A case of nasopharyngeal amyloidosis

A case of amyloidosis localized in the nasopharynx was reported. The patient was a 62-year-old female who complained of persistent nasal obstruction. The gross appearance of this tumor showed a thumb-sized red-yellowish mass, in part covered with white coating. The systemic amyloidosis was excluded by several clinical examinations including rectal biopsy. Histopathological examination revealed diffuse deposits of homogeneous eosinophilic substances. A polarized microscopic examination by congo-red stain showed that deposit's substances were amyloid. A typical amyloid fibril was observed under electron microscope. The mass was removed with Beckmann adenotome. Bleeding was minimal.     

Detection of viral antigen in the endolymphatic sac

A study was devised to determine whether or not any immune defense mechanism is present when a virus invades the human endolymphatic sac (ES). The ES was removed from 14 fresh autopsy cases having no known premortem diseases in the middle and inner ears. Specimens were then examined for viral antigens including herpes simplex (HSV) type 1 and 2, mumps and cytomegalovirus using immunohistochemical methods. DNA examination by in situ hybridization was also performed for HSV HSV antigen and DNA were observed in 9 of the 14 cases studied. These findings suggest that the virus invades the ES but is impeded by an immune defense mechanism under normal conditions. Since disease may alter host defenses, further studies are warranted to study the relationship between HSV and patients with Meniere's disease.     

Clinical efficacy of 5-fluorouracil (5-FU) topical cream for treatment of cholesteatoma

OBJECTIVE: In order to clarify clinical efficacy of commercially available 5-fluorouracil (5-FU) topical cream, a clinical study was conducted. METHODS: Two to three cubic millimetres of 5-FU topical cream (Kyowa, Roche) was applied on 50 cases of various types of cholesteatoma (50 patients) two to five times with the interval of 2 weeks, and its clinical efficacy was evaluated by the criteria we developed. RESULTS: In total, 59% of the cholesteatomas showed good effect, 29% of them showed fair effect, and the effect was poor in the remaining 12%. It was particularly effective in cholesteatomas in the EAC, attic cholesteatomas with an aerated mastoid, and in recurrent-type cholesteatomas. CONCLUSION: 5-FU topical cream appeared effective for the treatment of cholesteatomas.     

Cochlear implantations in visually impaired patients

We retrospectively review the cases to evaluate the outcome of cochlear implantation (CI) in patients with severe-to-profound hearing loss and visual impairment (VI). Six adults with severe or profound hearing loss and significant VI underwent multichannel CI. Follow-up period ranged from 17 months to 7 years. Case history, etiology of visual and hearing loss, and benefit from CI were evaluated. To measure the outcomes, we selected the pure-tone thresholds with CI, the speech discrimination scores (SDS) using the Japanese video SDS system, the speech perception rates using the Japanese CD SDS system by monosyllable and word, and the open-set and closed sentence score using live voice. All the patients live happily after CI. There was no significant difference between the present six patients and the patients with profound hearing loss without VI in evaluations of hearing and quality of life. CI can play a significant rehabilitative role in patients with severe hearing loss and VI.     

Experimental positional nystagmus induced by argon laser irradiation to the oval window

Experimental positional nystagmus (EPN) similar to benign paroxysmal positional nystagmus (BPPN) was induced in 8 of 20 rabbits by argon laser irradiation to the footplate. The power of the argon beam used was 2.0 watts and irradiation time was 1 or 2 sec. EPN and BPPN showed the following similarities maintained (1) When the head was maintained in the affected ear down position and the head positioned from the sitting to the supine position nystagmus directed to the affected ear or vertical nystagmus corresponding to rotatory nystagmus in man were provoked. (2) Delayed onset for provocation of nystagmus. (3) Limited duration. (4) Fatigability. (5) The direction of nystagmus was reversed when the head returned to the initial position (in two rabbits). Histological examination of the temporal bones showed separation of otoconia from the otolithic membrane of the saccule and bleeding in the saccule in both conditions. No lesions in the utricle and semicircular canals were evident. It was assumed that ablation of otoconia of the saccule causes this experimental positional nystagmus.     

A case of palmoplantar lichen planus in a patient with congenital sensorineural deafness

We report a case of palmoplantar lichen planus in a 7‐year‐old Japanese girl with congenital deafness, who presented with erythematous eruptions and hyperkeratosis, with peeling and fissures on her soles, palms and digits. On histological examination of a skin biopsy from the lesion on her wrist, lichen planus was identified. Using computed tomography of the inner ears, bilateral cochlear dysplasia was found. The patient’s DNA was sequenced; no sequence variants were detected in the GJB2 gene encoding connexin‐26, but she had a missense mutation in SLC26A4 (solute carrier family 26, member 4). Mutations in SLC26A4 are known causes of hearing loss, but this is a novel mutation, which has not been reported previously. In addition, there have been no reports of cutaneous symptoms in previously reported patients with mutations in SLC26A4. To our knowledge, therefore, this is the first report of palmoplantar lichen planus associated with sensorineural deafness accompanied by a mutation in the SLC26A4 gene.     

Endolymphatic hydrops induced by noise exposure.

In animal with cochlear deafness induced by firecracker explosion, fibrous degeneration of the endolymphatic sac was observed 4 months after the disappearance of Preyer's reflex. Endolymphatic hydrops induced by noise exposure may develop as the results of degeneration of the endolymphatic sac due to cochlear deafness by acoustic trauma after the lapse of a long period of time.