Search

In 2020, Advanced Bionics (AB) announced a recall of two cochlear implant (CI) models, the "HiRes Ultra" and "HiRes Ultra 3D", because of reports of hearing degradation. The present study examines clinical parameters and patient features in cases of device failure and evaluates outcomes after reimplantation.A series of 52 patients implanted with the recalled devices experienced suspected device failure and subsequently underwent revision CI placement at a tertiary academic medical center between December 2019 and November 2021.Consonant-nucleus-consonant scores and individual phonemes increased significantly between patients' preoperative evaluation and primary cochlear implantation. Performance declined significantly before revision and recovered after revision CI placement. Similarly, pure-tone average thresholds improved between preoperative and primary CI, fell before revision surgery, and were corrected with revision implantation. As a group, patients reached their peak hearing performance significantly faster after revision CI (mean ± standard deviation, 53.4 ± 51.8 d) compared with their primary CI (mean ± standard deviation, 260.6 ± 245.9 d). Electrical field imaging performed by AB and device impedance measurements were found to be abnormal in the basally positioned electrodes (electrodes 9-16).Hearing performance degradation is significant in AB Ultra device failures and seems to be linked to the basal-most electrodes in the array. Revision outcomes have been robust, necessitating continued monitoring of affected patients and support for reimplantation procedures.IV.

Internal auditory canal (IAC) diverticula, also known as IAC cavitary lesions or anterior cupping of the IAC, observed in otopathologic specimens and high-resolution computed tomography (CT) scans of the temporal bone are thought to be related to otosclerosis. Herein, we examined the usefulness of CT scans in identifying diverticula and determined whether IAC diverticula are associated with otosclerosis on otopathology.One hundred five consecutive specimens were identified from the National Temporal Bone Hearing and Balance Pathology Resource Registry. Inclusion criteria included the availability of histologic slides and postmortem specimen CT scans. Exclusion criteria included cases with severe postmortem changes or lesions causing bony destruction of the IAC.Ninety-seven specimens met criteria for study. Of these, 42% of the specimens were from male patients, and the average age of death was 77 years (SD = 18 yr). IAC diverticula were found in 48 specimens, of which 46% were identified in the CT scans. The mean area of the IAC diverticula was 0.34 mm 2 . The sensitivity and specificity of detecting IAC diverticula based on CT were 77% and 63%, respectively. Overall, 27% of specimens had otosclerosis. Histologic IAC diverticula were more common in specimens with otosclerosis than those without (37.5% versus 16%; p = 0.019). Cases with otosclerosis had a greater mean histologic diverticula area compared with nonotosclerosis cases (0.69 mm 2 versus 0.14 mm 2 ; p = 0.001).IAC diverticula are commonly found in otopathologic specimens with varied etiologies, but larger diverticula are more likely to be associated with otosclerosis. The sensitivity and specificity of CT scans to detect IAC diverticula are limited.

HYPOTHESIS: Computed tomography (CT) density measurement can be used to objectively distinguish otosclerosis from normal bone and to determine histologic grades of otosclerosis. BACKGROUND: Otosclerosis can be seen on CT as subtle radiolucent areas. An objective radiologic measurement that corresponds to known otosclerosis pathology may improve diagnostic accuracy, and could be used as a radiologic biomarker for otosclerosis grade. METHODS: A blinded, randomized evaluation of both histologic grade on histopathology slides and CT density measurement was performed on 78 human temporal bone specimens (31 with otosclerosis and 47 controls) that had undergone high-resolution multi-detector CT prior to histologic processing. Assessments were performed at 11 regions of interest (ROIs) in the otic capsule for each specimen. RESULTS: The CT density measurement mean (Hounsfield Units) ± standard deviation for all ROIs (#1-#9) was 2245 ± 854 for grade 0 (no otosclerosis, n=711), 1896 ±317 for grade 1 (inactive otosclerosis, n=109), and 1632 ±255 for grades 2 and 3 combined (mixed/active otosclerosis, n=35). There was a strong inverse correlation of CT density to histologic grade at ROIs #1–5 (ANOVA, p

OBJECTIVE: To investigate the influence of the COVID-19 pandemic on operative practices of otology and neurotology providers internationally. STUDY DESIGN: Cross-sectional survey. METHODS: A 78-question survey was distributed to otologists and neurotologists between May 12, 2020 and June 8, 2020 to assess the impact of the pandemic on surgical practices. Sections within the survey delineated time periods: prior to the crisis, onset of the crisis, during the crisis, postcrisis transition. RESULTS: Of 396 survey respondents, 284 participants from 38 countries met inclusion criteria.Respondents were 16.9% female and 82.4% male, with a most common age range of 40 to 49 years (36.3%). 69.8% of participants had been in practice for over 10 years and most respondents worked in an academic medical center (79.2%). The average operative weekly caseload was 5.3 (SD 3.9) per surgeon prior to the crisis, 0.7 (SD 1.2) during the COVID-19 crisis, and 3.5 (SD 3.3) for those who had begun a postcrisis transition at the time of survey administration (p 

Objectives To better distinguish NOG-related-symphalangism spectrum disorder (NOG-SSD) from chromosomal 17q22 microdeletion syndromes and to inform surgical considerations in stapes surgery for patients with NOG-SSD. Background Mutations in NOG cause a variety of skeletal syndromes that often include conductive hearing loss. Several microdeletions of chromosome 17q22 lead to severe syndromes with clinical characteristics that overlap NOG-SSD. Isolated deletion of NOG has not been described, and therefore the contribution of NOG deletion in these syndromes is unknown. Methods Two families with autosomal dominant NOG-SSD exhibited stapes ankylosis, facial dysmorphisms, and skeletal and joint anomalies. In each family, NOG was evaluated by genomic sequencing and candidate mutations confirmed as damaging by in vitro assays. Temporal bone histology of a patient with NOG-SSD was compared with temporal bones of 40 patients diagnosed with otosclerosis. Results Family 1 harbors a 555 kb chromosomal deletion encompassing only NOG and ANKFN1. Family 2 harbors a missense mutation in NOG leading to absence of noggin protein. The incus-footplate distance of the temporal bone was significantly longer in a patient with NOG-SSD than in patients with otosclerosis. Conclusion The chromosomal microdeletion of family 1 led to a phenotype comparable to that due to a NOG point mutation and much milder than the phenotypes due to other chromosome 17q22 microdeletions. Severe clinical findings in other microdeletion cases are likely due to deletion of genes other than NOG. Based on temporal bone findings, we recommend that surgeons obtain longer stapes prostheses before stapes surgery in individuals with NOG-SSD stapes ankylosis.

