Three cases of congenital cerebrospinal fluid (CSF) otorrhea from oval window with inner ear dysplasia (Mondini dysplasia) are reported. All three cases had congenital unilateral total deafness with recurrent meningitis. X-ray tomography and high resolution CT of the temporal bone revealed the large vestibule and cystic cochlea. At the operations, all three cases had fistulas on the oval windows and CSF drainages were done in all cases. In the second case a muscular mass was inserted in the cochlea from the enlarged oval window after stapedectomy. In the third case inner ear obliteration with muscle was done after stapedectomy and labyrinthectomy. The fistula was closed and the CSF leakages was controlled in all cases at first operation and they have no recurrence of meningitis.
Mondini dysplasia associated with recurrent meningitis was found in a 4-year-old boy who had 5 episodes of meningitis since he was 3 years old. His right ear was totally deaf and CT films showed hypoplastic cochlea and vestibule on that ear. Exploratory tympanotomy revealedcerebrospinal fluid (CSF) leakage through a bony defect of the stapedial footplate. The innerear was obliterated with small pieces of connective tissue and the oval window was sealed with auricular cartilaginous chips applied from within the vestibule. The patient has remained free from recurrent meningitis. Scrutiny of Japanese literature in recent 10 years showed that the CSF leakage occurred at the oval window area in all reported 14 cases. From this and our experiences in 2 cases, mastoidectomy is not essential for exploration and therapy. An attempt to reduce the CSF pressure is not necessary during surgery.
Incidence of bilateral traumatic facial palsies is rare. Of 116 cases of traumatic facial palsy encountered during the last 16 years in our clinic, bilateral involvements were seenin five patients (4.3 per cent). Causes of head injury in these patients were traffic accident, fall from stairs, struck on the head by a falling subject in one case, respectively, and nipping of the head in two. All of the patients were struck on their temporal portion of the head at the accidents. Mechanisms of the bilateral involvements were considered to be coup and contre coup injury in one case, low velocity crushing head injury on both sides in two and struck of the head bilaterally in two. Seven sides in four of them underwent decompression surgery; 4 by transmastoid approach and 3 by combination of transmastoid andmiddle cranial fossa approach. Recovery of the facial function in the operated patients were satisfactory, and no difference could be seen from that of unilateral palsy in terms ofthe duration between the onset and surgery. This indicated that there was no significant difference in the degree of facial nerve injury between bilateral and unilateral traumatic palsy. Although diagnosis of the bilateral facial palsy tends to be delayed due to lack ofasymmetrical facial appearance and to high incidence of unconsciousness, timing of operation should not be missed to assure good prognosis.
Subjective and objective signs and symptoms of middle ear barotrauma during hyperbaric oxyganation therapy (OHP) were investigated in 67 patients. The patients were classified on the basis of otoscopic examination into 5 groups. The incidence of middle ear barotraumaduring OHP assesed by objective criteria was 68.7%, and showed little variation with age or primary disease. On the other hand, the incidence assesed by subjective symptoms was lower than the objectively-based incidence in aged patients and in those with severe neurological diseases. Patients with a traumatic grade of 2 or more showed severe symptoms. The incidence of silent patients who did not mention any symptoms but otoscopy revealed severe signs was 14.9%. This group included patients with neurological diseases in poor condition. These results suggest that the middle ear barotrauma during OHP should be evaluated with otoscopic examination, especially in patients in poor condition.
A35-year-old man suffering from cerminous adenocarcinoma was complaining of otalgia, otorrhea and hard of hearing of the right ear. He had received surgeries of the right ear twice at the age of 20 and 30 years old. At that time the diagnosis was cerminous adenoma of the external auditory canal. A mass occuping the right external auditory canal with pulsating otorrhea and a mixed deafness of averaging 49dB was found. A radical mastoidectomy was performed and the tumor was found invading to the middle ear space. In spite of little difference of histopathological findings of the tumor from specimens of previous operations, it was diagnosed as cerminous adenocarcinoma based on clinical characteristics of local invasion and frequent recurrence. Careful histopathological evaluation and radical excision in the early stage may be required for treatment of cerminous gland tumors.
Congenital cholesteatoma of the middle ear have been detected more frequently in recent years. This article reports three patients, 5, 10 and 12 years of age, with double cholesteatomas of the middle ear. Microscopic examination of the tympanic membrane and high resolution CT scan of the temporal bone indicated attic cholesteatoma extending to the mastoid antrum in each case. Surgical exploration demonstrated the presence of a highly extensive cholesteatoma and a small lesion located in tegmental cells of the mastoid region, adjacent to the cochleariform process and superstructure of the stapes, respectively. There was no epithelial continuity between the large and small cholesteatomas. Based on the present findings, the large and small cholesteatomas may have possibly arisen from acquired and congenital origins, respectively. However, it seems reasonable to assume that both of them are due to congenital causes. As one of two small lesions expands, it would eventually perforate the tympanic membrane of the pars flaccida, become secondarily infected, and appear at a later date mimicking acquired attic cholesteatoma.
