scholarly journals CT Imaging Categorization and Biomarker Study of Anomalous Tympanic Segment of the Facial Nerves in Patients With Hearing Loss in the Absence of Microtia

2019 ◽  
Vol 98 (6) ◽  
pp. 340-345
Author(s):  
Nisa Oren ◽  
Daniel Thomas Ginat
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Temporal Bone ◽  
Charge Syndrome ◽  
Oval Window ◽  
High Resolution Computed Tomography ◽  
Facial Nerves ◽  
Ear Anomalies ◽  
Temporal Bones ◽  
Congenital Syndrome

The purpose of this study is to categorize anomalous tympanic facial nerve (FN) on high-resolution computed tomography (HRCT) and to determinate the significance of associated temporal bone anomalies and congenital syndromes without microtia in patients with hearing loss. A retrospective analysis of HRCT findings in 30 temporal bones in 18 patients with anomalous FN was performed. Abnormalities of the tympanic FN were categorized as follows: category 1: FN medially positioned, but above the oval window; category 2: FN in the oval window niche; and category 3: FN below the oval window. Potential associated findings that were assessed included stapes abnormalities, oval window atresia, and inner ear anomalies, as well as the presence of a known congenital syndrome with hearing loss. The most common type of anomalous tympanic FN was category 1 (67%, n = 20), following by group 2 (20%, n = 6) and group 3 (13%, n = 4). Stapes anomalies were detected in 77% of temporal bones (n = 23), oval window atresia was detected in 43% of temporal bones (n = 13), and inner ear anomalies were detected in 70% of temporal bones (n = 21). Anomalous tympanic facial nerves in temporal bone with conductive hearing loss were often (60%) not associated with oval window atresia. The combination of aberrant tympanic FN and inner ear anomalies was significantly ( P = .038) associated with a known congenital syndrome (6 patients), including CHARGE syndrome, oculo-auriculo-vertebral spectrum, Pierre-Robin sequences, and Down syndrome. Therefore, an anomalous tympanic FN in conjunction with inner ear anomalies appears to be a biomarker for certain congenital syndromes with hearing loss in the absence of microtia.

Auditory and Temporal Bone Abnormalities in Charge Association

1986 ◽  
Vol 95 (5) ◽  
pp. 480-486 ◽  
Author(s):  
Charles G. Wright ◽  
William L. Meyerhoff ◽  
O. E. Brown ◽  
J. C. Rutledge
Keyword(s):  
Hearing Loss ◽  
Facial Nerve ◽  
Temporal Bone ◽  
Choanal Atresia ◽  
Charge Syndrome ◽  
Oval Window ◽  
Congenital Defects ◽  
Auditory Brain Stem Response ◽  
Charge Association ◽  
Temporal Bones

CHARGE association is a recently described cluster of congenital defects including ocular coloboma, heart disease, choanal atresia, retarded development and/or CNS abnormalities, genital hypoplasia, and ear anomalies. Although congenital hearing loss has been reported in CHARGE association, no information regarding the underlying temporal bone disease is available in the literature to date. The authors evaluated four patients with multiple anomalies consistent with CHARGE syndrome. Two surviving patients have bilateral severe hearing loss on auditory brain stem response testing. Two patients did not survive, and their temporal bones were obtained at autopsy for histologic examination. All four temporal bones showed severe middle ear defects including ossicular deformities, absence of the stapedius muscle, absence of the oval window, aberrant course of the facial nerve, and dehiscence of the facial nerve canal. In the more severely affected case, a Mondini-type malformation of the cochlea was present, together with multiple anomalies of the vestibular apparatus. Vestibular defects also occurred in the other case; however, the cochleae were found to be normally developed.

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).

Effect of Aspiration of Perilymph during Stapes Surgery on the Endocochlear Potential of Guinea Pig

2011 ◽  
Vol 145 (5) ◽  
pp. 801-805 ◽  
Author(s):  
Ryoukichi Ikeda ◽  
Kazuhiro Nakaya ◽  
Hidetoshi Oshima ◽  
Takeshi Oshima ◽  
Tetsuaki Kawase ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Guinea Pig ◽  
Inner Ear ◽  
Oval Window ◽  
Stapes Surgery ◽  
Partial Recovery ◽  
Cochlear Function ◽  
Stapes Footplate ◽  
Guinea Pig Model ◽  
Temporal Bones

