Publications by authors named "Joseph Nadol"

Objectives: Current surgical techniques aim to preserve intracochlear structures during cochlear implant (CI) insertion to maintain residual cochlear function. The optimal technique to minimize damage, however, is still under debate. The aim of this study is to histologically compare insertional trauma and intracochlear tissue formation in humans with a CI implanted via different insertion techniques.

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We aimed to determine the prevalence of radiological temporal bone features that in previous studies showed only a weak or an inconsistent association with the clinical diagnosis of Meniere's disease (MD), in two groups of MD patients (n = 71) with previously established distinct endolymphatic sac pathologies; i.e. the group MD-dg (ES degeneration) and the group MD-hp (ES hypoplasia).

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Introduction: Dizziness is a common disease. However, approximately 10-40% of patients were diagnosed unknown dizziness even though general, neurological, and otological examinations were performed. The aim of this otopathological study was to investigate the histopathology of the peripheral vestibular system of patients who suffered from undiagnosed dizziness.

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Introduction: Internal auditory canal (IAC) diverticula, also known as IAC cavitary lesions or anterior cupping of the IAC, observed in otopathologic specimens and high-resolution computed tomography (CT) scans of the temporal bone are thought to be related to otosclerosis. Herein, we examined the usefulness of CT scans in identifying diverticula and determined whether IAC diverticula are associated with otosclerosis on otopathology.

Methods: One hundred five consecutive specimens were identified from the National Temporal Bone Hearing and Balance Pathology Resource Registry.

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Hypothesis: Computed tomography (CT) density measurement can be used to objectively distinguish otosclerosis from normal bone and to determine histologic grades of otosclerosis.

Background: Otosclerosis can be seen on CT as subtle radiolucent areas. An objective radiologic measurement that corresponds to known otosclerosis pathology may improve diagnostic accuracy, and could be used as a radiologic biomarker for otosclerosis grade.

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Cochlear implants provide effective auditory rehabilitation for patients with severe to profound sensorineural hearing loss. Recent advances in cochlear implant technology and surgical approaches have enabled a greater number of patients to benefit from this technology, including those with significant residual low frequency acoustic hearing. Nearly all cochleae implanted with a cochlear implant electrode array develop an inflammatory and fibrotic response.

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Objective: We aim to assess the histopathology of human temporal bones (TBs) with evidence of cochlear implantation (CI) electrode scalar translocation.

Study Design: Otopathology study.

Setting: Otopathology laboratory.

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Article Synopsis
  • The study investigates whether cochlear implantation leads to an increase in monocyte-derived macrophages within cochlear vessels, finding that macrophages are more prevalent in implanted ears compared to unimplanted ones.* -
  • Using light microscopy and anti-Iba1 immunostaining, researchers analyzed human cochlear samples, revealing that Iba1-positive macrophages were significantly more common in vessels near the round window of implanted ears.* -
  • Results indicate that the density of these macrophages correlates with the duration since implantation, suggesting that the immune response may play a key role in changes observed in the cochlea post-surgery.*
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Article Synopsis
  • The study focuses on the specific site of lesions causing vestibular function loss in CANVAS, an adult-onset genetic disorder leading to balance issues.
  • Histological analysis of temporal bones and brainstems from CANVAS patients revealed severe loss of vestibular ganglion cells while preserving auditory structures, indicating a selective impact on vestibular function.
  • The findings suggest that CANVAS involves a degeneration of certain cranial sensory ganglia, highlighting a common underlying cause for both sensory and balance impairments in the disease.
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Hypothesis: Epithelial ion transport pathologies of the endolymphatic sac (ES) are associated with large vestibular aqueduct syndrome (LVAS).

Background: LVAS is defined by the pathognomonic features of a widened bony vestibular aqueduct (VA) and an enlarged ES. The underlying cause of its associated cochleovestibular symptoms remains elusive.

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Objective: In cases of a severe to profound sensorineural hearing loss following head injury, the cochlear implant (CI) is the primary option for auditory rehabilitation. Few studies, however, have investigated long-term CI outcomes in patients following head trauma, including those without temporal bone fracture (TBF). Herein, the aim of this study is to examine CI outcomes following cases of head injury with and without TBF.

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Objective: The term "labyrinthine concussion" has evolved to mean audiovestibular dysfunction in the absence of a temporal bone fracture (TBF). Despite a multitude of case descriptions of labyrinthine concussion, the precise pathophysiology remains poorly understood. Herein, we explore the historical otopathologic underpinnings of the diagnosis of labyrinthine concussion with a focus on the auditory pathway during the late 19th to the mid-20th centuries and conclude with a discussion of its contemporary relevance.

