Fluctuating corticosteroid-responsive auditory neuropathy/dyssynchrony is suggestive of central nervous system pathology.
OBJECTIVE
To highlight diagnostic and management features of auditory neuropathy/auditory dyssynchrony (AN/DS) due to central demyelinating disorder.


METHODS
A child with AN/DS due to central nervous system pathologic findings.


METHODS
Audiometry, auditory brainstem response (ABR) test, otoacoustic emission test, magnetic resonance imaging (MRI) with gadolinium contrast, intravenous corticosteroid treatment, antiviral treatment, stereotactic biopsy, and cyclophosphamide immunomodulation.


METHODS
Pure-tone audiometry, speech discrimination testing, ABR, and MRI.


RESULTS
A 12-year-old girl presented with acute sensorineural hearing loss, abnormal ABR, and normal otoacoustic emissions consistent with AN/DS. The hearing loss demonstrated fluctuation and corticosteroid responsiveness. Magnetic resonance imaging and stereotactic biopsy revealed brainstem demyelination consistent with multiple sclerosis. Definitive treatment consisted of cyclophosphamide immunomodulation.


CONCLUSIONS
Although recent focus on pathophysiology of AN/DS has shifted from auditory nerve abnormalities to dyssynchrony within the cochlea, cases associated with fluctuating sensorineural hearing loss and responsiveness to corticosteroid therapy should raise the suspicion of central nervous system pathologic findings. Therefore, it is crucial to obtain brain MRI with contrast enhancement in all patients with AN/DS. This is critical in patients undergoing cochlear implantation because MRI may be contraindicated postoperatively.
Mallur
Pavan S
PS
Department of Otolaryngology, New York University School of Medicine, New York, New York 10016, USA.
Lalwani
Anil K
AK
D000212:Acyclovir / Q000627:therapeutic use; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000998:Antiviral Agents / Q000627:therapeutic use; D001298:Audiology; D001301:Audiometry, Pure-Tone; D002493:Central Nervous System Diseases / Q000150:complications*; D002648:Child; D003056:Cochlear Nerve; D003520:Cyclophosphamide / Q000627:therapeutic use; D016057:Evoked Potentials, Auditory, Brain Stem / Q000187:drug effects / Q000502:physiology; D005260:Female; D005682:Gadolinium; D046088:Hearing Loss, Unilateral / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D009103:Multiple Sclerosis / Q000175:diagnosis / Q000473:pathology; D009186:Myelin Sheath / Q000473:pathology; D013063:Speech Discrimination Tests; D000160:Vestibulocochlear Nerve Diseases / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology*
D000305:Adrenal Cortex Hormones; D000998:Antiviral Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D005682:Gadolinium; D000212:Acyclovir