Objective To describe the site of lesion responsible for the severe, bilateral, symmetrical, selective loss of vestibular function in Cerebellar Ataxia with Neuronopathy and Vestibular Areflexia Syndrome (CANVAS), an adult-onset recessively-inherited ataxia, characterized by progressive imbalance due to a combination of cerebellar, somatosensory, and selective vestibular impairment with normal hearing. Methods Histologic examination of five temporal bones and the brainstems from four CANVAS patients and the brainstem only from one more, each diagnosed and followed from diagnosis to death by one of the clinician authors. Results All five temporal bones showed severe loss of vestibular ganglion cells (cell counts 3-16% of normal), and atrophy of the vestibular nerves, whereas vestibular receptor hair cells and the vestibular nuclei were preserved. In contrast, auditory receptor hair cells, the auditory ganglia (cell counts 51-100% of normal), and the auditory nerves were relatively preserved. In addition, the cranial sensory ganglia (geniculate and trigeminal), present in two temporal bones, also showed severe degeneration. Conclusions In CANVAS there is a severe cranial sensory ganglionopathy neuronopathy (ganglionopathy) involving the vestibular, facial, and trigeminal ganglia but sparing the auditory ganglia. These observations, when coupled with the known spinal dorsal root ganglionopathy in CANVAS, indicate a shared pathogenesis of its somatosensory and cranial nerve manifestations. This is the first published account of both the otopathology and neuropathology of CANVAS, a disease that involves the central as well as the peripheral nervous system.

This combined American Neurotology Society, American Otological Society, and American Academy of Otolaryngology - Head and Neck Surgery Foundation document aims to provide guidance during the coronavirus disease of 2019 (COVID-19) on 1) "priority" of care for otologic and neurotologic patients in the office and operating room, and 2) optimal utilization of personal protective equipment. Given the paucity of evidence to inform otologic and neurotologic best practices during COVID-19, the recommendations herein are based on relevant peer-reviewed articles, the Centers for Disease Control and Prevention COVID-19 guidelines, United States and international hospital policies, and expert opinion. The suggestions presented here are not meant to be definitive, and best practices will undoubtedly change with increasing knowledge and high-quality data related to COVID-19. Interpretation of this guidance document is dependent on local factors including prevalence of COVID-19 in the surgeons' local community. This is not intended to set a standard of care, and should not supersede the clinician's best judgement when managing specific clinical concerns and/or regional conditions.Access to otologic and neurotologic care during and after the COVID-19 pandemic is dependent upon adequate protection of physicians, audiologists, and ancillary support staff. Otolaryngologists and associated staff are at high risk for COVID-19 disease transmission based on close contact with mucosal surfaces of the upper aerodigestive tract during diagnostic evaluation and therapeutic procedures. While many otologic and neurotologic conditions are not imminently life threatening, they have a major impact on communication, daily functioning, and quality of life. In addition, progression of disease and delay in treatment can result in cranial nerve deficits, intracranial and life-threatening complications, and/or irreversible consequences. In this regard, many otologic and neurotologic conditions should rightfully be considered "urgent," and almost all require timely attention to permit optimal outcomes. It is reasonable to proceed with otologic and neurotologic clinic visits and operative cases based on input from expert opinion of otologic care providers, clinic/hospital administration, infection prevention and control specialists, and local and state public health leaders. Significant regional variations in COVID-19 prevalence exist; therefore, physicians working with local municipalities are best suited to make determinations on the appropriateness and timing of otologic and neurotologic care.

Objective To investigate small-particle aerosolization from mastoidectomy relevant to potential viral transmission and to test source-control mitigation strategies. Study Design Cadaveric simulation. Setting Surgical simulation laboratory. Methods An optical particle size spectrometer was used to quantify 1- to 10-µm aerosols 30 cm from mastoid cortex drilling. Two barrier drapes were evaluated: OtoTent1, a drape sheet affixed to the microscope; OtoTent2, a custom-structured drape that enclosed the surgical field with specialized ports. Results Mastoid drilling without a barrier drape, with or without an aerosol-scavenging second suction, generated large amounts of 1- to 10-µm particulate. Drilling under OtoTent1 generated a high density of particles when compared with baseline environmental levels (P < .001, U = 107). By contrast, when drilling was conducted under OtoTent2, mean particle density remained at baseline. Adding a second suction inside OtoTent1 or OtoTent2 kept particle density at baseline levels. Significant aerosols were released upon removal of OtoTent1 or OtoTent2 despite a 60-second pause before drape removal after drilling (P < .001, U = 0, n = 10, 12; P < .001, U = 2, n = 12, 12, respectively). However, particle density did not increase above baseline when a second suction and a pause before removal were both employed. Conclusions Mastoidectomy without a barrier, even when a second suction was added, generated substantial 1- to 10-µm aerosols. During drilling, large amounts of aerosols above baseline levels were detected with OtoTent1 but not OtoTent2. For both drapes, a second suction was an effective mitigation strategy during drilling. Last, the combination of a second suction and a pause before removal prevented aerosol escape during the removal of either drape.

Supplemental Digital Content is available in the text, Background: COVID-19 has become a global pandemic with a dramatic impact on healthcare systems. Concern for viral transmission necessitates the investigation of otologic procedures that use high-speed drilling instruments, including mastoidectomy, which we hypothesized to be an aerosol-generating procedure. Methods: Mastoidectomy with a high-speed drill was simulated using fresh-frozen cadaveric heads with fluorescein solution injected into the mastoid air cells. Specimens were drilled for 1-minute durations in test conditions with and without a microscope. A barrier drape was fashioned from a commercially available drape (the OtoTent). Dispersed particulate matter was quantified in segments of an octagonal test grid measuring 60 cm in radius. Results: Drilling without a microscope dispersed fluorescent particles 360 degrees, with the areas of highest density in quadrants near the surgeon and close to the surgical site. Using a microscope or varying irrigation rates did not significantly reduce particle density or percent surface area with particulate. Using the OtoTent significantly reduced particle density and percent surface area with particulate across the segments of the test grid beyond 30 cm (which marked the boundary of the OtoTent) compared with the microscope only and no microscope test conditions (Kruskall–Wallis test, p = 0.0066). Conclusions: Mastoidectomy with a high-speed drill is an aerosol-generating procedure, a designation that connotes the potential high risk of viral transmission and need for higher levels of personal protective equipment. A simple barrier drape significantly reduced particulate dispersion in this study and could be an effective mitigation strategy in addition to appropriate personal protective equipment.

