Bony sheathed chorda tympani: A unique case of incudomalleolar fixation
To describe a unilateral conductive hearing loss caused by restriction of the movements of malleus and incus due to chorda tympani in a bony sheath. A 23-year-old man with a stable mild conductive hearing loss in the left ear and mild sensorineural hearing loss in the right ear. An exploratory tympanotomy was performed. Immobility of […]
Hearing Loss in Biotinidase Deficiency: Genotype-Phenotype Correlation
Children with symptoms of profound biotinidase deficiency with null mutations are more likely to have hearing loss develop than those with missense mutations, even if not treated for a period of time. Hearing loss appears to be preventable in children with null mutations if treatment is initiated soon after birth.
Is it possible to predict diffuse obliterative otosclerosis preoperatively by audiologic examination
Patients with diffuse obliterative otosclerosis have more extensive footplate pathology than annular cases. As a result of this more skill is required for diffuse otosclerosis cases, and postoperative hearing results are usually worse than annular cases. In this retrospective study we compared the preoperative audiological features of annular and diffuse otosclerosis patients. The subjects were […]
Effect of age on speech recognition in noise and on contralateral transient evoked otoacoustic emission suppression
We aimed to study the influence of age, in normal hearing individuals, on: the masking level difference test, the speech recognition in noise test, the transient evoked otoacoustic emissions test, and the contralateral transient evoked otoacoustic emission suppression test. We also aimed to research the effect of age when using these tests to evaluate the […]
Homozygous FGF3 mutations result in congenital deafness with inner ear agenesis, microtia, and microdontia
Homozygous mutations in the fibroblast growth factor 3 (FGF3) gene have recently been discovered in an autosomal recessive form of syndromic deafness characterized by complete labyrinthine aplasia (Michel aplasia), microtia, and microdontia (OMIM 610706 – LAMM). In order to better characterize the phenotypic spectrum associated with FGF3 mutations, we sequenced the FGF3 gene in 10 […]
Complete Labyrinthine Aplasia: Clinical and Radiologic Findings with Review of the Literature
Complete labyrinthine aplasia (CLA), also referred to as Michel aplasia, is a severe congenital anomaly of the inner ear, defined by the complete absence of inner ear structures. The purpose of this study was to document the imaging findings in a series of patients with CLA, with review of the literature, to better understand this […]
A Complex TFAP2A Allele Is Associated With Branchio-Oculo-Facial Syndrome and Inner Ear Malformation in a Deaf Child
We present a 4-year-old girl with congenital profound sensorineural deafness associated with inner ear malformation (incomplete partition type II, enlarged vestibule, and enlarged vestibular aqueduct). The proposita also had pseudocleft lips, skin defects, auricle abnormalities, and unilateral multicystic dysplastic kidney, leading to the diagnosis of branchio-oculo-facial (BOF) syndrome. Mutation analysis of the TFAP2A gene showed […]
Efficacy of nasopharyngeal culture in identification of pathogen in middle ear fluid in chronic otitis media with effusion
Chronic otitis media with effusion (OME) is the leading cause of hearing loss during childhood. In bacterial etiology of OME, the most frequent pathogens responsible are Haemophilus influenzae followed by Streptococcus pneumoniae and Moraxella catarrhalis. This study aimed at evaluating the accuracy of nasopharyngeal (NP) specimens in the identification of pathogens in the middle ear […]
Balance after stapedotomy: Analysis of balance with computerized dynamic posturography
The aim of this study was to investigate vestibular symptoms and their effect on the balance in otosclerosis patients undergoing stapedotomy operations. Prospective study at an academic tertiary referral centre. Thirty-three patients undergoing stapedotomy were included in the study. Sensory organisation test (SOT) protocol of computerized dynamic posturography was used to analyse the balance in […]
Preliminary Results of Auditory Brainstem Implantation in Prelingually Deaf Children With Inner Ear Malformations Including Severe Stenosis of the Cochlear Aperture and Aplasia of the Cochlear Nerve
The aim of our study is to present the results of 11 children where auditory brainstem implantation (ABI) was successfully performed to restore hearing. Case presentation. This study was conducted at the departments of Otolaryngology and Neurosurgery at Hacettepe University Ankara, Turkey. Between July 2006 and April 2008, 11 prelingual (30-56 mo) deaf children with […]
Successive Medical Treatment Versus Watchful Waiting in Chronic Otitis Media with Effusion
Objective: Management of otitis media with effusion (OME) is still an unresolved problem. Although watchful waiting gained popularity, it may not be applicable in the developing countries where medical and surgical treatments are being used more promptly. The aim of our study was to compare consecutive use of medical therapies with watchful waiting. Materials and […]
Cochlear Implantation in Inner Ear Malformations — A Review Article
Inner ear malformations constitute about 20% of congenital sensorineural hearing loss. In this review article an updated classification of cochlear malformations is provided. Incomplete partition and cochlear hypoplasia cases are each divided further into three groups. There are two main difficulties in the surgery of inner ear malformations; gusher and facial nerve abnormalities. Radiological features […]
Congenital facial paralysis: Facial nerve hypoplasia
Facial paralysis in a newborn is called congenital facial paralysis (CFP). The major role of the physician is to differentiate the etiology of the facial paralysis whether it is traumatic or developmental. This can be achieved by history and physical examination in some cases, however diagnosis may remain uncertain despite all efforts.We herein report a […]
Brain Stem Responses Evoked by Stimulation With an Auditory Brain Stem Implant in Children With Cochlear Nerve Aplasia or Hypoplasia
The inclusion criteria for an auditory brain stem implant (ABI) have been extended beyond the traditional, postlingually deafened adult with Neurofibromatosis type 2, to include children who are born deaf due to cochlear nerve aplasia or hypoplasia and for whom a cochlear implant is not an option. Fitting the ABI for these new candidates presents […]
Significance of the cochlear aqueduct size and type in the development of postmeningitic sensorineural hearing loss Type: Scientific Paper
Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to third-party sites or information are provided solely as a convenience to you and do not […]
Prevalance and relative frequencies of the congenital inner ear abnormalities in patients evaluated with temporal bone CT for sensorineural hearing loss Type: Scientific Paper
Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to third-party sites or information are provided solely as a convenience to you and do not […]
Evaluation of Objective Test Techniques in Cochlear Implant Users With Inner Ear Malformations
The aim of the study was to compare results of objective test techniques in cochlear implant users with inner ear malformations and incomplete partition anomalies with types I and II and to show which techniques should be used in the evaluation and fitting of cochlear implant users with inner ear malformations. Retrospective clinical study. The […]