, and corresponding antiviral and antiinflammatory therapy was given.

Results: The magnetic resonance imaging gadolinium enhancement of 4 cases of Ramsay Hunt syndrome was as follows: 3D-FLAIR sequence delay of 4.5 hours scanning 4 patients labyrinthine and/or middle ear signal was enhanced at the same time as the healthy side; T1VIBE sequence scanning disease in 3 cases of vestibular nerve development was enhanced than the healthy side, 2 cases of facial nerve development was enhanced than the healthy side, and 2 cases of cochlear nerve development was enhanced than the healthy side. All 4 patients were cured with related treatment.

Conclusion: Through the comparison of 3D-FAIR and T1VIBE sequence of 4.5 hours delay before intravenous gadolinium injection and 4.5 hours delay after intravenous gadolinium injection in 4 patients with Ramsay Hunt syndrome, it was found that (i) 3D-FLAIR sequence delay of 4.5 hours scan was more likely to show whether the inner ear labyrinth barrier permeability increased and (ii) Ramsay Hunt syndrome deep ear tissue damage can be manifested as labyrinthitis, vestibular cochlear neuritis, facial neuritis, and otitis media.

We report a case of primary bilateral tuberculous otitis media in a patient who underwent kidney transplantation. This case presents unusual clinical features and histopathology from those of classical tuberculous otitis media. A 75-year-old woman presented at the clinic with purulent ear discharge and hearing loss in both ears. She had undergone kidney transplantation 6 years prior and had been taking immunosuppressant medications. Otoscopic examination and imaging studies suggested acute otitis media, which was irresponsive to antibiotics. The patient underwent surgery to eradicate the disease, and histopathologic examination revealed multifocal granulomas with Langhans giant cells without caseous changes. Ziehl-Neelsen staining and polymerase chain reaction confirmed the diagnosis of tuberculous otitis media. While tuberculous otitis media is a very rare manifestation of extrapulmonary tuberculosis, this case is more noteworthy in that it occurred as a primary infection rather than as a reactivation of a prior infection. In addition, it did not show the classical triad of clinical manifestations, which occurred bilaterally, and its histopathology was different from those of classical tuberculous otitis media. This case presents a new clinical variation in tuberculous otitis media.