Objectives To present a histopathological case of a 91‐year‐old woman who was diagnosed with superior semicircular canal dehiscence postmortem. Methods The patient was a registered donor with the National Temporal Bone Donor Program at the NIDCD National Temporal Bone, Hearing and Balance Pathology Resource Registry. Computed tomography imaging was performed on each temporal bone. The temporal bones were decalcified with ethylenediaminetetracetate and embedded in celloidin, and tissue sections were stained with hematoxylin and eosin. Horizontal sections were taken through the left temporal bone, and vertical sections were taken through the right temporal bone. Results Histopathological sections taken through the right temporal bone demonstrated no bone between the membranous wall of the superior semicircular canal and the middle fossa dura. There was no histopathological evidence of superior semicircular canal dehiscence in the left temporal bone; however, a small dehiscence would not be identified on horizontal sections. Microcavitations were observed in the common crus of the left temporal bone. Conclusion This reports describes the case of a woman who was diagnosed with superior semicircular canal dehiscence postmortem. The presence of microcavitations in the temporal bone is consistent with osteoclastic activity, which may play a role in the development of superior canal dehiscence.

Cochlear implants provide effective auditory rehabilitation for patients with severe to profound sensorineural hearing loss. Recent advances in cochlear implant technology and surgical approaches have enabled a greater number of patients to benefit from this technology, including those with significant residual low frequency acoustic hearing. Nearly all cochleae implanted with a cochlear implant electrode array develop an inflammatory and fibrotic response. This tissue reaction can have deleterious consequences for implant function, residual acoustic hearing, and the development of the next generation of cochlear prosthetics. This article reviews the current understanding of the inflammatory/foreign body response (FBR) after cochlear implant surgery, its impact on clinical outcome, and therapeutic strategies to mitigate this response. Findings from both in human subjects and animal models across a variety of species are highlighted. Electrode array design, surgical techniques, implant materials, and the degree and type of electrical stimulation are some critical factors that affect the FBR and inflammation. Modification of these factors and various anti-inflammatory pharmacological interventions have been shown to mitigate the inflammatory/FBR response. Ongoing and future approaches that seek to limit surgical trauma and curb the FBR to the implanted biomaterials of the electrode array are discussed. A better understanding of the anatomical, cellular and molecular basis of the inflammatory/FBR response after cochlear implantation has the potential to improve the outcome of current cochlear implants and also facilitate the development of the next generation of neural prostheses.

Endoscopic ear surgery (EES) has become an integral part of otologic surgery. Training in EES involves learning fundamental techniques for endoscopic visualization, becoming proficient at one-handed dissection, mastering use of instruments designed for endoscopic ear surgery, and learning to optimize the operating room setup specifically for EES. Despite the steep learning curve, EES offers several advantages over the microscope for otologic procedures. With the rise in the demand for minimally invasive approaches, EES has a clear role in the future of otologic surgery. Identifying strategies to improve the training process of EES for the novice and experienced otolaryngologist is paramount.

Introduction COVID‐19 has significantly impacted endonasal surgery, and recent experimentation has demonstrated that sinonasal drilling and cautery have significant propensity for airborne particulate generation immediately adjacent to the surgical field. In the present investigation, we assess nasopharyngeal suctioning as a mitigation strategy to decrease particulate spread during simulated endonasal surgical activity. Methods Airborne particulate generation in the 1‐10μm range was quantified with an optical particle sizer in real‐time during cadaveric‐simulated anterior and posterior endonasal drilling and cautery conditions. To test suction mitigation, experiments were performed both with and without a rigid suction placed in the contralateral nostril, terminating in the nasopharynx. Results Both anterior (medial maxillary wall and nasal septum) and posterior (sphenoid rostrum) drilling produced significant particulate generation in the 1‐10μm range throughout the duration of drilling (p

Background: COVID-19 poses a potentially significant infectious risk during procedures of the head and neck due to high viral loads in the nasal cavity and nasopharynx. Facial plastic surgery has s...

Objectives: The aim of this study is to perform a histopathologic analysis of temporal bones with an intralabyrinthine schwannoma (ILS) in order to characterize its extension. Methods: Archival temporal bones with a diagnosis of sporadic schwannoma were identified. Both symptomatic and occult nonoperated ILS were included for further analysis. Results: A total of 6 ILS were identified, with 4 intracochlear and 2 intravestibular schwannomas. All intracochlear schwannomas involved the osseous spiral lamina, with 2 extending into the modiolus. The intravestibular schwannomas were limited to the vestibule, but growth into the bone next to the crista of the lateral semicircular canal was observed in 1 patient. Conclusions: Complete removal of an ILS may require partial removal of the modiolus or bone surrounding the crista ampullaris as an ILS may extend into these structures, risking damage of the neuronal structures. Due to the slow growth of the ILS, it remains unclear if a complete resection is required with the risk of destroying neural structures hindering hearing rehabilitation with a cochlear implant.

Objective To show the histologic correlate of the cochlear cleft, a small low density focus just anterior to the oval window seen on computed tomography (CT) in three temporal bone specimens. Patients Three temporal bone specimens donated to the National Temporal Bone Registry from patients aged 34 weeks gestation, 42 years, and 89 years with cochlear clefts seen on CT were studied. Intervention Review and comparison of postmortem high-resolution CT and temporal bone histopathology MAIN OUTCOME MEASURE:: Correlation of CT findings with temporal bone histopathology. Results The cochlear cleft visible on CT as a pericochlear lucency anterior to the oval window corresponds to fatty marrow in areas of incomplete endochondral ossification of the otic capsule. The cochlear cleft is distinct from the fissula ante fenestram and can be present in adults. Conclusion In these three cases, the cochlear cleft represents an area of fatty marrow from incomplete ossification of the otic capsule and can be present in adults. Care should be taken when interpreting temporal bone CT to avoid mistaking the cochlear cleft for true pathology (otosclerosis) of the temporal bone.

