Hearing loss in diffuse cutaneous systemic scleroderma

Patients with diffuse cutaneous systemic sclerosis (dcSSc) have distinct clinical manifestations, usually with more aggressive organ involvement and highermortality than limited cutaneous systemic sclerosis (lcSSC). Hearing loss had not yet been evaluated in this subtype, so our aim was to assess the auditory system of dcSSc patients in a controlled study.

Twenty-six patients with dcSSc, according to American College of Rheumatology (ACR) criteria, were evaluated. For each dcSSc patient included in the study, two healthy controls were matched for sex and age (± 2 years). All patients responded to a structured questionnaire regarding otological symptoms and risk factors with regard to hearing loss. A complete ear/nose/throat physical examination was performed followed by speech/pure tone audiometry and impedance measurements.

The mean age of the dcSSc patients was 47.0 ± 11.9 years; the mean disease duration was 9.4 ± 9.1 years, and there was a clear female predominance (83%). The questionnaire revealed auditory complaints in 54% of the dcSSc patients (25% hearing loss, 21% aural fullness, 21% tinnitus, and 21% dizziness) and normal otoscopy. Nearly half (46%) of the dcSSc patients were diagnosed as having hearing loss on audiogram compared to only 19% in the control group (p = 0.01). All dcSSc with hearing loss had a sensorineural component, eight (73%) with excellent discrimination on the Percentage Index of Speech Recognition (PISR). Descending configuration and Metz recruitment were observed in 54% of these patients, suggesting cochlear involvement.

Patients with dcSSc have a high prevalence of sensorineural audiometric hearing impairment and otological complaints, suggesting that the cochlea is an additional target organ in this disease.