Congenital choanal atresia and nerve deafness

1979 ◽  
Vol 93 (12) ◽  
pp. 1223-1228 ◽  
Author(s):  
Israel Brama ◽  
Dan Engelhard
Keyword(s):  
Congenital Anomaly ◽  
Hearing Loss ◽  
Early Diagnosis ◽  
Sensorineural Hearing Loss ◽  
Congenital Anomalies ◽  
Neonatal Period ◽  
Choanal Atresia ◽  
Surgical Correction ◽  
Electric Response ◽  
Electric Response Audiometry

SummaryFOUR cases of bilateral congenital choanal atresia are presented. Three of them (75 per cent) were shown by Electric Response Audiometry (ERA) tests to have, in addition, congenital nerve deafness; and two had other congenital anomalies. Thus, congenital nerve deafness should be considered as a possible congenital anomaly associated with choanal atresia. Early diagnosis and treatment of congenital choanal atresia can prevent death from asphyxia during the neonatal period, but after surgical correction of the atresia the possible existence of additional anomalies has to be determined and treated.By the use of electric response audiometry we are able to detect sensorineural hearing loss during the neonatal period and can plan the rehabilitation of deaf infants.

Aminoglycoside Ototoxicity in the Neonatal Period: Possible Etiologic Factor in Delayed Postural Control

1981 ◽  
Vol 89 (5) ◽  
pp. 818-821 ◽  
Author(s):  
Lydia Eviatar ◽  
Abraham Eviatar
Keyword(s):  
Hearing Loss ◽  
Postural Control ◽  
Sensorineural Hearing Loss ◽  
Neonatal Period ◽  
Vestibular Dysfunction ◽  
Untreated Group ◽  
Sensorineural Hearing ◽  
Etiologic Factor ◽  
Aminoglycoside Ototoxicity ◽  
Laboratory Evidence

Forty-three infants, treated with aminoglycosides during the neonatal period, were tested repeatedly over a period of two to five years for neurovestibular responses. Results of testing were compared with those obtained from a group of 276 healthy newborns followed simultaneously. No abnormalities were found in the untreated group. Among the treated infants, three had a sensorineural hearing loss and eight had laboratory evidence of vestibular dysfunction and delay of head and postural control.

scholarly journals Radiological Assessment of the Indian Children with Congenital Sensorineural Hearing Loss

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Sangeet Kumar Agarwal ◽  
Satinder Singh ◽  
Samarjit Singh Ghuman ◽  
Shalabh Sharma ◽  
Asish Kr. Lahiri
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Temporal Bone ◽  
Congenital Anomalies ◽  
Tertiary Care ◽  
Sensorineural Hearing ◽  
Tertiary Care Centre ◽  
Indian Children ◽  
3D Scan

Introduction. Congenital sensorineural hearing loss is one of the most common birth defects with incidence of approximately 1 : 1000 live births. Imaging of cases of congenital sensorineural hearing loss is frequently performed in an attempt to determine the underlying pathology. There is a paucity of literature from India and for this reason we decided to conduct this study in Indian context to evaluate the various cochleovestibular bony and nerve anomalies by HRCT scan of temporal bone and MRI with 3D scan of inner ear in a tertiary care centre.Material and Methods. A total of 280 children with congenital deafness (158 males and 122 females), between January 2002 to June 2013 were included in the study and they were assessed radiologically by HRCT scan of temporal bone and MRI with 3D scan of inner ear.Results. In the present study we found various congenital anomalies of bony labyrinth and vestibulocochlear nerve. Out of 560 inner ears we found 78 anomalous inner ears. Out of these 78 inner ears 57 (73%) had cochlear anomaly, 68 (87.1%) had anomalous vestibule, 44 (56.4%) had abnormal vestibular aqueduct, 24 (30.7%) had anomalous IAC, and 23 (29.4%) had abnormal cochleovestibular nerves.Conclusion. In present study, we found lower incidences of congenital anomalies comparative to existing literature.

scholarly journals Genetic and Non-genetic Workup for Pediatric Congenital Hearing Loss

2021 ◽  
Vol 9 ◽  
Author(s):  
Ryan Belcher ◽  
Frank Virgin ◽  
Jessica Duis ◽  
Christopher Wootten
Keyword(s):  
Hearing Loss ◽  
Genetic Testing ◽  
Sensorineural Hearing Loss ◽  
Congenital Anomalies ◽  
Anticipatory Guidance ◽  
Congenital Hearing Loss ◽  
Bilateral Hearing Loss ◽  
Genetic Causes ◽  
Genetic Hearing Loss ◽  
Over Time

Hearing loss is one of the most common concerns for presentation for a geneticist. Presentation prior to the age of one (congenital hearing loss), profound sensorineural hearing loss (SNHL), and bilateral hearing loss are sensitive and should raise concern for genetic causes of hearing loss and prompt referral for genetic testing. Genetic testing particularly in this instance offers the opportunity for anticipatory guidance including possible course of the hearing loss over time and also connection and evaluation for additional congenital anomalies that may be associated with an underlying syndrome vs. isolated genetic hearing loss.

