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Differential Diagnosis of Cochlear and Retrocochlear Disorders
Yukio Ohkouchi
1989 Volume 1989 Issue Supplement32 Pages
1-14
Published: March 25, 1989
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The relationship between hearing level and several indexes, for example wave V latency, I-V interpeak latency (IPL) and interaural latency difference for wave V (IT5) of auditory brainstem response (ABR) was examined, and best index for the differential diagnosis of lesions was determined. Moreover, whether differential diagnosis of cochlear and retrocochlear hearing loss was possible was examined using the only one chain of loud stimulus. ABR in 134 cochlear hearing losses,35 cerebellopontine angle (CPA) tumors,17 other brain tumors,20 cerebro-vascular disorders, and 10 systemic degenerative disorders were evaluated, and the criteria of differential diagnosis of lesions investigated.
I-V IPL was the best index because of its stability at any hearing level. Wave V latency was a good index considering hearing level except for the case of profound deafness over 70 dB. IT
5 is good too if interaural hearing level difference is within 40 dB.
Loud stimulation is good to diagnose lesions for high apearance of ABR and less dispersion of latency. It was no trouble to diagnose with only one chain of loud stimulus.
Increase of 1-V IPL, no responses or prolongation of wave V within 70 dB deafness, increase of IT5 within 40 dB interaural hearing level difference and partial disapperance of ABR were seen with retrocochlear lesions.
As the ABR abnormality is frequently seen in patients with infratentorial lesions, especially in mass lesion as CPA tumor, ABR is very useful to these cases, and ABR abnormality is not rare in patients with supratentorial lesions.
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An Experimental Study
Mchio Kobari
1989 Volume 1989 Issue Supplement32 Pages
15-38
Published: March 25, 1989
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The ultrastructural change on the outer hair cells and its long-term alteration by kanamycin (KM) administration was examined in rabbits. Spoendlin's method of the block surface preparation technique to rabbits was useful to observe the cochlea from beginning of the basal turn to the apical end and also to identify the same cell in the ultrastructural specimen as observed in the surface view.
Animals were injected intramuscularly with daily doses of 200 mg/Kg of KM for 10 consecutive days, and decapitated immediately after the last KM administration. The result showed the increase of secondary lysosomes at the subcuticular zone of the outer hair cells in all of the experimental animals and these findings were considered the primary ultrastructural change of KM injury.
The outer hair cells were observed at 7,40 and 100 days after 200 mg/Kg of KM administration for 10 consecutive days, and at 7,30,40,70 and 100 days after 400 mg/Kg of KM administration for 5 consecutive days to evaluate the long-term alteration of the primary ultrastructural change. The ultrastructural study revealed that the secondary lysosomes replaced to the primary lysosomes in the cells mildly damaged in the light microscopy. These cells were supposed to be able to survive and to live on. In the cells which had severe damage by light microscopic observation, almost all of the lysosomes had residual bodies with a myelin like structure. The latter cells were probably close to the critical level that led to cell death.
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Hitomi Kobari
1989 Volume 1989 Issue Supplement32 Pages
34-48
Published: March 25, 1989
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Potentiation of the cochlear damage was studied after systemic administration of kanamycin (KM) in the case of experimentally induced otitis media.
Left eustachian tube was obstructed in guinea pigs to induce otitis media. Right ear was left intact as control. There were three types of histopathological changes after the eustachian tube obstruction, i. e., purulent otitis media, serous otitis media and a state that was considered to be healed condition.
The animals were given 400 mg/kg of KM at 2 days and 21 days following left eustachian tube obstruction for 10 consecutive days. Histopathological changes of outer hair cells at 10 days after the last KM administration were evaluated by the graphic cochlear reconstruction method using a light microscope. Concurrently, the histopathology was studied at 2,3,4 and 21 days following left eustachian tube obstruction as a control group. In the 21-day group of control animals, outer hair cell damage was observed at only the beginning portion of basal turn of cochlea of the left (experimental ear). In the KM administered group, the experimental result revealed significantly severer outer hair cell damage in all turn of cochlea in the left (experimental ear) than in the right (control ear).
