Management of Cholesteatoma: Surgical Options for Disease Limited to Middle Ear/Mastoid.

Surgery remains the mainstay of cholesteatoma management. Through advancement in technique and technology, the available surgical approaches have expanded to include not only the traditional procedures, but also endoscopic procedures, canal wall reconstruction procedures, mastoid obliteration, and retrograde mastoidotomy. Selection of management technique will depend on disease characteristics, patient factors, and surgeon preference.

Contemporary Mechanics of Conductive Hearing Loss.

The middle ear plays a critical role for the conversion of acoustic energy to mechanical vibrations that subsequently enter the cochlea. It is middle ear impedance matching through ossicular coupling that has enabled land-dwelling vertebrates to hear soft airborne sounds. Conductive hearing loss may result from damage to the delicate middle ear structures following infection, trauma or rapid pressure changes. An understanding of the mechanics of the middle ear significantly improves the oto-surgeon's ability to effectively diagnose conductive hearing loss, localize the responsible lesion and then effectively correct the conduction abnormality. This article reviews some of the basic knowledge of middle ear mechanics for sound transmission, highlights recent advances in developing new techniques to assist in diagnosis of middle ear disease, and finally sheds light on future research aimed at improving the diagnosis and management of middle ear pathology.

Ossicular joint histopathology in cases of age-related hearing loss.

Objectives: Age-related hearing loss (presbycusis) is a prevalent condition traditionally attributed to inner ear dysfunction. Little is known about age-related changes in the ossicular joints or their contribution to presbycusis. Herein, we performed an otopathologic evaluation of the ossicular joints in cases of presbycusis without a clear sensorineural explanation. Methods: Histopathologic analysis of the incudomallear (IM) and incudostapedial (IS) joints was performed in specimens from the National Temporal Bone Registry with audiometrically confirmed presbycusis but without histologically observed sensorineural, strial, or mixed features; deemed cases of "indeterminate" presbycusis. Specimens identified as "indeterminate" presbycusis (IP, n = 18) were compared to specimens with histologically confirmed sensorineural presbycusis (n = 16) and strial presbycusis (n = 11). Presbycutic specimens were also compared to age-matched controls (n = 9) and young controls (n = 14). Results: The synovial space at the center of the IM joint was wider in the IP group (194 ± 36.8 µm) compared to age-matched controls (138 ± 36.5 µm), young controls (149 ± 32.2 µm), and ears with sensorineural presbycusis (148 ± 52.7 µm) (p < .05). The synovial space within the IS joint was wider in the IP group (105 ± 33.0 µm) when compared to age-matched controls (57.9 ± 13.1 µm) and ears with sensorineural presbycusis (62.3 ± 31.2 µm) (p < .05). Conclusion: IP ears have wider IM and IS joints when compared to ears with sensorineural presbycusis and age-matched controls. Findings point to a potential middle ear source of high frequency conductive hearing loss in a subset of presbycutic ears. Level of Evidence: Retrospective study.

The human middle ear in motion: 3D visualization and quantification using dynamic synchrotron-based X-ray imaging.

The characterization of the vibrations of the middle ear ossicles during sound transmission is a focal point in clinical research. However, the small size of the structures, their micrometer-scale movement, and the deep-seated position of the middle ear within the temporal bone make these types of measurements extremely challenging. In this work, dynamic synchrotron-based X-ray phase-contrast microtomography is used on acoustically stimulated intact human ears, allowing for the three-dimensional visualization of entire human eardrums and ossicular chains in motion. A post-gating algorithm is used to temporally resolve the fast micromotions at 128 Hz, coupled with a high-throughput pipeline to process the large tomographic datasets. Seven ex-vivo fresh-frozen human temporal bones in healthy conditions are studied, and the rigid body motions of the ossicles are quantitatively delineated. Clinically relevant regions of the ossicular chain are tracked in 3D, and the amplitudes of their displacement are computed for two acoustic stimuli.

Human otopathology in scleroderma.

