Hearing dysfunction in patients with Neuro-Sjögren: a cross-sectional study.

Area of Science:

• Neurology
• Otolaryngology
• Immunology

Background:

• Sjögren's syndrome is an autoimmune disease causing gland destruction and sicca symptoms.
• Extraglandular neurological manifestations, including cranial nerve involvement, are increasingly recognized.
• Hearing loss due to vestibulocochlear nerve damage is a significant disability in Neuro-Sjögren.

Purpose of the Study:

• To evaluate the prevalence and nature of hearing dysfunction in patients with Sjögren's syndrome and polyneuropathy (Neuro-Sjögren).

Main Methods:

• Thirty patients meeting ACR-EULAR criteria for Sjögren's syndrome and polyneuropathy were assessed.
• Auditory function was evaluated using pure tone audiometry, speech comprehension tests, otoacoustic emissions, and brainstem response audiometry.

Main Results:

• Hearing loss was detected in 33% of patients, ranging from mild to severe.
• Retrocochlear auditory dysfunction was found in an additional 47% of patients.
• Overall, 80% of patients exhibited pathological audiometric findings indicating hearing dysfunction.

Conclusions:

• Hearing dysfunction is significantly underestimated in patients with Neuro-Sjögren.
• Cranial neuropathy may be a more frequent cause of hearing impairment in this population than previously recognized.