A novel radiological software prototype for automatically detecting the inner ear and classifying normal from malformed anatomy.

BACKGROUND: To develop an effective radiological software prototype that could read Digital Imaging and Communications in Medicine (DICOM) files, crop the inner ear automatically based on head computed tomography (CT), and classify normal and inner ear malformation (IEM). METHODS: A retrospective analysis was conducted on 2053 patients from 3 hospitals. We extracted 1200 inner ear CTs for importing, cropping, and training, testing, and validating an artificial intelligence (AI) model. Automated cropping algorithms based on CTs were developed to precisely isolate the inner ear volume. Additionally, a simple graphical user interface (GUI) was implemented for user interaction. Using cropped CTs as input, a deep learning convolutional neural network (DL CNN) with 5-fold cross-validation was used to classify inner ear anatomy as normal or abnormal. Five specific IEM types (cochlear hypoplasia, ossification, incomplete partition types I and III, and common cavity) were included, with data equally distributed between classes. Both the cropping tool and the AI model were extensively validated. RESULTS: The newly developed DICOM viewer/software successfully achieved its objectives: reading CT files, automatically cropping inner ear volumes, and classifying them as normal or malformed. The cropping tool demonstrated an average accuracy of 92.25%. The DL CNN model achieved an area under the curve (AUC) of 0.86 (95% confidence interval: 0.81-0.91). Performance metrics for the AI model were: accuracy (0.812), precision (0.791), recall (0.8), and F1-score (0.766). CONCLUSION: This study successfully developed and validated a fully automated workflow for classifying normal versus abnormal inner ear anatomy using a combination of advanced image processing and deep learning techniques. The tool exhibited good diagnostic accuracy, suggesting its potential application in risk stratification. However, it is crucial to emphasize the need for supervision by qualified medical professionals when utilizing this tool for clinical decision-making.

Case Report: Diagnosis of Petrous Apex IgG4-Related Disease by Middle Cranial Fossa Craniotomy and Temporal Bone Biopsy.

Introduction: Primary IgG4-related disease (IgG4-RD) of the temporal bone is a rare condition. Unlike typical petrous apicitis or Gradenigo syndrome, our patient presented exclusively with unilateral cranial nerve VI palsy and symptoms of diplopia. Skull base imaging demonstrated a destructive bony lesion in the petrous apex. Imaging and systemic investigations were insufficient to support a diagnosis. The diagnosis was achieved histologically after acquiring the specimen by middle cranial fossa craniotomy and temporal bone biopsy. This case report is thought to be the first published description of a diagnosis of IgG4-RD proven with the middle cranial fossa approach. Case Report: We describe a 29-year-old female with primary IgG4-RD of the petrous apex of the temporal bone. This patient presented with a few-month history of left-sided headache and recent-onset diplopia due to paralysis of cranial nerve VI. Imaging demonstrated a petrous apex lesion, and comprehensive systemic investigations could not reach a diagnosis. A middle cranial fossa craniotomy and a biopsy of the temporal bone lesion were undertaken to establish the diagnosis. Histological confirmation of IgG4-RD was proven. Following treatment with corticosteroids, the patient experienced complete recovery and resolution of her symptoms. Conclusion: This study describes a case of primary IgG4-RD of the petrous apex of the temporal bone that presented with diplopia and was diagnosed by middle fossa craniotomy and temporal bone biopsy. To the best of our knowledge, this is the first case description where primary diagnosis was made based on middle cranial fossa craniotomy and temporal bone biopsy.

Wideband acoustic immittance in superior semicircular canal dehiscence.

OBJECTIVE: The apparent effect of superior semicircular canal dehiscence (SSCD) on middle ear- and cochlear impedance has led researchers to investigate the use of wideband acoustic immittance as a screening tool when SSCD is suspected. The purpose of the study was to describe the absorbance characteristics and tympanometric values of ears with confirmed SSCD measured at tympanometric peak pressure (TPP) and at ambient pressure. METHODS: Wideband Acoustic Immittance was performed at ambient pressure and at TPP on ten participants (12 ears) with confirmed SSCD, as well as on an age- and gender matched control group (12 ears). Inferential statistics were used to determine whether statistical differences existed for the absorbance values at each of the averaged frequencies, the resonance frequency (RF) and tympanometric data between the SSCD and control groups. RESULTS: The mean absorbance of the SSCD group reached a maximum at 890.9 Hz and a minimum at 6349.6 Hz. When testing absorbance at TPP, a statistically significant increase/peak in the absorbance values of the SSCD group (compared to those of the control group) was found from 630 to 890.9 Hz and a decrease from 4489.8 to 6349.6 Hz. Similar patterns were observed for absorbance at ambient pressure. A lower mean RF for ears with SSCD as well as an increased mean admittance magnitude (AM) value at RF was found compared to those of the control group. CONCLUSION: The use of SSCD as a screening tool when SSCD is suspected was strengthened by results similar to those of previous studies. As a result of the significant difference in RF of SSCD ears compared to the RF of the control group, the potential value of measuring the RF of the middle ear to differentiate between mass-and stiffness dominated pathologies, was also illustrated.