Objective: Scleroderma is a complex chronic progressive immune-mediated disease that causes fibrosis of the skin and internal organs, and vasculopathy.Ear involvement has been poorly studied in patients with scleroderma. Vasculitic and autoimmune mechanisms are considered as possible etiologies on hearing impairment, however, this etiology still unclear.Herein, we reviewed three cases of scleroderma from a temporal bone repository. Methods: The national temporal bone database was reviewed for cases with scleroderma. Clinical case review and correlative otopathologic analysis. Middle and inner ear otopathologic analysis was performed following hematoxylin and eosin staining under light microscopy. Findings were compared to three age-matched controls. Results: Two patients (three cases) with a history of serologically confirmed scleroderma were identified. Both individuals reported tinnitus and demonstrated bilateral moderate to severe down-sloping sensorineural hearing loss on audiometry. Histologically, the incudomallear joint space was diminished and ossicles appeared demineralized. A loss of hyaline cartilage, and obliteration of the incudomallear and incudostapedial joint synovial spaces was observed. Decreased caliber and intimal hyperplasia of arteries adjacent to ossicles was also identified. Mild diffuse atrophy of stria vascularis in the middle and apical turns of cochlea were found. Hair cell populations were normal. Total spiral ganglion neurons were lower in cases of scleroderma (range 29%-51%) compared to age-matched controls. Conclusion: Fibrosis, inflammation, and vascular changes were observed in the middle and inner ear in patients with scleroderma. Findings have implications for understanding hearing and vestibular dysfunction in this patient population. Level of evidence: Retrospective study.

Measurements of ear-canal geometry from high-resolution CT scans of human adult ears.

Description of the ear canal's geometry is essential for describing peripheral sound flow, yet physical measurements of the canal's geometry are lacking and recent measurements suggest that older-adult-canal areas are systematically larger than previously assumed. Methods to measure ear-canal geometry from multi-planar reconstructions of high-resolution CT images were developed and applied to 66 ears from 47 subjects, ages 18-90 years. The canal's termination, central axis, entrance, and first bend were identified based on objective definitions, and the canal's cross-sectional area was measured along its canal's central axis in 1-2 mm increments. In general, left and right ears from a given subject were far more similar than measurements across subjects, where areas varied by factors of 2-3 at many locations. The canal areas varied systematically with age cohort at the first-bend location, where canal-based measurement probes likely sit; young adults (18-30 years) had an average area of 44mm2 whereas older adults (61-90 years) had a significantly larger average area of 69mm2. Across all subjects ages 18-90, measured means ± standard deviations included: canals termination area at the tympanic annulus 56±8mm2; area at the canal's first bend 53±18mm2; area at the canal's entrance 97±24mm2; and canal length 31.4±3.1mm2.

Design of medical tympanostomy conduits with selective fluid transport properties.

Implantable tubes, shunts, and other medical conduits are crucial for treating a wide range of conditions from ears and eyes to brain and liver but often impose serious risks of device infection, obstruction, migration, unreliable function, and tissue damage. Efforts to alleviate these complications remain at an impasse because of fundamentally conflicting design requirements: Millimeter-scale size is required to minimize invasiveness but exacerbates occlusion and malfunction. Here, we present a rational design strategy that reconciles these trade-offs in an implantable tube that is even smaller than the current standard of care. Using tympanostomy tubes (ear tubes) as an exemplary case, we developed an iterative screening algorithm and show how unique curved lumen geometries of the liquid-infused conduit can be designed to co-optimize drug delivery, effusion drainage, water resistance, and biocontamination/ingrowth prevention in a single subcapillary-length-scale device. Through extensive in vitro studies, we demonstrate that the engineered tubes enabled selective uni- and bidirectional fluid transport; nearly eliminated adhesion and growth of common pathogenic bacteria, blood, and cells; and prevented tissue ingrowth. The engineered tubes also enabled complete eardrum healing and hearing preservation and exhibited more efficient and rapid antibiotic delivery to the middle ear in healthy chinchillas compared with current tympanostomy tubes, without resulting in ototoxicity at up to 24 weeks. The design principle and optimization algorithm presented here may enable tubes to be customized for a wide range of patient needs.

Characterization and Clinical Use of Bone Conduction Transducers at Extended High Frequencies.

