External Auditory Canal, Bilateral Atresia Of, With Congenital Vertical Talus

Last update 01 Nov 2024

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Synonyms

EXTERNAL AUDITORY CANAL, BILATERAL ATRESIA OF, WITH CONGENITAL VERTICAL TALUS, External Auditory Canal, Bilateral Atresia Of, With Congenital Vertical Talus, RASMUSSEN SYNDROME

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100 Clinical Results associated with External Auditory Canal, Bilateral Atresia Of, With Congenital Vertical Talus

100 Translational Medicine associated with External Auditory Canal, Bilateral Atresia Of, With Congenital Vertical Talus

0 Patents (Medical) associated with External Auditory Canal, Bilateral Atresia Of, With Congenital Vertical Talus

Literatures (Medical) associated with External Auditory Canal, Bilateral Atresia Of, With Congenital Vertical Talus

01 Jan 2022·Journal of Veterinary Medical ScienceQ4 · AGRICULTURAL & FORESTRY SCIENCE

Congenital malformations of the external and middle ear accompanied by temporal bone anomaly in a calf

Q4 · AGRICULTURAL & FORESTRY SCIENCE

Article

Author: OISHI, Motoharu ; KAMIIE, Junichi ; SATO, Reiichiro ; MOMOKI, Anna ; MURAKAMI, Hironobu ; HATTORI, Nanase ; YAMADA, Kazutaka ; AIHARA, Naoyuki ; NAGAI, Makoto ; GOTO, Yoshiaki

A 7-month-old female Holstein calf presented with bilateral microtia and absent external acoustic meatus. The real-time polymerase chain reaction test was negative for bovine viral diarrhea virus and bovine leukemia virus. The calf's dam had a normal reproductive history. Computed tomography confirmed bilateral atresia of external auditory canals, aplasia of tympanic cavities and the ossicular chain, and temporomandibular joint abnormality. Necropsy revealed a severe malformation of the temporal bone. In the tympanic region, the external acoustic pore, tympanic bulla, and muscular process were absent bilaterally. The bilateral inner ear structure was normal. Based on these findings, we diagnosed the present case as congenital malformations of the external and middle ear accompanied by temporal bone anomaly.

01 Sep 2021·Acta Otorrinolaringologica (English Edition)

Hearing rehabilitation with osseointegrated hearing implant in bilateral congenital external auditory canal atresia

Article

Author: Ferraz, Sara Moreira ; Coutinho, Miguel Bebiano ; Casanova, Maria Jorge ; Almeida E Sousa, Cecília ; Magalhães, António

INTRODUCTION AND OBJECTIVESCongenital atresia of the external auditory canal (EAC) is a congenital defect present in one in every 10,000-20,000 births. It causes conductive hearing loss, with an air-bone gap of 50-60dB. Early amplification is essential in bilateral cases to ensure normal language development. The aim of this study is to present the osseointegrated hearing implant as a treatment for bilateral EAC atresia, reviewing the audiometric results and the rate of complications.MATERIAL AND METHODSRetrospective analysis of patients diagnosed with bilateral congenital EAC atresia under follow-up in the pediatric ENT clinic of the ENT and Head and Neck Surgery department of a Portuguese Tertiary Hospital, between 2003 and 2019. We reviewed the medical records and collected information on the assessment of the initial audiometric status. In the cases submitted for implantation with an osseointegrated hearing implant, we analyzed the details of follow-up, including immediate and long-term post-operative complications, as well as the audiometric results.RESULTSWe present 8 pediatric patients, 6 girls and 2 boys, with a diagnosis of bilateral congenital EAC atresia. The audiometric assessment revealed moderate to severe bilateral conductive hearing loss with a mean speech recognition threshold (SRT) of 51dB. Six patients underwent osseointegrated hearing implantation. All 6 patients showed good audiometric results, with an average SRT of 20dB and closure of the air-bone gap.CONCLUSIONSThe osseointegrated hearing implant was an effective treatment option in these patients, without significant morbidity or complications. Osseointegrated hearing implantation should be considered first line treatment for children with bilateral congenital EAC atresia, as it presents good functional results and a high level of patient satisfaction.

05 Jan 2018·Oncotarget

Asymmetrically enlarged parietal foramina in a rare case of Goldenhar syndrome with a possible etiopathogenesis

Article

Author: Alhur, Nagham Younus ; Sarsam, Shamil Naji ; Al-Kzayer, Lika’a Fasih Y. ; Liu, Tingting ; Nakazawa, Yozo

Goldenhar´s syndrome (GS) also known as oculo-auriculo-vertebral spectrum (OAVS) is a relatively rare condition. GS is of multifactorial etiology that includes environmental and/or genetic factors, in addition to teratogens that disturb the blastogenesis. A 5-year-old girl from Saudi Arabia, was a member of dizygotic twins conceived by assisted reproductive technology (ART), and born with features of GS. She had asymmetrical face, cleft lip and palate, right microphthalmia and microtia. Radiological imaging showed right maxillary and mandibular bone hypoplasia, asymmetrically enlarged parietal foramina, a persistent midline occipital foramen, abnormal bone arising from occipital bone, extending along tentorium cerebelli, and a lipoma at the right tentorium cerebelli. A rudimentary right eye with dermoid cyst and pseudotumor as well as bilateral atresia of external auditory canals were present. Karyotyping was normal. ART and the risk of manipulation of ovum/embryo, was shown to be associated with multiple gestation and an increased risk of major birth defects. Given that our patient was from Eastern-province close to the South of Iraq, where Gulf wars took place and the reported incidence of birth defects, including orofacial malformation, jumped there to about seven-folds, after war, thus, environmental contamination, and the possible teratogenic effect of depleted uranium could not be excluded. In conclusion, our case of GS, disclosed a rare radiological finding in calvarial anatomy, and asserted that, careful clinical evaluation is recommended in cases of GS. ART fertilization risk along with the possible parental environmental exposure were regarded as the potential cooperators of multifactorial etiology in our case.

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External Auditory Canal, Bilateral Atresia Of, With Congenital Vertical Talus

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