Congenital Ear Anomalies

Author(s):  
Sabrina Cugno ◽  
Neil W. Bulstrode
Keyword(s):  
2021 ◽  
pp. 014556132199683
Author(s):  
Wenqi Liang ◽  
Line Wang ◽  
Xinyu Song ◽  
Fenqi Gao ◽  
Pan Liu ◽  
...  

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.


1971 ◽  
Vol 4 (2) ◽  
pp. 291-318 ◽  
Author(s):  
Isamu Sando ◽  
Raymond P. Wood II
Keyword(s):  

1974 ◽  
Vol 84 (7) ◽  
pp. 1188-1201 ◽  
Author(s):  
Makoto Igarashi ◽  
Donald B. Singer ◽  
Bobby R. Alford ◽  
Ted A. Cook
Keyword(s):  

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Alaa Nasser Hussain Zaher ◽  
Tougan Taha Abd El Aziz ◽  
Ahmed Samy Abdelrahman

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).


Author(s):  
Shay I. Duvdevani ◽  
Hadas Knoller ◽  
Noa Rozendorn ◽  
Eran E. Alon ◽  
Ory Madgar

AbstractCongenital ear anomalies are associated with psychological morbidity. Ear deformities can usually be corrected by nonsurgical techniques such as splinting or molding in the neonatal period, initiated before 6 weeks. Without early corrections, many will require otoplasty during childhood. We introduce a novel silicone-based custom mold technique for congenital ear anomalies. The highly malleable silicone was pushed into every part of the auricle, enabling the auricle to remain in the desired shape, with new molds made weekly. Of 31 newborns (18 males, 13 females), 54 ears were treated. Average age at treatment initiation was 26.8 days. The mean treatment duration was 43.2 days, with a median of 28 days. Normal appearance and parent's satisfaction were achieved in 30 patients with 49 deformed ears and also in four newborns older than 6 weeks. Three concha type microtia in two patients achieved great improvements and parents' satisfaction. No complications were reported. Marked aesthetic improvements and normal appearance were achieved for all deformed auricles treated, and improvements in the concha type microtia will partially alleviate future surgical corrections. The results are not inferior to other techniques. The advantages are as follows: cost-effective, time-saving, simple to master, no need for hair shaving, and easy use for parents. Patients older than 6 weeks of age achieved normal auricle appearance, enabling the correction in older newborns. Custom made silicone auricle molding offers a simple nonsurgical technique for correcting congenital ear anomalies, alleviating the need for future surgical corrections.


1985 ◽  
Vol 40 (5) ◽  
pp. 302-303
Author(s):  
IRA T. LOTT ◽  
MAUREEN BOCIAN ◽  
HENRY W. PRIBRAM ◽  
MARC LEITNER

Sign in / Sign up

Export Citation Format

Share Document