In 159 cases of cholesteatoma otitis media including 101 PF (pars flaccida) type and 58 PT (pars tensa) type, hearing results were evaluated in 137 cases. Other 18 cases underwent only one stage operation and 4 cases of radical operation were excluded from this study. Type I tympanolasty was performed in34ears of PF type (33.7%) and in 5 of PT type (8.6%). Type IV or modified type IV tympanoplasty was performed in 20 ears of PF type (19.8%) and in 17 of PT type (29.3%). As procedures for PF type, canal wall-down operation was performed in 74 ears (77.1%), canal wall-up in 8 (8.3%), canal wall reconstruction in 7 (7.3%) and mastoid obliteration in7 (7.3%). For PT type, canal wall-down operation was performed in 27 ears (65.9%), canal wall-up in 9 (22.0%), canal wall reconstruction in 2 (4.9%) and mastoid obliteration in 3 (7.3%). One stage tympanoplasty was performed in65ears of PF type (67.7%) and in 32 of PT type (78.0%), two staged tympanoplasty in 10 ears of PF type (10.4%) and in8of PT type (19.5%). Modified radical mastoidectomy was performed in 21 ears of PF type (21.9%). The successful rate of hearing was 81.3% in PF type and 75.6% in PT type. In both PF and PT types, canal wall-up operation showed better postoperative hearing than canal wall-down and two staged tympanoplasty better than one stage tympanoplasty. Recurrent cholesteatoma occurred in 3 ears of PF type and in 2 of PT type. Residual cholesteatoma was found in 2 ears of canal wall-up operation. Postoperative mastoid problem was found in 5 ears (4.2%) of canal wall-down operation.
The results of 32 ears that underwent tympanoplasty for cholesteatoma in children under 9 years old were compared with those of 22 ears of adolescent (10 to 15 years) and 63 ears of adult (over 16 years). The planned staged tympanoplasty was performed in 68.8% of children, 45.5% of adolescents, but only 19.0% of adults. The canal wall up procedure was employed in 71.9% of children and in 87.1% of patients over 10 years. At the second operation of planned staged tympanoplasty or the planned second look operation, a pearl-like residual cholesteatoma was found in 16% of children, and in 21.1% of patients over 10 years. But these planned revised operations were underwent in 78.1% of children, whereas only in 22.4% of patients over 10 years. The need for revised operation is greater in children than in adolescents and adults. Residual cholesteatoma was found in 15.2% treated by the canal wall up procedure and in 27.3% by the canal wall down procedure in all revised ears. Higher incidence of residual cholesteatoma in the canal wall down procedure than in the canal wall up procedure was thought to be caused that the former was performed in extensive and aggresive cholesteatoma. The incidence of recurrent cholesteatoma after the canal wall up procedure was the same in children (4.3%) and patients over 10 years (5.4%). Recurrence is closely related to dysfunction of the eustachian tube or tympano-mastoid aeration. We found that in ears with poor eustachian tube function, complete tympano-mastoid blockage by cortical bone fragments was the most effective preventative technique against recurrent cholesteatoma, though reconstruction of scutum and repair of tympano-mastoid aeration were useful for ears with good eustachian tube function.
The clinical pathology of middle ear cholesteatoma were classified from the surgical findings. And the surgical results were studied in each classified groups in order to evaluate the surgical techniques used in our clinic. The cases analyzed were 73 ears operated on from 1985 and 1989. They consisted of 30 ears of pars flaccida type (attic type), 23 ears of pars tensa type (adhesive type) and 20 ears of the recurrent cases. The conclusions obtained were as follows: 1. The extent of cholesteatoma was graded into 4 stages in the flaccida type and tensa type, which were seemed proper and useful to analyze the results of surgery. 2. For treatment of the pars flaccida type, the anterior tympanotomy is absolutely necessary although the obliterative technic is not necessary. There were no cases of recurrence in this group. 3. For treatment of the pars tensa type, transcanal atticotomy, lateral tympanotomy in combination with anterior tympanotomy were absolutely necessary, and in the extensive cases antrotomy, mastoidotomy and further obliterative technic were necessary. 4. Two cases of recurrence were found in the pars tensa type. They included each one of grade II and grade III cases, using canal-up technique combined with anterior tympanotomy and posterior tympanotomy.