Objective. Suction applied to the vestibule through the oval window during stapes surgery is considered a primary risk for postoperative sensorineural hearing impairment. This study investigated the mechanism of acute phase change in cochlear function caused by aspiration of the opened oval window. Study Design. Guinea pig model. Setting. Academic hospital laboratory. Subjects and Methods. Guinea pigs were divided into 3 groups: stapes footplate removed without suctioning (6 animals), with indirect suctioning (5 animals), and with direct suctioning of the vestibular perilymph (6 animals). Endocochlear potentials (EPs) were measured at the second turn of the cochlea, and temporal bones were examined histologically. Results. Removal of the stapes footplate without suctioning caused little change in the EP (original value, 80.12 ± 3.52 mV), indirect suctioning caused minor decline of the EP of 9.14 ± 1.84 mV, and partial recovery ensued, whereas direct but gentle suctioning, resulting in dry vestibule, caused reduction in the EP of 16.38 ± 6.63 mV. Recovery was not observed or incomplete. No animals showed profound decrease in the EP. Conclusion. Gentle suctioning and removal of the vestibular perilymph can cause a mild decrease in the EP even without damaging the inner ear structures. Therefore, suctioning of the perilymph should be avoided during stapes surgery because acute hearing loss can result even without damaging the inner ear structures. However, hearing loss may not be profound, if suctioning is not vigorous enough to cause damage to the inner ear structures.

Temporal Bone Histopathology in Charge Association

2002 ◽  
Vol 111 (5) ◽  
pp. 397-401 ◽  
Author(s):  
Shin-Ichi Haginomori ◽  
Makoto Miura ◽  
Isamu Sando ◽  
Margaretha L. Casselbrant
Keyword(s):  
Inner Ear ◽  
Temporal Bone ◽  
Middle Ear ◽  
Congenital Heart ◽  
The Other ◽  
Otologic Surgery ◽  
Charge Association ◽  
Ear Anomalies ◽  
Temporal Bones ◽  
Central Nervous System Anomalies

Three temporal bones obtained at autopsy from 2 patients with CHARGE association (Coloboma, congenital Heart disease, Atresia of choanae, mental Retardation and/or central nervous system anomalies, Genital hypoplasia, and Ear anomalies) were examined histopathologically. Both temporal bones from 1 patient showed multiple anomalies in the middle ear, inner ear, and facial nerve. However, the temporal bone obtained from the other patient showed almost normal structures in the inner ear, middle ear, and eustachian tube. These results are different from those of 2 previous reports of temporal bone histopathology regarding CHARGE association. This difference suggests that CHARGE association may arise not from one etiopathogenetic factor, but from complex factors. Special attention to dehiscent facial canal and perilymphatic gusher during otologic surgery in patients with CHARGE association is discussed.

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.

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.

scholarly journals The Effect of Pulling Out Cochlear Implant Electrodes on Inner Ear Microstructures: A Temporal Bone Study

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Ingo Todt ◽  
Rainer O. Seidl ◽  
Arne Ernst
Keyword(s):  
Cochlear Implant ◽  
Inner Ear ◽  
Temporal Bone ◽  
Basilar Membrane ◽  
Temporal Sequence ◽  
Pull Out ◽  
Temporal Bones

The exchange of an cochlear implant or the re-positioning of an electrode have become more frequently required than a decade ago. The consequences of such procedures at a microstructural level within the cochlea are not known. It was the aim of the present study to further investigate the effects of an CI electrode pull-out. Therefore 10 freshly harvested temporal bones (TB) were histologically evaluated after a cochlear implant electrode pull-out of a perimodiolar electrode. In additional 9 TB the intrascalar movements of the CI electrode while being pulled-out were digitally analysed by video- capturing. Histologically, a disruption of the modiolar wall or the spiral osseous lamina were not observed. In one TB, a basilar membrane lifting up was found, but it could not be undoubtedly attributed to the pull-out of the electrode. When analyzing the temporal sequence of the electrode movement during the pull-out, the electrode turned in one case so that the tip elevates the basilar membrane. The pull- out of perimodiolarly placed CI electrodes does not damage the modiolar wall at a microstructural level and should be guided (e.g., forceps) to prevent a 90 o turning of the electrode tip into the direction of the basilar membrane.

scholarly journals Radiographic Measurement of Cochlear in Sudanese Population using High Resolution Computed Tomography (HRCT)

2021 ◽  
Vol 29 (2) ◽  
Author(s):  
Lubna Bushara ◽  
Mohamed Yousef ◽  
Ikhlas Abdelaziz ◽  
Mogahid Zidan ◽  
Dalia Bilal ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
High Resolution ◽  
Temporal Bone ◽  
Healthy Subjects ◽  
Cochlear Nerve ◽  
High Resolution Computed Tomography ◽  
Mean Width ◽  
The Mean ◽  
The Right

