A case report of first hearing loss, then painful menarche: a young girl with Herlyn–Werner–Wunderlich syndrome (OHVIRA syndrome) and concomitant inner ear anomalies

2021 ◽  
pp. 1-3
Author(s):  
Ayşe Ö. Balık ◽  
Buket Yağcı ◽  
Murat Özoğul ◽  
Fisun Vural
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Inner Ear ◽  
Young Girl ◽  
Ohvira Syndrome ◽  
Ear Anomalies

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.

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).

Inner Ear Anomalies and Conductive Hearing Loss in Children With Apert Syndrome

2009 ◽  
Vol 30 (2) ◽  
pp. 184-189 ◽  
Author(s):  
Guangwei Zhou ◽  
Lynn Thomas Schwartz ◽  
Quinton Gopen
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Conductive Hearing Loss ◽  
Apert Syndrome ◽  
Ear Anomalies ◽  
Conductive Hearing

scholarly journals A Case of Perilymphatic Fistula with Inner Ear Anomaly Diagnosed Preoperatively by the Cochlin-Tomoprotein Detection Test

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Kana Lee ◽  
Naoki Ochi ◽  
Kohei Yamahara ◽  
Kunihiko Makino ◽  
Tetsuo Ikezono
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Clinical Course ◽  
Sudden Hearing Loss ◽  
Sensorineural Hearing ◽  
Fistula Repair ◽  
Perilymphatic Fistula ◽  
Ear Anomalies ◽  
Inner Ear Anomaly

We present a case of perilymphatic fistula (PLF) with inner ear anomalies having sudden, progressive sensorineural hearing loss and describe the fistula repair surgeries. We focus on the diagnosis methods of PLF and clinical course of PLF with inner ear anomaly. The cochlin-tomoprotein (CTP) detection test is very useful for the surgeons to encourage the earlier operation to sudden hearing loss cases. It is also helpful to define the diagnosis of PLF after operation. We could not get the good result as to hearing from the fistula repair surgery mainly because surgery was held 1 month after the onset. The results of the case, as well as recommendations of other reports, suggest that patients with sudden sensorineural hearing loss and PLF may need repair surgery within at most 2 weeks from the onset. We describe how to diagnose PLF more accurately using CTP detection combined with intraoperative findings.

Pathology of Congenital Deafness

1980 ◽  
Vol 89 (5_suppl) ◽  
pp. 31-42 ◽  
Author(s):  
LaVonne Bergstrom
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Genetic Disorders ◽  
Illustrative Case ◽  
Case Histories ◽  
Neonatal Life ◽  
Significant Incidence ◽  
Cytomegalic Inclusion Disease ◽  
Genetic Hearing Loss ◽  
Ear Anomalies

The pathology of congenital hearing loss is as varied as the etiologies. Severe external ear anomalies, such as microtia, and middle ear defects have a significant incidence of accompanying inner ear aplasias, more often of the vestibular than of the cochlear end-organs. Classic inner ear aplasias are discussed, including Michel, Mondini, Alexander and Scheibe. A case illustrating close resemblance between a genetically induced Scheibe malformation and rubella inner ear deformities is presented. A variety of ear disorders acquired during intrauterine or neonatal life, including cytomegalic inclusion disease, hypoxia, erythroblastosis fetalis and later-onset genetic disorders are also discussed. Illustrative case histories are presented as well as priorities for future investigations into the pathology of congenital and genetic hearing loss.

