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Objectives: The purpose of this study was to determine the normal volume ranges of cochlear duct, saccule, and utricle, and to assess endolymphatic hydrops in Ménière disease.Study Design: Retrospective temporal bone study.Methods: Three-dimensional (3-D) images of membranous labyrinth were reconstructed from 31 normal temporal bones, six temporal bones from three patients with bilateral Ménière disease, and 16 temporal bones from eight patients with unilateral Ménière disease. Volumes of each part of membranous labyrinth were measured in each temporal bone group after 3-D reconstruction.Results: The mean volumes and upper normal volume limits (over the 95% confidence interval) of the cochlear duct, saccule, and utricle were 7.67 and 9.77 mm(3), 2.42 and 3.68 mm(3), and 10.65 and 16.45 mm(3), respectively. All three patients with bilateral Ménière disease showed endolymphatic hydrops (excess of volume over normal limits) in both ears. Of eight patients with unilateral Ménière disease, five had no symptom in the contralateral ear, whereas three patients had histories of progression from unilateral to bilateral Ménière disease 13-21 years after the initial onset. All of the diseased and three of eight contralateral ears showed endolymphatic hydrops. In contrast, no hydrops was observed in any part of the membranous labyrinth in asymptomatic ears.Conclusions: Our findings suggest that cochleosaccular hydrops is a sensitive finding in Ménière disease. In addition, the volume data obtained from this study could be useful as a standard value for the assessment of hydrops in diagnostic imaging of the inner ear in Ménière disease. [ABSTRACT FROM AUTHOR]

Conclusion: The 3D models of endolymphatic and perilymphatic spaces enabled us to obtain normal and pathological volumes of each space and helped us to understand the 3D structure of various parts of the inner ear and of endolymphatic hydrops. Objective: To make a 3D model of the inner ear using sections of temporal bone with and without hydrops. Materials and methods: Every 10th 20 µm thick section of temporal bone was collected from two ears with endolymphatic hydrops and five ears without hydrops. Using ZedView, 3D Doctor, FreeForm as analytical software, a 3D model of the inner ear was obtained by reconstruction of these sections. The volumes of the endolymphatic (EV) and perilymphatic spaces (PV) were calculated in each part of the cochlea and vestibular apparatus including the semicircular canals, but the endolymphatic duct and sac were not included. Results: In normal ears (controls), the average cochlear EV was 5.1 µl and the PV was 41.9 µl, and the average vestibular EV was 24.0 µl and the PV 75.7 µl. In one hydropic ear, the cochlear EV was 17.5 µl, cochlear PV 30.7 µl, vestibular EV 42.5 µl, and vestibular PV 33.4 µl. In the other hydropic ear, cochlear EV was 31.2 µl, cochlear PV 30.1 µl, vestibular EV 25.6 µl, and vestibular PV 71.8 µl. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: Large vestibular aqueduct syndrome (LVAS) is one of the common causes of hearing loss (HL). All prior studies have reported some anomalies associated with LVAS by imaging techniques. This study was undertaken to determine prevalence of LVAS in our temporal bone (TB) collection and its relationship to other systemic or otologic anomalies. Study Design: Retrospective, TB histopathologic study. Methods: Anteroposterior diameters of internal (IA) and external (EA) apertures were measured in 40 normal TBs (40 cases). TBs were considered as large vestibular aqueduct (LVA) if width of apertures was 95% greater than "normals." Systemic and otologic anomalies and histopathology of ears with LVAS were noted. Result: Of 1,608 non-"normal" TBs, 63 had LVA. There was negative correlation between IA and EA in 48 TBs with only enlarged IA. Fifteen TBs with enlarged EA always had enlarged IA and were therefore considered as LVAS. The most common pathologic condition was congenital heart anomaly. The most common syndrome or dysplasia was Mondini's. The most common anomalies of external and middle ears were dehiscent facial nerves, low set auricles, and ossicular deformities. Inner ear anomalies included modiolar deficiencies, hair cell loss, interscalar septum defects, and strial atrophy. There was no record of family history of HL, head injury, or craniofacial, branchial, or thyroidal abnormalities. Conclusion: Because HL associated with LVAS may be attributed to other ear anomalies, it is important to investigate other inner ear problems and system diagnoses that may indicate a syndrome in patients with radiologically diagnosed LVAS. [ABSTRACT FROM AUTHOR]

