Abrupt Onset of Sensorineural Hearing Loss and Tinnitus in a Patient with Capillary Telangiectasia of the Pons

Capillary telangiectasia is often found incidentally on magnetic resonance imaging and may be associated with minor neurologic symptoms, but there has been little evidence about whether such lesions are responsible for these symptoms. Here we report a case of capillary telangiectasia of the right mid pons, which was associated with the abrupt onset of tinnitus and sensorineural hearing loss. Examination of the auditory brain stem responses showed abnormalities of waves III and IV on the left and bilateral prolongation of the interwave intervals. Optokinetic nystagmus showed slight left-right asymmetry.

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Significance of Auditory Brain Stem Response and Gadolinium-Enhanced Magnetic Resonance Imaging for Idiopathic Sudden Sensorineural Hearing Loss

Otolaryngology ◽

10.1016/s0194-5998(95)70117-6 ◽

1995 ◽

Vol 113 (3) ◽

pp. 271-275 ◽

Cited By ~ 8

Author(s):

Nicolas Y. Busaba ◽

Steven D. Rauch

Keyword(s):

Magnetic Resonance Imaging ◽

Hearing Loss ◽

Magnetic Resonance ◽

Sensorineural Hearing Loss ◽

Brain Stem ◽

Sudden Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Resonance Imaging ◽

Auditory Brain Stem Responses ◽

Auditory Brain Stem

Previous studies tried to correlate prognosis and response to oral corticosteroids in patients with idiopathic sudden sensorineural hearing loss to such factors as the age of the patient, presence of vertigo, shape of the audiogram, or severity of the hearing loss. However, temporal bone histopathologic evidence shows that idiopathic sudden sensorineural hearing loss may be caused by cochleitis or cochlear nerve neuritis. Herein we report results of a retrospective study of 96 consecutive patients with idiopathic sudden sensorineural hearing loss who were evaluated with auditory brain stem responses and gadolinium-enhanced magnetic resonance imaging. Results of the auditory brain stem response and magnetic resonance imaging were correlated with hearing outcome. Follow-up was available for 65 patients: 14 with abnormal and 51 with normal auditory brain stem responses. The overall rate of hearing recovery or improvement was 65% in the normal auditory brain stem response group compared with 43% in the abnormal auditory brain stem response group ( p = 0.07). Among the 38 patients treated with a tapering course of oral corticosteroids, the recovery or improvement rate was 83% for those with normal auditory brain stem responses and 56% for those with abnormal auditory brain stem responses ( p < 0.05). Of the 27 patients who did not receive steroid therapy, the improvement rate was 41% in those with normal auditory brain stem responses and 20% in those with abnormal auditory brain stem responses ( p = 0.09). Magnetic resonance imaging with gadolinium was obtained on all 14 patients with abnormal auditory brain stem responses but on none with normal auditory brain stem responses. Only 1 magnetic resonance image of 14 demonstrated an abnormality, showing a high signal intensity in the distal internal auditory canal; this resolved 6 weeks later on a follow-up magnetic resonance image. We conclude that idiopathic sudden sensorineural hearing loss patients with abnormal auditory brain stem responses have poorer hearing prognoses compared with those patients with normal auditory brain stem responses, irrespective of treatment. Idiopathic sudden sensorineural hearing loss patients with abnormal auditory brain stem responses may have cochlear neuritis causing their hearing loss or may have a more extensive involvement of their auditory system, and this “lesion” may have a lower spontaneous recovery rate and less response to therapy. Magnetic resonance imaging with gadolinium may show abnormal signal intensities along the course of the eighth nerve in patients with idiopathic sudden sensorineural hearing loss, but this is infrequent, and its prognostic implications are not clear.

