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Introduction: Dizziness is a common disease. However, approximately 10–40% of patients were diagnosed unknown dizziness even though general, neurological, and otological examinations were performed. The aim of this otopathological study was to investigate the histopathology of the peripheral vestibular system of patients who suffered from undiagnosed dizziness. Methods: Eighteen temporal bone specimens from 9 patients with undiagnosed dizziness and 20 temporal bone specimens from age-matched 10 normal controls were selected. Cases with a history of dizziness and vertigo caused by particular peripheral vestibular disease and central etiology were excluded. Specimens of the vestibular system were carefully assessed by light microscopy. The basophilic deposits adhered to cupulae of the semicircular canals and the wall of the labyrinth were investigated. Scarpa’s ganglion cell counts in the vestibular nerves were performed. Results: Fifteen ears of 9 patients had the findings of vestibular pathology such as a basophilic deposit on cupula (8 ears), on canal wall (7 ears), vestibular nerve loss (8 ears), or vestibular atelectasis (2 ears). Unclear pathological findings such as crista neglecta, subepithelial deposits of the crista ampullaris, and adhesion of the cupula to dark cell area were demonstrated. The mean size of basophilic deposits seen in the patients (mean: 191 µm) was larger than that of latent deposits seen in the normal controls (mean: 101 µm; p = 0.01). Conclusions: We demonstrated some peripheral vestibular pathological findings such as deposit within the semicircular canal, vestibular nerve loss, and vestibular atelectasis and suggested the possible diagnosis of dizziness (benign paroxysmal positional vertigo, presbyvestibulopathy, vestibular atelectasis). These findings will provide a better insight into the multiple etiologies of the unknown dizziness in the elderly.

Charcot-Marie-Tooth (CMT) syndrome is a clinically and genetically heterogeneous group of neuropathies affecting both peripheral motor and sensory nerves. Progressive sensorineural hearing loss, vestibular abnormalities, and dysfunction of other cranial nerves have been described. This is the second case report of otopathology in a patient with CMT syndrome. Molecular genetic testing of DNA obtained at autopsy revealed a missense variant in the MPZ gene (p.Thr65Ala), pathogenic for an autosomal-dominant form of CMT1B. The temporal bones were also prepared for light microscopy by hematoxylin and eosin and Gömöri trichome stains, and immunostaining for anti-myelin protein zero. Pathology was consistent with a myelinopathy of the auditory, vestibular, and facial nerves bilaterally. The pathophysiology of cranial nerve dysfunction in CMT is unknown. Findings in the current case suggested, at least in cranial nerves 7 and 8, that a myelinopathy may be causative.

Bone remodeling within the otic capsule has been reported to be inhibited especially at or near the cochlea, except under some pathological conditions such as otosclerosis, Paget's disease, or mastoiditis, when bone remodeling can occur. Microcavitations found in periosteal and endosteal layers of human temporal bone specimens without otosclerosis, Paget's disease, or inflammation as reported in the current study are consistent with osteoclastic bone resorption. Thirty-three temporal bones from 33 patients were prepared for light microscopy and classified into 4 groups: histologically proven dehiscence of the superior semicircular canal (SSCD) (n = 3, group 1), age 20 years or younger (n = 10, group 2), age 90 years or older and with otosclerosis (n = 10, group 3), and age 90 years or older without otosclerosis (n = 10, group 4). Microcavitation was seen at 7 anatomic locations in the temporal bone in all 4 groups, but not in the cochlea or vestibule. Microcavitation within the temporal bone is likely due to osteoclastic activity, and it is seen in both young and old patients, patients with and without otosclerosis, and in cases with SSCD.