The concentration of KM in perilymph was measured by bioassay at 4 hours and 24 hours after single administration of 400 mg/kg of KM and at 24 hours after the last administration of 400 mg/kg/day of KM for 10 consecutive days. There was no significant difference in the KM concentration in perilymph between experimental ear and control ear.
These results suggest that the increased cochlear damage after KM administration during otitis media was not due to the increase of KM level in the inner ear, and also suggest that two different kinds of ototoxic agents which are aminoglycoside antibiotics and otitis media can cause severer cochlear damage than the sum of each damage.
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Iwao Ohtani, Tomohiro Anzai, Tohru Aikawa, Hiro-oki Okamura
1989 Volume 1989 Issue Supplement32 Pages
49-55
Published: March 25, 1989
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The ototoxicity, nephrotoxicity and hematologic toxicity of carboplatin (CBDCA) were compared with those of cisplatin (CDDP) using the rat and guinea pig.
The oto-and nephrotoxicity of CBDCA was very low as compared with CDDP. Forced hydration and diuresis will not be necessary for CBDCA administration clinically.
The hematologic toxicity was remarkably high in comparison to CDDP. The dose limiting toxicity of CBDCA will be primarily bone marrow suppression.
The excretion of CBDCA from the bone marrow as well as serum, perilymphatic space, kidney was extremely fast. In conclusion the difference of severity of various toxicity induced by CBDCA is not only due to the concentration in the tissues.
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Tohru Aikawa, Iwao Ohtani, Masafumi Murakami, Tomohiro Anzai, Hitomi K ...
1989 Volume 1989 Issue Supplement32 Pages
56-60
Published: March 25, 1989
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Masafumi Murakami, Hitomi Kobari, Hidetaka Kanno, Tohru Aikawa, Tomohi ...
1989 Volume 1989 Issue Supplement32 Pages
61-66
Published: March 25, 1989
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We observed sensorineural hearing loss following X-ray irradiation to the malignant tumor of head and neck. There were 24 patients whose auditory organs lied within the irradiation field. Ten of these patients were affected by sensorineural hearing loss. Hearing loss occurred at a high frequency in elderly patients, epipharynx tumor and high dose of irradiation. Many cases revealed high tone hearing loss. Most cases showed about a 20∼30 dB hearing loss, so their impediment seemed not severe in daily life. In some of these cases, we could have temporal bone findings, but there were no particular findings relevant to sensorineural hearing loss.
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Yohko Sato, Iwao Ohtani, Tohru Aikawa, Masahumi Murakami, Tomohiro Anz ...
1989 Volume 1989 Issue Supplement32 Pages
67-71
Published: March 25, 1989
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The histopathological findings in the temporal bone of a 57-year-old man who died of drowning are described.
Diffuse hemor r hage and congestion were evident throughout the external auditory canal, tympanic membrane, middle ear cavity, mastoid cavities, scala vestibuli of cochlea, facial nerve, and greater petrosal nerve. There were no abnormal findings in vestibular organs.
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Hiro-oki Okamura, Tohru Aikawa, Yukio Ohkouti, Tomohiro Anzai, Iwao Oh ...
1989 Volume 1989 Issue Supplement32 Pages
72-78
Published: March 25, 1989
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The histopathological findings in the temporal bone of a 37-year-old male who had had a left facial palsy caused by lymphoma cell infiltration in the facial nerve, are reported.
Lymphoma cells infiltrated the internal auditory canal, facial nerve and bone marrow in both sides, and in the tympanic cavity of the left side. However in the external auditory canal, tympanic membrane, mastoid air cells, chorda tympani, cochlea, vestibule, and semicircular canal, lymphoma cells were not seen.
The left facial nerve was swollen and degenerated by marked infiltration of lymphoma cells. Lymphoma cells also infiltrated in the right facial nerve, but the facial nerve was. not swollen, and there was no facial palsy.
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Tomohiro Anzai, Iwao Ohtani, Tohru Aikawa, Yohko Sato, Masafumi Muraka ...