Objective: To correlate pathologic findings of the tympanic membrane with pathologic changes in the middle ear cleft in chronic otitis media.Study Design: Retrospective.Material and Methods: One hundred-fifty temporal bones from 97 subjects with chronic otitis media (defined as middle ear pathologic changes including granulation tissue, fluid, cholesteatoma, cholesterol granuloma, tympanosclerosis, and ossicular changes) were selected to correlate the presence of these middle ear pathologies with histopathologic changes of the tympanic membrane. The tympanic membrane pathologies included perforation, myringosclerosis, retraction, hemorrhage, fluid-filled cystic spaces, or dilated vessels. Temporal bones were also assessed for atelectasis. Fifty-six normal temporal bones were taken as controls for measurements.Results: Significant correlations between tympanic membrane and middle ear pathology included myringosclerosis and granulation tissue, myringosclerosis and ossicular pathology, retraction and cholesterol granuloma, retraction and cholesteatoma, retraction and ossicular pathology, perforation and ossicular pathology, and hemorrhage and granulation tissue. Additive effects of some pathologies were also observed. Almost half the bones with middle ear pathology had no associated tympanic membrane pathology, whereas multiple pathologic changes in the tympanic membrane generally showed underlying multiple pathologic changes in the middle ear.Conclusion: When tympanic membrane pathology is detected otoscopically, its presence, alone or in combination, can be a strong indicator of underlying middle ear pathology. However, a normal-appearing tympanic membrane does not exclude the possibility of middle ear pathology. These findings suggest the need for other diagnostic tools such as multifrequency tympanometry and otoacoustic emissions to complement otoscopy for diagnosis of middle ear pathology, especially in a tympanic membrane that appears "normal." [ABSTRACT FROM AUTHOR]

Objectives: To characterize glycoconjugate expression in normal human eustachian tubes and study the alterations in glycoconjugate expression found in eustachian tubes with otitis media.Study Design: Using lectin histochemistry, alterations in glycoconjugates were studied in three normal temporal bones, in four temporal bones with mucoid otitis media (MOM), and in five with serous otitis media (SOM).Methods: Sections of previously processed temporal bones were decelloidinized, and then incubated with seven biotinylated lectins--WGA, SNA, MAA, BPA, PNA, UEA-1, and LcH--that reflect seven carbohydrate residues of glycoconjugates, respectively: GlcNAc/NeuNAc, NeuNAc alpha(2-6)GalNAc, NeuNAc alpha(2-3)GalNAc, Gal beta(1-3) GalNAc, L-fucose, and alpha-mannose residues. Control sections were incubated with inhibitory carbohydrates or without biotinylated lectins.Results: In the normal temporal bones, five carbohydrate residues in goblet cells and cilia of the eustachian tube demonstrated moderate to strong activity--NeuNAc alpha(2-6)GalNAc, NeuNAc alpha(2-3)GalNAc, GalNAc, Gal beta(1-3)GalNAc, and L-fucose. Two residues demonstrated weak activity--GlcNAc/NeuNAc and alpha-mannose. Temporal bones with MOM revealed increases in sialic acid and alpha-mannose, and a decrease in L-fucose. Residues of carbohydrates in the cilia of bones with SOM were notably decreased, especially for GalNAc, Gal beta(1-3)GalNAc, and NeuNAc alpha(2-6)GalNAc.Conclusions: Glycoconjugates in the normal human eustachian tube are rich in GalNAc, Gal beta(1-3)GalNAc, L-fucose, and NeuNAc alpha(2-3/2-6) GalNAc, but low in alpha-mannose and sialic acid. Eustachian tubes from cases with SOM or MOM demonstrated alterations in glycoconjugate expression in cilia and goblet cells, which may reflect disorder of the carbohydrate metabolism during otitis media, especially in SOM. [ABSTRACT FROM AUTHOR]