Short-Term Test-Retest Reliability of Electrically Evoked Cortical Auditory Potentials in Adult Cochlear Implant Recipients.

Background: Late latency auditory evoked potentials (LLAEPs) provide objective evidence of an individual's central auditory processing abilities. Electrically evoked cortical auditory evoked potentials (eCAEPs) are a type of LLAEP that provides an objective measure of aided speech perception and auditory processing abilities in cochlear implant (CI) recipients. Aim: To determine the short-term test-retest reliability of eCAEPs in adult CI recipients. Design: An explorative, within-subject repeated measures research design was employed. Study Sample: The study sample included 12 post-lingually deafened, unilaterally implanted adult CI recipients with at least 9 months of CI experience. Method: eCAEPs representing basal, medial and apical cochlear regions were recorded in the implanted ears of each participant. Measurements were repeated 7 days after the initial assessment. Results: No significant differences between either median latencies or amplitudes at test and retest sessions (p > 0.05) were found when results for apical, medial and basal electrodes were averaged together. Mean intraclass correlation coefficient (ICC) scores averaged across basal, medial and apical cochlear stimulus regions indicated that both consistency and agreement were statistically significant and ranged from moderate to good (ICC = 0.58-0.86, p < 0.05). ICC confidence intervals did demonstrate considerable individual variability in both latency and amplitudes. Conclusion: eCAEP latencies and amplitudes demonstrated moderate to good short-term test-retest reliability. However, confidence intervals indicated individual variability in measurement consistency which is likely linked to attention and listening effort required from the CI recipients.

Vestibular involvement in adults with HIV/AIDS.

OBJECTIVE: HIV/AIDS is responsible for widespread clinical manifestations involving the head, and neck. The prevalence and nature of vestibular involvement is still largely unknown. This study, aimed to describe and compare the occurrence and nature of vestibular involvement among a group of, adults infected with HIV compared to a control group. It also aimed to compare the vestibular function, of symptomatic and asymptomatic HIV positive adults who receive antiretroviral (ARV) therapies to, subjects not receiving ARV. METHODS: A cross-sectional study was conducted on 53 adults (29 male, 24 female, aged 23-49 years, mean=38.5, SD=4.4) infected with HIV, compared to a control group of 38 HIV negative adults (18, male, 20 female, aged 20-49 years, mean=36.9, SD=8.2). A structured interview probed the subjective, perception of vestibular symptoms. Medical records were reviewed for CD4+ cell counts and the use of, ARV medication. An otologic assessment and a comprehensive vestibular assessment (bedside, assessments, vestibular evoked myogenic potentials, ocular motor and positional tests and bithermal, caloric irrigation) were conducted. RESULTS: Vestibular involvement occurred in 79.2% of subjects with HIV in all categories of disease, progression, compared to 18.4% in those without HIV. Vestibular involvement increased from 18.9% in CDC category 1 to 30.2% in category 2. Vestibular involvement was 30.1% in category 3. There were, vestibular involvement in 35.9% of symptomatic HIV positive subjects, and 41.5% in asymptomatic, HIV positive subjects. There was no significant difference in the occurrence of vestibular involvement, in subjects receiving ARV therapies compared to those not receiving ARV therapies (p=.914; chi-square, test). The odds ratio indicates that individuals with HIV have a 16.61 times higher risk of developing, vestibular involvement during their lifetime of living with the disease and that it may occur despite, being asymptomatic. CONCLUSION: Vestibular involvement was significantly more common in subjects with HIV. Primary health care providers could screen HIV positive patients to ascertain if there are symptoms of vestibular involvement. If there are any, then they may consider further vestibular assessments and subsequent vestibular rehabilitation therapy.

