Classification and Current Management of Inner Ear Malformations
Morphologically congenital sensorineural hearing loss can be investigated under two categories. Majority of the congenital hearing loss (80%) are membranous malformations. Here the pathology involves inner ear hair cells. There is no gross bony abnormality and therefore, in these cases, high resolution computerized tomography and MRI of the temporal bone reveal normal findings. Remaining 20% […]
Novel pathogenic variants underlie SLC26A4 -related hearing loss in a multiethnic cohort
Objectives: The genetics of sensorineural hearing loss is characterized by a high degree of heterogeneity. Despite this heterogeneity, DNA variants found within SLC26A4 have been reported to be the second most common contributor after those of GJB2 in many populations. Methods: Whole exome sequencing and/or Sanger sequencing of SLC26A4 in 117 individuals with sensorineural hearing […]
Bony cochlear nerve canal and internal auditory canal measures predict cochlear nerve status
Objectives The bony cochlear nerve canal is the space between the fundus of the internal auditory canal and the base of the cochlear modiolus that carries cochlear nerve fibres. This study aimed to determine the distribution of bony labyrinth anomalies and cochlear nerve anomalies in patients with bony cochlear nerve canal and internal auditory canal […]
See all › 35 References See all › 2 Figures Download citation Share Download full-text PDF Accuracy of turbo spin-echo diffusion-weighted imaging signal intensity measurements for the diagnosis of cholesteatoma
Purpose: We aimed to evaluate the diagnostic accuracy of turbo spin-echo diffusion-weighted imaging (TSE-DWI) at 3 T, for cholesteatoma (CS) diagnosis, using qualitative and quantitative methods with numerical assessment of signal intensity (SI), signal intensity ratios (SIR), and apparent diffusion coefficient (ADC) values. Methods: In this retrospective study, two blinded observers independently evaluated the preoperative […]
Incomplete partition type III: A rare and difficult cochlear implant surgical indication
Objective: Presenting the clinical features and treatment options for incomplete partition type-III. Methods: Nine primary and 1 revision incomplete partition type-III cochlear implant cases treated between 2004 and 2015 in Hacettepe University Department of Otolaryngology were included in the study. Treatment options and particularly cochlear implantation tecnique were described. Results: Nine primary and 1 revison […]
Audiologic and radiologic findings in cochlear hypoplasia
Objective: The aim of the current study is to evaluate audiologic and radiologic findings of cochlear hypoplasia which is a subgroup of inner ear malformations. Methods: This study was a prospective clinical study and based on voluntary participation from cases with cochlear hypoplasia diagnosis. The study was conducted at Hacettepe University, Department of Otolaryngology, Head […]
Audiological and Radiological Characteristics in Incomplete Partition Malformations
Objective: To compare the audiological and radiological findings of patients with incomplete partition malformations (IPs) and analyze the relationship between the audiological and radiological findings. Materials and methods: The study included 84 patients (168 ears) with IPs as follows: 26 patients with Type I;IP-I (41 ears), 54 patients with Type II;IP-II (108 ears), and 4 […]
Variations in Multiple Syndromic Deafness Genes Mimic Non-syndromic Hearing Loss
The genetics of both syndromic (SHL) and non-syndromic hearing loss (NSHL) is characterized by a high degree of genetic heterogeneity. We analyzed whole exome sequencing data of 102 unrelated probands with apparently NSHL without a causative variant in known NSHL genes. We detected five causative variants in different SHL genes (SOX10, MITF, PTPN11, CHD7, and […]
Long-term Results of ABI in Children With Severe Inner Ear Malformations
Objective: To report the long-term outcomes of children who received auditory brainstem implant (ABI) because of severe inner ear malformations. Study design: Retrospective chart review. Setting: Tertiary referral otolaryngology clinic. Subjects and methods: Between July 2006 and October 2014, 60 children received ABI at Hacettepe University. Preoperative work up included otolaryngologic examination, audiological assessment, radiological […]