Measurement of bone conduction (BC) hearing thresholds at extended high frequencies (EHF; above 8 kHz) is of clinical interest but is technically complicated by limitations in standard BC transducer output, a lack of calibration standards and sparse clinical data from human subjects. A recently described calibration scheme using an artificial mastoid and interposed accelerometer is applied in this study to characterize and compare acceleration and computed force outputs over the 4-20 kHz range of two standard BC transducers: the RadioEar® B71 and B81, as well as two non-standard, commercially available BC transducers: the Tascam® HP-F200 and the Aftershokz® AS400. Measures of linear output growth, harmonic distortion and acoustic radiation are assessed and compared across devices. A maximum linear input voltage is established for each BC transducer using measurements of linear output growth and total harmonic distortion. At maximum linear input level, the Tascam shows superior force output by 25 to 40 dB above 8 kHz and the widest dynamic EHF range. Acoustic radiation per output force was lowest for the Tascam, whereas the AS400 behaved more like an air conduction earphone than a force generator. In a cohort of 15 normal hearing volunteers, BC thresholds, measured with the Tascam and reported in dB re 1 rms µN, were consistent with historical measures of EHF BC thresholds in similar subjects using an alternative BC transducer.

Evaluating the accuracy of speech to text applications for cochlear implant candidates during COVID-19.

OBJECTIVES: Universal mask wearing due to COVID-19 has introduced barriers to clear communication. In hearing impaired individuals this can impact informed surgical consent. For cochlear implant candidates, who do not rely on sign language, real-time transcription with a stenographer (CART) is the gold-standard in assistive technologies. If CART is not available, speech to text (STT) applications have been advertised as solutions, but their transcription accuracy with or without an N95 mask is not well-established. Herein, we sought to investigate the transcription accuracy of three STT solutions for iPhone and compare their performance to the CART service at our institution. METHODS: Three native English speakers and three non-native English speakers read two passages (a cochlear implant consent and the non-medical 'Rainbow passage') with and without an N95 mask. Error rates from the comparison of the transcript (from either the STT app or CART) with the original passage were calculated. RESULTS: The CART service had the lowest error rate of all testing conditions (4.79-7.14%). Ava 24/7 (15.0 ± 9.49%) and the iPhone dictation (15.6 ± 6.65%) had significantly lower average error rates than the Live Transcribe (37.7 ± 20.3%) (P < 0.0001) application. Neither the presence of an N95 nor the type of passage had a statistically significant impact on the error rate. CONCLUSION: CART should be used to augment communication with patients who are hard of hearing. If CART is not available, a STT application such as Ava 24/7 or the native iPhone dictation application may be considered, even in the context of medical terminology.

Primary Sarcoidosis of the Temporal Bone a Clinical Pathologic Correlation.

Objective: This report describes a case of sarcoidosis that presented as a lytic bone lesion in the squamous part of the temporal bone. Patients: A 64-year-old woman presented with right-sided aural fullness, pulsatile tinnitus, and intermittent otalgia. Interventions: CT and MRI were performed without contrast and suggested an osseodestructive, lytic bone lesion. An excisional biopsy was performed, showing granulomatous infiltration suggestive of osseous sarcoidosis. Main Outcome Measures: Removal of mass and resolution of symptoms. Results: Initial findings from patient imaging suggested a lytic bone lesion. An excisional biopsy was required for diagnosis and was performed with little patient morbidity. Biopsy findings showed granulomatous infiltration suggestive of osseous sarcoidosis. Osseous involvement of sarcoidosis is a rare manifestation and typically occurs secondary to other disease manifestations. After the removal of the mass and a short unrelated course of steroids, the patient's symptoms resolved. Conclusions: Sarcoidosis should be added to the differential diagnosis of lytic bone lesions in the temporal bone.

Educational Quality of YouTube Cholesteatoma Surgery Videos: Areas for Improvement.

