Norimasa Morita

Membranous labyrinth volumes in normal ears and Meniere disease: a three-dimensional reconstruction study.

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.

Sudden hearing loss as the presenting symptom of systemic sclerosis.

Objectives: Sudden sensorineural hearing loss (SSHNL) is an emergency in otolaryngology. In most cases, the exact cause cannot be identified, but different immunologic disorders and microvascular events have been suggested to play a role in its pathogenesis. Sudden sensorineural hearing loss can be caused by collagen vascular disorders, but it has rarely been reported as the presenting symptom of these diseases. This case presented with bilateral hearing loss and was finally diagnosed as a systemic sclerosis (SSc) patient. This is the first case of SSc that presented with SSNHL as an initial symptom. Patient: A 65-year-old man presented with bilateral hearing loss. He also complained of generalized fatigue and intermittent paresthesia. Brain imaging and blood tests were negative except for antinuclear antibodies. Administration of high-dose prednisone did not make any improvement. After extensive workup, follow-up, and referral to rheumatologist, the diagnosis of SSc was made. The patients hearing improved after receiving intravenous immunoglobulin along with other symptoms of the disease. Conclusion: This case illustrates the importance of follow-up and appropriate of SSNHL patients with other systemic symptoms.

Vestibular system changes in sudden deafness with and without vertigo: a human temporal bone study.

Objective To investigate the vestibular system changes in sudden deafness with vertigo (SDwV) and sudden deafness without vertigo (SDwoV) and the cause of persistent canal paresis (CP) in SDwV patients. Study Design Retrospective study. Materials and Methods Four temporal bones from the affected ear in 4 patients with unilateral sudden deafness (SD), 2 SDwV and 2 SDwoV, were selected. Four contralateral temporal bones with normal-hearing ears were defined as the control. Morphologic findings of the labyrinth, the number of Scarpa’s ganglion cells, and the density of vestibular hair cells were investigated in all temporal bones. Clinical data and the results of vestibular tests of 11 patients with unilateral SD, as a separate group, also were investigated. Results Atrophic change of the organ of Corti, tectorial membrane, and stria vascularis in cochlea, and deposits and atrophic otoconial membrane in vestibular sense organs were seen on affected ears more than control ears. The density of Type I hair cells seemed to decrease on the saccular macula and the posterior semicircular canal crista on affected ears, and there was no remarkable difference between SDwV and SDwoV. In 1 patient with SDwoV who died 10 months after the onset of SD, there were large amount of deposits on the cupula, the atrophied otoconial membrane was peeling off from the saccular macula, and the saccular membrane collapsed to the saccular macula in the affected ear. In the clinical data, all SDwV who were examined within 2 years from the onset had CP, and all SDwV had profound hearing loss. Conclusion There is no remarkable difference between SDwV and SDwoV in the number of Scarpa’s ganglion cells and the density of vestibular hair cells. The damage of the extracellular superstructure is seen in SD with or without vertigo. The damage of extracellular superstructure is potentially one of the causes of persistent CP in patients with SD.

Cochlear changes in presbycusis with tinnitus.

OBJECTIVES The pathophysiology of tinnitus is obscure and its treatment is therefore elusive. Significant progress in this field can only be achieved by determining the mechanisms of tinnitus generation, and thus, histopathologic findings of the cochlea in presbycusis with tinnitus become crucial. We revealed the histopathologic findings of the cochlea in subjects with presbycusis and tinnitus. MATERIAL AND METHODS The subjects were divided into 2 groups, presbycusis with tinnitus (tinnitus) group and presbycusis without tinnitus (control) group, with each group comprising 8 temporal bones from 8 subjects. We quantitatively analyzed the number of spiral ganglion cells, loss of cochlear inner and outer hair cells, and areas of the stria vascularis and spiral ligament. RESULTS There was a significantly greater loss of outer hair cells in the tinnitus group compared with the control group in the basal and upper middle turns. The stria vascularis was more atrophic in the tinnitus group compared with the control group in the basal turn. CONCLUSIONS Tinnitus is more common in patients with presbycusis who have more severe degeneration of outer hair cells and stria vascularis.

