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Author: admin, 11.05.2014
Sudden deafness is a disorder characterized by an abrupt sensorineural hearing loss of unknown etiology. Sudden deafness is defined by a high degree of abrupt sensorineural hearing loss without clear causes for the loss.
Eighteen patients who had received MRI examinations were chosen from patients complaining of sudden deafness. Pure-tone audiometry and short-increment sensitivity index tests were conducted on all patients. To summarize, among the 18 subjects, 3 of the 5 patients (17%) with sudden deafness showing abnormal MRI findings had SBF of the VBS, 1 (5%) had an abnormal course of the VBS, and 1 (5%) had labyrinthine enhancement. Sudden deafness refers to abrupt sensorineural hearing loss of unknown etiology, often accompanied by tinnitus and vertigo.
Viral labyrinthitis causes sudden deafness owing to infections caused by such viruses as measles or mumps, and it may occur without any other general symptoms. In the past, MRI was mostly used for the diagnosis of lesions of the internal auditory canal and of the cerebellopontine angle, and particularly for acoustic neuroma in deaf patients [5]. Seltzer and Mark [4] also reported that the MRI results of all five patients experiencing sudden deafness showed Gd-DTPA enhancement of the cochlea and the vestibule. Besides viral infections, circulation disorders of the inner ear are presumed to be a major cause of sudden deafness. Regarding the relationship between the abnormal findings on MRI and vertigo, our study found that all the cases showing abnormalities on MRI also showed canal paresis. No significant relationship was found between MRI results and the type of audiogram or degree of hearing loss.
Until now, the exact cause of sudden deafness has not been clarified, but abnormal blood flow of the inner ear is known as a cause of this disorder.
To determine whether establishing the pathogenesis of this disease is possible, this study analyzed the magnetic resonance imaging (MRI) of early stages of sudden deafness in combination with the clinical results of treatments.
Many theories seek to explain the etiology of sudden deafness; however, its exact causes are not specified. If it is not cured at an early stage, deafness can be permanent; thus, an early and accurate diagnosis is essential. They were hospitalized at Pusan National University Hospital from November 2000 to May 2001. The vestibular function test consisted of air-caloric tests, and the results were measured with electronystagmography. Figure 1 shows the Gd-DTPA-enhanced T1-weighted coronal image of patient 9, who had vertigo and sudden deafness in the left ear; the image reveals increased gadolinium enhancement in the left labyrinth.


Gd-DTPA TI-weighted coronal image in left ear of subject with sudden deafness with vertigo.
The patient having an abnormal course of the VBS and the patient having labyrinthine enhancement also showed vertigo and canal paresis. Schuknecht and Donovan [2] reported that the pathological findings of the temporal bones of patients having idiopathic sudden deafness showed an atrophy of the striae vascularis, the tectorial membrane, and the organ of Corti; these pathological findings are similar to those of a viral cochlear inflammation. The inner ear artery, which supplies blood to the inner ear, is provided through the anterior inferior cerebellar artery, the basal artery, and the posteroinferior cerebellar artery with the rostral vertebral artery [6]. The response to treatment tended to be slow in patients with abnormal MRI findings, as compared with patients in whom MRI findings were normal. The MRI findings of 18 patients with sudden deafness included three cases of slow blood flow of the vertebrobasilar system, one case of abnormal course of the vertebrobasilar system, and one case of labyrinthine enhancement.
Recently, magnetic resonance imaging (MRI) has become an important method for evaluation of ear diseases. Various suspected causes of sudden deafness include circulation disorder of the inner ear, viral infection, rupture of the cochlear membrane, syphilis, and allergy. The 4-mm-thick axial and coronal MRI tests produced TI-weighted images, Gd-enhanced T1-weighted images, proton density images, and T2-weighted images. Figure 2 shows the proton density axial image of patient 2, who had vertigo and sudden deafness in the right ear; this image displays increased signals in the basal artery, due to the SBF of the basal artery.
In addition, the patients showing abnormal MRI findings did not respond well to treatment as compared to those with normal MRI findings (Table 2). Generally, labyrinthitis caused by viral infection, blood circulation disorders, or rupture of the inner ear membrane are believed to cause sudden deafness [1]. The inflammation in the inner ear membrane can be found by conducting an antibody (Ab) test for viruses in the blood of affected patients.
In many cases, the decrease in the blood supply to the inner ear artery is caused by a decrease in the blood supply to the VBS or by vascular obstruction of the inner ear artery itself [7,8]. However, the number of cases examined in this study was small; therefore, no statistical significance is claimed. Therefore, we concluded that MRI is indispensable for examining patients with sudden deafness, especially those patients with accompanying vertigo. Labyrinthine enhancement on gadolinium enhanced MRI in sudden deafness and vertigo:Correlation with audiologic and electronystagmographic studies. Contrast enhancement of labyrinth on MR scans in patients with sudden hearing loss and vertigo:Evidence of labyrinthine disease. Sudden sensorineural hearing loss associated with slow blood flow of the vertebrobasilar system.


MRI showed many abnormal findings in the temporal bone of sudden-deafness patients who also complained of vertigo. To determine whether establishing the pathogenesis of sudden deafness is possible, we analyzed MRI scans of early stages of sudden deafness in combination with the clinical results of treatments.
However, no theory has yet been established for the causes of this disease, so making a definite diagnosis is very difficult.
All patients were managed with same treatment protocol, which consists of high-dose steroid, low-molecular-weight dextran, and carbogen (95% O2 + 5% CO2) therapy.
Figure 3 depicts the proton density axial image of patient 6, who had vertigo and sudden deafness in the left ear; this image shows increased signals in the left vertebrobasilar artery, due to the SBF. These researchers further reported that when the enhancement was confined to certain segments of the cochlea, deafness existed at the frequency of the region predicted by the enhancement. Although the exact mechanism and significance of SBF in sudden deafness are unknown, a temporary decrease in blood flow occurred at the onset of sudden deafness [11]. In particular, 5 of the 11 (45%) patients with sudden deafness and accompanying vertigo showed abnormalities on MRI, and 3 of the 11 patients (27%) showed SBF. In CW Cummings, JM Fredrickson, LA Harker (eds), Otolaryngology Head and Neck Surgery, 3rd ed. As compared with normal cases, the cases showing abnormal findings on MRI did not respond well to treatment.
To discover the exact causes of sudden deafness, the authors examined the relationships between patients' symptoms and the results of acoustic and vestibular function tests by analyzing abnormal findings on MRI, in particular the slow blood flow (SBF) of the vertebrobasilar system (VBS) and labyrinthine enhancement on MRI augmented by gadolinium (Gd-DTPA). Figure 4 shows the proton density axial image of patient 5, who had vertigo and sudden deafness in the right ear; the image exhibits an abnormal course of the right vertebrobasilar artery. Therefore, gadolinium enhancement of the labyrinth is characteristic of labyrinthine diseases [3,4,6]. Additionally, they reported that viral labyrinthitis was diagnosed in nine patients and leutic labyrinthitis was diagnosed in two. We concluded that MRI is indispensable for examining patients with sudden deafness, especially those who have accompanying vertigo.
The latter group also reported that the SBF of the VBS demonstrated high-signal proton density images [12-14].



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