Postoperative condition of 101 cases of cholesteatoma were analyzed statistically. Cholesteatomas were categorized into PF (Pars flaccida) and PSQ (postero-superior quadrant) types. Seven cases were complicated by facial palsy and 3 cases by labyrinthine fistula. Canal wall-up operation was done in 37 ears of PF (48.7%) and15ears of PSQ type (57.7%). Only 16 ears underwent canal down operation. Tympanoplasty with columella III type was most frequently performed (37.5%) for ossicular chain reconstruction. Overall success rate in hearing was 63.2%. Regarding postoperative condition of the ear drum, 47.5% of the cases showed abnormal findings, such as retraction pocket, adhesion and otorrhea. Recurrent cholesteatoma was found in 17 ears in PF and 2 ears in PSQ types. Especially, the cases with cholesteatoma extending from the aditus through the mastoid cavity showed a high recurrence rate. In general, the cases without otorrhea yielded better postoperative hearing than the wet cases. The incidence of recurrence was especially high in the wet cases of PF type.
From January 1985 to September 1990, at the Medical College of Oita, we performed 100 tympanoplasties for patients with chronic otitis media with cholesteatoma. Sixty cases in this series were subjected to the reconstruction of the external auditory canal with the pedicled periosteo-osteal flap made on the mastoid after canal down tympanoplasty. In 12 cases, canal up tympanoplasy was performed, transcanal atticotomy was in 16 cases, and canal down tympanoplasty was in 12 cases. All cases were followed up for at least 12 months after surgery. Recurrent cholesteatoma was found in two cases of this series. Both of the recurrent cases were children with attic type of cholesteatoma. One of them was a 13-year-old boy from the reconstruction group, and the other was a 10-year-old boy from the canal up group. The recurrence of cholesteatoma was not significantly different among those 4 procedures. Mean duration of the re-epithelization of the postoperative canal wounds in the reconstruction group (30 days) was similar to those values of the canal up group (22 days) and the atticotomy group (33 days) and significantly shorter than that of the canal down group (74 days). After reconstruction of the canal wall with the pedicled periosteo-osteal flap, 48 canals (80%) showed normal appearance. Eight canals (13%) were slightly expanded by a backwards shift of the bone graft, and 2 canals (3%) were slightly narrowed. However, they required no postoperative treatments, such as local cleaning or crust removal. Retraction pocket with recurrent cholesteatoma was found in one case (2%). Only one case required the surgical removal of the bone graft because of infection and necrosis. Hearing improvement after surgery was achieved in 27 cases (45%) of the reconstruction group, 5 cases (42%) of the canal up group, 8 cases (50%) of the atticotomy group and 2 cases (17%) of the canal down group. Although the stapedial superstructure was frequently damaged by cholesteatoma in the reconstruction group, the postoperative hearing improvement of this group was a satisfactory result in comparison with that of the canal up group. These findings suggest that the reconstruction of the canal wall defect after canal down tympanoplasty solves so-called ‘cavity problems’ attributed to the canal down procedure and improves postoperative hearing by rebuilding the physiological structure of the external auditory canal and middle ear cleft. For the reconstraction of the canal wall defect, the pedicled periosteo-osteal flap is a useful material because it is a viable allograft receiving blood supply via the periosteal pedicle and well fuses with surrounding tissues. Since it is easy to make the bony graft on the mastoid cortex and does not require extensive surgery, this technique is effective for the reconstruction of the canal wall defect after canal down procedure.
A 6-year-old boy complained of hearing impairment, one month after the head injury caused by a traffic accident. Audiogram revealed conductive deafness. However, tympanometry and stapedial reflex test showed normal response. By exploratory tympanotomy, disruption of the ossicular chain was found. Restoration of the ossicular chain was perfomed with satisfactory results.
