Enlarged vestibular aqueducts and other inner-ear abnormalities in patients with Down syndrome

2016 ◽  
Vol 131 (4) ◽  
pp. 298-302 ◽  
Author(s):  
C M Clark ◽  
H H Patel ◽  
S G Kanekar ◽  
H Isildak
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
Down Syndrome ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Internal Auditory Canal ◽  
Sensorineural Hearing ◽  
Vestibular Aqueduct ◽  
Computed Tomography Images ◽  
Temporal Bones

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.

Causes and Types of Sensorineural Hearing Loss. Clinical Manifestations and Basic Principles of Treatment

2020 ◽  
pp. 9-16
Author(s):  
Sergey Armakov
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Clinical Manifestations ◽  
Sensorineural Hearing ◽  
Doppler Imaging ◽  
Comprehensive Treatment ◽  
Impaired Hearing ◽  
Temporal Bones ◽  
The Brain

Sensorineural hearing loss is a disorder associated with the damage to the inner ear structures: the cochlea (cortical organ), dysfunctioning of the vestibule-cochlear nerve or the central part of the auditory analyser (brain stem and cortical representation of the cortical temporal lobe). In recent years, there has been a steady increase in ensorineural hearing loss patients; they account for ca. 70% among the total patients with impaired hearing. The disease has numerous causes and a complex pathogenesis. Among the main factors contributing to hearing loss are genetic predisposition, perinatal pathology, including hypoxia at childbirth, exposure to infectious and toxic agents and metabolic disorders, injuries (mechanical, acoustic and altitude trauma). Vascular-rheological disorders in the vertebro-basilar system play an important part because blood is supplied to the inner ear from the anterior inferior cerebellar artery. There are sudden, acute and chronic sensorineural hearing loss. The ensorineural hearing loss isdiagnosed by examinations that allow to verify the diagnosis and to determine the sound analyser damage level. This complex includes audiometric examinations, including the tuning fork examination, speech audiometry, and acoustic impedancemetry. If necessary, ultrasound Doppler imaging of the main blood vessels of the brain, computed tomography of the temporal bones, and MRI of the brain are prescribed. The pattern of comprehensive treatment should include, first of all, the elimination of the disease cause and anti-hypoxic drugs, anti-oxidants and a number of physiotherapy procedures.

Pontine Tegmental Cap Dysplasia

2011 ◽  
Vol 145 (6) ◽  
pp. 992-998 ◽  
Author(s):  
Nilesh K. Desai ◽  
Lindsay Young ◽  
Mario A. Miranda ◽  
Joe Walter Kutz ◽  
Peter S. Roland ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Cranial Nerves ◽  
Internal Auditory Canal ◽  
Sensorineural Hearing ◽  
Imaging Findings ◽  
Membranous Labyrinth ◽  
Brainstem Response ◽  
Temporal Bones

Objectives. Pontine tegmental cap dysplasia (PTCD) is a rare congenital malformation. Clinical and imaging findings in 3 patients and the authors’ experience with bilateral cochlear implantation in 1 patient are described. Study Design. Retrospective review. Setting. Two tertiary medical centers. Subjects and Methods. Three patients were evaluated by an otolaryngologist and underwent magnetic resonance imaging (MRI) of the temporal bones and brain. High-resolution computed tomography (CT) scanning of the temporal bones was performed in 2 patients. Imaging findings of the brain, the presence and course of resolvable cranial nerves, the membranous labyrinth, and internal auditory canals were reviewed. Clinical data were reviewed. Results. All patients demonstrated typical brain characteristics of PTCD. Mild, bilateral cochlear dysplasia was noted in 2, and all had a normal vestibular labyrinth. The cochleovestibular nerves were universally absent bilaterally. The facial nerves were subjectively deficient bilaterally in 1 patient, unilaterally in the second patient, and normal in the third. An accessory canal for the seventh cranial nerve, referred to as a duplicated internal auditory canal, was present in all patients. Auditory brainstem response testing revealed profound bilateral sensorineural hearing loss in all of the patients; none suffered facial weakness. A single patient underwent bilateral cochlear implantation with only minimal response. Conclusion. The authors report 3 cases of PTCD with emphasis on imaging of the seventh and eighth cranial nerves and clinical neurotologic findings. All patients manifested duplicated internal auditory canals, a previously unreported finding in PTCD. Bilateral profound sensorineural hearing loss is due to absence of the cochleovestibular nerve. Prognosis for cochlear implantation is poor.

scholarly journals PATHOLOGICAL CHANGES IN THE INNER EAR IN EXPERIMENTAL MODELING OF SENSORINEURAL HEARING LOSS IN ANIMALS

2018 ◽  
Vol 25 (3) ◽  
pp. 82-87 ◽  
Author(s):  
V. G. OVSYANNIKOV ◽  
T. V. ZOLOTOVA ◽  
E. V. LOBZINA ◽  
N. V. DUBINSKAYA
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Broadband Noise ◽  
Sensorineural Hearing ◽  
Laboratory Animals ◽  
Pathological Changes ◽  
Noise And Vibration ◽  
Noise Vibration ◽  
Temporal Bones

