Hearing loss in autoimmune disorders: Prevalence and therapeutic options.

The objective of this study was to review our current knowledge relative to the correlation between sensorineural hearing loss (SNHL) and autoimmune diseases, focusing on the prevalence of hearing loss in different pathologies and possible therapeutic approaches. A review of the literature on hearing loss in different forms of autoimmune disease has been carried out, with emphasis on incidence and prevalence of SNHL. Therapeutic protocols have been assessed including both conservative medical and rehabilitative methods. Cochlear implant outcomes have been investigated. The prevalence of hearing loss in autoimmune and immune-mediated inner ear diseases, as referred by case reports or single-center statistics, is widely variable. More difficult is the evaluation of severe/profound SNHL, usually reported in relation to cochlear implantation. Though these patients represent ideal candidates for cochlear implantation, as they become deaf after years of hearing; the associated systemic disease, the specific damage on inner ear structures and the medication taken may influence the result of cochlear implantation. The main problem is the cochlear fibrosis or ossification that has been found to affect 50% of implanted ears in patients suffering from autoimmune and immune-mediated SNHL. Hence, in the presence of severe/profound SNHL earlier implantation may be indicated before post-inflammatory obliterative changes to the cochlea.

Adequate formal language performance in unilateral cochlear implanted children: is it indicative of complete recovery in all linguistic domains? Insights from referential communication.

OBJECTIVES: Referential communication (RC) is a key element in achieving a successful communication. This case series aimed to evaluate RC in children with unilateral cochlear implants (CIs) with formal language skills within the normal range. METHODS AND MATERIALS: A total of 31 children with CIs, with language development within the normal range, were assessed using the Pragmatic Language Skills test (MEDEA). RESULTS: Of the children with CIs, 83.9% reached performance levels appropriate for their chronological ages. The results confirmed a positive effect of cochlear implantation on RC development, although difficulties remained in some CI users. CONCLUSIONS: The outcomes emphasize the need to pay greater attention to the pragmatic aspects of language, assessing them with adequate testing in the early phase after cochlear implantation. Clear knowledge of children's communicative competence is the key in optimizing their communicative environments in order to create the basis for future successful interpersonal exchanges and social integration.

Long-term results of intratympanic prednisolone injection in patients with idiopathic sudden sensorineural hearing loss.

CONCLUSION: The results demonstrate the stability of the efficacy over time of the intratympanic prednisolone protocol and they suggest that the spontaneous recovery does not significantly influence the long-term evaluation of intratympanic therapy. A 10-day follow-up after 3 days of intratympanic prednisolone administration can be considered a sufficient period to evaluate the effectiveness of the undertaken therapy. OBJECTIVE: To assess the stability of the efficacy over time of intratympanic steroid therapy for patients with idiopathic sudden sensorineural hearing loss. METHODS: A total of 122 patients received an intratympanic steroid injection of prednisolone daily for 3 days. Audiograms were performed before therapy and at 10 days and 1 year after therapy. Successful recovery was defined as complete and partial recovery using Siegel's criteria and complete and marked recovery following Furuhashi's criteria. RESULTS: The comparison of audiometric data at study entry, at 10 days, and at 1 year after the treatment showed a statistically significant improvement of the mean pure tone audiometry. The evaluation of the hearing outcomes demonstrated a statistically significant improvement, with similar success rates when evaluated 10 days and 1 year after the treatment. Comparing the hearing outcomes at 1 year with the results at 10 days, no significant difference was detected between these two time points.