The aeration in several parts of the middle ear space and the presence of direct communication between the meso-and epitympanum were examined in cholesteatoma extending from the pars flaccida using high resolution computed tomography. In order to investigate the effect of the extension of cholesteatoma, pars flaccida cholesteatoma was divided into two subgroups according to the presence or absence of extension into the antrum (extended type and non-extended type). Chronic otitis media with central perforation and cholesteatoma extending from the pars tensa were also examined as a control. The degree of aeration in the middle ear space increased in the order of pars tensa cholesteatoma, pars flaccida cholesteatoma, and chronic otitis media with central perforation. In pars flaccida cholesteatoma, there was greater aeration of the middle ear space in the non-extended type than in the extended type. The tympanic isthmus was aerated in47.8% of the non-extended type and 18.5% of the extended type. Direct communication between the meso-and epitympanum was found in 30.4% of the non-extended type and 3.7% of the extended type. In two cases, pars flaccida cholesteatoma had developed from a retraction pocket at the pars flaccida, with aeration in all middle ear spaces and with direct communication between the mesoand epitympanum. Various theories have been presented about causative and progressive factors of cholesteatoma. The loss of direct communication between the meso-and epitympanum caused by obstruction of the tympanic isthmus is considered to be an important factor in etiology and progress of pars flaccida cholesteatoma. However, this study showed that the meso-and epitympanum had direct communication in some cases of pars flaccida cholesteatoma, even in the extended type. This result suggests that the obstruction of the tympanic isthmus, intercepting the route between the meso-and epitympanum, is not a necessary condition for the etiology and progress of pars flaccida cholesteatoma.
Juvenile functional deafness is classified as psychogenic deafness and examination deafness which is defined as a hearing impairment only shown on audiogram in school without complaint of deafness. Thirty eight cases of functional deafness who visited in recent 10 years were studied. Females are more frequent rather than males and the male/female ratio is about 1: 2. The number of bilateral cases are 32 and the unilateral cases are 6. Pure tone audiograms showed a flat-shaped sensorineural hearing loss in 57 ears (84.1%). Békésy audiogram showed type V of Jerger's classification in 43 ears (74.1%). ABR and evoked oto-acoustic emission (e-OAE) recordings were useful for diagnosis of functional deafness. And especially in unilateral cases, these examinations were valuable tools in differential diagnosis of functional deafness from sudden deafness.
A 56-year-old woman first complained of hoarseness, dysphagia and weight loss due to paralyses of the pharyngeal and the vagal nerves without symptoms of otological disease nor fever. Then she developed headache and paralyses of the accessory, hypoglossal and the facial nerves. Jugular fossa tumor had been suspected on CTscan and MRI. An exploratory mastoidectomy did not reveal any otogenic intracranial complications, but showed slight mucosal fibrosis and microabscess compatible with chronic mastoiditis. Craniotomy revealed an occlusion of jugular bulb with a white thrombus and sigmoid sinus with a red thrombus. Removal of thrombi and administration of antibiotics, steroid hormone improved paralyses of the VII, IX, XI and XII th cranial nerves.
A 64- year-old man was complaining of hoarseness, dysphagia, swallowing pain and right otalgia. Herpetic eruption on his right auricle was found. On examination, the following findings were also found, redness and sensory disturbance of the right pharyngeal posterior wall, paralysis of the right soft palate and right vocal cord palsy. After hospitalization vertigo, hearing loss on the right side and facial palsy on the right side appeared. The serum IgM antibody titer to herpes zoster virus was elevated. The diagnosis of Hunt's syndrome affecting the 5th, 7th, 8th, 9th and 10th cranial nerves was made. On examination of hearing and vestibular function, a cochlear hearing loss on the right side was found, but the vestibular nerve was not impaired. The facial nerve was impaired at the tympanic portion. From these findings, we speculated that inflammation in the ganglion of the glossopharyngeal nerve spread to other cranial nerves.
