DFNA9 Hearing Loss and Balance Disorder
The otopathology webcasts are recordings of the Otopathology Conferences hosted on a quarterly basis at Massachusetts Eye and Ear. Each webcast is approximately 60 minutes in length. If you would like to be added to the email list for upcoming conferences, please email firstname.lastname@example.org.
Upcoming conference dates all starting at 5:00 pm EST:
DFNA9 Hearing Loss and Balance Disorder
Charge Syndrome: Otopathology and Radiologic Correlates
Meniere’s Disease: Endolymphatic Shunt Surgery and MRI Diagnostics
Cochlear Nerve Schwannoma and Otosclerosis
Enlarged vestibular aqueduct and incomplete partition type II
Immune-mediated hearing loss
Autosomal Dominant Sensorineural Hearing Loss
Meniere’s disease with endolymphatic sac decompression, intratympanic gentamicin injections, and suboccipital vestibular neurectomy.
Congenital anomalies of the ossicles
Otopathology of head injury.
Squamous cell carcinoma of the temporal bone.
Asymptomatic schwannoma of the facial nerve.
Otopathology of the CHARGE Association.
Rapidly progressive sensorineural hearing loss.
Surgical pathology of the temporal bone following resection of an acoustic neuroma.
Lateral canal fenestration procedure for otosclerosis.
Aberrant internal carotid artery.
Angiosarcoma of the temporal bone.
Squamous cell carcinoma arising in a surgical mastoid bowl.
Cerebellar ataxia-neuropathy-vestibular areflexia syndrome (CANVAS).
Perineural spread of squamous cell carcinoma in the facial nerve.
Role of immunostaining in quantitative otopathology.
Surgical pathology following labyrinthectomy for Meniere’s syndrome.
Type II Collagenopathy.
Otopathology of a cochlear implant.
Bilateral sensorineural hearing loss and cysts of the internal auditory canals.
A 78-year-old man with bilateral conductive hearing loss.
An 83-year-old woman with otosclerosis who underwent a stapedotomy. Includes a presentation by Dr. Alicia Quesnel on radiologic-pathologic correlations in otosclerosis.
A 74-year-old patient with bilateral mixed hearing loss, bilateral stapedectomy, and bilateral cochlear implants.
A 94-year-old woman with a history of sudden unilateral sensorineural hearing loss and vertigo.
Dandy’s Syndrome (bilateral vestibular areflexia and oscillopsia).
A 62-year-old woman with superior semicircular canal dehiscence (SCD) syndrome. Includes a review by Dr. John Carey of SCD in a histological survey of 1,000 temporal bones.
A 53-year-old man with bilateral Meniere’s syndrome. Includes a discussion by Dr. Joe Adams on mechanisms involved in fluid-ion homeostasis within the inner ear.
A newborn child with sensorineural hearing loss caused by selective loss of inner hair cells. Includes results of a study on the same topic by Drs. Amatuzzi, Liberman, and Northrop, and a discussion of the possible role of thiamine deficiency in causing this type of pathology.
A 90-year-old woman with episodic vertigo who had undergone a cochleosacculotomy procedure. Includes a discussion about Meniere’s syndrome, vestibular migraine, and capacity of the cochlea to mount a healing response.
Features cases of the nonsyndromic deafness, DFNA9, including clinical features, histopathology, and genetics. It includes a discussion of the many insights in basic science and in clinical medicine gained as a result of the study of DFNA9.
A 77-year-old man with otosclerosis who underwent a stapes mobilization in the right ear and a stapedectomy in the left ear. Both procedures were unsuccessful. Includes a discussion of causes of residual and recurrent conductive hearing loss after stapedectomy.
A 51-year-old woman with hearing loss due to Susac’s syndrome. Includes an overview of the syndrome by Dr. John Susac, a description of ophthalmic pathology by Dr. Gerard Lutty, and a description of the otopathology.
A 52-year-old woman with hearing loss due to MELAS syndrome. Includes an overview of mitochondrial disorders by Dr. Katherine Sims, a description of the otopathology, and a discussion of basic and clinical implications.
A 57-year-old man with profound hearing loss due to superficial siderosis; he was treated with a cochlear implant. Includes a review of siderosis with emphasis on its otologic manifestations and a discussion of otopathology after cochlear implantation.
An 82-year-old woman with chronic otitis media. Includes a discussion of pathogenesis of congenital cholesteatoma and histopathology of mastoidectomy with surgical implications.
A 48-year-old man with sensorineural hearing loss occurring in early childhood. Includes a discussion by Prof. Lisbeth Tranebjærg about Wolfram syndrome and genetics of hearing loss in the setting of other neurodegenerative diseases.
A newborn baby boy with bilateral congenital aural atresia. Surgical implications are discussed. Also includes a presentation of a study of the Scala Communis anomaly, with implications for its radiological diagnosis.
A 92-year-old woman with hearing loss and vertigo due to Meniere’s syndrome. Also, includes a study of the histopathology after endolymphatic sac decompression surgery for Meniere’s syndrome.
A 91-year-old woman with bilateral severe hearing loss due to extensive otosclerosis. She had a stapedectomy and labyrinthectomy in one ear. The discussion includes histologic aspects of otosclerosis with clinical and surgical relevance. Also included is a demonstration of various resources featured on our former website.
A 34-year-old woman with bilateral hearing loss due to neurofibromatosis type 2 (NF2). Also includes a general discussion of NF2 and determinants of hearing loss in vestibular schwannoma.
A 77-year-old man with history of noise exposure and accelerated presbycusis. Includes a discussion of correlation of audiometric and histopathologic data and recent basic research on long-term effects of noise exposure.
A 100-year-old woman with unoperated acoustic neuroma. Includes a discussion of the histopathology and mechanisms of hearing loss from acoustic neuromas.
A 74-year-old woman with otosclerosis and stapedectomy. Includes a discussion of pathogenesis of otosclerosis.
A 24-year-old man with sensorineural hearing loss due to Wolfram’s syndrome. Includes a discussion of clinical manifestations of the syndrome and its neuropathology and otopathology.
An 88-year-old woman with sensorineural hearing loss due to strial atrophy. Includes a discussion of phenotypes of strial atrophy, as well as physiology and pathophysiology of the lateral cochlear wall.
A 68-year-old woman with progressive hearing loss due to cochlear atrophy. Includes a discussion of relevance of the pathology to cochlear implantation.
An 88-year-old man with hearing loss due to a temporal bone fracture. Includes a discussion on types of temporal bone fractures, their histopathology, and long-term complications arising from fractures.