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Patient with an SLC26A4 gene mutation who had low-frequency sensorineural hearing loss and endolymphatic hydrops

The Journal of Laryngology & Otology ◽

10.1017/s0022215114003399 ◽

2015 ◽

Vol 129 (1) ◽

pp. 95-97 ◽

Cited By ~ 3

Author(s):

T Yoshida ◽

M Sone ◽

S Naganawa ◽

T Nakashima

Keyword(s):

Magnetic Resonance Imaging ◽

Hearing Loss ◽

Magnetic Resonance ◽

Sensorineural Hearing Loss ◽

Gene Mutation ◽

Endolymphatic Hydrops ◽

Low Frequency ◽

Sensorineural Hearing ◽

Resonance Imaging ◽

The Right

AbstractObjective:To report magnetic resonance imaging findings in a patient with an SLC26A4 gene mutation who had low-frequency sensorineural hearing loss.Case report:A 13-year-old girl had bilateral and symmetric low-frequency sensorineural hearing loss. Upon genetic testing, a heterozygous c.1105A > G (p.K369E) mutation of the SLC26A4 gene was detected. Mild endolymphatic hydrops in the right cochlea and marked endolymphatic hydrops in the left vestibulum were seen by magnetic resonance imaging 4 hours after an intravenous gadolinium injection.Conclusion:This is the first reported case of a patient with the SLC26A4 gene mutation c.1105A > G (p.K369E) who had low-frequency sensorineural hearing loss. Co-occurrence of cochlear and vestibular endolymphatic hydrops suggests an association with that pathology.

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Intralabyrinthine Schwannomas: Disease Presentation, Tumor Management, and Hearing Rehabilitation

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0039-1678731 ◽

2019 ◽

Vol 80 (02) ◽

pp. 196-202 ◽

Cited By ~ 8

Author(s):

Baishakhi Choudhury ◽

Matthew Carlson ◽

Daniel Jethanamest

Keyword(s):

Magnetic Resonance Imaging ◽

Hearing Loss ◽

Magnetic Resonance ◽

Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Resonance Imaging ◽

Hearing Rehabilitation ◽

Rare Tumors ◽

Auditory Rehabilitation ◽

Tumor Management

AbstractIntralabyrinthine schwannomas (ILS) are rare tumors that frequently cause sensorineural hearing loss. The development and increased use of magnetic resonance imaging in recent years have facilitated the diagnosis of these tumors that present with otherwise nondiscriminant symptoms such as tinnitus, vertigo, and hearing loss. The following is a review of the presentation, pathophysiology, imaging, and treatment with a focused discussion on auditory rehabilitation options of ILS.

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Cochlear nerve anomalies in paediatric single-sided deafness – prevalence and implications for cochlear implantation strategies

The Journal of Laryngology & Otology ◽

10.1017/s002221512000225x ◽

2020 ◽

pp. 1-4

Author(s):

K Pollaers ◽

A Thompson ◽

J Kuthubutheen

Keyword(s):

Magnetic Resonance Imaging ◽

Hearing Loss ◽

Magnetic Resonance ◽

Sensorineural Hearing Loss ◽

Cochlear Nerve ◽

Sensorineural Hearing ◽

Resonance Imaging ◽

Bilateral Sensorineural Hearing Loss ◽

Single Sided Deafness ◽

Cochlear Nerve Hypoplasia

Abstract Objective To determine the prevalence of cochlear nerve anomalies on magnetic resonance imaging in patients with unilateral or bilateral sensorineural hearing loss. Methods A retrospective case series was conducted at a tertiary referral centre. The inclusion criteria were paediatric patients with bilateral or unilateral sensorineural hearing loss, investigated with magnetic resonance imaging. The primary outcome measure was the rate of cochlear nerve hypoplasia or aplasia. Results Of the 72 patients with unilateral sensorineural hearing loss, 39 per cent (28 cases) had absent or hypoplastic cochlear nerves on the affected side. Fifteen per cent (11 cases) had other abnormal findings on magnetic resonance imaging. Eighty-four patients had bilateral sensorineural hearing loss, of which cochlear nerve hypoplasia or aplasia was identified only in 5 per cent (four cases). Other abnormal findings were identified in 14 per cent (12 cases). Conclusion Paediatric patients with unilateral sensorineural hearing loss are more likely to have cochlear nerve anomalies than those patients with bilateral sensorineural hearing loss. This has important implications regarding cochlear implantation for patients with single-sided deafness.