This study aimed to determine the measurements of the cochlea among healthy subjects and hearing deafness subjects using a High Resolution Computed Tomography (HRCT). A total of 230 temporal bone HRCT cases were retrospectively investigated in the period spanning from 2011 to 2015. Three 64-slice units were used to examine patients with clinical complaints of hearing loss conditions at three Radiology departments in Khartoum, Sudan. For the control group (A) healthy subjects, the mean width of the right and left cochlear were 5.61±0.40 mm and 5.56±0.58 mm, the height were 3.56±0.36 mm and 3.54±0.36 mm, the basal turn width were 1.87±0.19 mm and 1.88 ±0.18 mm, the width of the cochlear nerve canal were 2.02±1.23 and 1.93±0.20, cochlear nerve density was 279.41±159.02 and 306.84±336.9 HU respectively. However, for the experimental group (B), the mean width of the right and left cochlear width were 5.38±0.46 mm and 5.34±0.30 mm, the height were 3.53±0.25 mm and 3.49±0.28mm, the basal turn width were 1.76±0.13 mm, and 1.79±0.13 mm, the width of the cochlear nerve canal were 1.75±0.18mm and 1.73±0.18mm, and cochlear nerve density were 232.84±316.82 and 196.58±230.05 HU, respectively. The study found there was a significant difference in cochlea’s measurement between the two groups with a p-value < 0.05. This study had established baseline measurements for the cochlear for the healthy Sudanese population. Furthermore, it found that HRCT of the temporal bone was the best for investigation of the cochlear and could provide a guide for the clinicians to manage congenital hearing loss.

scholarly journals Bilateral temporal bone fractures: a case report

2017 ◽  
Vol 4 (1) ◽  
pp. 271
Author(s):  
Kiran Natarajan ◽  
Koka Madhav ◽  
A. V. Saraswathi ◽  
Mohan Kameswaran
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Facial Nerve ◽  
Temporal Bone ◽  
Facial Paralysis ◽  
Bone Fractures ◽  
Clinical Manifestations ◽  
Csf Leak ◽  
The Right ◽  
Temporal Bones

<p>Bilateral temporal bone fractures are rare; accounting for 9% to 20% of cases of temporal bone fractures. Clinical manifestations include hearing loss, facial paralysis, CSF otorhinorrhea and dizziness. This is a case report of a patient who presented with bilateral temporal bone fractures. This is a report of a 23-yr-old male who sustained bilateral temporal bone fractures and presented 18 days later with complaints of watery discharge from left ear and nose, bilateral profound hearing loss and facial weakness on the right side. Pure tone audiometry revealed bilateral profound sensori-neural hearing loss. CT temporal bones &amp; MRI scans of brain were done to assess the extent of injuries. The patient underwent left CSF otorrhea repair, as the CSF leak was active and not responding to conservative management. One week later, the patient underwent right facial nerve decompression. The patient could not afford a cochlear implant (CI) in the right ear at the same sitting, however, implantation was advised as soon as possible because of the risk of cochlear ossification. The transcochlear approach was used to seal the CSF leak from the oval and round windows on the left side. The facial nerve was decompressed on the right side. The House-Brackmann grade improved from Grade V to grade III at last follow-up. Patients with bilateral temporal bone fractures require prompt assessment and management to decrease the risk of complications such as meningitis, permanent facial paralysis or hearing loss. </p>

scholarly journals Facial nerve anomalies in paediatric cochlear implant candidates: radiological evaluation

2016 ◽  
Vol 131 (1) ◽  
pp. 26-31 ◽  
Author(s):  
F B Palabiyik ◽  
K Hacikurt ◽  
Z Yazici
Keyword(s):  
Magnetic Resonance Imaging ◽  
Computed Tomography ◽  
Magnetic Resonance ◽  
High Resolution ◽  
Facial Nerve ◽  
Cochlear Implant ◽  
Inner Ear ◽  
Resonance Imaging ◽  
High Resolution Computed Tomography ◽  
Ear Anomalies

AbstractBackground:Pre-operative radiological identification of facial nerve anomalies can help prevent intra-operative facial nerve injury during cochlear implantation. This study aimed to evaluate the incidence and configuration of facial nerve anomalies and their concurrence with inner-ear anomalies in cochlear implant candidates.Methods:Inner-ear and concomitant facial nerve anomalies were evaluated by magnetic resonance imaging and temporal high-resolution computed tomography in 48 children with congenital sensorineural hearing loss who were cochlear implant candidates.Results:Inner-ear anomalies were present in 11 out of 48 patients (23 per cent) and concomitant facial nerve anomalies were present on 7 sides in 4 patients (7 per cent of the total). Facial nerve anomalies were accompanied by cochlear or vestibular malformation.Conclusion:Potential facial nerve abnormalities should always be considered in patients with inner-ear anomalies. Pre-operative facial nerve imaging can increase the surgeon's confidence to plan and perform cochlear implantation. Magnetic resonance imaging should be used to detect inner-ear anomalies; if these are identified, temporal high-resolution computed tomography should be used to evaluate the facial nerve.

Sign in / Sign up

Export Citation Format

Share Document