Sudden Deafness and Facial Diplegia in Guillain-Barré Syndrome: Radiological Depiction of Facial and Acoustic Nerve Lesions

Takazawa, Takanori; Ikeda, Ken; Murata, Kiyoko; Kawase, Yuji; Hirayama, Takehisa; Ohtsu, Motoharu; Harada, Hisanobu; Totani, Takeshi; Sugiyama, Kunio; Kawabe, Kiyokazu; Kano, Osamu; Iwasaki, Yasuo

doi:10.2169/internalmedicine.51.7737

Cited by 17 publications

(15 citation statements)

References 16 publications

(28 reference statements)

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“…Our findings indicate that HHL can also arise from pathologies of cochlear Schwann cell (schwannopathy) or nodal structures (nodopathy), rather than synaptopathy. Indeed, some patients with demyelinating disorders such as CMT disease and GBS have auditory deficits, associated with reduced ABR amplitudes and increased latencies 60 61 62 63 64 . Our animal model shares mechanistic similarities with GBS, where Schwann cells are specifically and transiently damaged 52 .…”

Section: Discussionmentioning

confidence: 99%

Transient auditory nerve demyelination as a new mechanism for hidden hearing loss

2017

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Hidden hearing loss (HHL) is a recently described auditory neuropathy believed to contribute to speech discrimination and intelligibility deficits in people with normal audiological tests. Animals and humans with HHL have normal auditory thresholds but defective cochlear neurotransmission, that is, reduced suprathreshold amplitude of the sound-evoked auditory nerve compound action potential. Currently, the only cellular mechanism known for HHL is loss of inner hair cell synapses (synaptopathy). Here we report that transient loss of cochlear Schwann cells results in permanent auditory deficits characteristic of HHL. This auditory neuropathy is not associated with synaptic loss, but rather with disruption of the first heminodes at the auditory nerve peripheral terminal. Thus, this study identifies a new mechanism for HHL, highlights the long-term consequences of transient Schwann cell loss on hearing and might provide insights into the causes of the auditory deficits reported in patients that recover from acute demyelinating diseases such as Guillain–Barré syndrome.

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Section: Discussionmentioning

confidence: 99%

Transient auditory nerve demyelination as a new mechanism for hidden hearing loss

2017

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“…Although it's a rare finding, there are few case reports describing this entity (Takazawa, et al . )[ 14 ]. All these patients had multiple cranial nerve palsies including facial and bulbar palsies.…”

Section: Discussionmentioning

confidence: 99%

A study of Guillain–Barré syndrome with reference to cranial neuropathy and its prognostic implication

Bhargava¹,

Banakar²,

Pujar³

et al. 2014

Journal of Neurosciences in Rural Practice

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Background:Focused studies on cranial neuropathy in Guillain–Barré syndrome (GBS) and its prognostic implication are not done previously.Aim:To study the clinical profile of GBS patients with special reference to cranial neuropathy and its prognostic implication.Materials and Methods:The study included 61 patients with GB syndrome, fulfilling Asbury Cornblath's criteria for GB syndrome. A pre-designed semi-structured questionnaire was used to obtain data regarding demographic profile and clinical profile. All patients underwent detailed neurological examination, investigations including nerve conduction studies and CSF examination and treated according to the severity of the illness. Patients were followed up for 6 months. During analysis two groups were made depending on cranial nerve involvement, and compared with respect to various parameters.Results:Out of 61 patients 38 (62.3%) patients had cranial nerve palsies, in that 25 had multiple cranial nerve palsies, and 13 had single isolated nerve palsy. A majority of 30 (49.2%) had bulbar palsy, 28 (46%) had facial nerve palsy, and all had bilateral involvement except 3 patients who had unilateral palsy. Hypoglossal nerve involvement was seen in six (10%) patients and four (6.5%) patients had ophthalmoplegia. Only one had bilateral vestibulocochlear nerve palsy. On comparing various clinico-electrophysiological parameters among patients of GB syndrome with and without cranial nerve involvement, the presence of respiratory paralysis, IVIg and ventilatory support requirement had significant association with cranial nerve involvement in GBS.Conclusion:Our study found a correlation between cranial nerve palsies and severity of the illness. Cranial nerve innervated muscles recover earlier as compared to distal limb muscles. No association was found between outcome at 6 months and cranial nerve involvement.

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“…Guillain-Barre syndrome (GBS) is a peripheral neuropathy caused by Schwann cell damage, possibly through an autoimmune mechanism. Although the incidence is relatively low, a portion of GBS patients show abnormal ABRs (Ropper and Chiappa, 1986;Nelson et al, 1988;Schiff et al, 1985;Ueda and Kuroiwa, 2008;Takazawa et al, 2012). Intriguingly, although hearing thresholds gradually recover in some patients, their ABR waveforms show persistent increases in interpeak latencies (Takazawa et al, 2012), suggestive of permanent myelin deficits in these patients.…”

Section: Peripheral Neuropathymentioning

confidence: 99%

“…Although the incidence is relatively low, a portion of GBS patients show abnormal ABRs (Ropper and Chiappa, 1986;Nelson et al, 1988;Schiff et al, 1985;Ueda and Kuroiwa, 2008;Takazawa et al, 2012). Intriguingly, although hearing thresholds gradually recover in some patients, their ABR waveforms show persistent increases in interpeak latencies (Takazawa et al, 2012), suggestive of permanent myelin deficits in these patients. Our recent study using mouse models showed that when Schwann cells are lost, they regenerate and remyelinate SGNs, but heminodes remain disrupted and ABR latencies prolonged (Wan and Corfas, 2017).…”

Section: Peripheral Neuropathymentioning

confidence: 99%

Myelin development, plasticity, and pathology in the auditory system

Long

Wan

Roberts

et al. 2017

Developmental Neurobiology

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Myelin allows for the rapid and precise timing of action potential propagation along neuronal circuits and is essential for healthy auditory system function. In this review we discuss what is currently known about myelin in the auditory system with a focus on the timing of myelination during auditory system development, the role of myelin in supporting peripheral and central auditory circuit function, and how various myelin pathologies compromise auditory information processing. Additionally, in keeping with the increasing recognition that myelin is dynamic and is influenced by experience throughout life, we review the growing evidence that auditory sensory deprivation alters myelin along specific segments of the brain’s auditory circuit.

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Sudden Deafness and Facial Diplegia in Guillain-Barré Syndrome: Radiological Depiction of Facial and Acoustic Nerve Lesions

Cited by 17 publications

References 16 publications

Transient auditory nerve demyelination as a new mechanism for hidden hearing loss

Transient auditory nerve demyelination as a new mechanism for hidden hearing loss

A study of Guillain–Barré syndrome with reference to cranial neuropathy and its prognostic implication

Myelin development, plasticity, and pathology in the auditory system

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