![]() ![]() We report findings of central auditory dysfunction and bilateral (asymmetrical) auditory neuropathy in a patient with iSS and correlate these with the self-report measures and the brain magnetic resonance images (MRI). Central auditory function in iSS may be affected since haemosiderin is frequently deposited in the surfaces of key auditory processing areas including temporal cortices but there are no detailed studies ( 2, 4). Comprehensive systematic analysis of the audiovestibular function in iSS is lacking and it is difficult to ascertain the exact site of lesion ( 4). Balance dysfunction in iSS can be of both central (cerebellar) and peripheral vestibular origin ( 4, 5). Reports of the auditory brainstem responses and stapedial reflexes findings are variable, with some also reporting cochlear involvement ( 3, 4). The choice of audiological (and vestibular) tests is guided by the patient's signs and symptoms and the overall clinical presentation ( 4, 5). ![]() It may resemble age-related hearing loss (ARHL) ( 1, 3, 4). Hearing loss is usually described as high-frequency sensorineural, bilateral and often asymmetrical, ranging from mild-moderate to severe-profound ( 1, 3). The most commonly identified cause of iSS is a dural defect, usually due to previous trauma or neurosurgery the bleeding may originate from damaged capillaries at the dural breach margins ( 1, 2).Ĭlinically, iSS is characterized by a triad of hearing loss (most frequent symptom), imbalance (ataxia) and myelopathy. It is characterized by haemosiderin deposition on the surfaces of the brain, cerebellum, brainstem and spinal cord due to chronic continuous or intermittent low volume and low pressure bleeding into the subarachnoid space ( 1). Infratentorial (classical) superficial siderosis (iSS) is a rare but increasingly recognized disabling neurological condition ( 1– 3). We correlate the audiological and vestibular findings with self-report measures and the siderosis appearances on brain magnetic resonance images. ![]() We confirm the presence of (asymmetrical) auditory neuropathy and identify central auditory processing deficits, suggesting involvement of the central auditory pathway beyond the brainstem. We describe such findings in a patient with iSS in an attempt to precisely localize the site of the audiovestibular dysfunction, determine its severity and functional impact. To the best of our knowledge, there have been no previous reports assessing central auditory function in iSS. In addition, monitoring disease progression and response to treatment is challenging and currently mainly guided by subjective patient reports and magnetic resonance imaging. There are few comprehensive descriptions of audiovestibular function in iSS and therefore limited understanding of the affected segment(s) of the audiovestibular pathway. Hearing and balance impairment are the most frequently reported features of infratentorial (classical) superficial siderosis (iSS).
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