Otolaryngology surgical education continues to evolve where trainees increasingly use videos to learn technical skills. Trainees commonly use YouTube, but no study to date has evaluated the educational quality (EQ) of otologic surgical videos on YouTube. We aim to assess the EQ of cholesteatoma surgical videos. Cholesteatoma surgical videos were queried using YouTube search terms, assessed using LAParoscopic surgery Video Educational GuidelineS (LAP-VEGaS), a validated assessment tool for publication, and categorized into low (0-6), medium (7-12), and high (13-18) EQ groups. In total, 74 videos were identified (mean LAP-VEGaS score = 9.6 ± 4.0) and 44.6% had medium EQ. Videos commonly lacked graphic aids to highlight anatomy (71.6%) and postprocedural outcomes (68.9%). LAP-VEGaS scores were greater in videos originating from US surgeons compared to non-US surgeons (12.4 ± 3.4 vs 8.0 ± 3.5; P < .001). Our study highlights that otolaryngology trainees may experience difficulty finding high-EQ cholesteatoma surgery videos on YouTube. Areas for improved EQ content are discussed. Level of evidence: IV.

Methods for the calibration of bone conduction transducers at frequencies from 5 to 20 kHz.

Techniques for standardizing the output of bone conduction transducers over the 5-20 kHz range are presented. The techniques include definitions of the standard artificial mastoid (AM) impedance and force sensitivity in that high-frequency range using an impedance head coupled to a vibration source. The AM impedance is shown to vary with the contact area of the vibration source. The AM force sensitivity does not vary with the contact area but does exhibit sharp frequency dependences over the 14-20 kHz range. The sharp frequency dependence complicates the use of the AM force sensor as a stand-alone calibration device at these high frequencies. An alternative calibration scheme that uses an accelerometer interposed between the vibrator and the AM impedance is described. Comparisons of the two schemes demonstrate that the accelerometer method produces more consistent results. Comparisons of the force and acceleration output of one bone conduction vibrator at high frequencies suggest those outputs depend on the driven load. The loads used in the two calibration schemes are compared to what is known of the impedance of the skin-covered head.

Information scarcity among otolaryngology applicants: A review of the largest database of programs.

Objective: Otolaryngology residency applicants face challenges finding accurate information about training programs. Social media platforms are not verified, and official training program websites are not standardized. Currently, the American Medical Association's Fellowship and Residency Electronic Interactive Database (FREIDA) is the most comprehensive sanctioned and verified otolaryngology residency program database. However, the exact amount of information shared by individual programs included is not presently known. Methods: Herein, we analyzed the available data on all 124 otolaryngology residency programs in FREIDA to assess the completeness of the database. Results: While every program listed an address, contact email, and the name of the program director, more than half of programs (n = 65, 52.4%) did not provide additional information. Most programs (70.2%) did not include a one-paragraph program description. Conclusion: Our findings suggest that while FREIDA is the only sanctioned online database for residency programs, it is inadequately populated with detailed program information. Level of Evidence: N/A.

Ex vivo transtympanic permeation of the liposome encapsulated S. pneumoniae endolysin MSlys.

An increase in bacterial resistance to systemic antibiotics has sparked interest into alternative antimicrobial compounds as well as methods for effective local, non-invasive drug delivery. Topical treatments, however, may be hindered by the presence of biological barriers, such as the tympanic membrane in the case of otitis media. Herein, the transtympanic permeation ability of liposomes loaded with the pneumococcal endolysin MSlys and of free MSlys was evaluated ex vivo. MSlys loaded in PEGylated liposomes showed an increased permeation across human tympanic membranes, as compared to its free form, being able to reduce the pneumococcal cell load after 2 h of permeation. However, antipneumococcal activity was no longer detected after 4 h of permeation and hydrolysis of the endolysin was observed after an extended incubation time (≥48 h). This work provides a first assessment of a successful, non-invasive delivery method for endolysins across an intact tympanic membrane. Findings have implications for non-systemic, local treatment of otitis media.

Assessment of Sudden Sensorineural Hearing Loss After COVID-19 Vaccination.