Objective To determine the efficacy of fibroblast growth factor-2 (FGF-2) in treating chronic nonhealing tympanic membrane (TM) perforations. Method Double-blinded, randomized placebo controlled phase 2 clinical trial for patients with chronic TM perforations of more than 3 months duration with a cross-over arm. Patients received either FGF-2 or placebo (sterile water) saturated gelatin sponge in the perforation after rimming the perforation under topical anesthesia. The perforation was then covered with Tisseel fibrin glue. The primary endpoint was complete closure of the TM perforation. Secondary end points included change in hearing and partial TM closure rates. The TM was examined every 3 weeks with otoendoscopy for closure. The treatment was repeated if there was incomplete closure every 3 weeks up to a total of three treatments per arm. Results Seventy four patients were recruited for the study. Fifty seven met eligibility criteria and fifty four completed the study. Ten of 14 perforations closed completely in the placebo group (71.4%) and 23 of 40 perforations closed completely in the FGF-2 treatment group (57.5%), P value = .36. Pure tone averages and word recognition scores were not statistically significantly different between study groups post-treatment. After initial complete closure, re-perforation occurred in seven FGF-2 treated patients and two placebo patients making the effective final closure rate 40% for FGF and 57% for placebo, respectively. Conclusion No statistically significant difference in tympanic membrane perforation closure rate was found between the FGF-2 and placebo groups. There were no differences in hearing outcomes between the groups. Level of evidence 1b.

Hypothesis Cochlear implantation may cause an increase in the number of macrophages in the human cochlea similar to previous findings in the vestibular endorgans. Background Macrophages play a key role in both an inflammatory response and homeostatic maintenance. Recently, an increase in the prevalence of macrophages was demonstrated in the human vestibular endorgans after implantation. However, the prevalence of macrophages in the cochlea after implantation is unclear. The aim of this study was to compare the distribution and prevalence of macrophages in implanted human cochleae and the contralateral unimplanted ears. Methods The prevalence of macrophages in the cochlea in 10 human subjects who had undergone unilateral cochlear implantation was studied by light microscopy using anti-Iba1 immunostaining. The densities of macrophages in the osseous spiral lamina (OSL) and Rosenthal's canal (RC) in implanted cochleae were compared with the contralateral unimplanted ears. The distribution of macrophage morphology (amoeboid, transitional, and ramified) was also compared. Results There were activated and phagocytosing macrophages within the fibrotic sheath surrounding the electrode track and within fibrous tissue with lymphocytic infiltration in implanted ears. The densities of macrophages in OSL and RC in implanted ears were significantly greater than in unimplanted ears in some areas. There was also a difference in the prevalence of macrophage phenotype between the OSL and RC. Conclusion An increase in the density of macrophages in the cochlea after cochlear implantation was demonstrated. Both phagocytosis and anti-inflammatory activity of macrophages were suggested by the distribution and prevalence of macrophages in the implanted cochlea.

Objective To assess long-term hearing outcomes in patients treated with third-generation bisphosphonates for otosclerosis-related progressive sensorineural hearing loss (SNHL). Study Design Retrospective case series review Methods We performed a retrospective case series review of patients with otosclerosis and progressive SNHL. Patients were treated with either risedronate or zoledronate after a diagnosis of otosclerosis with a significant SNHL component. Bone conduction pure tone threshold averages (BC-PTAs) and word recognition scores (WRS) before and after bisphosphonate administration in long-term follow-up was analyzed. Significant change in BC-PTA was defined as greater than 10dB or between 4% and 18% in WRS based on binomial variance. Results Seven patients were identified and 14 ears met inclusion criteria. Three patients were female and the mean age was 48.3 ± 10.3 years. The mean duration between treatment with bisphosphonate administration and long-term post-treatment follow-up audiometry was 87.6 ± 18.3 months, with a range of 61.6 to 109.1 months and median of 89.2 months. Analysis using BC-PTA and WRS demonstrated that 11 ears remained stable while 2 improved and 1 worsened. No patient experienced any major complication as the result of bisphosphonate therapy. Conclusion Treatment with third-generation bisphosphonates is associated with stability in otosclerosis-related sensorineural hearing over 5- to 9-year period. These results suggest that such medications may prevent the progression of SNHL in patients with otosclerosis. Level of Evidence 4 (Case series)

An inflammatory tissue reaction around the electrode array of a cochlear implant (CI) is common, in particular at the electrode insertion region (cochleostomy) where mechanical trauma often occurs. However, the factors determining the amount and causes of fibrous reaction surrounding the stimulating electrode, especially medially near the perimodiolar location, are unclear. Temporal bone (TB) specimens from patients who had undergone cochlear implantation during life with either Advanced Bionics (AB) Clarion ™ or HiRes90K™ (Sylmar, CA, USA) devices that have a half-band and a pre-curved electrode, or Cochlear ™ Nucleus (Sydney, Australia) device that have a full-band and a straight electrode were evaluated. The thickness of the fibrous tissue surrounding the electrode array of both types of CI devices at both the lower (LB) and upper (UB) basal turns of the cochlea was quantified at three locations: the medial, inferior, and superior aspects of the sheath. Fracture of the osseous spiral lamina and/or marked displacement of the basilar membrane were interpreted as evidence of intracochlear trauma. In addition, post-operative word recognition scores, duration of implantation, and post-operative programming data were evaluated. Seven TBs from six patients implanted with AB devices and five TBs from five patients implanted with Nucleus devices were included. A fibrous capsule around the stimulating electrode array was present in all twelve specimens. TBs implanted with AB device had a significantly thicker fibrous capsule at the medial aspect than at the inferior or superior aspects at both locations (LB and UB) of the cochlea (Wilcoxon signed-ranks test, p 0.01). TBs implanted with a Nucleus device had no difference in the thickness of the fibrous capsule surrounding the track of the electrode array (Wilcoxon signed-ranks test, p 0.05). Nine of fourteen (64%) basal turns of the cochlea (LB and UB of seven TBs) implanted with AB devices demonstrated intracochlear trauma compared to two of ten (20%) basal turns of the cochlea (LB and UB of five TBs) with Nucleus devices, (Fisher exact test, p 0.05). There was no significant correlation between the thickness of the fibrous tissue and the duration of implantation or the word recognition scores (Spearman rho, p = 0.06, p = 0.4 respectively). Our outcomes demonstrated the development of a robust fibrous tissue sheath medially closest to the site of electric stimulation in cases implanted with the AB device electrode, but not in cases implanted with the Nucleus device. The cause of the asymmetric fibrous sheath may be multifactorial including insertional trauma, a foreign body response, and/or asymmetric current flow.

