scholarly journals Syphilis of the inner ear causing fluctuating hearing loss responsive to steroid therapy.

1989 ◽  
Vol 82 (2) ◽  
pp. 207-211
Author(s):  
Tadahito Saito ◽  
Mituo Mukudai ◽  
Kyoko Tamaki ◽  
Tosihide Tabata
1983 ◽  
Vol 59 (4) ◽  
pp. 703-705 ◽  
Author(s):  
Orhan Barlas ◽  
Hüsameddin Gökay ◽  
M. İnan Turantan ◽  
Nermin Başerer

✓ Two cases of aqueductal stenosis presenting with fluctuating hearing loss, tinnitus, and vertigo are presented. Audiovestibulometric assesment of both cases disclosed the characteristic pattern of disorder of the membranous inner ear. Non-tumoral aqueductal stenosis was demonstrated by computerized tomography in one case and by positive contrast ventriculography in the other. Shunting procedures of the cerebrospinal fluid resulted in resolution of inner ear dysfunction in both patients.


2021 ◽  
pp. 1-8
Author(s):  
Ramin A. Morshed ◽  
Nicole T. Jiam ◽  
Elaina J. Wang ◽  
Stephen T. Magill ◽  
Renata M. Knoll ◽  
...  

OBJECTIVE Ménière’s disease is an inner ear disorder classically characterized by fluctuating hearing loss, tinnitus, and aural fullness accompanied by episodic vertigo. While the pathogenesis of Ménière’s remains under debate, histopathological analyses implicate endolymphatic sac dysfunction with inner ear fluid homeostatic dysregulation. Little is known about whether external impingement of the endolymphatic sac by tumors may present with Ménière’s-like symptoms. The authors present a case series of 7 patients with posterior fossa meningiomas that involved the endolymphatic sac and new onset of Ménière’s-like symptoms and review the literature on this rare clinical entity. METHODS A retrospective review of patients undergoing resection of a posterior petrous meningioma was performed at the authors’ institution. Inclusion criteria were age older than 18 years; patients presenting with Ménière’s-like symptoms, including episodic vertigo, aural fullness, tinnitus, and/or hearing loss; and tumor location overlying the endolymphatic sac. RESULTS There were 7 cases of posterior petrous face meningiomas involving the vestibular aperture presenting with Ménière’s-like symptoms. Imaging and intraoperative examination confirmed no cranial nerve VIII compression or labyrinthine artery involvement accounting for audiovestibular symptoms. Of the 7 patients in the series, 6 experienced significant improvement or resolution of their vertigo, and all 7 had improvement or resolution of their tinnitus after resection. Of the 5 patients who had preoperative hearing loss, 2 experienced improvement or resolution of their ipsilateral preoperative hearing deficit, whereas the other 3 had unchanged hearing loss compared to preoperative evaluation. CONCLUSIONS Petrous face meningiomas overlying the endolymphatic sac can present with a Ménière’s syndrome. Early recognition and microsurgical excision of these tumors is critical for resolution of most symptoms and stabilization of hearing loss.


2012 ◽  
Vol 9 (3) ◽  
pp. 231-242 ◽  
Author(s):  
Teresa Rivera ◽  
Lorena Sanz ◽  
Guadalupe Camarero ◽  
Isabel Varela-Nieto

2021 ◽  
pp. 014556132199683
Author(s):  
Wenqi Liang ◽  
Line Wang ◽  
Xinyu Song ◽  
Fenqi Gao ◽  
Pan Liu ◽  
...  

The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyndromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient’s hearing was rehabilitated with bilateral cochlear implantation.


2021 ◽  
Vol 22 (12) ◽  
pp. 6497
Author(s):  
Anna Ghilardi ◽  
Alberto Diana ◽  
Renato Bacchetta ◽  
Nadia Santo ◽  
Miriam Ascagni ◽  
...  

The last decade has witnessed the identification of several families affected by hereditary non-syndromic hearing loss (NSHL) caused by mutations in the SMPX gene and the loss of function has been suggested as the underlying mechanism. In the attempt to confirm this hypothesis we generated an Smpx-deficient zebrafish model, pointing out its crucial role in proper inner ear development. Indeed, a marked decrease in the number of kinocilia together with structural alterations of the stereocilia and the kinocilium itself in the hair cells of the inner ear were observed. We also report the impairment of the mechanotransduction by the hair cells, making SMPX a potential key player in the construction of the machinery necessary for sound detection. This wealth of evidence provides the first possible explanation for hearing loss in SMPX-mutated patients. Additionally, we observed a clear muscular phenotype consisting of the defective organization and functioning of muscle fibers, strongly suggesting a potential role for the protein in the development of muscle fibers. This piece of evidence highlights the need for more in-depth analyses in search for possible correlations between SMPX mutations and muscular disorders in humans, thus potentially turning this non-syndromic hearing loss-associated gene into the genetic cause of dysfunctions characterized by more than one symptom, making SMPX a novel syndromic gene.


Molecules ◽  
2021 ◽  
Vol 26 (12) ◽  
pp. 3626
Author(s):  
Yi-Chun Lin ◽  
Yuan-Yung Lin ◽  
Hsin-Chien Chen ◽  
Chao-Yin Kuo ◽  
Ai-Ho Liao ◽  
...  

The application of insulin-like growth factor 1 (IGF-1) to the round window membrane (RWM) is an emerging treatment for inner ear diseases. RWM permeability is the key factor for efficient IGF-1 delivery. Ultrasound microbubbles (USMBs) can increase drug permeation through the RWM. In the present study, the enhancing effect of USMBs on the efficacy of IGF-1 application and the treatment effect of USMB-mediated IGF-1 delivery for noise-induced hearing loss (NIHL) were investigated. Forty-seven guinea pigs were assigned to three groups: the USM group, which received local application of recombinant human IGF-1 (rhIGF-1, 10 µg/µL) following application of USMBs to the RWM; the RWS group, which received IGF-1 application alone; and the saline-treated group. The perilymphatic concentration of rhIGF-1 in the USM group was 1.95- and 1.67- fold of that in the RWS group, 2 and 24 h after treatment, respectively. After 5 h of 118 dB SPL noise exposure, the USM group had the lowest threshold shift in auditory brainstem response, least loss of cochlear outer hair cells, and least reduction in the number of synaptic ribbons on postexposure day 28 among the three groups. The combination of USMB and IGF-1 led to a better therapeutic response to NIHL. Two hours after treatment, the USM group had significantly higher levels of Akt1 and Mapk3 gene expression than the other two groups. The most intense immunostaining for phosphor-AKT and phospho-ERK1/2 was detected in the cochlea in the USM group. These results suggested that USMB can be applied to enhance the efficacy of IGF-1 therapy in the treatment of inner ear diseases.


2020 ◽  
Vol 53 (2) ◽  
pp. 16424-16429
Author(s):  
Milka C.I. Madahana ◽  
Otis T.C. Nyandoro ◽  
John E.D. Ekoru

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