The article offers advice on the selection and location of ventilation tubes for acute otitis media, chronic otitis media with effusion, chronic mastoiditis, chronic intractable eustachian tube ventilation dysfunction and post-tympanoplasty atelectasis. It suggests the use of tubes with a lumen of 1.1 millimeters (mm) or 1.5mm. It says that the tubes should be inserted into the posterior superior quadrant and posterior inferior to prevent damage to ear structures. Also recommended is the insertion of the tubes into the anterior superior quadrant.

Objective: To perform an otopathologic analysis of temporal bones (TBs) with CHARGE syndrome., Study Design: Otopathologic study of human TB specimens., Setting: Otopathology laboratories., Methods: From the otopathology laboratories at the University of Minnesota and Massachusetts Eye and Ear Infirmary, we selected TBs from donors with CHARGE syndrome. These TBs were serially sectioned at a thickness of 20 µm, and every 10th section was stained with hematoxylin and eosin. We performed otopathologic analyses of the external ear, middle ear (middle ear cleft, mucosal lining, ossicles, mastoid, and facial nerve), and inner ear (cochlea, vestibule, internal auditory canal, and cochlear and vestibular nerves). The gathered data were statistically analyzed., Results: Our study included 12 TBs from 6 donors. We found a high prevalence of abnormalities affecting the ears. The most frequent findings were stapes malformation (100%), aberrant course of the facial nerve (100%) with narrow facial recess (50%), sclerotic and hypodeveloped mastoids (50%), cochlear (100%) and vestibular (83.3%) hypoplasia with aplasia of the semicircular canals, hypoplasia and aplasia of the cochlear (66.6%) and vestibular (91.6%) nerves, and narrowing of the bony canal of the cochlear nerve (66.6%). The number of spiral ganglion and Scarpa's ganglion neurons were decreased in all specimens (versus normative data)., Conclusions: In our study, CHARGE syndrome was associated with multiple TB abnormalities that may severely affect audiovestibular function and rehabilitation.

Hypothesis/background: We hypothesize that following head trauma there is a difference in temporal bone (TB) pathology in cases with and without skull fracture. Although conductive, sensorineural, mixed hearing loss, and TB pathology following head trauma have been reported, to our knowledge, there are no studies that have compared the pathology of the TB in cases with and without skull fracture., Methods: We analyzed 34 TBs from donors who had a history of head trauma (20 with skull fracture and 14 without fracture), and 25 age-matched controls without clinical or histological evidence of otologic disorders. We documented the presence and location of TB fracture, ossicular injury, and cochlear hemorrhage and evaluated the loss of spiral ganglion cells and sensory hair cells, damage to the stria vascularis, and the presence of endolymphatic hydrops., Results: We found a significant loss of outer hair cells in the upper basal, lower, and upper middle turns of the cochlea (p = 0.009, =0.019, =0.040, respectively), a significant loss of spiral ganglion cells (p = 0.023), and cochlear hemorrhage predominantly in the basal turns secondary to head trauma. Interestingly, these findings were significantly observed in TBs from donors with a history of head trauma without skull fracture., Conclusion: The greatest damage was to the cochlear basal turn. Our findings suggest that head trauma may result in tonotopic high frequency sensorineural hearing loss. TBs from donors with skull fracture have less pathologic changes than those without., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