Objectives 1) To evaluate the long-term incidence and degree of the sensorineural component of hearing loss (SNHL) in patients with otosclerosis after accounting for expected age-related hearing loss. 2) To identify variables that might predict development of sensorineural hearing loss due to otosclerosis. Study design Retrospective audiometric database and chart review. Setting Tertiary referral center. Patients Consecutive patients with otosclerosis observed between 1994 and 2004, with ≥10 years follow-up, excluding patients with postoperative hearing loss or surgery before the initial audiogram. Intervention Bone conduction (BC) thresholds at 0.5, 1, 2, and 4 kHz and Word Recognition. Main outcome measure BC threshold change (BCTC) over ≥10 years minus estimated age-related threshold change (ARTC) specific to age and sex for each patient (based on ISO 7029 reference population). Results Three-hundred fifty-seven ears (290 patients) met study criteria, including 217 ears that had undergone stapedectomy during the study period. Mean follow-up was 14.0 years. The average BCTC after subtracting estimated ARTC was 4.6, 2.6, 3, and 2.7 dB for 0.5, 1, 2, and 4 kHz frequencies, respectively. However, 34% of ears (122 ears) had clinically significant progression of SNHL during the study period (>10 dB BCTC beyond expected ARTC at ≥2 frequencies). Multivariate analysis demonstrated that the probability of developing clinically significant SNHL was higher for women (odds ratio 1.86, p = 0.018) and lower for operated patients (odds ratio 0.46, p = 0.002). Conclusion The average long-term sensorineural hearing loss due to otosclerosis was statistically significantly more than for age alone at each frequency, but these average values (from 2.6 to 4.6 dB for tested frequencies) were clinically insignificant. Approximately one-third of patients with otosclerosis demonstrated a clinically significant progression of the sensorineural component of hearing loss, with the average BCTC above expected age-related changes ranging from 10.2 to 14.6 dB for tested frequencies among this subgroup.

Postmortem temporal bone computed tomography (CT) and histopathologic findings in an infant with CHARGE syndrome revealed bilateral cochleovestibular hypoplasia, including cochlear pathology relevant to cochlear implant candidacy. Both ears had absence of the superior semicircular canals (SCCs), severely hypoplastic posterior SCCs, and hypoplastic (right ear) or absent (left ear) lateral SCCs seen on CT and histopathology. Histopathology further revealed the absence of all SCC ampullae except the right lateral SCC ampulla and atrophic vestibular neuroepithelium in the saccule and utricle bilaterally. The right cochlea consisted of a basal turn with patent round window, and malformed middle turn (type IV cochlear hypoplasia), with a small internal auditory canal (IAC) but near normal cochlear nerve aperture (fossette). Quantification of spiral ganglion neurons (SGNs) on histologic sections revealed a reduced SGN population (35% of normal for age), but this ear would still have likely achieved benefit from a cochlear implant based on this population. The left cochlea consisted of only a basal turn with patent round window (type III cochlear hypoplasia) with a small IAC and very small cochlear nerve aperture. Notably, histology revealed that there were no SGNs in the cochlea, and therefore, this ear would not have been a good candidate for cochlear implantation. Level of evidence: IV.

Objective To describe the presentations and treatment results for patients with concurrent otosclerosis and superior semicircular canal dehiscence (SCD). Study design Retrospective case series and literature review. Setting Tertiary academic medical center. Patients Patients with concurrent diagnoses of otosclerosis (radiographically and/or surgically confirmed) and SCD (radiographically confirmed) in the same ear. Intervention(s) Review of medical records. Main outcome measure(s) Clinical presentations, outcomes following stapedotomy. Results Eight patients with 10 affected ears were identified. All patients presented with slowly progressive conductive hearing loss, normal otoscopy, absent acoustic reflexes, and without other symptoms of SCD syndrome. Seven patients were treated with stapedotomy and 1 with hearing aids. Of those treated with stapedotomy, a persistent conductive hearing loss was the most common hearing result. One patient had near-complete closure of their air bone gap. None had a profound sensorineural hearing loss. Four patients had unmasking of SCD symptoms. Conclusions The clinical and audiometric presentations of patients with concurrent otosclerosis and SCD are often indistinguishable from those of patients with only otosclerosis. Computed tomography of the temporal bone is the only way to identify concurrent SCD. Stapedotomy in these patients typically results in a persistent conductive hearing loss, though 14 to 33% of patients experience near-complete closure of their air bone gap. SCD symptoms are unmasked in 57 to 63% of patients who undergo stapedotomy. Further work is needed to delineate the utility of routine preoperative computed tomography scan in otosclerosis patients, and to identify prognostic factors for patients with concurrent otosclerosis and SCD who wish to undergo stapedotomy.

A study of clinical records and temporal bone histopathology from a woman with bilateral otosclerosis who was treated with lateral semicircular canal fenestration procedures as well as stapedectomy.

OBJECTIVES To explore the relationship between hearing loss and the internal auditory canal (IAC) diverticula. To determine whether diverticula exist within or medial to the otic capsule and the prevalence in a control population. METHODS Retrospective review of adult patients with radiologic evidence of an IAC diverticulum, no evidence of otosclerosis, and audiometric testing. Analyzed degree of hearing loss and width, length, height, and volume of diverticulum. Hounsfield unit (HU) measurements lateral and medial to the diverticulum. RESULTS Pure tone average (PTA), air-bone gap, and WRS (word recognition score) did not correlate with length, width, height, and volume of the diverticula. In patients with a unilateral diverticulum, there was no difference in mean PTA or WRS when comparing the diverticulum and nondiverticulum sides. Mean HU lateral to the diverticulum (2104 HU) was found to be significantly higher than medial to the diverticulum (1818 HU). There is a 5.6% prevalence of IAC diverticula in patients who underwent high-resolution computed tomography (CT) scans for chronic sinusitis (control group). CONCLUSION These data support the notion that hearing loss in this population is a product of sampling bias. The size of IAC diverticula does not correlate with the degree of hearing loss, and there is no statistically significant association between sensorineural hearing loss (SNHL) and the presence of an IAC diverticulum. IAC diverticula may exist medial to, rather than within, the otic capsule given the significant difference in mean HUs medial and lateral to the diverticula. LEVEL OF EVIDENCE 4 Laryngoscope, 130:1011-1015, 2020.