Girl with accelerated growth, hearing loss, inner ear anomalies, delayed myelination of the brain, and del(22)(q13.1q13.2)

2000 ◽  
Vol 92 (3) ◽  
pp. 195-199 ◽  
Author(s):  
Yasuko Fujita ◽  
Daishi Mochizuki ◽  
Yosuke Mori ◽  
Natsue Nakamoto ◽  
Masaaki Kobayashi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Accelerated Growth ◽  
Ear Anomalies ◽  
The Brain ◽  
Delayed Myelination

scholarly journals Audiological and Vestibular Functions in Patients With Lateral Semicircular Canal Dysplasia and Aplasia

2020 ◽  
Vol 13 (3) ◽  
pp. 255-260 ◽  
Author(s):  
Sang Hyun Kwak ◽  
Min Ki Kim ◽  
Sung Huhn Kim ◽  
Jinsei Jung
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Semicircular Canal ◽  
Pure Tone ◽  
Lateral Semicircular Canal ◽  
Vestibular Loss ◽  
Horizontal Canal ◽  
Canal Paresis ◽  
Bilateral Hearing Loss ◽  
Ear Anomalies

Objectives. The aim of the present study was to evaluate audiologic and vestibular functions in patients with lateral semicircular canal (LSCC) dysplasia/aplasia.Methods. We conducted a retrospective study of a patients with LSCC dysplasia and aplasia at tertiary referral center. The subjects included 15 patients with LSCC dysplasia or aplasia, with or without combined inner ear anomalies. Medical history, temporal bone computed tomography scans, pure-tone audiograms, and vestibular function test results were analyzed.Results. LSCC anomaly was identified in 15 patients (20 ears). Nine patients had unilateral LSCC dysplasia only and showed a mean pure-tone average of 45.5±28.7 dB, while three patients (33.3%) among them had normal hearing. Six patients had bilateral LSCC dysplasia/aplasia combined with other inner ear anomalies and profound bilateral hearing loss. Notably, only four out of 15 patients (26.7%) had dizziness symptoms. On caloric test, patients with isolated LSCC dysplasia showed a 51.8%±29.3% level of canal paresis (eight out of nine patients showed anomalies), whereas patients with bilateral LSCC dysplasia/aplasia presented bilateral vestibular loss. One patient with isolated LSCC underwent video-head impulse test; horizontal canal gain decreased to 0.62 (17% asymmetry) and anterior canal gain was 0.45 (52.6% asymmetry), whereas posterior canal gain was normal.Conclusion. Bilateral LSCC dysplasia/aplasia is comorbid with other inner ear anomalies and presents as profound bilateral hearing loss and vestibulopathy. In contrast, isolated unilateral LSCC dysplasia presents as ipsilateral horizontal canal paresis. Hearing function in isolated LSCC dysplasia is usually, but not always, impaired with varying severity.

Ayurvedic Management of Otosclerosis - A Case report

2019 ◽  
Vol 7 (02) ◽  
Author(s):  
GANGAPRASAD ANANTRAO WAGHMARE ◽  
PRASHANT SURU
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Inner Ear ◽  
Key Words ◽  
Bone Tissue ◽  
Bone Remodeling ◽  
Female Patient ◽  
Middle Ear ◽  
Clinical Findings ◽  
Outpatient Department

Otosclerosis is a term derived from oto, meaning“of the ear,”and sclerosis, meaning“abnormal hardening of body tissue.”The condition is caused by abnormal bone remodeling in the middle ear. Bone remodeling is a lifelong process in which bone tissue renews itself by replacing old tissue with new. In otosclerosis, abnormal remodeling disrupts the ability of sound to travel from the middle ear to the inner ear. A Hindu,married 50 yr old Female patient visited the outpatient department of Institute with complaint of Diminished of hearing in both ear (Left ear > Right ear), Ringing sensation in both ear & Blocking of both ear. The patient was diagnosed as case of Otosclerosis with clinical findings & Investigations done. Tablet Lakshadi guggulu 2TDS(each250mg), Tablet Amarsundari Vati2TDS(each250mg),Powder combination of Aamalaki churna (1gm)+ AshwagandhaChurna(1gm)+PunarnavaChurna(500mg)+JatamansiChurna(500mg)+Sunhi churna(250mg)+ Godanti churna (250mg) And Syp Dashmooladi Kwath10ml TDS were administered for 6 months. After the 6 months treatment ,a significant response in various symptoms such as Diminished of hearing, Ringing sensation & Blocking of ear was found.   Key words- Otosclerosis, Hearing loss, Diminished of hearing,Audiometry, tinnitus, Badhirya  

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