Auditory and otological manifestations in adults with HIV/AIDS.

OBJECTIVES: This study describes the prevalence and nature of auditory and otological manifestations in adults with HIV/AIDS through clinical examinations and self-reported symptoms across stages of disease progression. DESIGN: Descriptive cross-sectional group design. STUDY SAMPLE: Two hundred HIV positive adult patients (56.5% male; 43.5% female; mean age: 37.99 ± 6.66 years) attending the Infectious Disease Clinic of a tertiary referral hospital in Pretoria, South Africa were included. Patients were interviewed, medical files were reviewed, and clinical examinations, including otoscopy, tympanometry, pure-tone audiometry, and distortion product otoacoustic emissions, were conducted. A matched HIV negative control group was used to compare hearing loss prevalence. RESULTS: Tinnitus (26%), vertigo (25%) hearing loss (27.5%), otalgia (19%), and ear canal pruritis (38%) were prevalent self-reported symptoms. Abnormalities in otoscopy, tympanometry, and otoacoustic emissions were evident in 55%, 41%, and 44% of patients respectively. Pure-tone average (PTA) hearing loss > 25 dBHL was evident in 14% of patients and 39% for hearing loss > 15 dBHL (PTA). Significant differences across average thresholds in the HIV positive and HIV negative control group was present. An increase in self reported vertigo, self reported hearing loss, OAE abnormalities, and hearing loss (PTA > 15 dBHL and PTA > 25 dBHL) was seen with disease progression but was not statistically significant. A significant increase (p <.05) in sensorineural hearing loss was however evident with disease progression. CONCLUSIONS: Auditory and otological symptoms are more common in patients with HIV with a general increase of symptoms, especially sensorineural hearing loss, towards advanced stages of disease progression.

An unusual presentation of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA) in an adult: anterior papillary muscle rupture causing severe mitral regurgitation.

We describe a 29-year-old male, previously in good health, with no history of angina pectoris and no risk factors for ischemic heart disease presenting with biventricular failure and severe mitral valve regurgitation. There were no signs or serological test results to suggest infective endocarditis. Transthoracic echocardiography (TTE) revealed severe anterior mitral valve prolapse secondary to papillary muscle rupture, severe mitral valve regurgitation, as well as an anterior myocardial wall hypokinesis. Parasternal short-axis view showed an anomalous left coronary artery arising from the pulmonary artery (ALCAPA), which was confirmed on coronary angiography. This is an unusual presentation of ALCAPA in an adult.

Too much bone: the middle ear in sclerosing bone dysplasias.

The middle ear changes in Sclerosteosis and Van Buchem disease are described. Reduced bone resorption occurs due to faulty activity of the sclerostin molecule, a product of the recently discovered SOST gene in chromosome 17. Syndactyly draws attention to scleroteosis, and a conductive hearing loss develops before age six in both conditions. Acute, repeated attacks of facial palsy, similar to Bell's palsy, are usually the first symptoms in both conditions. Total facial nerve decompression can stop the attacks of facial paralysis. The hearing loss is a problem because new bone formation continues up to age 21. Life saving craniectomy becomes necessary when increased intracranial pressure develops, and this may have to repeated. The sclerostin molecule is now of major interest to the researchers who want to develop a treatment for osteoporosis.

Sclerosing bone dysplasias: neurologic assessment and management.

PURPOSE OF REVIEW: Sclerosing bone dysplasias are rare genetic disorders of bone remodeling in which excessive bone formation takes place, resulting in encroachment on neural structures. The infant usually appears normal at birth, and the first sign of a problem only comes when a neurologic deficit develops, usually in the form of an acute facial palsy. Although less than 300 cases have been published, these conditions should always be considered in the differential diagnosis of facial nerve palsy, especially in children. RECENT FINDINGS: This review highlights the neurologic presentation and assessment as well as the management of sclerosing bone dysplasias. An exciting development is the recent discovery of the sclerosteosis (SOST) gene, which is involved with excessive bone formation in sclerosteosis and Van Buchem disease. Researchers in bone metabolism and pharmaceutical companies are now utilizing this knowledge to develop a medicine for osteoporosis. SUMMARY: In children and young adults an acute facial palsy, especially if it is recurrent, as well as a conductive hearing loss may be the first sign of a sclerosing bone dysplasia.