1989 Volume 1989 Issue Supplement32 Pages
79-85
Published: March 25, 1989
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A histopathological study of the temporal bones in three cases of occult schwannomas of the vestibular nerve is reported. Four small occult schwannomas of the vestibular nerve were discovered on routine examination of 315 serially sectioned temporal bones of 165 individuals.
In the first patient who was a 67-year-old woman, the tumor arose from the left inferior division of vestibular nerve without damaging the vestibular end organs.
In the second patient who was a 77-year-old man, the tumor arose from the right superior division of vestibular nerve without the damage of utricular macula.
In the third patient who was a 37-year-old man, the right ear had two tumors which had a multicentric origin from inferior division of vestibular nerve and cochlear nerve with severe degeneration of cochlear nerve fibers.
It is presently impossible to diagnose this entity during life, because of very small tumors and no damage of vestibular organs.
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Chiaki Suzuki, Iwao Ohtani, Tohru Aikawa, Yohko Satoh, Hiroshi Ogawa
1989 Volume 1989 Issue Supplement32 Pages
86-90
Published: March 25, 1989
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man in whom audiometric study, about a month before death, had shown deafness in the left ear. About 10 years previously, he noticed hearing loss and tinnitus in the left ear. The left hearing became progressively worse, but no vestibular symptoms were present while he was alive. A tumor occupied the lumen of the cochlea and had penetrated the modiolus, and protruded partly into the internal auditory canal. The membranous labyrinth had degenerated. The tumor seemed to have originated in the Rosenthal's canal of basal turn, and seemed to have expanded into the modiolus and the internal auditory canal.
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Hitoshi Sakuma, Tetsuya Akaike, Tohru Aikawa, Iwao Ohtani
1989 Volume 1989 Issue Supplement32 Pages
91-96
Published: March 25, 1989
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The histopathological findings in the temporal bone of a 3-year-old male who died of fourth ventricle ependymoma are reported. The tumor was growing pressing the cerebellum and medulla oblongata, and invaded the bilateral internal auditory canals and had enlarged them. The cochlear and vestibular nerves in the bilateral internal auditory canals were degenerated, but spiral ganglion cells, stria vascularis, hair cells of the organ of Corti, utricle, saccule and semicircular canals were well preserved.
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Kazuo Takeuchi, Iwao Ohtani, Tohru Aikawa, Yohko Sato
1989 Volume 1989 Issue Supplement32 Pages
97-99
Published: March 25, 1989
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We report temporal bone findings of a case of multiple endocrine neoplasia (MEN)type 2B. The pathological findings in bilateral bones was as follows. Ear drum was thickened remarkably, and neuromas were observed in its lamina propria. The Facial nerve exhibited an obtuse angle of geniculum, and no horizontal portion. Cochlear aqueduct was markedly large and patent.
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Syu Yamanobe, Tohru Aikawa, Tomohiro Anzai, Masafumi Murakami, Iwao Oh ...
1989 Volume 1989 Issue Supplement32 Pages
100-105
Published: March 25, 1989
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The temporal bone findings in a case of Cornelia de Lange syndrome are reported.
The temporal bones showed shortened cochlea, maldevolopment of the p osterior semicircular canal, malformations of the auditory ossicles, mesenchymal tissue in the tympanic cavity, wide angle of the facial genu and large bony defect of the facial canal.
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Yukio Ohkouchi, Hidetaka Kanno, Hiroshi Ogawa, Iwao Ohtan
1989 Volume 1989 Issue Supplement32 Pages
106-111
Published: March 25, 1989
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Six hundred and twelve patients (754 ears), consisting of 253 males and 359 femeles complaining of fullness in the ear were investigated in our clinic during the past two years. Although most cases were between 30 and 60 years old, the high rate of complaining of aural fullness to ear disease was seen in the twenties to forties. Four fifth of them were unilateral, and no laterality was seen. The sensation of aural fullness was more seen in cerumen, secretory otitis media, acute otitis media, auditory tube obstruction, sudden deafness, perilymph fistula and Mèniére's disease. Deafness, tinnitus and hyperacusis accompanied aural fullness, but vertigo did so less often. Pure tone audiometry was performed in 671 ears, which were classified into four group; 150 normal ears,68 with conductive hearing loss,322 with sensorineural hearing loss and 131 mixed type. No relation was seen between the amount of aural fullness and hearing level.