IMPORTANCE: Emerging reports of sudden sensorineural hearing loss (SSNHL) after COVID-19 vaccination within the otolaryngological community and the public have raised concern about a possible association between COVID-19 vaccination and the development of SSNHL. OBJECTIVE: To examine the potential association between COVID-19 vaccination and SSNHL. DESIGN, SETTING, AND PARTICIPANTS: This cross-sectional study and case series involved an up-to-date population-based analysis of 555 incident reports of probable SSNHL in the Centers for Disease Control and Prevention Vaccine Adverse Events Reporting System (VAERS) over the first 7 months of the US vaccination campaign (December 14, 2020, through July 16, 2021). In addition, data from a multi-institutional retrospective case series of 21 patients who developed SSNHL after COVID-19 vaccination were analyzed. The study included all adults experiencing SSNHL within 3 weeks of COVID-19 vaccination who submitted reports to VAERS and consecutive adult patients presenting to 2 tertiary care centers and 1 community practice in the US who were diagnosed with SSNHL within 3 weeks of COVID-19 vaccination. EXPOSURES: Receipt of a COVID-19 vaccine produced by any of the 3 vaccine manufacturers (Pfizer-BioNTech, Moderna, or Janssen/Johnson & Johnson) used in the US. MAIN OUTCOMES AND MEASURES: Incidence of reports of SSNHL after COVID-19 vaccination recorded in VAERS and clinical characteristics of adult patients presenting with SSNHL after COVID-19 vaccination. RESULTS: A total of 555 incident reports in VAERS (mean patient age, 54 years [range, 15-93 years]; 305 women [55.0%]; data on race and ethnicity not available in VAERS) met the definition of probable SSNHL (mean time to onset, 6 days [range, 0-21 days]) over the period investigated, representing an annualized incidence estimate of 0.6 to 28.0 cases of SSNHL per 100 000 people per year. The rate of incident reports of SSNHL was similar across all 3 vaccine manufacturers (0.16 cases per 100 000 doses for both Pfizer-BioNTech and Moderna vaccines, and 0.22 cases per 100 000 doses for Janssen/Johnson & Johnson vaccine). The case series included 21 patients (mean age, 61 years [range, 23-92 years]; 13 women [61.9%]) with SSNHL, with a mean time to onset of 6 days (range, 0-15 days). Patients were heterogeneous with respect to clinical and demographic characteristics. Preexisting autoimmune disease was present in 6 patients (28.6%). Of the 14 patients with posttreatment audiometric data, 8 (57.1%) experienced improvement after receiving treatment. One patient experienced SSNHL 14 days after receiving each dose of the Pfizer-BioNTech vaccine. CONCLUSIONS AND RELEVANCE: In this cross-sectional study, findings from an updated analysis of VAERS data and a case series of patients who experienced SSNHL after COVID-19 vaccination did not suggest an association between COVID-19 vaccination and an increased incidence of hearing loss compared with the expected incidence in the general population.

Intracochlear New Fibro-Ossification and Neuronal Degeneration Following Cochlear Implant Electrode Translocation: Long-Term Histopathological Findings in Humans.

OBJECTIVE: We aim to assess the histopathology of human temporal bones (TBs) with evidence of cochlear implantation (CI) electrode scalar translocation. STUDY DESIGN: Otopathology study. SETTING: Otopathology laboratory. PATIENTS: TBs from patients who had a history of CI and histopathological evidence of interscalar translocation. Specimens with electrode placed entirely within the ST served as controls. INTERVENTION: Histopathological assessment of human TBs. MAIN OUTCOME MEASURES: TBs from each patient were harvested postmortem and histologically analyzed for intracochlear changes in the context of CI electrode translocation and compared to controls. Intracochlear new fibro-ossification, and spiral ganglion neuron (SGN) counts were assessed. Postoperative word recognition scores (WRS) were also compared. RESULTS: Nineteen human TBs with electrode translocation and eight controls were identified. The most common site of translocation was the ascending limb of the basal turn (n = 14 TBs). The average angle of insertion at the point of translocation was 159°â€Š±â€Š79°. Eighteen translocated cases presented moderate fibroosseous changes in the basal region of the cochlea, extending to the translocation point and/or throughout the electrode track in 42%. Lower SGN counts were more pronounced in translocated cases compared to controls, with a significant difference for segment II (p = 0.019). Although final postoperative hearing outcomes were similar between groups, translocated cases had slower rate of improvement in WRS (p = 0.021). CONCLUSIONS: Cochlear implant electrode translocation was associated with greater fibroosseous formation and lower SGN population. Our findings suggest that scalar translocations may slow the rate of improvement in WRS overtime as compared to atraumatic electrode insertions.Level of evidence: IV.

