Sensorineural Hearing Loss

tone chart

Sensorineural Hearing Loss will affect the majority of Superficial Siderosis patients and can be one of the first clinical symptoms to present. The eighth cranial nerve is a sensory nerve with two main components: the vestibular nerve, which is responsible for balance, and the cochlear nerve, which is responsible for hearing. it is especially sensitive to damage from superficial siderosis caused by the breakdown of slow or repeated episodes of subarachnoid hemorrhage into hemosiderin (Fig 13). It is postulated that the sensitivity of cranial nerve VIII to subpial hemosiderin deposition is due to its long cisternal segment, which exposes it to a high concentration of iron, in combination with the fact that the transition from central to peripheral myelin occurs near the IAC in cranial nerve VIII but relatively close to the brainstem in cranial nerves III–XII. The central myelin and the microglia that produce it are especially sensitive to siderosis. The high iron concentration lining the brain and cranial nerves will cause local magnetic inhomogeneity and is most easily visualized on susceptibility-weighted or gradient-echo T2*-weighted images (15,16). ¹   Hemosiderin and the associated free iron attaches to areas of the eighth cranial nerve slowly causing degeneration resulting in bilateral, asymmetric sloping sensorineural…

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The Tinnitus experienced by a large percentage of Superficial Siderosis patients points to a disorder of the cochleovestibular system and the neurodegeneration of the eighth cranial nerve. Commonly linked with Sensorineural Hearing Loss, Superficial Siderosis patients may experience multiple tone patterns such as high-pitched ringing, swooshing, chains clinking, chirping or buzzing intermittently or in some cases continuously. This abnormal auditory sensory perception can be a horribly challenging problem for those who are sufferers and has a very negative impact on the quality of life. Audiologists struggle to help Superficial Siderosis patients find relief from the sounds they are hearing in their head. A number of patients who have a 100% hearing loss in either one or both ears will report they are still tormented by tinnitus. White noise will be an ineffective masking tool for this patient group. Depression, fatigue, and stress is known to increase the intensity of tinnitus. There is no cure or proven medications for tinnitus.   tinnitus-sounds-bta from British Tinnitus Association on Vimeo.   Source: British Tinnitus Association  

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