The histopathology of the inner ear in a patient with hearing loss caused by the p.L114P COCH mutation and its correlation with the clinical phenotype are presented. To date, 23 COCH mutations causative of DFNA9 autosomal dominant sensorineural hearing loss and vestibular disorder have been reported, and the histopathology of the human inner ear has been described in 4 of these. The p.L114P COCH mutation was first described in a Korean family. We have identified the same mutation in a family of non-Asian ancestry in the USA, and the temporal bone histopathology and clinical findings are presented herein. The histopathology found in the inner ear was similar to that shown in the 4 other COCH mutations and included degeneration of the spiral ligament with deposition of an eosinophilic acellular material, which was also found in the distal osseous spiral lamina, at the base of the spiral limbus, and in mesenchymal tissue at the base of the vestibular neuroepithelium. This is the first description of human otopathology of the COCH p.L114P mutation. In addition, it is the only case with otopathology characterization in an individual with any COCH mutation and residual hearing, thus allowing assessment of primary histopathological events in DFNA9, before progression to more profound hearing loss. A quantitative cytologic analysis of atrophy in this specimen and immunostaining using anti-neurofilament and anti-myelin protein zero antibodies confirmed that the principal histopathologic correlate of hearing loss was degeneration of the dendritic fibers of spiral ganglion cells in the osseous spiral lamina. The implications for cochlear implantation in this disorder are discussed.

Alström's syndrome is an autosomal recessive syndromic genetic disorder caused by mutations in the ALMS1 gene. Sensorineural hearing loss occurs in greater than 85% of patients. Histopathology of the inner ear abnormalities in the human has not previously been fully described. Histopathology of the inner ear in Alström's syndrome is presented in 2 genetically confirmed cases. The predominant histopathologic correlates of the sensorineural loss were degeneration of the organ of Corti, both inner and outer hair cells, degeneration of spiral ganglion cells, and atrophy of the stria vascularis and spiral ligament.

In our laboratory, human temporal bone specimens from patients who in life have undergone cochlear implantation are routinely processed with the implant in situ, embedded in Araldite, sectioned at 20 µm and serially photographed during cutting, stained with toluidine blue and mounted on glass slides. From the images, two-dimensional and three-dimensional reconstructions can be made and a very accurate implant insertion depth can be calculated from the three-dimensional reconstructions. However, this method precludes subsequent special stains and further molecular investigations of the tissue including proteomics and immunostaining, which is now possible with celloidin-embedded tissue. In this study, we correlated measurement of the implant array insertion depth calculated from histologic three-dimensional reconstruction with that measured from three-dimensional radiologic multiplanar reconstruction. Four human temporal bones with cochlear implants underwent postfixation preprocessing CT imaging with a Siemens Somatom Sensation Scanner. The CT scans from these four bones were downloaded into the Voxar software application, reformatted using the multiplanar reconstruction tool, viewed in three dimensions and measurements of intracochlear insertion lengths of the implants were obtained. The bones were processed routinely for in situ Araldite embedding, serial images were made of the block during sectioning, postprocessed using PV-Wave® software, aligned with Amira® software, and used to create histologic three-dimensional reconstructions. From these three-dimensional reconstructions, the insertion depth of the electrode array was mathematically calculated. The range of insertion depths was 15.9 mm (case 1) to 26.6 mm (case 4). The two methods, radiographic multiplanar reconstruction and three-dimensional reconstruction, differed by 0.4-0.9%. This provides confidence that important localization information about the electrode in situ can be gleaned from CT scans, thereby allowing us to extract the implants prior to processing for celloidin embedment and allow further techniques such as special stains and immunostaining to be accomplished in order to evaluate molecular mechanisms involved in cochlear implantation.

The current study evaluates histopathologic changes in the temporal bones of 4 human subjects who underwent revision cochlear implantation. Specimens were removed at autopsy, fixed and prepared for histological study by standard techniques. Specimens were serially sectioned, reconstructed by two-dimensional methods, and the tracks of the initial and revision cochlear-implant electrodes identified. The tracks were of three types: a ‘common track’ (shared by the reimplantation electrode and initial electrode), ‘two tracks’ (where the reimplantation electrode was in a different track than that of the initial electrode) and ‘one track’ (where the reimplantation electrode extended beyond the initial electrode, forming a single track). Associated histopathologic findings (new bone formation, fibrosis or inflammatory cells, and cochlear fluid) were evaluated for the three types of tracks. In all 4 subjects, the insertion depth of the revision cochlear implant was deeper than that of the initial cochlear implant. The primary track of the initial implantation did not interfere with insertion of a revision cochlear implant, and the trajectory of the revision electrode did not always follow the primary track. In cochlear segments with a common track or two tracks, the mean (across-subject) percent area of the extraelectrode cochlear duct filled with abnormal (new bone or fibrotic) tissue (43.2%) was significantly greater than the mean percent area occupied by fluid (13.4%; t = 3.12, d.f. = 19.9, p = 0.003).

