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

Author(s):  
K Pollaers ◽  
A Thompson ◽  
J Kuthubutheen

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.

2009 ◽  
Vol 123 (11) ◽  
pp. 1266-1270 ◽  
Author(s):  
A Muthu ◽  
S Stevenson ◽  
P Bird

AbstractObjective:Magnetic resonance imaging is a routine investigation in cases of asymmetric sensorineural hearing loss, but it is not routinely used to investigate bilateral sensorineural hearing loss.Method:This case report illustrates the benefits of magnetic resonance image scanning in the latter patient group.Results:A 53-year-old man with rapidly progressive, symmetrical, bilateral, sensorineural hearing loss was found also to have anosmia, imbalance and incoordination. Magnetic resonance image scanning demonstrated leptomeningeal haemosiderosis. Progressive, bilateral, sensorineural hearing loss is the most common presentation of this condition and magnetic resonance imaging is the diagnostic investigation of choice.Conclusion:There are potential treatments for leptomeningeal haemosiderosis which prevent further irreversible damage, if a bleeding source can be found. Hearing loss may be due to cochlear or retrocochlear pathology. Cochlear implantation may be indicated.


2002 ◽  
Vol 116 (10) ◽  
pp. 804-810 ◽  
Author(s):  
Levent Sennaroglu ◽  
Isil Saatci ◽  
Ayse Aralasmak ◽  
Bulent Gursel ◽  
Ergin Turan

Recent reports indicate that the cochlear nerve may be absent in some cases of congenital sensorineural hearing loss. The aim of this prospective study was to determine the incidence of cochlear nerve anomaly in cochlear implant candidates with congenital hearing loss using magnetic resonance imaging (MRI). Twenty-seven patients with congenital profound bilateral sensorineural hearing loss who were being evaluated for the cochlear implant procedure were studied.These patients had high-resolution computerized tomography (CT), through the petrous bone in axial sections. MRI examinations consisted of T1 and turbo spin echo (TSE) T2-weighted 3 mm axial images, and additional 3D Fourier Transform T2-weighted TSE sequences obtained on three different planes (axial, perpendicular and parallel to the internal auditory canal (IAC) i.e. oblique sagittal and coronal, respectively) for the purpose of cochlear nerve demonstration. Results showed that all of the 14 patients with normal CT of the temporal bone, had four distinct nerves in the distal part of the IAC on TSE-MRI. Thirteen patients demonstrated various bony malformations of the cochleovestibular system on CT. MRI revealed the absence of the cochleovestibular nerve in four patients where the IAC was very narrow or completely absent on CT. One patient with severe Mondini malformation who had an enlarged IAC demonstrated an isolated absent cochlear nerve.


2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Abolfazl Mollasadeghi ◽  
Amir Houshang Mehrparvar ◽  
Saeid Atighechi ◽  
Mohammad Hossein Davari ◽  
Pedram Shokouh ◽  
...  

Magnetic resonance imaging (MRI) devices produce noise, which may affect patient’s or operators’ hearing. Some cases of hearing impairment after MRI procedure have been reported with different patterns (temporary or permanent, unilateral or bilateral, with or without other symptoms like tinnitus). In this report, a case of bilateral sensorineural hearing loss in an otherwise healthy patient underwent brain MRI was described. The patient’s hearing loss was accompanied with tinnitus and was not improved after 3 months of followup.


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 80 (02) ◽  
pp. 196-202 ◽  
Author(s):  
Baishakhi Choudhury ◽  
Matthew Carlson ◽  
Daniel Jethanamest

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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