This is a report of three cases of mixed hearing loss that resulted from inner ear disorders. Two cases were unilateral and the third was bilateral. The diagnosis was based on the findings of normal middle and external ears in association with the absence of round window reflexes. The contralateral stapedial reflex was present in the two unilateral cases. This is the first documentation of conductive deafness due to inner ear abnormality. This diagnosis should be considered in cases of conductive hearing loss if the middle and external ears are normal. More studies are needed to establish the pathophysiology of this entity.

Ear damage in systemic lupus erythematosus (SLE) patients has been occasionally reported but the frequency and the mechanisms of ear involvement are not well documented. In an attempt to investigate the presence of hearing loss and the possible causes for it we prospectively evaluated 43 SLE patients. All patients underwent a complete ear-nose-throat physical examination and audiological evaluation with pure tone, impedance and speech audiometry. In addition, systemic manifestations of the disease and drug therapy were recorded. Finally, all patients were tested for the presence of autoantibodies. The results were compared with those of 50 age-matched healthy subjects.

Hearing loss (HL) was found in nine patients (22.5 per cent). More specifically, eight patients presented sensorineural hearing loss (SNHL) (21.5 per cent) and only one had conductive hearing loss (CHL) (2.63 per cent). From the patients with SNHL, one had bilateral symmetrical damage, four had bilateral but no symmetrical damage and three patients showed unilateral SNHL. Finally, the patient with CHL had unilateral involvement. There were no statistically significant differences between patients with HL and those without regarding age, disease duration, clinical disease manifestations, autoantibody profile and drug therapy.

In conclusion, one fourth of our SLE patients presented HL, expressed as SNHL affecting mainly the middle and high frequencies, while only one patient had CHL. This is a lower percentage of ear involvement in SLE than that reported by other investigators. The mechanism of ear damage remains unknown. Thus, additional prospective studies are needed to elucidate its pathogenesis.