Internal auditory canal enlargement (Giant IAC) and defective fundus in a child with congenital neurosensorial hearing loss

2007 ◽  
Vol 71 (6) ◽  
pp. 991
Keyword(s):  
Hearing Loss ◽  
Internal Auditory Canal ◽  
Canal Enlargement

scholarly journals Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

2021 ◽  
pp. 014556132199683
Author(s):  
Wenqi Liang ◽  
Line Wang ◽  
Xinyu Song ◽  
Fenqi Gao ◽  
Pan Liu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Next Generation Sequencing ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Transverse Diameter ◽  
Congenital Hearing Loss ◽  
Canal Stenosis ◽  
Ear Anomalies ◽  
Generation Sequencing

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.

Clinical Value of Measurement of Internal Auditory Canal in Pediatric Cochlear Implantation

2019 ◽  
Vol 128 (6_suppl) ◽  
pp. 61S-68S
Author(s):  
Hantai Kim ◽  
Dong Young Kim ◽  
Eun Ju Ha ◽  
Hun Yi Park
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Negative Impact ◽  
Internal Auditory Canal ◽  
Normal Hearing ◽  
Clinical Value ◽  
Cutoff Values ◽  
Auditory Performance ◽  
Bilateral Sensorineural Hearing Loss ◽  
Hearing Children

Objectives: The aims of this study were to clarify the clinical value of the bony cochlear nerve canal (BCNC) and internal auditory canal (IAC) in children with bilateral sensorineural hearing loss (b-SNHL) and to reveal the correlation between these parameters and outcomes after cochlear implantation (CI). Methods: Ninety-four ears with b-SNHL that received CI and 100 ears with normal hearing were enrolled. Parameters of IAC and pre- and post-CI categories of auditory performance scores were analyzed. Results: The width of the BCNC and the width, height, and length of the IAC were shorter in the b-SNHL group. BCNC and IAC width were associated with b-SNHL. The calculated cutoff values for BCNC and IAC width were 2.055 mm in the BCNC and 4.245 mm in the IAC, setting the sensitivity to 90%. Patients with narrow BCNCs and IACs had significantly worse post-CI auditory performance. Conclusions: BCNC and IAC widths were narrower in children with b-SNHL than in normal-hearing children. Narrow BCNC and IAC width had a negative impact on post-CI outcomes. The proposed cutoff values for BCNC and IAC width were meaningful when predicting the auditory outcome after CI, especially considering both.

scholarly journals Diagnostic Yield of MRI for Sensorineural Hearing Loss – An Audit

2020 ◽  
Vol 47 (5) ◽  
pp. 656-660
Author(s):  
Helen Wong ◽  
Yaw Amoako-Tuffour ◽  
Khunsa Faiz ◽  
Jai Jai Shiva Shankar
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Diagnostic Yield ◽  
Internal Auditory Canal ◽  
Auditory Brainstem ◽  
Sensorineural Hearing ◽  
Vestibular Evoked Myogenic Potentials ◽  
Clinical Indication ◽  
Presenting Symptoms ◽  
Brainstem Response

ABSTRACT:Purpose:Contrast-enhanced magnetic resonance imaging (CEMRI) of the head is frequently employed in investigations of sensorineural hearing loss (SNHL). The yield of these studies is perceptibly low and seemingly at odds with the aims of wise resource allocation and risk reduction within the Canadian healthcare system. The purpose of our study was to audit the use and diagnostic yield of CEMRI for the clinical indication of SNHL in our institution and to identify characteristics that may be leveraged to improve yield and optimize resource utilization.Materials and methods:The charts of 500 consecutive patients who underwent CEMRI of internal auditory canal for SNHL were categorized as cases with relevant positive findings on CEMRI and those without relevant findings. Demographics, presenting symptoms, interventions and responses, ordering physicians, and investigations performed prior to CEMRI testing were recorded. Chi-squared test and t-test were used to compare proportions and means, respectively.Results:CEMRI studies revealed relevant findings in 20 (6.2%) of 324 subjects meeting the inclusion criteria. Pre-CEMRI testing beyond audiometry was conducted in 35% of those with relevant positive findings compared to 7.3% of those without (p < 0.001). Auditory brainstem response/vestibular-evoked myogenic potentials were abnormal in 35% of those with relevant CEMRI findings compared to 6.3% of those without (p < 0.001).Conclusion:CEMRI is a valuable tool for assessing potential causes of SNHL, but small diagnostic yield at present needs justification for contrast injection for this indication. Our findings suggest preferred referral from otolaryngologists exclusively, and implementation of a non-contrast MRI for SNHL may be a better diagnostic tool.

