scholarly journals Autosomal dominant asymmetrical radial dysplasia, dysmorphic facies, and conductive hearing loss (facioauriculoradial dysplasia)

1982 ◽  
Vol 19 (2) ◽  
pp. 110-115 ◽  
Author(s):  
A E Harding ◽  
C M Hall ◽  
M Baraitser
Keyword(s):  
Hearing Loss ◽  
Autosomal Dominant ◽  
Conductive Hearing Loss ◽  
Conductive Hearing

scholarly journals Ear Surgery in Treacher Collins Syndrome

1995 ◽  
Vol 104 (1) ◽  
pp. 31-41 ◽  
Author(s):  
Henri A. M. Marres ◽  
H. M. A. Marres ◽  
W. R. J. Cremers ◽  
Patrick L. M. Huygen
Keyword(s):  
Hearing Loss ◽  
Autosomal Dominant ◽  
Conductive Hearing Loss ◽  
Branchial Arch ◽  
Treacher Collins Syndrome ◽  
Ear Surgery ◽  
Arch System ◽  
Conductive Hearing ◽  
Outcome Of Surgery ◽  
Insight Into

The autosomal dominant hereditary Treacher Collins syndrome manifests itself phenotypically in dysmorphogenesis of particularly the first, but also the second branchial arch system. Consequently, 50% of patients with Treacher Collins syndrome have a congenital, generally pure conductive hearing loss resulting from a major or minor ear anomaly. The outcome of surgery to improve patients' hearing varies and is sometimes even disappointing. Thorough analysis of 33 cases (39 operated ears) and the strict application of a classification for the anomaly to each ear enabled us to gain insight into the most suitable surgical policy and to form a prognosis for reconstructive ear surgery.

The p.(Pro170Leu) variant in NOG impairs noggin secretion and causes autosomal dominant congenital conductive hearing loss due to stapes ankylosis

2019 ◽  
Vol 46 (9) ◽  
pp. 445-449 ◽  
Author(s):  
Yilai Shu ◽  
Lijun Wang ◽  
Xiaoting Cheng ◽  
Chayada Tangshewinsirikul ◽  
Weili Shi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Autosomal Dominant ◽  
Conductive Hearing Loss ◽  
Conductive Hearing

scholarly journals Cone-Beam CT compared to Multi-slice CT for the diagnostic analysis of conductive hearing loss, a pilot study

2016 ◽  
Vol 130 (S3) ◽  
pp. S188-S188
Author(s):  
Pieter Kemp ◽  
Jiska van Stralen ◽  
Pim de Graaf ◽  
Erwin Berkhout ◽  
Jan Wolff ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Pilot Study ◽  
Conductive Hearing Loss ◽  
Cone Beam Ct ◽  
Cone Beam ◽  
Diagnostic Analysis ◽  
Conductive Hearing

Long-term Compliance and Satisfaction With Percutaneous Bone Conduction Devices in Patients With Congenital Unilateral Conductive Hearing Loss

2015 ◽  
Vol 36 (5) ◽  
pp. 826-833 ◽  
Author(s):  
Rik C. Nelissen ◽  
Emmanuel A. M. Mylanus ◽  
Cor W. R. J. Cremers ◽  
Myrthe K. S. Hol ◽  
Ad F. M. Snik
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Bone Conduction ◽  
Conductive Hearing

scholarly journals Successive ipsilateral surgery of Vibrant Soundbridge and Bonebridge devices for congenital bilateral conductive hearing loss: a case report

2020 ◽  
Vol 48 (12) ◽  
pp. 030006052097228
Author(s):  
Yujie Liu ◽  
Ran Ren ◽  
Shouqin Zhao
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Conductive Hearing Loss ◽  
Vibrant Soundbridge ◽  
Patient’S Experience ◽  
Hearing Devices ◽  
Conductive Hearing ◽  
Subjective Outcomes

The Bonebridge and Vibrant Soundbridge systems are semi-implanted hearing devices, which have been widely applied in patients with congenital conductive hearing loss. However, comparison between these two hearing devices is rare, especially in the same patient. We report a 23-year-old man who underwent successive implantation of Vibrant Soundbridge and Bonebridge devices in the same ear because of dysfunction of the Vibrant Soundbridge. We provide insight on the patient’s experience and compare the audiological and subjective outcomes of satisfaction.

Unexplained Conductive Hearing Loss

1980 ◽  
Vol 73 (3) ◽  
pp. 335-338 ◽  
Author(s):  
FRED H. BESS ◽  
G. W. MILLER ◽  
MICHAEL E. GLASSCOCK ◽  
GENE W. BRATT
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Conductive Hearing

Ossicular Discontinuity and Exostoses in Proteus Syndrome: A Case Report

2005 ◽  
Vol 114 (3) ◽  
pp. 242-246
Author(s):  
Joni K. Doherty ◽  
Dennis R. Maceri
Keyword(s):  
Hearing Loss ◽  
High Frequency ◽  
Conductive Hearing Loss ◽  
Ossicular Chain ◽  
Typical Feature ◽  
Proteus Syndrome ◽  
Frequency Range ◽  
Pure Tone Average ◽  
Ossicular Chain Reconstruction ◽  
Conductive Hearing

Proteus syndrome (PS) is a rare hamartomatous disorder characterized by mosaic overgrowth of multiple tissues that manifests early in life and is progressive. The presence of unilateral external auditory canal exostoses in a patient who is not a swimmer or surfer is suggestive of PS. However, hearing loss is not a typical feature. Here, we describe exostoses and ossicular discontinuity with conductive hearing loss in a patient with PS. The treatment consisted of canalplasty and ossicular chain reconstruction. A postoperative reduction was demonstrated in the patient's air-bone gap, from 21 dB to 13 dB for the pure tone average (four frequencies) and from 41 dB to 15 dB in the high-frequency range (6,000 to 8,000 Hz). Causes of ossicular discontinuity are discussed. Routine annual audiometric and otolaryngological evaluation should be considered in all patients with temporal bone inyolvement of PS.

Exploratory Tympanotomy Revealing Incus Discontinuity and Stapedial Otosclerosis as a Cause of Conductive Hearing Loss

2006 ◽  
Vol 27 (4) ◽  
pp. 466-468 ◽  
Author(s):  
Anita Jeyakumar ◽  
Todd M. Brickman ◽  
Kim Murray ◽  
Paul Dutcher
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Conductive Hearing
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