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). 6Department of Neurology, National Hospital for Neurology and Neurosurgery, London, United Kingdom.5Department of Brain Repair and Rehabilitation, Stroke Research Centre, Institute of Neurology, University College London, London, United Kingdom. 4Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, United Kingdom.3Department of Neuro-Otology, Royal National Throat, Nose and Ear Hospital, London, United Kingdom.2National Institute for Health Research, University College London Hospitals Biomedical Research Centre (Deafness and Hearing Problems Theme), London, United Kingdom.1Ear Institute, University College London, London, United Kingdom.June 19–21, 2008), a group of leading authorities on the condition reached a consensus and renamed it as auditory neuropathy spectrum disorder.Natallia Kharytaniuk 1,2,3 *, Peter Cowley 4, David J. Newborn Hearing Screening Conference, Como, Italy, Of Infants with Auditory Neuropathy, International In 2008 at a meeting convened at Lake Como in Italy (Guidelines DevelopmentĬonference on the Identification and Management The condition was originally termed auditory neuropathy (AN) and in 2001 as Auditory Neuropathy / Auditory Dys-synchrony (AN/AD) (to include those cases where no true neuropathy was apparent). Very few (1 in 14) will go on to develop normal speech and language but with poor speech perception in background noise and in others, no speech perception and therefore language development is possible. Individuals presenting with this recently recognised hearing loss appear to display sporadic windows of hearing and not. Auditory neuropathy spectrum disorder ( ANSD) is a specific form of hearing loss defined by the presence of normal or near-normal otoacoustic emissions (OAEs) but the absence of normal middle ear reflexes and severely abnormal or completely absent auditory brainstem response (ABRs).
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