RIOR REPORTS SUGGESTED THAT rapidly progressive, bilateral, asymmetric inner ear dysfunction may be caused by immunological attack or may be associated with autoimmune disorders. 1-3 McCabe 4 renewed interest in this disorder as a distinct clinical entity and motivated investigators to identify the most efficacious treatment regimens for management of this potentially reversible form of deafness. Initial experience with this disorder has underscored the risk of developing bilateral profound deafness and vestibulopathy if patients are left untreated or treated inadequately.On empirical grounds, glucocorticoids and cytotoxic agents were initially proposed as treatments. In a fol-

Endolymphatic hydrops (EH) is a pathological condition which is the final common manifestation of a variety of otologic insults. In this paper we develop a classification which, on the basis of clinical and pathological data, distinguishes symptomatic and asymptomatic forms. Clinical case histories and temporal bone studies are presented to illustrate and substantiate this classification. The symptomatic form becomes evident by the hallmark symptoms of episodic vertigo and fluctuating hearing loss. The asymptomatic form is silent. Inter-conversion from one form to another may occur over time. Each of the forms can be subdivided, according to etiology, into embryopathic, acquired, and idiopathic types. The embryopathic type comprises those cases in which some noxious influence interferes with prenatal labyrinthine development. The acquired type includes those cases in which a documented insult, be it inflammatory or traumatic, is suffered by a previously normal labyrinth. The inflammation may be viral, bacterial, or spirochetal in nature, while the traumatic event may be either accidentally or surgically sustained. The idiopathic type includes cases in which the instigating event precipitating the EH is unknown. Menière's disease represents merely one example of the EH group of disorders, namely symptomatic idiopathic EH.

Steroid treatment in AIED-mediated hearing loss produce variable but significant hearing gains. Neither a focal, cochleotopic region of greatest vulnerability to AIED nor frequency-specific amenability to treatment were evident. We did observe that analysis of predictors and the degree of treatment effect vary with different approaches to measuring change in the WIS. Depending on the approach adopted, the size of the treatment effect may be greatest across intermediate hearing levels at baseline. These observations offer an audiometric database that may enable greater precision in judging clinically meaningful parameters for future studies of AIED treatment and other interventions for sensorineural hearing loss.

Sudden deafness with or without vertigo presents a difficult diagnostic problem. This article describes 12 patients with enhancement of the cochlea and/or vestibule on gadolinium-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (MRI), correlating the enhancement with the auditory and vestibular function. All patients were studied with T2-weighted axial images taken through the whole brain, enhanced 3-mm axial T1-weighted images taken through the temporal bone, and enhanced T1-weighted sagittal images taken through the whole brain. Cochlear enhancement on the side of hearing loss was found in all the patients. The vestibular enhancement correlated with both subjective vestibular symptoms and objective measures of vestibular function on electronystagmography. In 2 patients, the resolution of symptoms 4 to 6 months later correlated with resolution of the enhancement on MRI. No labyrinthine enhancement was seen in a series of 30 control patients studied with the same MRI protocol. Labyrinthine enhancement in patients with auditory and vestibular symptoms is a new finding and is indicative of labyrinthine disease. While abnormalities on electronystagmograms and audiograms are nonspecific and only indicate a sensorineural problem, enhanced MRI may separate patients with retrocochlear lesions, such as acoustic neuromas, from those in whom the abnormal process is in the labyrinth or the brain.