scholarly journals Bilateral Internal Auditory Canal (IAC) Duplication with Congenital Sensorineural Hearing Loss (SNHL)-A Case Report

2021 ◽  
Vol 20 (4) ◽  
Author(s):  
Nik Adilah Nik Othman ◽  
Nur Saadah Mohamad ◽  
Ahmad Aizuddin Mohamad Jamali ◽  
Mohd Shafie Abdullah ◽  
Mohd Khairi Md Daud

Duplication of the internal auditory canal is a rare entity. It can be unilateral or bilateral involvement and usually found during an investigation of sensorineural hearing loss or facial nerve palsy. We report this image finding in a child with Down syndrome with bilateral profound sensorineural hearing loss. There was no facial nerve palsy. We highlight the rarity of this image finding and the importance of imaging for detection of middle and inner ear abnormality in a case of congenital sensorineural hearing loss as it helps in decision making for the suitable treatment. Keywords: Duplicate IAC; Bilateral Duplicate IAC; sensorineural hearing loss; Down syndrome

2003 ◽  
Vol 117 (10) ◽  
pp. 784-787 ◽  
Author(s):  
Seung Kuk Baek ◽  
Sung Won Chae ◽  
Hak Hyun Jung

Congenital internal auditory canal stenosis is a rare cause of sensorineural hearing loss in children. A retrospective analysis including clinical manifestation and radiological findings was made for seven patients who were diagnosed with congenital internal auditory canal stenosis from 1996 to 2002. Chief presenting symptoms were hearing loss, facial nerve palsy, dizziness, and tinnitus. Hearing loss including deafness was found in five cases, vestibular function loss in four cases, and profound functional loss of facial nerve in two cases. In all cases, the diameter of the internal auditory canal was less than 2 mm on high-resolution temporal bone computed tomography (CT) scan. Two cases revealed bilateral internal auditory canal stenosis, and others were unilaterally involved cases. Congenital internal auditory canal stenosis can be an important cause of sensorineural hearing loss, facial nerve palsy, and vestibular dysfunction. High resolution temporal bone CT scan and magnetic resonance (MR) imaging were important tools for diagnosis.


2014 ◽  
Vol 29 (1) ◽  
pp. 20-22
Author(s):  
Mee Ling Tang ◽  
Govindaraju Revadi ◽  
Raman Rajagopalan ◽  
Sushil Brito-Mutunayagam

Objective: To report a case of vertebrobasilar dolichoectasia presenting with ipsilateral facial nerve paresis and concomitant severe sensorineural hearing loss.   Methods:                Design: Case Report                Setting: Secondary Government Hospital                Patient: One   Results:  We report a case of vertebrobasilar dolichoectasia with concomitant ipsilateral facial nerve paresis and severe sensorineural hearing loss in an elderly female.  She presented to us with left facial nerve palsy House-Brackmann Grade III with prior history of ipsilateral sensorineural hearing loss.  MRI of the brain showed normal inner ear structures, but revealed a dilated and tortuous basilar artery with compression on the left medulla and possible branches of anterior inferior cerebellar artery as it coursed superiorly, and possible partial thrombosis of proximal basilar artery.    Conclusion:  Concommitant facial nerve paresis and sensorineural hearing loss can be the clinical presentations of this rare but important condition.  MRI is vital in diagnosing vertebrobasilar dolichoectasia.   Keywords: Vertebrobasilar dolichoectasia, facial nerve palsy, sensorineural hearing loss, basilar artery


2016 ◽  
Vol 131 (4) ◽  
pp. 298-302 ◽  
Author(s):  
C M Clark ◽  
H H Patel ◽  
S G Kanekar ◽  
H Isildak

AbstractBackground:Histopathological anomalies of inner-ear structures in individuals with Down syndrome have been well documented; however, few studies have examined the radiological features.Methods:A retrospective study was conducted of temporal bone computed tomography images in 38 individuals (75 ears) with Down syndrome to evaluate the prevalence of inner-ear abnormalities and assess vestibular aqueduct widths.Results:Inner-ear anomalies were identified in 20 of the 38 individuals (52.6 per cent). Seven of the 75 temporal bones (9.3 per cent) were found to have higher than previously reported. A dilated internal auditory canal and vestibule were more common among the present study group, while prior studies have demonstrated internal auditory canal stenosis and decreased vestibule size.Conclusion:Down syndrome patients exhibit a high prevalence of dysplastic inner-ear features that confer substantial risk of sensorineural hearing loss. Computed tomography is a useful screening aid to detect inner-ear abnormalities, particularly enlarged vestibular aqueducts, which cause preventable sensorineural hearing loss in this population.


2017 ◽  
Vol 4 (3) ◽  
pp. 1115
Author(s):  
Akanksha Jaiswal ◽  
Thumjaa Annamalai ◽  
Nirupama M ◽  
Shafath Ahmed M ◽  
Ramya V

We report a case of 10 months old child with a variant of Goldenhar syndrome in the form of microtia of right ear, microsomia of the right-side face, right facial nerve palsy with cardiac anomaly, renal anomaly and sensorineural hearing loss on the right side which were rare associations prompted to report this case.


Author(s):  
Helen Wong ◽  
Yaw Amoako-Tuffour ◽  
Khunsa Faiz ◽  
Jai Jai Shiva Shankar

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.


2018 ◽  
Vol 127 (9) ◽  
pp. 649-652 ◽  
Author(s):  
Thomas Muelleman ◽  
Hannah Kavookjian ◽  
James Lin ◽  
Hinrich Staecker

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.


Author(s):  
James Ramsden

Hearing loss must be divided into conductive hearing loss (CHL) and sensorineural hearing loss (SNHL). CHL is caused by sound not reaching the cochlear (abnormality of the ear canal, tympanic membrane, middle ear, or ossicles), whereas SNHL is a condition affecting the cochlear or auditory (eighth cranial) nerve. Hearing loss may be accompanied by other cardinal signs of ear disease, such as pain or discharge from the ear, vertigo, facial nerve palsy, and tinnitus, which guide the diagnosis. This chapter describes the approach to the patient with hearing loss.


1990 ◽  
Vol 93 (5) ◽  
pp. 716-722
Author(s):  
KAZUAKI OGAWA ◽  
SOIKU WATANABE ◽  
YASUO IWABUCHI ◽  
KANEAKI HARAGUCHI ◽  
JOHJI HIROTA ◽  
...  

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