Objectives: Atraumatic and complete insertion of the electrode array is a stated objective of cochlear implant surgery. However, it is known that obstructions within the cochlea such as new bone formation, cochlear otosclerosis, temporal bone fracture, and cochlear anomalies may limit the depth of insertion of the electrode array. In addition, even among patients without obvious clinical or radiographic indicators of obstruction, incomplete insertion may occur. The current study is a histopathologic evaluation of possible sources of resistance to insertion of the electrode array using the temporal bone collection of the Massachusetts Eye and Ear Infirmary. Methods: Forty temporal bones from patients who in life had undergone cochlear implantation were evaluated. Temporal bones were removed at autopsy and fixed and prepared for histologic study by standard techniques. Specimens were then serially sectioned and reconstructed by 2-dimensional methods. Two electrode metrics were determined for each bone: the inserted length (IL: the distance measured from the cochleostomy site to the apical tip of the electrode) and the active electrode length (AEL: the distance between the most basal and most apical electrodes on the electrode array). The ratio of these two metrics (IL/AEL) was used to split the temporal bones into two groups: those with incomplete insertion (n = 27, IL/AEL Results: Obvious obstruction by abnormal intracochlear bone or soft tissue accounted for only 6 (22%) of the 27 partial insertions. Of the remaining 21 bones with incomplete insertions and 13 bones with complete insertions, dissection of the spiral ligament to the lateral cochlear wall was the only histopathologic indicator of insertion resistance identified with significantly higher frequency in the partial-insertion bones than in the complete-insertion bones (p = 0.003). An observed trend for the percentage of complete insertions to decrease with the number of times the electrode penetrated the basilar membrane did not reach significance. In the bones without an obvious obstruction, the most frequently observed indicator of insertion resistance was dissection of the spiral ligament (with no contact of the lateral cochlear wall) identified in 67% (14/21) of partial-insertion bones and in 92% (12/13) of complete-insertion bones. Conclusion: These results are consistent with the view that (1) electrode contact with cochlear structures resulting in observable trauma to the basilar membrane, osseous spiral lamina and/or spiral ligament does not necessarily impact the likelihood of complete insertion of the electrode array and (2) once contact trauma to the spiral ligament reaches the point of dissection to the cochlear wall, the likelihood of incomplete insertion increases dramatically.

The formation of new bone and fibrous tissue in the human inner ear following cochlear implantation was evaluated by computer-assisted 3-D reconstruction. Seven temporal bones from patients who in life had undergone cochlear implantation were prepared for histological study with the implant in situ. The specimens were sectioned in the axial plane at a thickness of 20 mu m. At least every tenth section was digitally reconstructed in three dimensions and volumes of new bone and fibrous tissue were calculated per millimeter length of the cochlea. New bone and fibrous tissue were found in all seven specimens, particularly at the cochleostomy site. In addition, new bone and fibrous tissue had extended to variable lengths along the track of the cochlear implant and in some cases extended beyond the distal end of the implanted electrode. This methodology provides a quantitative tool for evaluation of new bone and fibrous tissue in the inner ear following implantation. This should assist in correlating psychophysical and speech perception tests with intracochlear pathology, evaluating both electrode design and the techniques of preserving residual auditory function. Copyright (c) 2007 S. Karger AG, Basel.

The innervation densities, i.e. the number of afferent endings per inner and outer hair cell, number of synaptic specializations per inner hair cell and the number of efferent endings per outer hair c