Potential cause of positional vertigo in Ménière's disease

Objectives: To compare the incidence of deposits in the semicircular canals between the temporal bones with Ménières disease and normal controls and to investigate the relationship between the incidence of deposits and the symptoms of positional vertigo, often seen in patients with Ménières disease. Study Design: Retrospective histopathologic human temporal bone study. Methods: Twenty-two temporal bones from 11 patients with bilateral Ménières disease, 28 from 14 with unilateral and 50 age-matched normal temporal bones from 30 individuals were histopathologically examined. Medical records were reviewed for clinical history of positional vertigo and duration of disease. Results: Significant differences were found in the incidence of cupular and free-floating deposits in the posterior semicircular canals between temporal bones with and without Ménières disease. The incidence of free-floating deposits in the lateral semicircular canals was significantly higher in cases with unilateral Ménières disease compared with controls. The incidence of these deposits was associated with the duration of disease rather than with aging. All 5 patients with positional vertigo (3 of 11 patients of bilateral Ménières disease and 2 of 14 of unilateral) had free-floating deposits in at least 1 semicircular canal. Conclusion: Our findings suggest a possible causative relationship between cupular and free-floating deposits in the semicircular canals and the symptom of positional vertigo in patients with Ménières disease.

Pathologic correlations of otologic symptoms in acute lymphocytic leukemia

OBJECTIVES To assess the clinicopathologic correlations of otologic complaints in patients with acute lymphocytic leukemia. DESIGN Otologic complaints and histologic findings were evaluated in 25 temporal bones of 13 acute lymphocytic leukemia patients. RESULTS Nine patients had a history of otologic complaints, including hearing loss, otalgia, otorrhea, and vertigo in 5, 3, 3, and 2 patients, respectively. Hemorrhage was most commonly observed in the middle ear (6 patients, 9 temporal bones) and was also observed in cochlea (4 patients, 4 temporal bones), and vestibule (6 patients, 6 temporal bones). Leukemic infiltration was observed in the petrous apex (13 patients, 24 temporal bones), middle ear (7 patients, 14 temporal bones), cochlea (3 patients, 4 temporal bones), vestibule (3 patients, 4 temporal bones), and internal auditory canal (5 patients, 8 temporal bones). Inflammatory changes were observed in the cochlea (5 patients, 8 temporal bones) and vestibule (5 patients, 8 temporal bones). Middle ear effusion containing floating tumor cells was observed in 4 temporal bones of 3 patients. Irreversible histopathologic changes of the middle ear, such as the destruction of the ossicles, perforation of the tympanic membrane, and granulation tissues were observed in 5 temporal bones of 4 patients. CONCLUSIONS Ear involvement is common in acute lymphocytic leukemia patients. With prolonged survival due to the progress of treatment, the diagnosis and treatment of nonhematopoietic system symptoms, such as ear problems due to acute lymphocytic leukemia, have become more important.

Polyarteritis nodosa: a human temporal bone study

PURPOSE Polyarteritis nodosa is one of the common forms of vasculitis with multiorgan involvement. Hearing loss may be the presenting symptom of this disease. The aim of this histopathologic study was to evaluate temporal bone changes in polyarteritis nodosa and assess the correlation between otologic manifestations and histopathologic findings. MATERIALS AND METHODS A retrospective human temporal bone analysis was performed at an otopathology laboratory in a tertiary academic medical center. Three cases (5 temporal bones) with pathologically confirmed diagnosis of polyarteritis nodosa were selected for study. The processed temporal bone sections were studied under light microscopy. RESULTS Two of the 3 cases presented with hearing loss, and one of them had facial palsy. Audiometric data available for one patient demonstrated a bilateral, rapidly progressive, mixed hearing loss. Histopathologically, we found generalized vasculitis involving small- and medium-sized arteries in all cases. Of the 5 temporal bones available for study, 3 had chronic otitis media and 2 had serous otitis media. Arteritis was seen in all middle ears. There was disruption of the organ of Corti and spiral ganglion cells in 4 temporal bones. One temporal bone showed fibrosis and osteogenesis in the scala tympani of the basal turn. Vasculitis of stylomastoid and branches of labyrinthine artery were also found in temporal bones. CONCLUSIONS Significant temporal bone findings were seen in patients with polyarteritis nodosa, including otitis media, cochlear damage, neo-osteogenesis, and middle and inner ear vasculitis. This temporal bone series also suggests an interesting relationship between sensorineural hearing loss and labyrinthine vasculitis.