Cochlear, Brain Stem, and Cortical Evoked Responses in Nonorganic Hearing Loss

1977 ◽  
Vol 86 (2) ◽  
pp. 227-234 ◽  
Author(s):  
H. Sohmer ◽  
L. Bauberger-Tell ◽  
M. Feinmesser ◽  
E. Edelstein
Keyword(s):  
Hearing Loss ◽  
Brain Stem ◽  
Emotional Problem ◽  
Psychiatric Evaluation ◽  
Electric Response ◽  
Evoked Responses ◽  
Hearing Tests ◽  
Electric Response Audiometry ◽  
The Subject ◽  
True Threshold

After complete but inconclusive audiometric evaluation, 30 suspected cases of nonorganic hearing loss (NOHL) were referred to this laboratory for a complete battery of electric response audiometry tests (recording of cochlear, brain stem and cortical evoked responses). The chief advantages of these tests lie in their ability to more objectively confirm the presence of NOHL and to elicit the true threshold of the subject. Such tests along with psychiatric evaluation, indicate that NOHL can be subdivided into categories, examples of which are presented and discussed: Malingering or conscious simulation of deafness for obvious personal gain, and psychogenic deafness in which an emotional problem ( e.g., combat stress, anxiety) is unconsciously converted into a hearing problem in an escape mechanism. The basis of NOHL in some cases may be a mixture of both conscious components (malingering) and unconscious components (psychogenic). Several cases of NOHL are really nonorganic overlays or exaggeration on a small to moderate organic hearing loss. The psychogenic hearing loss seen in soldiers with combat neurosis may have originally developed as a psychogenic exaggeration of a temporary threshold shift induced by the noise of battle. Thus objective electrophysiological hearing tests and psychiatric evaluation have contributed to a better understanding of NOHL.

H-Type Tracheoesophageal Fistula Masquerading as Grade V Gastroesophageal Reflux in a Neonate

2021 ◽  
pp. 097321792110406
Author(s):  
Nasreen Banu ◽  
VVS Chandrasekharam ◽  
Durga Prasad Koduru
Keyword(s):  
Congenital Anomaly ◽  
Early Diagnosis ◽  
Gastroesophageal Reflux ◽  
Esophageal Atresia ◽  
Tracheoesophageal Fistula ◽  
Neonatal Period ◽  
Postoperative Outcome ◽  
Clinical Suspicion ◽  
Rare Congenital Anomaly ◽  
Cervical Approach

Tracheoesophageal fistula (TEF) without associated esophageal atresia is a rare congenital anomaly. Diagnosis in neonatal period is usually not made and most of the patients are treated as cases of pneumonia. We report a case of H-type TEF, which was initially diagnosed as grade V gastroesophageal reflux on contrast esophagogram and bronchoscopy done revealed H-type fistula. Through cervical approach, fistula was repaired and baby had uneventful postoperative outcome. High index of clinical suspicion and early diagnosis can provide a better prognosis.

ALGORITHM FOR EARLY DIAGNOSIS AND PREVENTION OF PROFESSIONAL SENSORINEURAL HEARING LOSS

2021 ◽  
Author(s):  
E.A. Preobrazhenskaya ◽  
◽  
A.V. Sukhova ◽  
E.N. Kryuchkova
Keyword(s):  
Hearing Loss ◽  
Early Diagnosis ◽  
Sensorineural Hearing Loss ◽  
Working Conditions ◽  
Preventive Measures ◽  
Hearing Preservation ◽  
Hearing Research ◽  
Auditory Analyzer ◽  
Risk Categorization ◽  
Selection Of

Abstract: The aim of the study is to develop an algorithm for early diagnosis and prevention of professional neurosensory hearing elasticity (PNST). A comprehensive audiological survey of 600 miners aged 37 to 58, with 10 to 35 years of experience in noise conditions above the PDU for 7-21 dBA (class 3.1-3.3) was carried out. Based on the studies, an algorithm of early diagnosis and prevention of PNST, consisting of 3 blocks: I - hygienic (assessment of working conditions and risk categorization), II - clinical diagnostic, based on modern audiological methods of hearing research with the selection of diagnostic markers of risk of PNST, III - preventive, including the development of individual hearing preservation programs. The developed algorithm allows in 65-75% of cases to identify changes in the auditory analyzer at the preclinical stage, to clarify the nature and localization of the auditory analyzer, which is important in the development of therapeutic and preventive measures.

Cochlear Implants in Young Children with Usher'S Syndrome

1995 ◽  
Vol 104 (9_suppl2) ◽  
pp. 342-345 ◽  
Author(s):  
N. M. Young ◽  
J. C. Johnson ◽  
M. B. Mets ◽  
T. C. Hain
Keyword(s):  
Hearing Loss ◽  
Early Diagnosis ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Autosomal Recessive Disorder ◽  
Sensorineural Hearing ◽  
Special Importance ◽  
Sensory Deficits ◽  
Usher’S Syndrome

Usher's syndrome (US) is an autosomal recessive disorder characterized by congenital sensorineural hearing loss and retinitis pigmentosa. The majority of affected individuals have severe to profound sensorineural hearing loss and are therefore potential cochlear implant candidates. A goal of our otology program has been early identification and implantation of children with US. At our center, early diagnosis of US has been achieved by comprehensive ophthalmologic evaluation including electroretinography. Four of our 19 pediatric cochlear implant recipients with congenital sensorineural hearing loss were identified with US and implanted prior to the onset of signs or symptoms of visual loss. All children have received measurable benefit from cochlear implantation. In light of the dual sensory deficits present in US, the need for early diagnosis and cochlear implantation is of special importance in this population.

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