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Related Concept Videos

Bone Disorders01:29

Bone Disorders

Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
Compact Bone01:27

Compact Bone

Most bones contain compact and spongy osseous tissue, but their distribution and concentration vary based on the bone's overall function.
Compact bone, also called cortical bone, is the denser, stronger of the two types of bone tissue. It is found under the periosteum and in the diaphyses of long bones, where it provides support and protection. The microscopic structural unit of compact bone is called an osteon, or haversian system. Each osteon is composed of concentric rings of calcified...
Sutures of the Skull01:22

Sutures of the Skull

The human skull is composed of several bones that come together to protect the brain and support the structures of the face. The junctions where these bones meet are called sutures.
Sutures are immobile joints between adjacent bones of the skull. The narrow gap between the bones is filled with dense, fibrous connective tissue that unites the bones. The long sutures located between the skull bones are not straight but instead follow irregular, tightly twisting paths. These twisting lines tightly...
Bone Formation by Intramembranous Ossification01:29

Bone Formation by Intramembranous Ossification

Intramembranous ossification is one of the two processes involved in the development of bones within an embryo. The flat bones of the face, most of the cranial bones, and the clavicles are formed via this process. During intramembranous ossification, the bones develop directly from sheets of undifferentiated mesenchymal connective tissue.
The process begins when mesenchymal cells in the embryonic skeleton gather together and differentiate into osteogenic cells, which then develop into...
Cranial Bones: Lateral View01:27

Cranial Bones: Lateral View

The lateral view of the cranium is dominated by temporal, sphenoid, and ethmoid bones.
The temporal bone forms the lower lateral side of the skull. The temporal bone is subdivided into several regions. The flattened upper portion is the squamous portion of the temporal bone. Below this area and projecting anteriorly is the zygomatic process of the temporal bone, which forms the posterior portion of the zygomatic arch. Posteriorly is the mastoid portion of the temporal bone. Projecting...
Gross Anatomy of Bone01:17

Gross Anatomy of Bone

The two main features of a long bone are the diaphysis and the epiphysis.
The diaphysis is the tubular shaft that runs between the proximal and distal ends of the bone. The walls of the diaphysis are composed of dense and hard compact bone made of numerous osteons — the functional unit of the compact bone. The hollow region in the diaphysis is called the medullary cavity, which harbors the bone marrow. In infants and children, this marrow cavity is filled with red marrow, whereas in adults, it...

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Related Experiment Video

Updated: Jun 4, 2026

High-Speed Human Temporal Bone Sectioning for the Assessment of COVID-19-Associated Middle Ear Pathology
03:42

High-Speed Human Temporal Bone Sectioning for the Assessment of COVID-19-Associated Middle Ear Pathology

Published on: May 18, 2022

Temporal bone abnormalities in children with GJB2 mutations.

Margaret A Kenna1, Heidi L Rehm, Anna Frangulov

  • 1Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, Boston, Massachusetts, USA. margaret.kenna@childrens.harvard.edu

The Laryngoscope
|February 8, 2011
PubMed
Summary
This summary is machine-generated.

Temporal bone imaging in children with GJB2 mutations and hearing loss revealed few subtle abnormalities in only 10%. No correlation was found between malformations, genotype, or hearing loss severity.

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Area of Science:

  • Genetics
  • Otolaryngology
  • Radiology

Background:

  • Biallelic GJB2 mutations are a common cause of congenital sensorineural hearing loss (SNHL).
  • Temporal bone imaging is crucial for identifying structural abnormalities that may contribute to hearing loss.

Purpose of the Study:

  • To investigate the incidence and types of temporal bone abnormalities in children with SNHL and pathogenic biallelic GJB2 mutations.
  • To determine if there is a correlation between imaging findings, genotype, and SNHL severity.

Main Methods:

  • Retrospective analysis of 113 pediatric patients with biallelic GJB2 mutations and SNHL.
  • Blinded review of temporal bone computed tomographic (CT) and magnetic resonance imaging (MRI) studies.

Main Results:

  • Subtle inner ear abnormalities were identified in 12% (12/113) of patients.
  • Observed malformations included semicircular canals (SCC), internal auditory canal, and cochlear nerve canal (CNC).
  • No correlation was found between SNHL severity, malformation presence/type, or genotype.

Conclusions:

  • This is the largest study to date on temporal bone imaging in this patient group.
  • The low incidence of abnormalities suggests GJB2 mutations primarily affect cochlear function rather than gross structure.
  • Discrepancies with prior studies may stem from differences in mutation types, populations, and imaging interpretation.