Objectives/hypothesis Investigate the otopathology of angiosarcoma of the temporal bone, which has not been previously described in the literature. Study design Postmortem evaluation and literature review. Methods Postmortem histological evaluation of the temporal bones and review of the literature for the treatment and prognosis of this rare disease were performed. Results A 50-year-old male with right chronic otitis media presented with progressive hearing loss, disequilibrium, otalgia, and acute facial paresis. Biopsy of the external auditory canal was unrevealing, but specimens from a canal wall down tympanomastoidectomy later showed high-grade angiosarcoma. Magnetic resonance imaging demonstrated an unresectable middle ear and mastoid mass extending superiorly into the temporal lobe. The patient received induction chemotherapy followed by proton beam radiation therapy and concurrent paclitaxel and bevacizumab. His course was complicated by a cerebrospinal fluid leak and cauda equina syndrome from leptomeningeal sarcomatosis. The patient died after developing meningitis and a temporal lobe abscess. Postmortem otopathology revealed persistent angiosarcoma in the internal auditory canal, although none was found in the middle ear or mastoid. There was inflammatory infiltrate throughout the mastoid, with direct extension of neutrophils and bacteria into the cochlea and through the tegmen into the middle cranial fossa. Conclusions Angiosarcoma of the temporal bone can arise in the setting of chronic otitis media. In this case, postmortem temporal bone sections demonstrated viable cancer despite chemoradiation. Inflammatory infiltrates crossing from the middle ear/mastoid into the labyrinth and central nervous system illustrate pathways for the development of otogenic meningitis. Level of evidence 4 Laryngoscope, 129:737-742, 2019.

OBJECTIVES 1) To evaluate the long-term (≥10 year) clinical incidence of endolymphatic hydrops (EH) after stapedectomy for otosclerosis, using low-frequency sensorineural hearing loss (LFSNHL) as a marker for EH. 2) To determine the histologic incidence of EH in human temporal bone specimens (TBS) with a history of stapedectomy for otosclerosis. 3) To determine the histologic incidence of EH in a control group of human TBS. STUDY DESIGN Retrospective review and temporal bone study. SETTING Tertiary medical center and temporal bone pathology laboratory. PATIENTS Patients with otosclerosis, human TBS with otosclerosis, and human TBS with presbycusis as the control group. INTERVENTION Pure-tone audiometry, temporal bone pathology. MAIN OUTCOME MEASURES 1) LFSNHL, defined as >10 decibel elevation of bone conduction thresholds at 250 and 500 Hz, after correcting for age-related hearing loss (per ISO 7029). 2) Histologic assessment of EH. RESULTS In patients with otosclerosis, 8 of 110 (7.3%) operated patients versus 3 of 123 (2.4%) nonoperated patients developed LFSNHL (p = 0.08). No patients with LFSNHL had other symptoms of EH. In TBS with otosclerosis, 11 of 93 (11.8%) operated TBS versus 3 of 156 (1.9%) nonoperated TBS had evidence of EH (p

Objective To correlate the computed tomography (CT) and magnetic resonance imaging (MR) findings in a patient with incomplete partition-type II (IP-II) anomaly with histopathology in a similar human temporal bone specimen. To discover the histologic correlate of a common finding on MR of an "apparent" interscalar septum (IS). Patients A patient with sensorineural hearing loss and imaging findings characteristic of IP-II, and a patient with histopathologic IP-II anomaly. Intervention High-resolution CT, MR, and review of postmortem temporal bone histopathology. Main outcome measure Correlation of temporal bone histopathology with CT and MR findings. Results Consistent findings of IP-II anomaly on CT, absence of the IS between the more distal turns and flattening of the interscalar ridge between the distal basal turn and the middle turn, were present. The signal void surrounding the cochlea on MR also demonstrated flattening of the interscalar ridge. However, a thin band-like area of low T2 signal was seen, which could be mistaken for an IS. Correlation with temporal bone histopathology revealed that the modiolus was foreshortened, and the spiral ganglion neuron dendritic processes continued toward the upper middle turn through the osseous spiral lamina, likely accounting for the MR finding. Conclusion Correlation of CT, MR, and histopathology in IP-II shows an "apparent" segmentation representing a continuum of neurosensory elements in approximately the same location of the expected location of a normal IS. Care should be taken when interpreting MR imaging in isolation. Understanding the bony outline of the cochlea on imaging may prove to be complementary.

Objective: To describe the human temporal bone histopathology in NOG-related symphalangism spectrum disorder, a spectrum of congenital stape fixation syndromes caused by mutations in the NOG gene. To discuss implications for clinical management. Patient: A patient with a mutation in the NOG gene. Intervention(s): Removal of temporal bones, postmortem temporal bone computed tomography, histologic processing, and review of temporal bones. Main Outcome Measure(s): Temporal bone histopathology and correlation with clinical, genetic, audiologic, and radiologic evaluations. Results: Both temporal bones demonstrated fixation of the stapes footplate to the otic capsule because of a circumferential bridge of calcified cartilage. In the right ear (unoperated), there was no additional abnormality of the ossicles or ossicular joints. In the left ear, fenestrations of the stapes footplate and the lateral semicircular canal were seen, consistent with a history of stapedectomy and fenestration procedure. Severe loss of spiral ganglion neurons throughout the left cochlea accounted for the profound sensorineural hearing loss; there was a normal number of spiral ganglion neurons in the right ear. In both ears, the cochleae demonstrated grossly preserved organs of Corti. Conclusion: The temporal bone pathologic correlate for conductive hearing loss in this patient with a NOG mutation was circumferentially calcified cartilage bridging the stapedovestibular joint space. The temporal bone histopathology findings suggest that conductive hearing loss related to NOG mutation should be improved after stapedectomy; however, care must be taken in extrapolating to all patients with NOG mutations because there may be variability in the pathology, especially given the variability of NOG spectrum disorders.

