Hearing loss associated with large internal auditory meatus: a report of five paediatric cases

1999 ◽  
Vol 113 (11) ◽  
pp. 1015-1019 ◽  
Author(s):  
Catherine S. Birman ◽  
William P. R. Gibson
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
Ct Scans ◽  
Otic Capsule ◽  
Internal Auditory Meatus ◽  
Progressive Hearing Loss ◽  
Profound Hearing Loss ◽  
Vestibular Aqueduct ◽  
Large Vestibular Aqueduct

AbstractThis paper describes the abnormality of a large internal auditory meatus (LIAM). Computed tomography (CT) scans show the otic capsule to be affected by a widened, bulbar internal auditory meatus with loss of or reduction of the bony wall dividing the lateral fundus of the meatus from the cochlea. The vestibule is abnormally dilated. We report five cases of children with LIAM and profound hearing loss. Three of these children are girls and two children were boys. Three had congenital progressive hearing loss, one of these had an accompanying large vestibular aqueduct and dysplasia of the cochlea. Two patients had had meningitis resulting in profound loss.

scholarly journals Two siblings with progressive, fluctuating hearing loss after head trauma, treated with cochlear implantation

2009 ◽  
Vol 124 (1) ◽  
pp. 86-89 ◽  
Author(s):  
M J F de Wolf ◽  
J Honings ◽  
F B M Joosten ◽  
L Hoefsloot ◽  
E A M Mylanus ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
Case Report ◽  
Cochlear Implant ◽  
Head Trauma ◽  
Cochlear Implantation ◽  
Progressive Hearing Loss ◽  
Enlarged Vestibular Aqueduct ◽  
Vestibular Aqueduct ◽  
Fluctuating Hearing Loss

AbstractObjective:Description of two siblings with unexplained, progressive, perceptive hearing loss after head trauma.Design:Case report.Subjects:Two siblings aged six and eight years old with bilateral, intermittent but progressive hearing loss.Results:These patients had a c.1172G>A (p.Ser391Asn) mutation in the SLC26A4 gene, which has not previously been reported and which caused Pendred or enlarged vestibular aqueduct syndrome. The diagnosis was based on the perceptive hearing loss, computed tomography findings and mutation analysis. The patients were each fitted with a cochlear implant because of their severe, progressive, perceptive hearing loss with deep fluctuations. The results were good.Conclusion:Further testing for the presence of an enlarged vestibular aqueduct is recommended when children present with sudden progression in perceptive hearing loss, whether or not in combination with head trauma. Cochlear implantation is indicated in patients with persistent, progressive hearing loss that leads to deafness. Implantation can be undertaken successfully despite cochlear hypoplasia.

Role of Computed Tomography and Magnetic Resonance Imaging in detecting the Prevalence of inner ear anomalies among cochlear implant candidates

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Alaa Nasser Hussain Zaher ◽  
Tougan Taha Abd El Aziz ◽  
Ahmed Samy Abdelrahman
Keyword(s):  
Magnetic Resonance Imaging ◽  
Computed Tomography ◽  
Hearing Loss ◽  
Cochlear Implant ◽  
Inner Ear ◽  
Resonance Imaging ◽  
High Resolution Computed Tomography ◽  
Vestibular Aqueduct ◽  
Incomplete Partition ◽  
Ear Anomalies

Abstract Background Hearing loss management using cochlear implants in patients with inner ear anomalies has long been discussed in the otology community. Magnetic resonances imaging (B,/IRI) and Computed tomography (CT) play important roles in the preoperative assessment of inner ear abnormalities such as cochlear nerve deficiency and variant anatomy as these abnormalities may not only affect the decision of the implantation procedure or the patient's prognosis regarding auditory improvement, but also the risk of complications. Objective To examine the prevalence of inner ear anomalies among cochlear implant recipients in patients with congenital sensorineural hearing loss among the pediatric age group in the Demerdash hospital, Ain Shams university using High resolution computed tomography (HRCT) and MRI imaging. Methods A retrospective descriptive study over the course of 9 months that included all patients that are candidates for cochlear implant referred to the Radiology department, Ain Shams University Hospitals for a preoperative imaging in the form of CT and VIRI scans. Results CT and MRI scans of 33 patients who had congenital hearing loss and were candidates for cochlear implantation with total 66 ears were reviewed. Inner ear anomalies were identified in 8 patients representing a prevalence (24.2%) with 14 ear diseased. Anomalies were seen bilaterally in 6 patients and unilaterally in 2 patients. Among the 14 diseased ear, 9 ears (64.3%) were seen with incomplete partition Il, 7 ears (50%) were seen with enlarged vestibular aqueduct, 4 ears (28.6%) were seen with cochlear hypoplasia, 3 ears (21.4%) were seen with semicircular canal aplasia, 2 ears (14.3%) were seen with incomplete partition type I, 2 ears (14.3%) were seen with cochlear nerve aplasia, 2 ears with cochlear aplasia (14.3%), I ear (7.1%) was seen with common cavity ear (7.1%) with complete labyrinthine aplasia. Conclusion Prevalence of inner ear anomalies among cochlear implant candidates was 24.2%. This result is consistent with results worldwide and the most common anomalies were Incomplete partition Il and large vestibular aqueduct. Abbreviations Computed tomography (CT), Magnetic resonance imaging (MRI), High resolution computed tomography (HRCT), Internal auditory canal (IAC), Cerebellopontine angle (CPA).