Forty patients of an acute low-tone sensorineural hearing loss (ALHL) were studied based on their clinical diary in which the patients described six subjective symptoms such as tinnitus, ear fullness, autophonia, headache and dizziness. Twenty-one patients complained of fluctuation of subjective symptoms in during a day. In seven cases, decrease of the fluctuation of subjective symptoms were observed as ALHL has been improved. The fluctuation of hearing or the relapse of hearing loss was indicated by audiograms in twenty-two patients. Audiograms in some cases did not correlate to their subjective symptoms. As it is difficult to perform frequent audiograms in the patients of ALHL, the clinical diary as well as the audiogram are very useful to study the clinical course of ALHL.
Two cases of osteoma of the external auditory canal are reported and the pathophysiologic mechanisms reported in the literature for osteomas of the head and neck region are reviewed. The first patient was a 22-year-old man with otorrhea who showed a hard mass of 6 mm in diameter in the left external auditory canal. The second patient was a 7-year-old boy with no complaints who had a hard mass of 3 mm in diameter in the left external auditory canal. Both masses were attached to the tympanomastoid suture line and were easily removed surgically. Clinically and histopathologically, both masses were diagnosed as osteoma. Review of the literature revealed no clear explanation of the pathophysiologic mechanisms of osteomas of the external auditory canal, temporal bone, or paranasal sinus. However, the literature does show that in all cases the site of osteoma formation was along the suture lines and that the overlying skin and subcutaneous tissue or the overlying mucosa were thinner than that overlying other sites.
We encountered 3 cases of congenital cholesteatoma of the petrous apex. There were 2 female and 1 male. The average age at the time that the diagnosis was made was 24.3 year-old. These patients all showed a left-sided lesion. The first symptom in 2 of 3 patients (66.6%) was unilateral progressive hearing loss. The periods between the presence of the first symptom and the diagnosis were 7 years, 11 years and 2 months respectively. The symptoms presented at the time of the original diagnosis included mixed type hearing loss, facial palsy, headache, facial paresthesia, vertigo and decreased caloric response. CT scanning is very useful in the diagnosis as well as in defining the exact location and the extent of the lesion. Total removal was attempted in two cases and marsupialization was done in one case. We suggest to keep the possibility of a petrous apex cholesteatoma in mind whenever encountering an unexplained unilateral hearing loss with deficits of facial nerve or trigeminal nerve in young patients.
Two cases, a female and her mother, of van der Hoeve syndrome are reported. They showed a conductive or mixed hearing loss. A stapedotomy was performed on each case. The findings at surgery were similar in both cases. The stapedial footplates in both cases were fixed firmly, and were normal in shape. The histopathological findings of stapes indicated similar type to that of otosclerosis in one patient, and those were almost normal in the other patient. After the stapedotomy, ecxellent hearing level was obtained in both patients.
Planned staged tympanoplasty was designed in 49 ears for 5 years in our department. The two-staged operation was performed in 85% of the 49 cases, but the planned 2nd staged operation was not performed in 15% cases. The indication of planned staged tympanoplasty should be carefully decided because two admissions to hospital for one patient seemed to be diffcult. The posterior wall in all cases was removed at the 1st stage operation. Reconstraction of the posterior wall or oblitalation of the mastoid cavities were chosen at the 2nd stage operation. In 50% of the cases of chronic suppurative otitis media with cholesteatoma and in 10% of chronic simple suppurative otitis media the planned staged tympanoplasty was performed. Cholesteatoma was found at the 2nd stage operation in 30% of cases. Hearing level after the 2nd staged operation was same as that at 1st staged operation.
The clinical findings of 12 patients (6males and 6 females) with inner ear damage due to acute otitis media were studied. The age of patients ranged between 16 to 58 years old (average 39.8 years old). All patients complained of hearing loss after otalgia and three of them experienced vertigo. Six patients showed hyperemia of both the tympanic membrane and external auditory meatus under otoscopic examination, but no bullous myringitis was observed. All patients revealed a mixed hearing loss on pure tone audiometry. Positional nystagmus was seen in 11 patients and seven showed decreased caloric function of the damaged ear. They were treated by systemic administration of antibiotics, steroids and vitamins, and the majority of these cases showed complete recovery of hearing and disappearance of vertigo.
