Association between hearing loss and hereditary ATTR amyloidosis.

Hereditary transthyretin (TTR) related amyloidosis (ATTRv) is a life-threatening condition, which can potentially affect all organs. The objective was to identify the hearing status of patients with cardiac ATTRv and describe their audiological pattern. Nineteen patients with confirmed diagnosis of ATTRv cardiac amyloidosis (CA) underwent otoscopy and audiological tests, including pure tone and speech audiometry.

Pharyngo-laryngeal involvement in systemic amyloidosis with cardiac involvement: a prospective observational study.

Systemic amyloidosis with cardiac involvement (CA) is a severe disease caused by the aggregation of misfolded proteins infiltrating organs and tissues and leading to their dysfunction. No study has yet focused on potential pharyngo-laryngeal impairments associated to CA. Our objective was to define its prevalence and describe pharyngo-laryngeal involvement patterns in a population with CA (light chain: AL, wild-type transthyretin: ATTRwt, variant transthyretin: ATTRv).

Stevens-Johnson syndrome and toxic epidermal necrolysis: ear, nose, and throat description at acute stage and after remission.

Importance: Ear, nose, and throat (ENT) lesions are frequently involved in Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN), although a detailed description is lacking in the literature.

Objectives: To describe ENT lesions at the acute stage and follow-up in a large series of patients with SJS/TEN and identify factors associated with the severe ENT form.

Design, Setting, And Participants: Retrospective study of 49 patients with SJS/TEN hospitalized in a referral care center from 2005 to 2010.

Cochlear implant outcomes in the elderly.

Objective: This study aimed to review cochlear implantation with respect to surgical and auditory outcomes in subjects aged 70 years and older.

Study Design: Retrospective chart review.

Setting: Tertiary referral centers.