Hypothesis: In temporal bones with otitis media, fibrin and neutrophil extracellular traps (NETs) form a fibrous network with bacteria, which is involved in growth of bacterial clusters/biofilms and chronicity of disease., Background: NETs and fibrin are important in host defense against pathogens; however, their role in otitis media is not well understood., Methods: Eight human temporal bones with serous otitis media, 30 with serous-purulent otitis media, 7 with mucoid otitis media, 23 with mucoid-purulent otitis media (OM), 30 with purulent OM, and 30 with chronic otitis media were selected based on histopathologic findings. Fibrous material with bacteria was detected with hematoxylin-eosin, Gram-Weigert, and propidium iodide stains; and its composition was analyzed with immunohistochemistry., Results: Extensive formations of fibrous material with bacteria were observed in 30% of temporal bones with serous-purulent otitis media, 29% with mucoid otitis media, 50% with mucoid-purulent OM, 57% with purulent OM, and 67% of temporal bones with histological evidence of chronic otitis media. Some of these formations showed large bacterial clusters or biofilms. Immunohistochemical analysis showed that fibrous structures were composed of fibrin or NETs., Conclusions: Formations of fibrous material with bacteria were detected in human temporal bones with different types of otitis media. Inflammatory cells were observed mostly in areas with low presence of fibrous structures. The network of fibrous material seems to prevent clearance of bacteria by phagocytic cells and thus influences growth of bacterial clusters or biofilms. Fibrin and NETs may be important for the recurrences and chronicity of disease, and contribute to clogging of tympanostomy tubes in children., Competing Interests: The authors disclose no conflict of interests., (Copyright © 2021, Otology & Neurotology, Inc.)

Objective: The purpose of this study was to investigate the morphological changes of round window membrane (RWM) in chinchillas with Streptococcus pneumoniae (S. pneumoniae) serotype 7F induced acute otitis media (AOM) by two dimensional (2D) and three dimensional (3D) measurements., Methods: Temporal bone specimens taken from 12 chinchillas were divided into two groups. The control group consisted of healthy animals that were injected with intrabullar saline. The subjects in the experimental group were induced with AOM by intrabullar injection of S. pneumoniae 7F. The 2D and 3D measurements of RWM were compared between the groups., Results: Dramatic changes were noted in the RWM of the experimental group compared to the control group. The thickness [mean ± standard deviation (SD)] of the RWM was significantly (p<0.05) increased in the experimental group compared to the control group by 2D measurements taken at three different points of RWM. Moreover, 3D measurements revealed that the volume (mean ± SD) of RWM was significantly (p=0.009) increased in the experimental group., Conclusion: The results of our study, which indicated significant change in RWM in both 2D and 3D measurements, may shed light on the relationship between AOM and inner ear diseases. Based on our results, we recommend evaluating 3D analyses of RWM, which provide useful data, to better understand the changes in the membrane., Competing Interests: Conflict of Interest: The authors declared that there is no conflict of interest., (©Copyright 2021 by Official Journal of the Turkish Society of Otorhinolaryngology and Head and Neck Surgery.)

Purpose: In this study, we aimed to determine whether or not COM leads to loss of spiral and Scarpa ganglion neurons., Methods: From the human temporal bone (HTB) collection at the University of Minnesota we selected human temporal bones with COM, defined as the presence of clinically intractable tissue abnormalities in the middle ear (cholesteatoma, perforation of the eardrum, granulation tissue, fibrosis, tympanosclerosis, and cholesterol granuloma). We also selected HTBs from donors with no ear diseases as controls. We quantitatively analyzed the number of spiral and Scarpa ganglion cells and compared the results obtained in the control and study groups., Results: In both COM and control groups we observed a significant negative correlation between age and number of both spiral (R = -0.632; P < 0.001; 95% CI - 0.766 to - 0.434) and Scarpa ganglion (R = - 0.404; P = 0.008; 95% CI - 0.636 to - 0.051) cells. We did not find any significant differences in the number of spiral ganglion cells (in total or per segment) or in the density of Scarpa ganglion cells (in each vestibular nerve or both) in the COM group as compared with controls (P > 0.05)., Conclusions and Relevance: Our results did not demonstrate significant loss of cochlear or vestibular peripheral ganglion neuron loss in HTBs with COM as compared with controls.