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Isamu Sato, Iwao Ohtani, Hidetaka Kanno, Hitomi Kobari, Tetsuya Akaike ...
1989 Volume 1989 Issue Supplement32 Pages
112-119
Published: March 25, 1989
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Two cases of malignant external otitis are reported.
One patient was a 33-year-old male who wa s affected with diabetes mellitus and complained of headache and left ear discharge. Staphylococcus aureus was isolated from the discharge.
The other patient was a 51-year-old male who was affected with diabetes mellitus and complained of left ear discharge, earache and impaired hearing. Pseudomonas aeruginosa was isolated from the discharge.
Local and systemic antibiotic therapy, debridement of necrotized tissue and strict control of the diabetes are very important in the management of malignant external otitis.
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Tetsuya Akaike, Iwao Ohtani, Hiroshi Ogawa, Tohru Aikawa, Tomohiro Anz ...
1989 Volume 1989 Issue Supplement32 Pages
120-127
Published: March 25, 1989
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Forty-eight ears that underwent tympanoplasty for cholesteatoma in children under 15 years old (25 ears were under 9 years and 23 ears were over 10 years) were compared. with 74 ears in adults. The cholesteatoma extended to the mastoid air cells and the mesoor hypotympanum in children more than in adults. The rate of planned staged tympanoplasty was 64% in children under 9 years, and 56.5% in children over 10 years, but it was only 29.7% in adults. The closed method was used in more than 50% in both children and adults.
At the second operation of planned staged tympanoplasty, residual cholesteatoma was noted more frequently in children under 9 years (25%) than in chileren over 10 years (16.7%) and in adults (11.1%). In the one-stage tympanoplasty group followed for more than 1 year, residual cholesteatoma was found more frequently in children under 9 years (12.5%) and over 10 years (11.1%) than in adults (2.7%). In children over 10 years and in adults, all residual cholesteatoma cases were treated by the closed method, but in children under 9 years, residual cholesteatoma appeared more frequently in the open method group than in the closed method group.
Retraction cholesteatoma was found m o re frequently in children under 9 years (14.3%) than in children over 10 years (0%) and in adults (3.2%) in the closed me thod group followed for more than 1 year.
Postoperative hearing impro v ement was better in children than in adults.
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Tomoko Suzuki, Michio Kobari, Tetsuya Akaike, Iwao Ohtani, Kohsei Ohts ...
1989 Volume 1989 Issue Supplement32 Pages
128-132
Published: March 25, 1989
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Three cases of bony obliteration of oval window are reported. All cases were in children who had cholesteatomas.
Generally, oste o lytic change is observed in cholesteatoma. However, osteolytic and osteoplastic changes were observed at the same time in these cases. We supposed that new bone formation arose from inflammation in young growing children.
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Chiaki Suzuki, Iwao Ohtani, Michio Kobari, Tomoko Suzuki
1989 Volume 1989 Issue Supplement32 Pages
133-137
Published: March 25, 1989
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A 17-year-old patient had tinnitus and hearing impairment in the right ear since her elementary school days. She had no history of aural infections. Otoscopic examination revealed an intact right tympanic membrane with normal landmarks. Audiometric test revealed a 65 dB conductive hearing loss on the affected side. X-ray findings were normal. Through the probe tympanotomy matrix of cholesteatoma was identified. It was not a closed pearl but an ‘open’ cholesteatoma. It was surrounded by loose fibrous tissue presumed to be unresolved mesenchyme. The long process of the incus and the head and crura of the stapes were missing. They were presumed to be ossicular anomalies.
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Yohko Baba, Michio Kobari, Masafumi Murakami, Hitomi Kobari, Syu Yaman ...
1989 Volume 1989 Issue Supplement32 Pages
138-143
Published: March 25, 1989
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Two cases of congenital cholesteatoma whose origin could be identified are reported.
Case 1: A 4-year-old boy was incidentally suspected to have left cholesteatom a when he went to a hospital because of right otorrhea. Operation revealed the cholesteatoma was derived from tendon of tensor tympani muscle.
