Branchio-oto-renal dysplasia (BOR dysplasia) is an autosomal dominant disorder characterized by ear malformation, hearing loss, cervical fistulae and renal abnormalities. In this report we presented three cases of children with BOR dysplasia accompanied by mild or severe deafness. In one case exploratory myringotomy revealed an adhesion of the malleus neck to the scutum. Mobilization of the adhesive region did not improve the hearing loss. In mother of this case a sensorineural hearing loss in both ears deteriorated gradually in several years. In another case tympanoplasty was carried out in both ears for middle ear cholesteatoma. However, hearing loss did not improve postoperatively. The audiological findings indicated the malformation and the vulnerability of the inner ear. So audiological management over a long period of time should be needed and the indications of surgical treatment should be considered carefully.
Thirteen patients (15 ears, 4 males, 9 females) who suffered from acute otitis media associated were treated in our hospital between December 1994 through May 1995. The age of the patients ranged from 25 to 62 years. All patients complained of typical symptoms of severe acute otitis media such as otalgia, otorrhea, and hearing loss. Pure tone audiometry revealed mixed hearing loss in all cases. They were treated by intravenous or oral antibiotics, steroids and vitamins, and myringotomy or tympanostomy tube insertion was performed in most cases. Although complete recovery of hearing was obtained in 10 of 15 ears, there was a considerable amount of insufficient recovery in the bone conduction threshold. The onset of sensorineural hearing loss in these cases was thought to be related to the prevalence of influenza in the area of our hospital during the same period.
A 27-year-old female was complaining of headache and gait disturbance. Although the tympanic membrane had a normal appearance, a CT scan revealed a soft tissue density in the tympanic cavity and mastoid air cells in the left side. Adjacent to this lesion, there was a ring-form shadow in the left cerebellar hemisphere enhanced with contrast medium. Under the diagnosis of congenital cholesteatoma with a cerebellar abscess, antibiotics were administered intensively. After relief of cerebellar abscess, left tympanoplasty (two-staged operation) was performed. The middle ear structures were markedly destroyed by cholesteatoma. The ossicular chain was reconstructed by using an autologous cartilage graft from the auricle (columella type IV procedure). The postoperative hearing was improved by 20 dB. There has been no evidence of cholesteatoma recurrence.
A 46-year-old man complaining of a hearing loss in the right ear for three years was found a large size cholesteatoma involving the petrous apex by CT and MRI. The patient had no vertigo, facial palsy and tinnitus. A defect of the attic bone and the retracted paras fracida of his right ear drum were observed. The cholesteatoma was surgically removed under general anesthesia by transmastoid approach and the labyrinth was removed for complete extirpation of the lesion extended into the retro and infralabyrinthine areas. The widely exposed dura mata of the middle and posterior cranial fossa was covered with a temporal muscle flap and postoperative spaces of the temporal bone were filled with a graft of abdominal subcutaneous fat. No reccurence was found after the surgical procedure.
We investigated 22 patients with sudden deafness whose hearing became worse after the initial examination. Sixteen cases (Group A) made progression of hearing loss during the first 10 days after the onset. The remaining 6 cases (Group B) showed improvement of hearing loss but became worse again. In those who had progression of hearing loss, their audiograms divided into 2 types. Thirteen cases mainly showed the involvement of the low and middle frequencies. Hearing loss pattern of the remaining nine cases was flat type. We concluded that progression of hearing loss observed in Group A was caused by natural course of the disease. On the other hand, in Group B, we suspected that the steroid administration affected viral infection in the inner ear and made a relaps of sensorineural hearing loss.
Electrocochleogram (ECochG), evoked otoacoustic emission (EOAE) and ABR were examined in 5 patients with acute sensori-neural hearing loss which were diagnosed as sudden deafness by the conventional audiometry. In these 5 patients, recruitment phenmenon was found, but there were no lesions on CT and MRI and the patients recovered completely. ECochG and EOAE were examined at the time of first visit and at the time of recovery. Three of 5 cases showed broad waveform of wave I and prolonged wave I latency on ABR at the first visit. And on ECochG, at the first visit, AP showed broad waveform, lower amplitude and higher threshold. CM threshold was high but lower than the threshold of conventional audiometry. On EOAE, at the first visit, the threshold was similar to CM. These findings showed that the lesion of these 3 cases was not only in inner ear but also retrocochlear region. Another 2 of 5 cases showed normal ABR at the first visit. And on ECochG, at the first visit, one case showed normal AP, in the other it was showed lower amplitude AP and higher threshold, but normal waveform. These changes could be explained by the CM changes. CM threshold was higher and similar to the conventional audiometry threshold. On EOAE, one case showed no change between the first test and the second test and the threshold was a slightly high but lower than the threshold of conventional audiometry. In the other case, the threshold was a slightly high but lower either at the first test. These fibdings showed that the lesion of these 2 cases was in the inner ear, especially outer hair cell (OHC). There was gap between CM threshold and EOAE threshold. So it was hypothesized that the mobility of OHC was normal or slightly damaged and the electrical potential of OHC was mainly damaged. Therfore it is proved that there are two lesions in acute sensori-neural hearing loss including sudden deafness. One is in the inner ear and the other is in retrocochlear region. The ECochG and the EOAE are very useful to detect the lesion of acute sensori-neural hearing loss.
It is known that Ramsay Hunt's syndrome is caused by reactivation of varicella-zoster virus in ganglion cells and its spread to the cranial nerves and cutaneous tissue. This syndrome consists of facial nerve paralysis and vestibulocochlear symptoms associated with vesicular eruptions of the auricle and external auditory canal, and other cranial nerves are occasionally involved. The mechanism of the spread of inflammation to these organs has not been sufficiently clarified. Therefore, this clinical study was performed concerning the spread of inflammation in Ramsay Hunt's syndrome. 72 patients with Ramsay Hunt's syndrome were investigated their progressive course of vesicular eruptions and facial nerve palsy. There were 38 males and 34 females ranging in age from 15 to 79 years. Of the 42 patients without vestibulocochlear symptoms, 9 were eruptions preceding cases (22%), 19 were palsy preceding cases (46%) and 14 were simultaneously occurred cases (33%). Of the 30 patients with vestibulocochlear disorders, however, 20 were simultaneously occurred cases (67%) and 10 were eruptions proceeding cases (33%), and there were no palsy preceding cases. In this study, it was suggested that patients with neural spread of inflammation in the initial stage may have accompanied vestibulocochlear disorders in addition to their facial nerve paralysis.
Surgical exposure of the internal auditory canal by the middle cranial fossa approach for acoustic neurinoma is much more difficult than by the trans labyrinthine approach, because there are so few surgical landmarks in the middle cranial fossa to approach to the internal auditory canal. We developed a new method to identify the internal auditory canal easily and accurately by the following teckniques: 1) tracing bilateral internal auditory canal and external auditory canal from high-rsolution CT in several slice levels and a tumor from enhanced MRI and the fluoroscopic image was composed. 2) determination of the drilling line on the middle cranial fossa which showed the relationship of the external auditory canal and internal auditory canal. As the result, among 6 tumor excisions operated on with this new method, the internal auditory canal was easily reached without causing damage to the inner ear structure.