Fluctuating hearing loss secondary to spontaneous intracranial hypotension: A case report and review of the literature.

ABSTRACT

OBJECTIVE: Fluctuating sensorineural hearing loss (SNHL) has multiple etiologies, most commonly Ménière's disease (MD), recurrent sudden SNHL, and autoimmune inner ear disorders. Fluctuating SNHL has rarely been described as a symptom of spontaneous intracranial hypotension (SIH). PATIENT A 39-year-old previously healthy female presented with "Ménière's like" symptoms responsive to steroid treatment, which worsened during the day and improved in the supine position. Conservative treatment for MD consisting of low salt and caffeine diet and betahistine medication yielded no improvement. Secondary revision of brain imaging scans showed signs indicative of SIH, and a spinal cerebrospinal fluid leak was ultimately found and treated by a novel technique of transvenous fistula embolization by means of Onyx® glue, leading to gradual clinical improvement and near-complete resolution of symptoms. CONCLUSION: SIH should be considered as part of the differential diagnosis of fluctuating SNHL. Clinical and radiological features should be known and sought. We suspect that early diagnosis and treatment can lead to cure and prevent permanent auditory damage.