Screening of hearing disorders was performed for three-year-old children by using questionnaire, audiometry and tympanometry. The incidence of sensori-neural hearing loss was 0.3%(1 of 288 children) and otitis media with effusion (OME) was 11.5%(33 of them). Audiometry wasnot useful at least for the screening of OME. As to questionnaire, we could select only less than half of OME. It seems necessary to reexamine each content and to find out some adequate items again. Tympanometry was useful for OME especially when it was not detected by other methods. Auditory test by using six whispered words may be helpful to find out moderate or mild hearing loss.
To examine deterioration of hearing after glycerol intake, hearing was determined after intake in 56 ears with definite Ménière's disease and 26 ears with suspected Ménière's disease. In 37 ears, hearing level deteriorated after intake of glycerol, but in 21 ears, it improved after intake. Eleven ears showed both deterioration and improvement of hearing. In19of56ears with definite Ménierè's disease, hearing improved after glycerol intake, and in 21ears, it deteriorated. On the other hand, in 16 of 26 ears suspected Ménière's disease, hearing deteriorated after intake and improved in only 2 ears. Improvement of hearing was occurred mainly in the low and middle frequencies 2 or 3 hours after glycerol intake. In contrast, deterioration of hearing occurred mainly in the low and high frequencies at least 1 hour after glycerol intake. The results of this study suggested that the mechanism of deterioration in hearingis different from that of improvement.
A new model of an acoustic probe N-1for e-OAE measurement was designed by utilizing a box of a behind-the-ear type hearing aid as the power source for its microphone. The probe consisted of an alminium holder connected with a minuature microphone (Knowles, EM3056) and an earphone (Cortitone, 20W). Instead of barrelled tubes in the probe two acoustic holes were bored at the metal plane of the extremity of the probe tip. In the recording of OAE, a plastic earplug of appropriate size was slipped onto the tip of the probe, as in tympanometry. The frequency sensitivity response curve of the earphone for the input voltage of 1×10-3W was measured by 2cc coupler (B & K, HA-2) varied within 5dB between 150Hz and 4kHz. While the frequency sensitivity response curve of the microphone for the input of 94dB SPL varied within 10dB in the range from 150Hz to 9kHz. Thus, the probe N-1had anexcellent frequency characteristic similar to that of the probe K-3which we have been usedconventionally. As for its transient characteristic, this probe produced clear sonud wave forms without any residual oscillation in response to short tone bursts examined at thesound intensity of 10dB nHL when examined in a dummy ear. In comparison between the results of e-OAE obtained by the probe N-1and those by the probe K-3, there was no difference at all. The probe N-1 with a case box included a battery weighed 13.5g. The probe securely fitted to the external auditory canal owing to its holding system with the battery case of behind-the-ear type and it was possible to examine OAE at the sitting position. This probe is feasible for performing the examination even in a soundproof room for the single-person use. In consequence it is useful for the measurement of OAE in out-patient clinics.
It has been recognized that the endotoxins of gram-negative bacteria are responsible in the pathogenesis of otitis media with effusion, sustaining the inflammatory response after an acute primary infection has been cleared. Using a scanning electron microscope, the authors studied the change of middle ear mucosa of guinea pigs after applying endotoxin through the tympanic membrane into the middle ear cavity. Serous effusions were observed from the 2nd through the12th day after the local application. Edema and congestion of the middle ear mucosa were also found. The density and the activity of secretory cells were increased. An increase in number of microvilli on the non-ciliated cells was also noted. Some ciliated cells showed the abnormal figures such as short cilia, decreased number of cilia, knobformation, compound cilia and elongated cilia. The changes were similar to those observed in the human middle ear mucosa of otitis media with effusion. From these findings, it is conjectured that endotoxin is one of the causative factors in the pathogenesis of otitis media with effusion.
The distribution of type IV collagen (C-IV) and laminin, which are important components of basement membrane (BM), in the endolymphatic sac (ES), kidney, spleen and lymph node of healthy Hartley guinea pigs was studied immunohistologically. Antibodies against C-IV and laminin were used in this study. After proteolytic digestion, these components were able to be detected on the paraffin embedded tissue sections. The distribution of C-IV in the examined tissue sections was almost the same as that of laminin whereas C-IV was localized in subepithelial connective tissue of the ES where laminin was not seen. The epithelial BM of the rugose portion of the ES as well as tubular BM of the kidney showed the most intense labeling by anti-C-IV and-laminin antibodies while these components were weakly found in the epithelial BM of the distal portion of the ES. Additionally, C-IV and laminin were confirmed to be localized in vascular BM. These results suggest that the epithelial BM of the rugose portion may play an important role in fluid transport in the endolymphatic sac.
The vestibular ganglion cells of the rat were classified with regard to the cytoplasmic structure by light and electron microscopic observation. Light microscopy revealed four types of cells, depending upon the characteristics and distribution of Nissl granules. Electron microscopy gave a definite classification of these types by the arrangement and distribution of the rough surfaced endoplasmic reticulum and neurofilament. The characteristics ofthe ganglion cells were discussed in relation to the cell size and their neurotransmittingfunction.