Peripheral Vestibular System in Down Syndrome Quantitative Assessment of Vestibular Histopathology

Objective. To evaluate the maturity of the peripheral vestibular system in Down syndrome by examining the number of Scarpa’s ganglion cells and the density of vestibular hair cells. Study Design. Case-control study using human temporal bones. Setting. Tertiary academic center, otopathology laboratory. Subjects and Methods. Sixteen temporal bones from 8 patients with Down syndrome and 15 control temporal bones from 8 individuals with no history of otologic disease were selected. Hypoplasia of the lateral semicircular canal (LSC) and vestibule was investigated by measuring the dimensions of the structures. Scarpa’s ganglion cells were counted under light microscopy. The vestibular hair cells were counted in the LSC crista and the utricular and saccular maculae under differential interference contrast (Nomarski) microscopy and expressed as density. Results. The patients with Down syndrome were divided into 2 groups: with and without LSC hypoplasia. The number of Scarpa’s ganglion cells and the density of vestibular hair cells were significantly smaller in both groups of patients with Down syndrome than in the control group. There was no significant difference in the number of Scarpa’s ganglion cells or the density of vestibular hair cells between the groups with and without LSC hypoplasia. Conclusion. The peripheral vestibular system, including Scarpa’s ganglion cells and vestibular hair cells, is hypoplastic irrespective of the vestibular malformation in Down syndrome.

Marrow-middle Ear Connections: A Potential Cause of Otogenic Meningitis

Hypothesis: We hypothesize that the connections between the hematopoietic bone marrow and middle ear is a potential cause of childhood otogenic meningitis. Background: Although it is known that there is a causal relationship between otitis media and bacterial meningitis, the relationship has never been satisfactorily established. Human fetal and infant temporal bones prepared for light microscopic evaluation revealed direct connections between the hematopoietic bone marrow and middle ear. We noted this difference in anatomy between the infant middle ear and the adult middle ear. Methods: We studied 10 temporal bones from 5 infants in each group: meningitis group with otitis media who died of meningitis, control Group 1 without otitis media, and control Group 2 with otitis media who died of diseases other than meningitis. A quantitative analysis of the frequency of connections between the hematopoietic bone marrow and middle ear was performed. The correlation between unabsorbed mesenchyme and otitis media also was investigated. Results: The frequency of connections was significantly higher in order of the meningitis group, control Group 2, and control Group 1. The degree of unabsorbed mesenchyme tended to be more severe in order of the meningitis group, control Group 2, and control Group 1. Conclusion: The prevalence of connections between the hematopoietic bone marrow and middle ear in patients with meningitis and otitis media is high. A higher prevalence of connections in infants with otitis media could increase the risk for otogenic meningitis in them.

Evaluation of Fluoride-18-Labeled Boronophenylalanine-Positron EmissionTomography Imaging for the Assessment of Boron Neutron Capture Therapy inPatients with Recurrent Head and Neck Squamous Cell Carcinoma

Introduction: A role for fluoride-18-labeled boronophenylalanine positron emission tomography (18FBPA-PET) in boron neutron capture therapy (BNCT) has not been fully elucidated. We investigated the role of 18FBPA-PET in BNCT for recurrent head and neck squamous cell carcinoma (HNSCC) patients. Materials and methods: 18FBPA-PET images were obtained from 10 histologically verified recurrent HNSCC patients who received BNCT. The intratumoral accumulation of 18FBPA was calculated as the ratio of maximum and minimum radioactivity counts to that of normal tissue (Tmax/N and Tmin/N ratios, respectively). The percentage volume at which the radioactivity count ratio was >2.5 compared to normal tissue was also calculated (Vo2.5). Moreover, mean and minimum irradiation dose to the tumor was calculated. We investigated which parameters could predict the treatment effect of BNCT. Results: Treatment effects of local lesions were as follows: complete remission (CR) in 5 cases and non-CR in 5 cases. Only Tmin/N ratio showed a significant difference between the CR and non-CR groups (P=0.008). Conclusion: The Tmin/N ratio of 18FBPA-PET can predict the treatment effect of BNCT for recurrent HNSCC.