: Hypothesis: There may be findings peculiar to the temporal bones of children with Down's syndrome (DS). The purpose of this study is to investigate the temporal bone histopathology of the children with DS., Background: Otitis media with effusion is a highly prevalent condition with DS. Knowledge of the volume of the tympanic compartments and the area of the tympanic isthmus might be important to find out the pathogenesis of highly prevalent otitis media with effusion in those patients., Methods: We compared the volume of the epitympanum, mesotympanum, and the areas of the tympanic isthmus and tympanic orifice of eustachian tube in temporal bones from patients with DS. We also investigated the eustachian tube histopathologically., Results: The mean volume of the epitympanum and the mesotympanum was significantly smaller in the DS group than the control group. We found no significant difference in the mean diameter of the protympanic opening and tympanic orifice between the two groups. The mean narrowest area of the aerated and bony tympanic isthmus also was not significantly different between the two groups. An immature development of eustachian tube and cartilage was seen. We found mesenchyme remaining at the epitympanum and/or mesotympanum in all specimens in the DS group, and in five specimens in the control group., Conclusion: In the presence of the small middle ear, poorly developed eustachian tube, and tensor muscle, a vicious circle occurs, making otitis media with effusion difficult to resolve.

Objective: To demonstrate the cochlear turns area changes among patients with a history of meningitis, through otopatologic study., Methods: We performed an analysis of the area of the bony cochlear turns and the cochlear lumen of the horizontal sections containing the modiolus and the area of the basal turn at the level of round window, in temporal bones obtained from patients with a history of meningitis and compared to a nondiseased control group., Results: The mean area of the bony walls and the lumen of all cochlear turns are reduced within the meningitis group. Patients who presented a time from the diagnosis of meningitis to death longer than 30 days had a significant reduction in the cochlear turns area, as compared to the control group., Conclusion: Future studies may further correlate audiologic outcomes, cochlear volume, and cochlear area among patients with meningitis., Competing Interests: The authors declare no potential conflict of interest., (© 2020 The Authors. Laryngoscope Investigative Otolaryngology published by Wiley Periodicals, Inc. on behalf of The Triological Society.)

Background: Evidence to support potential links between chronic otitis media (COM) and vestibular impairment/postural balance control issues is lacking., Objective: To investigate whether COM associates with vestibular symptoms, balance problems, and abnormalities in vestibular function tests., Methods: We selected 126 patients with COM and excluded patients with any identifiable underlying causes for vestibular dysfunction. Fifty-two healthy volunteers were included as controls. All subjects underwent anamnesis, physical examination, posturography, and video-head impulse tests., Results: We found a high prevalence of vestibular symptoms (58.4%) among patients with COM, while only 2% of the controls had vestibular symptoms. There was a positive correlation between COM activity with the presence of tinnitus and vestibular symptoms (P < 0.05). Clinical vestibular tests were abnormal in 63% of patients with COM, and those positively associated with presence of vestibular symptoms. Posturography results shown worse postural balance control in patients with COM as compared with controls, especially in the limit of stability (LOS) (Mean LOS, COM = 157.56 cm2; controls = 228.98 cm2; p < 0.001) and worse results in the test with eyes closed while standing on a foam mattress (sway area, COM = 10.91 cm2; controls = 5.90 cm2; p < 0.001) in patients with COM as compared with controls. We did not observe differences in the average vestibuloocular reflex gains in the video-head impulse test between our COM and control groups., Conclusions: Our results show that COM associates with higher prevalence of vestibular symptoms and abnormalities in clinical vestibular function tests, and worse postural control as compared with controls. Among patients with COM, the activity of the middle-ear inflammation seemed to positively associate with the severity of hearing and balance problems.

