scholarly journals Sudden Sensorineural Hearing Loss with Auditory Neuropathy: Evidence Based on Audiological Test and Inner Ear MRI

Author(s):  
Yu Ai ◽  
Na Hu ◽  
Mingming Wang

Abstract Background: Sudden sensorineural hearing loss (SSNHL), defined as a hearing loss of ≥30 dB affecting at least three consecutive frequencies occurring over a 72-hour period, is commonly in audiologic and otolaryngologic practice. However, SSNHL with auditory neuropathy is rarely reported. We described the clinical characteristics of a patient with SSNHL and auditory neuropathy, together with its etiologic mechanism. Case presentation: A 27-year-old woman was referred to our otological clinic with acute diminished hearing on the left ear, associated with persistent tinnitus, aural fullness and vertigo, all for a duration of 20 days. Audiological examination showed presence of otoacoustic emissions and cochlear microphone with absent ABR on the left ear, which was consistent with the criteria of auditory neuropathy. Magnetic resonance images of the brain and inner ear implicated demyelinating lesions in the auditory nerve. Forty months after onset, all the auditory retests were normal and symmetric T2-FLAIR signals of both auditory nerves were found in inner ear magnetic resonance images.Conclusion: We showed that the SSNHL with auditory neuropathy could be caused by demyelination. The case also suggests that some SSNHL caused by demyelination is reversible.

PLoS ONE ◽  
2017 ◽  
Vol 12 (10) ◽  
pp. e0186038 ◽  
Author(s):  
Jiwon Cho ◽  
Hanjae Cheon ◽  
Jung Hye Park ◽  
Hyo-Jeong Lee ◽  
Hyung-Jong Kim ◽  
...  

2016 ◽  
Vol 29 (4) ◽  
pp. 595-602 ◽  
Author(s):  
Andrea Ciorba ◽  
Virginia Corazzi ◽  
Chiara Bianchini ◽  
Claudia Aimoni ◽  
Henryk Skarzynski ◽  
...  

Electrolytic disorders of the inner ear represent a model that could be implicated in partially explaining the pathogenesis of sudden sensorineural hearing loss (SSNHL). Different types of electrolytes and different inner-ear loci are involved in cochlear homeostasis physiologically, to ensure the maintenance of an ion-balanced cochlear environment allowing a normal hair cell function. It has been hypothesized that a sudden loss of endocochlear potential, due to a rapid disruption of the inner ear fluid osmolality, could be responsible for a deterioration of the hearing function caused by damaged hair cells. The aim of this paper was to review the current literature and identify sources which might validate/fortify the hypothesis that inner ear electrolytic disorders have a role in the etiopathogenesis of SSNHL. The data in the literature underline the importance of ionic homeostasis in the inner ear, but they do not support a direct link between SSNHL and electrolyte disorders/imbalances. There is marginal evidence from otoacoustic emissions research that an indirect link might be present.


2020 ◽  
Vol 24 (4) ◽  
pp. 198-203
Author(s):  
Min Bum Kim ◽  
Jihyun Lim ◽  
Il Joon Moon

Background and Objectives: We sought to evaluate the diagnostic and prognostic value of measurable parameters of internal auditory canal (IAC) magnetic resonance imaging (MRI) in patients with idiopathic sudden sensorineural hearing loss (ISSNHL).Subjects and Methods: We retrospectively reviewed the patients with ISSNHL who underwent IAC MRI from January 2008 to March 2019. Measurable parameters of IAC MRI, such as the diameter of the IAC, bony cochlear nerve canal, and cross-sectional area of the cochlear nerve, were measured by a single examiner. These parameters were then compared between the affected and healthy sides. Inner-ear abnormalities such as intralabyrinthine hemorrhage or labyrinthitis were also evaluated. The relationship between the surveyed parameters and the diagnosis of ISSNHL was assessed.Results: A total of 208 patients with ISSNHL were included. The measured parameters of IAC MRI were not different between the affected and healthy sides and were also not associated with the diagnosis of ISSNHL. However, inner-ear abnormalities of IAC MRI in ISSNHL displayed a significant association with worse hearing before and after treatment. An age that was older than 40 years also correlated with poorer outcomes. Further, inner-ear abnormalities were more frequently detected when IAC MRI was performed early after ISSNHL onset.Conclusions: Patients with ISSNHL and inner ear abnormalities such as intralabyrinthine hemorrhage or labyrinthitis identified via IAC MRI may experience poorer hearing outcomes. To detect such abnormal findings, it is recommended to perform IAC MRI early after the onset of ISSNHL.


2019 ◽  
Vol 40 (2) ◽  
pp. 274-278
Author(s):  
Kaitian Chen ◽  
Lanying Wen ◽  
Ling Zong ◽  
Min Liu ◽  
Jincangjian Sun ◽  
...  

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