Sudden hearing loss and autoimmune inner ear disease

Area of Science:

• Audiology
• Immunology
• Otolaryngology

Background:

• Autoimmune inner ear disease (AIED) is an immune-mediated condition causing sensorineural hearing loss.
• AIED typically presents as asymmetric, bilateral hearing loss with an atypical configuration and rapid progression.

Observation:

• The case report details audiologic evaluations including basic battery, immittance audiometry, otoacoustic emissions, and auditory brainstem responses.
• Medical findings were monitored over a two-year period.
• Otoacoustic emissions and auditory brainstem responses indicated both cochlear and retrocochlear involvement, potentially misaligned with pure-tone thresholds.

Findings:

• Immune-mediated sensorineural hearing loss (AIED) often leads to disproportionately poor word recognition.
• Hearing loss in AIED is typically fluctuant but shows a pattern of rapid progression without early medical intervention.
• The case demonstrated findings suggestive of both cochlear and retrocochlear pathology.

Implications:

• Audiologists must be adept at identifying AIED due to the critical need for early diagnosis and intervention.
• An immunologic basis should be considered in various auditory disorders, including Meniere's disease.
• Prompt medical intervention is vital to mitigate the rapid progression of hearing loss in AIED.