Hypothesis: The presence of bony inner ear malformations may associate with a number of anatomical abnormalities affecting the middle ear structures. Those malformations may create pitfalls and complications for cochlear implantation., Background: Inner ear malformations associate with varying degrees of hearing loss, and frequently require cochlear implantation for hearing rehabilitation. Therefore, the abnormalities affecting the middle- and inner-ear structures may increase the risk of surgical complications., Methods: We examined 38 human temporal bones from donors with bony inner ear malformations. Using light microscopy, we analyzed the presence of abnormalities in the structures of the middle- and inner-ear., Results: Our collection comprises of 38 specimens with inner-ear malformations (cochlear aplasia, n = 3; cochlear hypoplasia, n = 30; incomplete partition, n = 3; isolated vestibular malformation, n = 2). The anatomy of the middle ear was abnormal in most temporal bones with cochlear aplasia, cochlear hypoplasia, and incomplete partition type I (40%-100%). Some of those abnormalities (hypoplastic or obliterated mastoid, 55.2%; aplastic or obliterated round window, 71.0%; aberrant course of the facial nerve, 36.8%) may hinder the access to the round window using the conventional facial recess approach for cochlear implantation. The cochlear nerve and associated bony structures (internal auditory canal and bony canal for cochlear nerve) were normal in 71.0% of all temporal bones with inner ear malformations., Conclusion: Each different type of malformation may create specific surgical challenges to surgeons. Comprehensive preoperative imaging is fundamental toward the surgical success of cochlear implants in patients with malformations. Alternatives to circumvent those middle- and inner-ear abnormalities and potential complications are further discussed.

The objective of this study was to evaluate any otopathologic changes in temporal bone specimens from dogs with deafness related to cochleosaccular (Scheibe) dysplasia (CSD). We used the canine temporal bone collections of the Otopathology Laboratory at the University of Minnesota and of the Massachusetts Eye and Ear Infirmary at Harvard University in Boston. Our morphometric analysis included measuring the areas of the stria vascularis and the spiral ligament and counting the number of spiral ganglion cells. In addition, we noted the presence of the organ of Corti and cochlear hair cells, assessed the location of Reissner's membrane and the saccular membrane, and counted the number of both Type I and Type II vestibular hair cells in the macule of the saccule and vestibular ganglion cells. In the group of specimens from dogs with cochleosaccular dysplasia, we observed generalized degeneration in the cochlea and a significantly decreased number of Type I and Type II vestibular hair cells and vestibular ganglion cells. As hereditary deafness is presently untreatable with known therapeutic methods, dogs with cochleosaccular dysplasia should not be considered for breeding. Future therapeutic approaches, such as stem cell therapies, should be designed to target all the elements of the cochlea in addition to the saccule as it was found that both are affected in dogs with CSD.

Objective: To investigate the temporal bone histopathology of vasculitis, especially in the vestibular organs, in granulomatosis with polyangitis (GPA)., Methods: Using light and differential interference contrast microscopy, we examined 12 human temporal bones from six deceased GPA patients and 12 histopathologically normal human temporal bones from six deceased age-matched patients., Results: In the GPA group, three patients had undergone tympanostomy tube placement. Two of them had suffered mixed hearing loss; one, sensorineural hearing loss; and one, conductive hearing loss. Of the 12 specimens in the GPA group, the granulation tissue invaded the round window niche in seven; cochlear hair cells were not preserved in five. Hemosiderin was deposited in the stria vascularis in eight specimens, in the ampulla or semicircular duct in 10, and in the vestibule in three. The spiral ligament showed severe loss of cellularity in two specimens. In the GPA group, type I vestibular hair cell density was significantly decreased; however, type II vestibular hair cell density did not significantly differ between the GPA group and the control group., Conclusion: Our histopathologic findings in human temporal bone specimens of GPA patients delineated changes in the tympanic membrane, middle ear cavity, round window membrane, organ of Corti, stria vascularis, spiral ligament, ampulla, semicircular duct, and vestibule. Type I vestibular hair cell density significantly decreased in the GPA group, as compared with the control group., Level of Evidence: N/A.