Case 2: A 3-year-old boy with congenita l hearing loss. He was suspected of having right cholesteatoma during an ABR examination. An operation revealed the cholesteatoma derived from maleus manibrium.
We discussed the origin of congenital cholesteatoma. Many congenital cholesteatomas are reported to have derived from the junction of the first and second branchial arch or tympanic isthmus. These two cases are in agreement with these reports.
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Yumi Sota, Tohru Aikawa, Hiro-oki Okamura, Iwao Ohtani, Masao Hattori
1989 Volume 1989 Issue Supplement32 Pages
144-150
Published: March 25, 1989
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Two cases of mastoid cyst are reported. One patient was a 44-year-old man who had had an operation on his left ear 25 years ago. Multiple cysts which occupied a wide area of the left temporal bone were removed surgically.
The other patient was a 7-year-old boy w h o had had chronic otitis media of the left ear. Mastoid cyst of the left ear was surgically removed.
Histologically both cases were cholesterin gran u lomas.
Most cases of mastoid cyst reported in th e Japanese literature were found to be related to an inflammatory disease in their past history. Therefore the chronic inflammation was thought to be an important factor of the formation of mastoid cyst.
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Yasuo Kakuta, Yukio Ohkouchi, Iwao Ohtani
1989 Volume 1989 Issue Supplement32 Pages
151-156
Published: March 25, 1989
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The patient was a 53-year-old female who was diagnosed to have the complete type of Hunt's syndrome with sensorineural hearing loss, herpes oticus and facial palsy. About 40 days later, hearing loss recovered almost completly. However, after discharge, impairment and restoration of her hearing recurred several times. It was reported that fluctuating sensorineural hearing loss might be caused by steroid-responsive sensorineural hearing loss, disorder of endolymph, inflammation of cochlear nerve, vertebral artery insufficiency, acoustic neurinoma, etc.. A disorder of endolymph was probably the cause of fluctuation of hearing in this case.
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Hiro-oki Okamura, Tohru Aikawa, Iwao Ohtani, Kohsei Ohtsuki
1989 Volume 1989 Issue Supplement32 Pages
157-163
Published: March 25, 1989
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We observed two patients with hearing loss caused by relapsing polychondritis during long term, and compared hearing levels with the general state.
The first case was in a 38-year-old male who was diagnosed with relapsing polychondritis when he was 35 years old when his hearing level was 77.5 dB in the right ear and 55 dB in the left ear. Then he had ocular inflammation, and his hearing level decreased to about 4 dB in the both ear. However after ocular inflammation, his hearing loss improved in the right ear.
The second case was in a 61-y e ar-old male who was diagnosed with relapsing polychondritis when he was 56 years old. When he was 59 years old, he had a respiratory disease, but his left hearing level was about 20 dB. However, during his remission, his left hearing level dropped about 5 dB. When he was 60 years old, he had a respiratory disease, but his hearing loss was unchanged. After this, his left hearing loss gradually took a turn for the worse.
We speculated t h at hearing loss of relapsing polychondritis was not affected by the patientis general state.
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Hidetaka Kanno, Tohru Aikawa, Yukio Ohkouchi, Manabu Honda, Tetsuya Ak ...
1989 Volume 1989 Issue Supplement32 Pages
164-168
Published: March 25, 1989
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We evaluated neurotological findings in a pedigree of 4 cases of Wagner's syndrome. Sensorineural hearing loss was present in 3 of the 4 cases. Two of the 3 cases exhibited a sensorineural hearing loss which was always at 2000 Hz.
Another exhibited a high-frequency sensorineural hearing loss which showed positive recruitment at 8000 Hz.
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A Case Report
Manabu Honda, Hidetaka Kanno, Yukio Ohkouchi, Yumi Sota, Tetsuya Akaik ...
1989 Volume 1989 Issue Supplement32 Pages
169-172
Published: March 25, 1989
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Neuro-otological findings of a Kabuki make-up syndrome in a 18-year-old woman are reported. She had had hearing impairment, equilibrium disturbance and spontaneous pathological eye movement.
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