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Objective: While cochlear ossification is a common sequalae of meningitic labyrinthitis, less is known about the effects of meningitis on peripheral vestibular end organs. Herein, we investigate histopathologic changes in the peripheral vestibular system and cochlea in patients with a history of meningitic labyrinthitis.

Methods: Temporal bone (TB) specimens from patients with a history of meningitis were evaluated and compared to age-matched controls.

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Objectives/hypothesis: To describe the histopathology of the invasion patterns of advanced-stage external auditory canal (EAC) squamous cell carcinoma (SCC). Study Design Retrospective cohort study.

Methods: Retrospective analysis of medical records of patients diagnosed with EAC SCC available at the Massachusetts Eye and Ear temporal bone (TB) collection.

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The classic view of cochlear partition (CP) motion, generalized to be for all mammals, was derived from basal-turn measurements in laboratory animals. Recently, we reported motion of the human CP in the cochlear base that differs substantially from the classic view. We described a human soft tissue "bridge" (non-existent in the classic view) between the osseous spiral lamina (OSL) and basilar membrane (BM), and showed how OSL and bridge move in response to sound.

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Postmortem temporal bone computed tomography (CT) and histopathologic findings in an infant with CHARGE syndrome revealed bilateral cochleovestibular hypoplasia, including cochlear pathology relevant to cochlear implant candidacy. Both ears had absence of the superior semicircular canals (SCCs), severely hypoplastic posterior SCCs, and hypoplastic (right ear) or absent (left ear) lateral SCCs seen on CT and histopathology. Histopathology further revealed the absence of all SCC ampullae except the right lateral SCC ampulla and atrophic vestibular neuroepithelium in the saccule and utricle bilaterally.

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Objectives: Describe the histopathology of the temporal bones in MELAS (myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) syndrome. The syndrome results from a known point mutation in mitochondrial DNA.

Methods: Histopathology analysis of a pair of temporal bones from the oldest surviving MELAS syndrome temporal bone donor.

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Contemporary histopathology of the ear is based on an evolution of equipment and histological techniques over the last 500 years, including the invention of the light microscope and protocols for fixation, embedment, sectioning, and staining of tissue samples, and visual documentation of findings. Several recent techniques which can be utilized in otopathology hold promise for significant improvement in methods and a better understanding of pathologic processes in diseases of the ear.

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Objectives: To present a histopathological case of a 91-year-old woman who was diagnosed with superior semicircular canal dehiscence postmortem.

Methods: The patient was a registered donor with the National Temporal Bone Donor Program at the NIDCD National Temporal Bone, Hearing and Balance Pathology Resource Registry. Computed tomography imaging was performed on each temporal bone.

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Article Synopsis
  • - The study hypothesizes that cochlear implantation increases macrophage presence in the cochlea, akin to previous observations in vestibular endorgans.
  • - Researchers analyzed macrophage prevalence in the cochlea of 10 patients following unilateral cochlear implantation, comparing it to their unoperated ear.
  • - Results indicated a significant rise in macrophage density in the cochlea of implanted ears, along with varying macrophage types, suggesting their role in both inflammation and homeostasis post-surgery.
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Kleefstra syndrome is a rare neurogenetic disorder caused by a subtelomeric 9q34.3 deletion or by an intragenic mutation of the euchromatin histone methyl transferase 1 gene (EHMT1). Approximately 20% to 30% of individuals have hearing loss.

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A study of clinical records and temporal bone histopathology from a woman with bilateral otosclerosis who was treated with lateral semicircular canal fenestration procedures as well as stapedectomy.

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Objective: To describe the histopathologic findings and clinical presentation of intra-temporal facial nerve invasion in primary and metastatic malignancies of the human temporal bone (TB).

Materials And Methods: Retrospective analysis of all medical records of patients diagnosed with peripheral facial nerve palsy (PFnP) of a malignant origin was performed. Temporal bones underwent standard processing for histologic examination.

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Article Synopsis
  • Cochlear implantation may lead to a higher density of macrophages in vestibular endorgans, which are areas in the inner ear responsible for balance.
  • A study involving 10 human subjects showed that macrophage counts were significantly greater in implanted ears compared to unimplanted ones, except in specific zones of the utricle and posterior semicircular canal.
  • The findings suggest a possible immune response, indicated by macrophages migrating from the subepithelial area into the neuroepithelium after the cochlear implantation procedure.
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