Aim. Study of pathological changes in the inner ear in the modeling of sensorineural hearing loss in laboratory animals.Materials and methods. A pilot study involving 27 laboratory animals − white outbred rats – was conducted. Modeling of sensorineural hearing loss was conducted by exposing them for 10 days to broadband noise of 90 dB, vibration and simultaneous immobilization of laboratory animals divided into 3 groups according to exposure conditions. The development of hearing loss was confirmed by the results of the registration of delayed caused emissions and emissions at the frequency of distortion products. After removing rats from the experiment histological medication from animals cochlear was produced and they were studied by the method of light microscopy.Results. In animals exposed to noise and vibration effects on the background of immobilization, the formation of persistent sensorineural hearing loss was achieved, which was confirmed by objective registration of violations of auditory function, and also by the results of morphological study of cochlear of the temporal bones of animals. More pronounced histological changes were noted in animals exposed to noise and vibration on the background of immobilization compared with the group of rats which were exposed only to noise impact and immobilization without applying vibration. Dystrophic and destructive changes in the structures of the spiral organ, signs of apoptotic way of cell death in the inner ear were detected. In addition, pronounced changes occurred in the spiral ganglia.Conclusion. Modeling of sensorineural hearing loss in laboratory animals on the basis of noise and vibration exposure in terms of immobilization leads to the formation of persistent sensorineural hearing loss, as evidenced by functional and morphological methods. Pathological changes in the inner ear show themselves through dystrophic and destructive changes in the spiral organ, including apoptosis of cells, and especially in the spiral ganglia. The use of this noise-vibration model of hearing loss can be a promising basis for future studies of drugs for the treatment of sensorineural hearing loss.

Computed Tomography in the Diagnosis of Cochlear Otosclerosis

1986 ◽  
Vol 94 (4) ◽  
pp. 434-438 ◽  
Author(s):  
Brian W. Blakley ◽  
Peter A. Hilger ◽  
Saul Taylor ◽  
Jerome Hilger
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
High Resolution ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
High Resolution Computed Tomography ◽  
Temporal Bones

Twelve patients with surgically proven otosclerosis and sensorineural hearing loss were studied with high-resolution computed tomography (CT). The purpose was to Identify abnormal bony changes that could be diagnostic of cochlear otosclerosis. Of the 24 temporal bones thus studied, 10 showed positive findings. The findings in our study—as well as those of others—show a pericochlear zone of radiolucency. The precise origin of this is undetermined.

Sensorineural Hearing Loss after Otitis Media with Effusion and Subacute Mastoiditis after Viral Infections of the Upper Respiratory Tract: A Comparative Study of Conservative and Surgical Treatment

2016 ◽  
Vol 95 (9) ◽  
pp. E18-E27 ◽  
Author(s):  
Thomas Wilhelm ◽  
Tim Stelzer ◽  
Rudolf Hagen
Keyword(s):  
Hearing Loss ◽  
Surgical Treatment ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Otitis Media ◽  
Viral Infections ◽  
Otitis Media With Effusion ◽  
Sensorineural Hearing ◽  
Upper Respiratory Tract ◽  
Temporal Bones

Involvement of the middle ear after viral infections of the upper airways may lead to serous otitis media with effusion in the absence of bacterial infection. This can be accompanied by a concomitant shading of the mastoid air cells, which could manifest as a reduced opacity on computed tomography (CT) in the absence of a history of chronic mastoiditis or acute inflammatory signs. This can lead to a subsequent impairment of inner ear function. CT scans reveal an extended pneumatization of the temporal bones in affected patients. Inner ear hearing impairment can probably be attributed to a concomitant labyrinthine reaction—the so-called toxic inner ear lesion. If no remission occurs within 5 days after initial conservative treatment (paracentesis or hemorrheologic infusions), surgical treatment with a mastoidectomy can accelerate hearing restoration. We conducted a retrospective, nonrandomized study of short- and long-term hearing outcomes in patients with a toxic inner ear lesion who had been treated with conservative measures alone (CONS group) or with surgery (SURG group) in a tertiary care referral center. Our study group was made up of 52 consecutively presenting patients (57 ears) who had been seen over a 10-year period; there were 20 patients (21 ears) in the CONS group and 32 patients (36 ears) in the SURG group. Initially, 15 CONS patients (75%) and 18 SURG patients (56%) complained of dizziness or a balance disorder. The initial averaged sensorineural hearing loss (over 0.5, 1.0, 2.0, and 3.0 kHz) was 32.4 ± 15.6 dB in the CONS group and 35.4 ± 12.0 dB in the SURG group. At follow-up (mean: 31.7 mo), the SURG group experienced a significantly greater improvement in hearing (p = 0.025). We conclude that patients with viral otitis media and concomitant noninflammatory mastoiditis with impairment of inner ear function (sensorineural hearing loss) experience a better hearing outcome when a mastoidectomy is performed during primary treatment.

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 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
Keyword(s):  
Hearing Loss ◽  
Down Syndrome ◽  
Facial Nerve ◽  
Sensorineural Hearing Loss ◽  
Nerve Palsy ◽  
Facial Nerve Palsy ◽  
Internal Auditory Canal ◽  
Sensorineural Hearing ◽  
Image Finding ◽  
Suitable Treatment

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

Drug Delivery to the Inner Ear: Strategies and Their Therapeutic Implications for Sensorineural Hearing Loss

2012 ◽  
Vol 9 (3) ◽  
pp. 231-242 ◽  
Author(s):  
Teresa Rivera ◽  
Lorena Sanz ◽  
Guadalupe Camarero ◽  
Isabel Varela-Nieto
Keyword(s):  
Drug Delivery ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Therapeutic Implications
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