Facial nerve schwannomas are benign peripheral nerve sheath tumors that arise from Schwann cells, and most commonly present with facial paresis and/or hearing loss. Computed tomography and MRI are critical to diagnosis. Management decisions are based on tumor size, facial function, and hearing status. Observation is usually the best option in patients with good facial function. For patients with poor facial function, the authors favor surgical resection with facial reanimation. There is growing evidence to support radiation treatment in patients with progressively worsening moderate facial paresis and growing tumors.

BACKGROUND: Neurofibromatosis Type 2 (NF2) is a dominantly inherited tumour syndrome with a phenotype which includes bilateral vestibular (eighth cranial nerve) schwannomas. Conventional thinking suggests that these tumours originate at a single point along the superior division of the eighth nerve. METHODS: High resolution MRI was performed in children genetically proven to have NF2. The superior vestibular nerve (SVN) and inferior vestibular nerve (IVN) were visualised along their course with points of tumour origin calculated as a percentage relative to the length of the nerve. RESULTS: Out of 41 patients assessed, 7 patients had no identifiable eighth cranial nerve disease. In 16 patients there was complete filling of the internal auditory meatus by a tumour mass such that its specific neural origin could not be determined. In the remaining 18 cases, 86 discrete separate foci of tumour origin on the SVN or IVN could be identified including 23 tumours on the right SVN, 26 tumours on the right IVN, 18 tumours on the left SVN and 19 tumours on the left IVN. DISCUSSION: This study, examining the origins of vestibular schwannomas in NF2, refutes their origin as being from a single site on the transition zone of the superior division of the vestibular nerve. We hypothesise a relationship between the number of tumour foci, tumour biology and aggressiveness of disease. The development of targeted drug therapies in addition to bevacizumab are therefore essential to improve prognosis and quality of life in patients with NF2 given the shortcomings of surgery and radiation treatments when dealing with the multifocality of the disease.

Otologic trauma was the most common physical injury sustained after the April 15, 2013, Boston Marathon bombings. The goal of this study is to describe the resultant otologic morbidity and to report on early outcomes.Multi-institutional prospective cohort study.Children and adults seen for otologic complaints related to the Boston Marathon bombings comprised the study population. Participants completed symptom assessments, quality-of-life questionnaires, and audiograms at initial and 6-month visits. Otologic evaluation and treatment, including tympanoplasty results, were reviewed.More than 100 patients from eight medical campuses have been evaluated for blast-related otologic injuries; 94 have enrolled. Only 7% had any otologic symptoms before the blasts. Ninety percent of hospitalized patients sustained tympanic membrane perforation. Proximity to blast (RR = 2.7, p0.01) and significant nonotologic injury (RR = 2.7, p0.01) were positive predictors of perforation. Spontaneous healing occurred in 38% of patients, and tympanoplasty success was 86%. After oral steroid therapy in eight patients, improvement in hearing at 2 and 4 kHz was seen, although changes did not reach statistical significance. Hearing loss, tinnitus, hyperacusis, and difficulty hearing in noise remain persistent and, in some cases, progressive complaints for patients. Otologic-specific quality of life was impaired in this population.Blast-related otologic injuries constitute a major source of ongoing morbidity after the Boston Marathon bombings. Continued follow-up and care of this patient population are warranted.

BACKGROUND AND PURPOSE: Incomplete partition type II of the cochlea, commonly coexisting with an enlarged vestibular aqueduct, can be a challenging diagnosis on MR imaging due to the presence of a dysplastic spiral lamina–basilar membrane neural complex, which can resemble the normal interscalar septum. The purpose of this study was to determine a reproducible, quantitative cochlear measurement to assess incomplete partition type II anomalies in patients with enlarged vestibular aqueducts using normal-hearing ears as a control population. MATERIALS AND METHODS: Retrospective analysis of 27 patients with enlarged vestibular aqueducts (54 ears) and 28 patients (33 ears) with normal audiographic findings who underwent MR imaging was performed. Using reformatted images from a cisternographic 3D MR imaging produced in a plane parallel to the lateral semicircular canal, we measured the distance (distance X) between the osseous spiral lamina-basilar membrane complex of the upper basal turn and the first linear signal void anterior to the basilar membrane. RESULTS: The means of distance X in patients with normal hearing and prospectively diagnosed incomplete partition type II were, respectively, 0.93 ± 0.075 mm (range, 0.8–1.1 mm) and 1.55 ± 0.25 mm (range, 1–2.1 mm; P < .001). Using 3 SDs above the mean of patients with normal hearing (1.2 mm) as a cutoff for normal, we diagnosed 21/27 patients as having abnormal cochleas; 4/21 were diagnosed retrospectively. This finding indicated that almost 20% of patients were underdiagnosed. Interobserver agreement with 1.2 mm as a cutoff between normal and abnormal produced a κ score of 0.715 (good). CONCLUSIONS: Incomplete partition type II anomalies on MR imaging can be subtle. A reproducible distance X of ≥1.2 mm is considered abnormal and may help to prospectively diagnose incomplete partition type II anomalies.

Objective To assess long‐term hearing outcomes in patients treated with third‐generation bisphosphonates for otosclerosis‐related progressive sensorineural hearing loss (SNHL). Study Design Retrospective case series review Methods We performed a retrospective case series review of patients with otosclerosis and progressive SNHL. Patients were treated with either risedronate or zoledronate after a diagnosis of otosclerosis with a significant SNHL component. Bone conduction pure tone threshold averages (BC‐PTAs) and word recognition scores (WRS) before and after bisphosphonate administration in long‐term follow‐up was analyzed. Significant change in BC‐PTA was defined as greater than 10dB or between 4% and 18% in WRS based on binomial variance. Results Seven patients were identified and 14 ears met inclusion criteria. Three patients were female and the mean age was 48.3 ± 10.3 years. The mean duration between treatment with bisphosphonate administration and long‐term post‐treatment follow‐up audiometry was 87.6 ± 18.3 months, with a range of 61.6 to 109.1 months and median of 89.2 months. Analysis using BC‐PTA and WRS demonstrated that 11 ears remained stable while 2 improved and 1 worsened. No patient experienced any major complication as the result of bisphosphonate therapy. Conclusion Treatment with third‐generation bisphosphonates is associated with stability in otosclerosis‐related sensorineural hearing over 5‐ to 9‐year period. These results suggest that such medications may prevent the progression of SNHL in patients with otosclerosis. Level of Evidence 4 (Case series).