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Inner ear and retrocochlear pathology on magnetic resonance imaging for sudden and progressive asymmetrical sensorineural hearing loss

ANZ Journal of Surgery ◽

10.1111/ans.14957 ◽

2018 ◽

Vol 89 (6) ◽

pp. 738-742

Author(s):

Samantha M. Baird ◽

Kevin Nguyen ◽

Daman D. S. Bhatia ◽

Benjamin P. C. Wei

Keyword(s):

Magnetic Resonance Imaging ◽

Hearing Loss ◽

Magnetic Resonance ◽

Sensorineural Hearing Loss ◽

Inner Ear ◽

Sensorineural Hearing ◽

Resonance Imaging ◽

Retrocochlear Pathology

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Neuroanatomic Differences in Children With Unilateral Sensorineural Hearing Loss Detected Using Functional Magnetic Resonance Imaging

Archives of Otolaryngology - Head and Neck Surgery ◽

10.1001/archoto.2009.208 ◽

2010 ◽

Vol 136 (1) ◽

pp. 22 ◽

Cited By ~ 33

Author(s):

Evan J. Propst ◽

John H. Greinwald ◽

Vincent Schmithorst

Keyword(s):

Magnetic Resonance Imaging ◽

Hearing Loss ◽

Magnetic Resonance ◽

Functional Magnetic Resonance Imaging ◽

Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Functional Magnetic Resonance ◽

Resonance Imaging

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Assess the hearing of contralateral ear in patients with unilateral chronic squamosal otitis media

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20201680 ◽

2020 ◽

Vol 6 (5) ◽

pp. 886

Author(s):

Raphella Khan ◽

Anirudh Kasliwal

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Otitis Media ◽

Conductive Hearing Loss ◽

Sensorineural Hearing ◽

Retraction Pocket ◽

Mixed Hearing Loss ◽

Contralateral Ear ◽

The Right ◽

Conductive Hearing

Background: Chronic squamosal otitis media can occur due to many conditions affecting the middle ear. Most common sign of developing a chronic squamosal otitis media is formation of a retraction pocket in the tympanic membrane leading to further development of a cholesteatoma and if not treated properly, may lead to development of dangerous complication in the affected ear. These etiological factors may also affect the other ear. It is therefore very necessary to assess and diagnose the contralateral ear, so that the disease can be intervened and treated at the right time, to prevent any deterioration in hearing of the contralateral ear.Methods: The prospective study was done in 100 patients with unilateral chronic squamosal otitis media, where the contra lateral ear was examined and assessed for any hearing loss. Results: We found hearing loss in the contra lateral ear ranging from mild conductive hearing loss to sensorineural hearing loss with the maximum patients with mild conductive hearing loss (42%) and lowest in sensorineural hearing loss (1%). Conclusions: In our study, 76 patients were seen with conductive hearing loss. Out of that, 42% patients were seen with mild conductive hearing loss, 30% with moderate conductive hearing loss and 4% with severe conductive hearing loss. 20% patients were seen with normal hearing. 3% patients were seen with mixed hearing loss and only 1% patient was seen with sensorineural hearing loss in contralateral ear.

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Prevalence and classification of asymmetric forms of sensorineural hearing loss in older age group

Russian otorhinolaryngology ◽

10.18692/1810-4800-2021-5-8-12 ◽

2021 ◽

Vol 20 (5) ◽

pp. 8-12

Author(s):

T. Yu. Vladimirova ◽

◽

A. B. Martynova ◽

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Hearing Aids ◽

Hearing Threshold ◽

Older Age ◽

Sensorineural Hearing ◽

Age Group ◽

The Difference ◽

The Right

The significance of asymmetric sensorineural hearing loss (ASNHL) is due to a special approach to diagnosis, followed by the process of hearing aids and auditory rehabilitation. Currently, there is no standard audiometric criterion for determining the forms of asymmetry, which significantly affects the assessment of the prevalence of ASNHL. The study aimed to assess the prevalence and classification of ASNHL forms in the older age group using two methods of calculation: 1) the difference in the average hearing threshold at speech frequencies (in the range of 0,5–4 kHz) ≥15 dB was detected in 14,14% of cases; 2) the different degree of hearing loss, according to the International classification, in the right and left ear was 35,98%. In most cases, asymmetry was manifested by bilateral sensorineural hearing loss of varying severity, prevailing in the group of long-livers – 82,6%. Given the potentially high prevalence of asymmetry depending on the audiological criterion, the results of the work are a reason for further research in the development of a unified method for verifying a clinically significant form of ASNHL.

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