The Size of Internal Auditory Canal Diverticula Is Unrelated to Degree of Hearing Loss

2020 ◽  
Vol 130 (4) ◽  
pp. 1011-1015 ◽  
Author(s):  
Thomas J. Muelleman ◽  
Kaley Pippin ◽  
Matthew Shew ◽  
Mark Villwock ◽  
James Lin ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Internal Auditory Canal

Acoustic Schwannoma Presenting as a Tumor of the External Auditory Canal

1987 ◽  
Vol 96 (4) ◽  
pp. 415-418 ◽  
Author(s):  
Patrice Tran Ba Huy ◽  
Jean Michel Hassan ◽  
Michel Wassef ◽  
Jacqueline Mikol ◽  
Claude Thurel
Keyword(s):  
Hearing Loss ◽  
External Auditory Canal ◽  
Cerebellopontine Angle ◽  
Internal Auditory Canal ◽  
Unilateral Hearing Loss ◽  
Acoustic Neurinoma ◽  
History Of ◽  
Acoustic Schwannoma ◽  
Initial Episode ◽  
Central Compensation

An acoustic neurinoma involving the internal auditory canal, the vestibule, the cochlea, the middle ear, and extending into the cerebellopontine angle and the external auditory canal, is described in a 56-year-old woman. An initial episode of vertigo was followed by a 27-year history of progressive unilateral hearing loss leading to complete deafness and areflexia with central compensation. The tumor was removed by a two-step surgical procedure, and the histologic features were those of a schwannoma.

Hypertrophic Pachymeningitis of the Internal Auditory Canal: A Rare Case of Unilateral Sudden Sensorineural Hearing Loss

2018 ◽  
Vol 127 (9) ◽  
pp. 649-652 ◽  
Author(s):  
Thomas Muelleman ◽  
Hannah Kavookjian ◽  
James Lin ◽  
Hinrich Staecker
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Internal Auditory Canal ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Hypertrophic Pachymeningitis ◽  
Eighth Cranial Nerve ◽  
Mri Scans ◽  
Magnetic Resonance Imaging Mri

Objectives: To describe and increase awareness of a rare cause of unilateral sudden sensorineural hearing loss. Methods: Case report and literature review. Results: We present a 66-year-old female who suffered left-sided sudden sensorineural hearing loss and dizziness. Diagnostic magnetic resonance imaging (MRI) did not reveal masses or lesions along the eighth cranial nerve or in the inner ear. Upon eventual referral to neurotology clinic, hypertrophic pachymeningitis of her left internal auditory canal and adjacent middle and posterior fossa dura were identified. The ensuing laboratory workup for autoimmune and infectious etiology revealed mild elevation of ACE 93 (9-67) but otherwise normal results. Conclusions: Idiopathic hypertrophic pachymeningitis is a diagnosis of exclusion. Neoplastic, infectious, and autoimmune causes must be ruled out. The prevailing treatment for this condition is high-dose corticosteroids. This entity should be considered when evaluating MRI scans obtained in the setting of sudden sensorineural hearing loss.

Cavernous angioma of the internal auditory canal

2004 ◽  
Vol 118 (5) ◽  
pp. 368-371 ◽  
Author(s):  
Kristian Aquilina ◽  
Jagdeep S. Nanra ◽  
Francesca Brett ◽  
Rory McConn Walsh ◽  
Daniel Rawluk
Keyword(s):  
Hearing Loss ◽  
Facial Nerve ◽  
Sensorineural Hearing Loss ◽  
Internal Auditory Canal ◽  
Cavernous Angioma ◽  
Dynamic Nature ◽  
First Report ◽  
Cavernous Angiomas ◽  
Nerve Paresis

Cavernous angiomas of the internal auditory canal are rare lesions. The authors present a case of a 29-year-old lady with multiple infratentorial cavernous angiomas, whose sister had previously undergone surgery for a similar supratentorial lesion. She initially presented with an acute brainstem haematoma, secondary to a pontine cavernous angioma. Three years later she developed progressive right-sided sensorineural hearing loss and facial nerve paresis due to an internal auditory canal lesion. This was removed via the translabyrinthine approach and was found to be a cavernous angioma. This report underlines the multiple and dynamic nature of familial cavernous angiomas, as well as the importance of follow up to determine whether new symptoms are due to the enlargement of known angiomas or the development of new ones. As far as the authors are aware, this is the first report describing a cavernous angioma of the internal auditory canal in the context of familial and multiple infratentorial angiomas.

Sudden deafness in vestibular schwannoma

1994 ◽  
Vol 108 (2) ◽  
pp. 116-119 ◽  
Author(s):  
David A. Moffat ◽  
David M. Baguley ◽  
Hubertus von Blumenthal ◽  
Richard M. Irving ◽  
David G. Hardy
Keyword(s):  
Hearing Loss ◽  
Vestibular Schwannoma ◽  
Tumour Size ◽  
Internal Auditory Canal ◽  
Sudden Deafness ◽  
Aetiological Factor ◽  
Caloric Test ◽  
Significant Difference ◽  
Facial Numbness ◽  
The Mean

AbstractTwelve per cent of a series of 284 patients with vestibular schwannoma presented with sudden deafness. If sudden sensorineural hearing loss is present then it is very likely to be the main presenting symptom. The mean length of patients' history is eight months shorter in this group than in the non-sudden deafness group. Sixteen per cent of vestibular schwannoma patients without sudden deafness present with a 'dead' ear whereas 29.5 per cent of those presenting with sudden deafness have total hearing loss. There was no significant difference between the sudden deafness group and the 'all others' group with regard to tumour size, udiogram shape, caloric test, imbalance, and facial numbness. Although the numbers of patients with sudden deafness in this series were too small to reach significance, on the basis of the clinical correlation of vestibular schwannoma morphology it is possible to postulate that compression of the vasculature within the bony internal auditory canal by a laterally arising tumour may be the aetiological factor and may be more likely to occur than in more medially arising tumours.

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