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PURPOSE: To analyze the clinical presentation, management, and outcome of two sisters affected by hereditary transthyretin related amyloidosis (ATTRv) and bilateral severe-to-profound hearing loss treated with cochlear implant. METHODS: A description of two patients underwent three cochlear implant (CI) surgery was performed. Age at time of implantation ranged from 48 to 53 years. Both patients were initially equipped with a bilateral hearing aid and subsequently underwent cochlear implant. A bimodal stimulation was maintained thereafter. One case underwent bilateral surgery. Hearing outcomes were assessed by comparing pre- and post-operatively tonal and vocal hearing thresholds and speech perception tests (Common Evaluation Protocol Results in Rehabilitation Audiology). RESULTS: In both patients, a significant and stable improvement of hearing threshold and speech perception was observed up to the last follow-up. No post-operative complication were observed during the follow-up. CONCLUSIONS: ATTRv amyloidosis may be the cause of cochlear function impairment. Restoring the hearing in these group of patients is of paramount importance since the vision is often compromised too. Cochlear implant could be a viable strategy of rehabilitation for selected patients affected by severe-to-profound sensorineural hearing loss caused by ATTRv amyloidosis.

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PURPOSE: Both hearing poor comprehenders (PCs) and deaf or hard-of-hearing (DHH) PCs have problems in understanding causal and temporal coherence relations signaled by connectives. The study examined whether hearing and DHH PCs' problems with connective understanding are similar and mainly related to their limited vocabulary, including knowledge of connective words, or to their poor reading comprehension abilities more generally. METHOD: Three groups of 7- to 10-year-old readers, matched on grade level (hearing PCs, DHH PCs, and hearing good comprehenders [GCs]) performed a reading comprehension task, a vocabulary task, and causal and temporal connective understanding tasks. Hearing and DHH PCs were also matched on reading comprehension and decoding abilities. RESULTS: The DHH PCs performed significantly worse than both the hearing GCs and PCs in temporal and causal connective understanding. Significant differences between hearing PCs and GCs were found only in causal connective understanding. DHH readers' difficulties in causal connective understanding were significantly associated with poorer vocabulary knowledge. In contrast, vocabulary knowledge did not uniquely contribute to hearing PCs' difficulties with causal connective understanding, once their reading comprehension skills were controlled for. CONCLUSIONS: The results suggest that despite a similar reading profile, DHH PCs' difficulties with causal connective understanding are more closely related to their vocabulary delay, whereas hearing PCs' difficulties are more strongly influenced by their poor text integration processes (as indexed by their reading comprehension skills). Neither vocabulary knowledge nor reading comprehension skills contributed to the explanation of DHH readers and hearing PCs' temporal connective understanding.

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BACKGROUND: Recently, an increasing number of articles have appeared on central auditory processing disorders, but in the literature there is only one study that evaluated the possible correlation between migraine in the critical phase and central auditory processing. The aim of our study was to assess the correlation between auditory processing information and childhood primary headaches in the intercritical phase. METHODS: This is an observational study. We enrolled 54 patients, 30 with primary headache (migraine and tension headache) and 24 normal controls, matched for sex and age. The mean age at first observation was 9 years 10 months; the duration of observational follow-up was 2 years. Both groups had normal audiological and neurological profiles, normal peripheral hearing acuity and normal cognitive and behavioral skills. We excluded patients who had undergone pharmacological prophylactic treatment for headaches in the 6 months preceding the study and subjects with a frequency of headache lower than one every two months. After enrolment, both groups were analyzed with a computerized test battery for Speech Perception Tests in silence and in noise background to assess speech perception disabilities. In addition, with a test battery of Speech Perception Tests, we compared patients with migraines and tension-type headaches. The non-parametric χ2 test, the Mann-Whitney U-test and the Wilcoxon signed ranks test were used for statistical analysis. P-values <0.05 were considered significant and STATA 10 software was used for statistical analyses. RESULTS: Our results showed that patients with primary headache (migraine and tension-type headache), had a deficit of auditory processing in noisy background compared to control cases, but we found no significant differences when we compared patients with migraine and tension-type headache. CONCLUSIONS: This is a work in progress and further studies are needed to assess the relationship between the impairment of auditory processing and primary headache, not only to improve the diagnostic approach to primary headache, but also to improve therapeutic intervention.

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