Serum zinc level was measured in 88 patients with a sensorineural hearing loss and/or tinnitus and in 42 control group, and correlations between serum zinc level and the degree of sensorineural hearing loss or tinnitus were studied. The average and standard deviation of serum zinc level in 79 patients with a sensorineural hearing loss was 0.831±0.184μg/ml and was significantly lower than that (0.951±0.156μg/ml) of the controls (p<0.01). But we were unable to find any statistical correlations between the degree of sensorineural hearing loss or types of audiograms and the serum zinc level. The average and standard deviation of serum zinc level in 68 patients with tinnitus was 0.829±0.173μg/ml and was significantly lower than that of the control group (p<0.01). But it was impossible to find any statistical correlations between intensity of tinnitus and the serum zinc level. We suggested that there were some correlations between serum zinc level and sensorineural hearing loss or tinnitus.
From 1987 we have performed multichannel cochlear implantation in 20 patients with a profound sensorineural hearing impairment. Three patients were prelingually deaf. The postoperative results of two of these three patients are reported. Both of them showed reduced tolerance for sound. One of them easily became familiar with the environmental sounds such as horn of cars, birds'singing, etc. The vowel and consonant confusion abilities of this patient was fairy good three months after operation. On the other hand, the other patient needed more period to become familiar with the environmental sounds. Two years after operation the speech comprehension ability of this patient is still poor. From this result, it is considered that the prelingually deaf patients needed more period for the rehabilitation than the postlingually deaf patients, and they seem easy to hear the environmental sounds, and that there were considerable variations in performance among the prelingually deaf patients.
Two cases of cholesterol granuloma with the intact tympanic cavity were reported. Neither case showed chronic tympanic membrane pathology, such as perforation, cholesteatoma and blue ear drum. Combination of CT and MRI was of value for the preoperative diagnosis of cholesterol granuloma localized in the mastoid area. Each case received simple mastoidectomy, which revealed a membranous blockade within the attic in Case 1 and at the aditus ad antrum in Case2. Clinical significances of localized cholesterol granuloma were discussed as one of the forms of silent otitis media.
We have recently found labyrintine ossification throughout the posterior semicircular in a case of acoustic neurinoma. We discussed the pathogenesis and histopathology of this lesion. Although, we regarded the chronic partial obstruction of blood circulation by the tumor in the internal auditory meatus as the cause of the labyrintine ossification, there still remain the unsolved problems on this subject. Moreover, we mentioned the possibility of the preoperative diagnosis for this lesion by means of X-ray, CT or MRI.
We present the temporal bone histopathological findings in a case of 6q partial monosomy. Clinically, malformation of the external ear was noted bilaterally in equal extent. Histopathological examination revealed hypoplasia of the ossicles, the cochlea, the lateral semicircular canal and the endolymphatic sac, as well as dilatation of the utricle, and there was no difference of laterality. In addition, three-dimensional reconstruction method of the bony labyrinth of the temporal bone was obtained, making it possible to clearly understand the three-dimensional anatomy of the bony labyrinth, including the hypoplasia of the lateral semicircular canals and the decrease of the cochlear turns. Three-dimensional reconstruction of the temporal bone was found to be very useful for understanding the complicated bony labyrinth in anomaly syndrome.
We report a high jugular bulb with Mondini dysplasia visualized by high resolution CT and Magnetic Resonance Angiography (MRA) in a 12-year-old girl. High resolution CT showed the empty cochlea and high jugular fossa. MRA performed for the purpose of confirming diagnosis of a high jugular bulb and sigmoid sinus enlargement in the left side, and hypoplasia of the sigmoid sinus in the right. Audiogram of the left ear showed a profound deafness, and ABR showed no response. Vestibular function was examined by the caloric test, eye tracking test, opto-kinetic pattern, stepping test, and stabilometry. The results were as follows: 1) We speculate that a high jugular bulb with Mondini dysplasia may be due to interruption of development at early fetal life, and that high jugular bulb is caused by hypoplasia of the contralateral sinuso-jugular system. 2) Vestibular dysfunction was compensated for by contralateral vestibular function, and the extrapyramidal and pyramidal tracts.