scholarly journals Tomographic diagnosis and relevant aspects of otosclerosis

2013 ◽  
Vol 46 (5) ◽  
pp. 307-312 ◽  
Author(s):  
Juliana Oggioni Gaiotti ◽  
Natália Delage Gomes ◽  
Ana Maria Doffémond Costa ◽  
Caroline Laurita Batista Couto Villela ◽  
Wanderval Moreira ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
Differential Diagnosis ◽  
Multidetector Computed Tomography ◽  
Surgical Planning ◽  
Otic Capsule ◽  
Metabolic Derangement ◽  
Pictorial Essay ◽  
Temporal Bone Anatomy ◽  
Endochondral Layer

A literature review and pictorial essay were developed to discuss the importance of knowing the main findings and locations of otosclerosis at multidetector computed tomography (MDCT). The authors performed a retrospective review of cases of otosclerosis diagnosed in their institution by means of high resolution multidetector computed tomography. Otosclerosis corresponds to otic capsule dysplasia characterized by metabolic derangement of its endochondral layer. Such condition constitutes a relevant cause of sensorineural hearing loss, affecting about 7% to 10% of the general population. The diagnosis is usually clinical, but imaging methods play a significant role in the anatomical detailing, differential diagnosis, surgical planning and evaluation of postoperative complications. Among such methods, the relevance of MDCT is highlighted. Radiologists should be familiar with the MDCT findings of otosclerosis, as well as with the temporal bone anatomy to assist in the appropriate clinical management of this disease.

The large vestibular aqueduct: A new definition based on audiologic and computed tomography correlation

2007 ◽  
Vol 136 (6) ◽  
pp. 972-977 ◽  
Author(s):  
Mark Boston ◽  
Mark Halsted ◽  
Jareen Meinzen-Derr ◽  
Judy Bean ◽  
Shyan Vijayasekaran ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Vestibular Aqueduct ◽  
Large Vestibular Aqueduct

Bilateral Evoked Otoacoustic Emissions in a Child with Bilateral Profound Hearing Loss

1996 ◽  
Vol 105 (4) ◽  
pp. 286-288 ◽  
Author(s):  
Laurence Laccourreye ◽  
Esther Tran BA Huy ◽  
Martine François ◽  
Philippe Narcy
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
Brain Stem ◽  
Inner Ear ◽  
Otoacoustic Emissions ◽  
Review Of The Literature ◽  
Profound Hearing Loss ◽  
Normal Limits

Recording of bilateral evoked otoacoustic emissions in a 3-year-old girl with bilateral profound hearing loss is presented. No component of auditory-evoked brain stem potentials was recorded. Findings on computed tomography of the inner ear were within normal limits. At retest 3 months later, these results were unchanged. Explanations for this particular condition are presented together with a review of the literature.

scholarly journals The Sustained-Exposure Dexamethasone Formulation OTO-104 Offers Effective Protection against Cisplatin-Induced Hearing Loss

2016 ◽  
Vol 21 (1) ◽  
pp. 22-29 ◽  
Author(s):  
Rayne Fernandez ◽  
Anne Harrop-Jones ◽  
Xiaobo Wang ◽  
Luis Dellamary ◽  
Carl LeBel ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Nuclear Receptor ◽  
Chronic Administration ◽  
Weekly Interval ◽  
Acute Administration ◽  
Effective Protection ◽  
Progressive Hearing Loss ◽  
Experimental Conditions ◽  
Profound Hearing Loss ◽  
Intratympanic Injection

The otoprotective effects of OTO-104 were investigated following both acute and chronic administration of cisplatin. The acute administration of cisplatin to guinea pigs resulted in profound hearing loss (70-80 dB SPL) across all frequencies tested. A single intratympanic injection of 6% OTO-104, but not of lower doses, almost completely protected against cisplatin ototoxicity. In contrast, a dexamethasone solution administered under the same experimental conditions offered no otoprotection. OTO-104 was also very effective in protecting against the progressive hearing loss observed with the chronic administration of cisplatin (3 injections at a weekly interval). The otoprotection was found to be dependent upon the activation of dexamethasone-dependent classical nuclear receptor pathways.

scholarly journals Unilateral Enlarged Vestibular Aqueduct Syndrome and Bilateral Endolymphatic Hydrops

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Massimo Ralli ◽  
Giuseppe Nola ◽  
Luca Sparvoli ◽  
Giovanni Ralli
Keyword(s):  
Hearing Loss ◽  
Intracranial Pressure ◽  
Endolymphatic Hydrops ◽  
Correct Diagnosis ◽  
Ct Scans ◽  
Vestibular Disorders ◽  
Hearing Improvement ◽  
Enlarged Vestibular Aqueduct ◽  
Vestibular Aqueduct ◽  
Inner Ear Malformation

Enlarged vestibular aqueduct (EVA) syndrome is a common congenital inner ear malformation characterized by a vestibular aqueduct with a diameter larger than 1.5 mm, mixed or sensorineural hearing loss that ranges from mild to profound, and vestibular disorders that may be present with a range from mild imbalance to episodic objective vertigo. In our study, we present the case of a patient with unilateral enlarged vestibular aqueduct and bilateral endolymphatic hydrops (EH). EH was confirmed through anamnestic history and audiological exams; EVA was diagnosed using high-resolution CT scans and MRI images. Therapy included intratympanic infusion of corticosteroids with a significant hearing improvement, more evident in the ear contralateral to EVA. Although most probably unrelated, EVA and EH may present with similar symptoms and therefore the diagnostic workup should always include the proper steps to perform a correct diagnosis. Association between progression of hearing loss and head trauma in patients with a diagnosis of EVA syndrome is still uncertain; however, these individuals should be advised to avoid activities that increase intracranial pressure to prevent further hearing deterioration. Intratympanic treatment with steroids is a safe and well-tolerated procedure that has demonstrated its efficacy in hearing, tinnitus, and vertigo control in EH.

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