Inner-ear malformations as a cause of single-sided deafness

2020 ◽  
Vol 134 (6) ◽  
pp. 509-518
Author(s):  
E Tahir ◽  
M D Bajin ◽  
S Jafarov ◽  
M Ö Yıldırım ◽  
B Ç Çınar ◽  
...  
Keyword(s):  
Inner Ear ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Resonance Imaging ◽  
Radiological Findings ◽  
Inner Ear Malformations ◽  
Disease Prognosis ◽  
Inner Ear Malformation ◽  
Ear Malformations ◽  
Single Sided Deafness

AbstractObjectiveTo determine the prevalence and distribution of inner-ear malformations in congenital single-sided deafness cases, as details of malformation type are crucial for disease prognosis and management.MethodsA retrospective study was conducted of 90 patients aged under 16 years with congenital single-sided deafness. Radiological findings were evaluated using computed tomography and magnetic resonance imaging. Inner-ear malformations were identified and cochlear nerve status was determined in affected ears.ResultsOut of 90 ears, 42 (46.7 per cent) were found to have inner-ear malformation. Isolated cochlear aperture stenosis was the most common anomaly (n = 18, 20 per cent), followed by isolated cochlear aperture atresia (n = 11, 12.2 per cent) and cochlear hypoplasia (n = 7, 7.8 per cent). Cochlear nerve deficiency was encountered in 41 ears (45.6 per cent). The internal auditory canal was also stenotic in 49 ears (54.4 per cent).ConclusionInner-ear malformations, especially cochlear aperture anomalies, are involved in the aetiology of single-sided deafness more than expected. The cause of single-sided deafness differs greatly between congenital and adult-onset cases. All children with single-sided deafness should undergo radiological evaluation, as the prognosis and management, as well as the aetiology, may be significantly influenced by inner-ear malformation type.

scholarly journals Appropriate Imaging Modality for the Etiologic Diagnosis of Congenital Single-Sided Deafness in Children

2018 ◽  
Vol 7 (12) ◽  
pp. 515 ◽  
Author(s):  
Sang-Yeon Lee ◽  
Shin Kim ◽  
Yun Bae ◽  
Eun Kim ◽  
Ja-Won Koo ◽  
...  
Keyword(s):  
Diagnostic Yield ◽  
Imaging Modality ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Original Cohort ◽  
Ear Malformations ◽  
Mutual Comparison ◽  
Novel Concept ◽  
Single Sided Deafness ◽  
Etiologic Diagnosis

We aimed to compare the diagnostic yield between temporal bone computed tomography (TBCT) and internal auditory canal MRI (IAC MRI) for the etiologic diagnosis of children with congenital single-sided deafness (SSD) and the evaluation of cochlear implant (CI) candidacy. In the original cohort, 24 subjects with congenital SSD were enrolled and underwent both TBCT and IAC MRI. We recruited an additional 22 consecutive infants with congenital SSD (the supplementary cohort) and evaluated in particular the cochlear nerve (CN) integrity using IAC MRI. Cochlear nerve deficiency (CND) was classified as ‘absent’, ‘small’, and ‘indeterminate’ via mutual comparison between optical and parameters based on the MRI results. The most common etiologies were CND in the original cohort (19 out of 24). Notably, accurate evaluations of CN status (‘small CN’ = 2, ‘indeterminate CN’ = 2), inner ear malformations, and brain abnormalities were possible only with MRI. The ‘indeterminate CN’ tended to be more frequently detected in SSD ears than in unaffected ears. MRI appeared to be more accurate than TBCT in a meticulous differentiation of CN, which is crucial for the selection of appropriate CI candidacy among congenital SSD children. Additionally, we introduced the novel concept of ‘indeterminate CN’, of which the causal relationship with SSD awaits confirmation.

scholarly journals Congenital Inner Ear Malformations as a Cause of Single Sided Deafness

2016 ◽  
Vol 130 (S3) ◽  
pp. S33-S33
Author(s):  
Emel Tahir ◽  
Münir Demir Bajin ◽  
Mehtap Öztürk ◽  
Levent Sennaroğlu
Keyword(s):  
Inner Ear ◽  
Inner Ear Malformations ◽  
Ear Malformations ◽  
Single Sided Deafness

Surface-Coil Magnetic Resonance Imaging of the Internal Auditory Canal and the Inner Ear Preliminary Report

1995 ◽  
Vol 104 (10) ◽  
pp. 776-782 ◽  
Author(s):  
Iwao Honjo ◽  
Eri Naito ◽  
Yasushi Natto ◽  
Haruo Takahashi ◽  
Kazumasa Nishimura ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Surface Coil ◽  
Cochlear Nerve ◽  
Acquisition Time ◽  
Computer Assisted ◽  
Resonance Imaging ◽  
Eighth Nerve

Parasagittal surface-coil magnetic resonance imaging of the internal auditory canal and the inner ear was performed. We used T2-weighted fast spin-echo sequences to visualize the inner ear and the individual nerves in the internal auditory canal with high contrast in a short acquisition time. Computer-assisted quantitative measurement of the nerves was performed to estimate the cross-sectional areas and the diameters of the nerves. The average diameters of the facial nerve, the cochlear nerve, and the vestibular nerve of normal-hearing individuals were, respectively, 1.1 ± 0.2 mm (mean ± SD), 1.2 ± 0.2 mm, and 1.5 ± 0.2 mm. In the cerebellopontine angle, the average diameter of the eighth nerve was 1.8 ± 0.2 mm. Two patients with unilateral and bilateral hearing loss were also presented. In the patient with unilateral deafness, the cochlear nerve of the diseased side was not identified and the eighth cranial nerve diameter was smaller than that of the normal side. In the patient with bilateral deafness, fibrosis of the inner ear and atrophy of the eighth nerve were demonstrated in the ear with posttraumatic deafness. The present method may represent a new approach to the assessment of pathologic processes involving the inner ear and the nerves in the internal auditory canal.

scholarly journals Anatomical and Pathological Findings of Magnetic Resonance Imaging in Idiopathic Sudden Sensorineural Hearing Loss

2020 ◽  
Vol 24 (4) ◽  
pp. 198-203
Author(s):  
Min Bum Kim ◽  
Jihyun Lim ◽  
Il Joon Moon
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Sudden Sensorineural Hearing Loss ◽  
Cochlear Nerve ◽  
Sensorineural Hearing ◽  
Resonance Imaging

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.

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.

scholarly journals De novo variants in GREB1L are associated with non-syndromic inner ear malformations and deafness

2018 ◽  
Vol 137 (6-7) ◽  
pp. 459-470 ◽  
Author(s):  
Isabelle Schrauwen ◽  
Elina Kari ◽  
Jacob Mattox ◽  
Lorida Llaci ◽  
Joanna Smeeton ◽  
...  
Keyword(s):  
Inner Ear ◽  
De Novo ◽  
Inner Ear Malformations ◽  
Ear Malformations

A new cochlear implant electrode with a “cork”-type stopper for inner ear malformations

2014 ◽  
Vol 41 (4) ◽  
pp. 331-336 ◽  
Author(s):  
Levent Sennaroğlu ◽  
Gamze Atay ◽  
Münir Demir Bajin
Keyword(s):  
Cochlear Implant ◽  
Inner Ear ◽  
Inner Ear Malformations ◽  
Ear Malformations

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

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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