Cochlear implant outcomes in prelingually deafened adults with and without sound deprivation: Are there differences in quality of life?

Background: Indications for cochlear implantation (CI) are constantly being updated, and with them, the audiometric results achieved by patients. Patient satisfaction should always be considered, even in patients with lower audiological results. The aim of the present study was to compare quality of life (QoL), self-perceived hearing benefit, and audiometric results between prelingually and postlingually deafened patients, with and without sound deprivation, after CI. Material/Methods: The sample included 46 patients with bilateral sensorineural hearing loss: 22 postlingually deafened and 24 prelingually deafened, further subdivided into sound-deprived (n=10) and non-sound-deprived (n=14). Auditory performance was evaluated with pure tone audiometry, speech recognition scores (SRS), and self-perceived hearing benefit, whereas QoL was evaluated with 2 self-reported questionnaires (Comprehensive Cochlear Implant Questionnaire and World Health Organization Quality of Life-BREF). Results: Audiometric results were worse in the prelingually deafened than in the postlingually deafened group, and worse in the prelingually deafened patients with sound deprivation. There was no marked difference in perceived CI benefit or QoL between the 2 groups or within the 2 prelingually deafened subgroups. No correlation was found between SRS and duration of CI use or between QoL and SRS in the prelingually and postlingually deafened groups. Conclusions: Our findings demonstrate better auditory performance for the postlingually deafened group and no differences in perceived QoL or benefit of CI between the groups. The sound-deprived patients had equal scores on the perceived QoL questionnaire. These analyses suggest that sound-deprived, prelingually deafened patients may benefit from CI

Stapedial Reflex: A Possible Novel Biomarker of Early Bulbar Involvement in Amyotrophic Lateral Sclerosis Patients

Background and Aim: Amyotrophic lateral sclerosis (ALS) is a neuromuscular progressive disorder, characterized by limb and bulbar muscle wasting and weakness. 30% of patients present a bulbar onset, while 70% a spinal outbreak, although most of them develop bulbar impairment later on. Due to the lack of an early biomarker of bulbar involvement, we chose to evaluate the role of stapedial reflex (SR) in order to predict preclinical bulbar impairment in ALS. Materials and Methods: We enrolled 36 ALS patients. We assessed revised-ALS functional-rating-scale and SR for a total of 4 visits. We established the presence of SR, acoustic reflex latency test (ARLT), and SRs Decay. Patients who had not develop bulbar signs at fourth visit continued follow-up up to 15 months. Data were analyzed by using Mann-Whitney U test, Friedman test, and Cox regression analysis. Results: We observed that SRs Decay at 500 and 1,000 Hz is the first parameter of SR to get altered in all ALS patients before the development of bulbar impairment. Twenty-eight patients developed bulbar impairment during the study. We highlighted a correlation between the progression rate of disease and both time of SRs Decay alteration and time of bulbar impairment from disease onset. Four patients who did not develop bulbar impairment had a progression rate lower than the other ones (p < 0.05). Discussion and Conclusions: This study shows that SR Decay test could be a sensitive measure for detecting pre-symptomatic bulbar involvement in ALS and could represent a simple, noninvasive, and useful biomarker of disease progression