"Transcanal view" computed tomography reformat: Applications for transcanal endoscopic ear surgery.

PURPOSE: Transcanal endoscopic ear surgery (TEES) is an increasingly used surgical approach for otologic surgeries, but no en face preoperative imaging format currently exists. We aim to assess the utility of a transcanal high resolution computed tomography (HRCT) reformat suitable for TEES preoperative planning. MATERIALS AND METHODS: Preoperative HRCTs of patients with middle ear pathologies (cholesteatoma, otosclerosis, and glomus tympanicum) who underwent TEES were obtained. Axial image series were rotated and reformatted -90 or +90 degrees for left and right ear surgeries, respectively, where additional rotation along the left-right axis was performed to align the transcanal series with the plane of the external auditory canal. Quantitative measurements of middle ear structures were recorded. Consecutive transcanal reformatted sections were then reviewed to identify critical middle ear anatomy and pathology with corresponding TEES cases. RESULTS: The aforementioned methodology was used to create three transcanal view HRCTs. The mean left-right axis degree of rotation was 4.0 ± 2.2 degrees. In the cholesteatoma transcanal HRCT, areas of cholesteatoma involvement in middle ear compartments (e.g. epitympanum) and eroded ossicles were successfully identified in the corresponding case. In the otosclerosis transcanal HRCT, areas for potential otosclerotic involvement were visualized such as the round window as well as a low-hanging facial nerve. In the glomus tympanicum transcanal HRCT, the span of the glomus tympanicum was successfully visualized in addition to a high riding jugular bulb. CONCLUSION: A transcanal HRCT reformat may aid preoperative planning for middle ear pathologies. This novel reformat may help highlight patient-specific anatomy.

A Subset of Intracanalicular Vestibular Schwannomas Demonstrates Minimal Growth Over a 10-Year Period.

OBJECTIVE: Vestibular schwannomas (VS) commonly undergo magnetic resonance imaging (MRI) surveillance, but long-term data to support the ideal frequency is limited. Herein, we aim to investigate intracanalicular VS growth predictors and long-term growth rates (GR). STUDY DESIGN: Retrospective chart review. SETTING: Two tertiary care centers. PATIENTS: Sporadic intracanalicular VS with initial conservative management and at least two sequential MRIs. INTERVENTION: Serial MRI. MAIN OUTCOME MEASURES: VS were categorized by baseline internal auditory canal tertile sublocalization (fundus, midpoint, porus) and size (≤100, 100-200, >200 mm3). Throughout follow-up, volumetric GR (mm3/yr) were determined (baseline-3 yrs, 3-5 yrs, 5-10 yrs) and treatment rates were assessed. RESULTS: Ninety-nine intracanalicular VS were identified (mean follow-up of 6.1 ±â€Š4.5 yrs). Mean GR before 5-year follow-up were comparable for baseline tertile involvement and size. After 5-year follow-up, mean GR of VS involving the fundus at baseline were lower than those involving the midpoint and fundus (6.17 ±â€Š21.16 and 119.74 ±â€Š117.57 mm3/yr, respectively; p = 0.034). Mean GR of VS with less than or equal to 100 mm3 at baseline (-7.29 ±â€Š25.44 mm3/yr) were lower than those with 100 to 200 mm3 (86.55 ±â€Š103.99 mm3/yr; p = 0.011) and more than 200 mm3 (45.70 ±â€Š35.71 mm3/yr; p = 0.031). Vestibular schwannomas involving the midpoint and fundus had greater treatment rates compared with VS involving only the fundus (p < 0.001). CONCLUSIONS: Baseline tertile involvement and size may predict long-term intracanalicular VS growth where fundal tumors or those less than or equal to 100 mm3 exhibit little long-term growth. Extending surveillance after 5-year follow-up may be reasonable for fundal VS.