Author(s): Ishiyama, Akira; Doherty, Joni; Ishiyama, Gail; Quesnel, Alicia M; Lopez, Ivan; Linthicum, Fred H | Abstract: ObjectiveTo evaluate for potential causes of delayed loss of residual hearing that variably occurs with hybrid cochlear implants.Study designHistopathological evaluation of 29 human temporal bone (HTB) with cochlear implant (CI).SettingThe Neurotology and House HTB Laboratory of UCLA (House-UCLA).Subjects and methodsHTB from CI patients from the House-UCLA HTB Laboratory (n = 28) and one courtesy of Massachusetts Eye and Ear Infirmary (MEEI). Histopathological analysis to identify the location of cochleostomy, fibrosis, and bone formation in the scala vestibuli and tympani, and endolymphatic hydrops. Spiral ganglion neuron counts were obtained. Statistical analysis compared presence of cochleostomy and location with the histopathological findings.ResultsSeventeen of 29 bones with fibrosis in the scala vestibule (SV) and tympani had evidence of a cochleostomy involving the SV containing the ductus reunions, all of which had hydrops. Ten of 11 bones had no SV fibrosis, and a cochleostomy limited to the scala tympani, of which all had no hydrops. One HTB had moderate SV fibrosis not involving the ductus reuniens, and was without hydrops. One HTB had a SV cochleostomy but the electrode ruptured Reissner's membrane, and was without hydrops. Cochleostomy was significantly associated with SV fibrosis and hydrops (p l 0.01), those without hydrops had no SV atrophy (p l 0.01). Round window insertion was associated with no fibrosis and no hydrops.ConclusionWe hypothesize that cochleostomies involving scala vestibuli incite fibrosis, compromising the ductus reuniens, causing hydrops which may cause the delayed loss of residual low frequency hearing in CI.

HYPOTHESIS Nonotosclerotic stapes fixation does not represent a significant cause of stapes ankylosis in patients undergoing stapedectomy; the vast majority have otosclerosis. BACKGROUND Nonotosclerotic stapes fixation has been proposed as the diagnosis in 30 to 40% of patients undergoing stapedectomy (after excluding rare congenital, systemic, and syndromic causes of stapes fixation and tympanosclerosis). This finding was based on the histopathologic evaluation of total stapedectomy surgical specimens. Since these specimens do not include the surrounding otic capsule, the histopathologic evidence of otosclerosis may be missed. METHODS Human temporal bone specimens from patients who underwent stapes mobilization, stapedotomy, or stapedectomy during life were evaluated for histologic evidence of otosclerosis. Patients with a history of temporal bone trauma, tympanosclerosis, and congenital, systemic, or syndromic causes of stapes fixation were excluded. Therefore, most temporal bone donors carried a clinical diagnosis of otosclerosis. RESULTS Two hundred ten specimens from three temporal bone collections were independently evaluated. Otosclerosis was found on histology in 99% (207/210). Therefore, the incidence of nonotosclerotic stapes fixation was 1% (3/210). In two of the three patients who did not have otosclerosis, the contralateral temporal bone had otosclerosis on histopathologic evaluation. These patients may have had otosclerosis in the footplate only (which was removed at the time of surgery and not available for review). CONCLUSION Nonotosclerotic stapes fixation is not likely a distinct pathologic classification from otosclerosis. Most patients diagnosed with nonotosclerotic stapes fixation likely have otosclerosis, but do not have otosclerotic foci in the stapes itself.

OBJECTIVE Pfeiffer syndrome is a rare craniosynostotic disorder resulting in premature bony fusion of the skull, which can result in abnormal temporal bone and vascular anatomy and hearing loss. Cochlear implantation in these patients requires thoughtful surgical planning given the potential for limited access to the cochlea because of subcutaneous or intratemporal vasculature. Herein, we present a patient with Pfeiffer syndrome who underwent successful cochlear implantation using preoperative Doppler ultrasound to identify large extracranial venous anatomy followed by a modified transcanal surgical approach. PATIENTS An adult female patient with Pfeiffer syndrome. INTERVENTION(S) Preoperative axial computed tomography, catheter angiography, Doppler ultrasound, and right side cochlear implant. MAIN OUTCOME MEASURE(S) Surgical feasibility and audiometric outcomes including aided thresholds and word recognition score. RESULTS Successful cochlear implantation was performed via a modified transcanal approach with blind sac closure of the external auditory meatus. Full electrode insertion was obtained through a round window approach. No extracranial or intratemporal vessels were encountered during surgery. CONCLUSION Patients with significant craniosynostoses and vascular malformations of the temporal bone can undergo successful cochlear implantation. Careful preoperative planning with high-resolution CT, MRA, and MRV, and/or traditional catheter angiography can assist in determining surgical feasibility and minimizing risk. We recommend preoperative Doppler ultrasound of abnormal extracranial vessels to assist in safe placement of incisions and hardware.

Objective To evaluate hearing outcomes in patients treated with third generation bisphosphonates for otosclerosis-related sensorineural hearing loss (SNHL). Hypothesis Otosclerosis is a disease of abnormal bone remodeling in the otic capsule. In recent years, third generation bisphosphonates, with more powerful anti-resorptive properties and increased bone affinity, have demonstrated effectiveness in the treatment of osteoporosis and other metabolic bone diseases. We hypothesized that newer generation bisphosphonates, such as risedronate and zoledronate, would be effective in slowing the progression of SNHL in patients with otosclerosis. Study design Retrospective review. Setting Tertiary referral center, ambulatory care. Interventions Risedronate or zoledronate administration. Main outcome measures Bone conduction pure tone threshold averages (PTAs) and word recognition (WR) scores were examined for each ear before and after bisphosphonate treatment. Criteria for significant change were defined as greater than 10 decibels in PTA or between 4% and 18% in WR based on binomial variance. Results All 10 patients had audiometric progression of SNHL in the pretreatment monitoring interval and 12 ears met criteria for significant progression. All 10 patients (19 ears) showed at least no significant progression of SNHL (i.e., stabilization) at an average follow-up of 13 months. Two patients (3 ears) showed improvement by defined audiometric criteria. There were no major complications. Conclusion Treatment with zoledronate or risedronate stabilized progressive SNHL related to otosclerosis in this small group of patients. Further evaluation of third-generation bisphosphonate treatments is warranted.