Histopathologic features of the temporal bone in usher syndrome type I.

Temporal bones of 2 patients with Usher syndrome type I were examined using light microscopy. In both patients, findings from histopathologic examination of the cochlea were characterized by degeneration of the organ of Corti, which was most marked in the basal turn, atrophy of the stria vascularis, and a decrease in the number of spiral ganglion cells. The cochlear nerve appeared to be diminished.

Temporal bone pathology in a case of Cogan's syndrome.

Cogan's syndrome (CS) presents typical and atypical types. Typically, there are episodes of nonsyphilitic keratitis and audiovestibular dysfunction. Atypically, there are inflammatory changes in other eye structures and other organ systems, particularly the cardiovascular system.

Auditory and cytocochlear correlates of inner ear disorders.

Pure-tone threshold audiograms showing sensorineural hearing loss, when plotted on a data-based anatomic frequency scale, show a close spatial correlation with their respective cytocochleograms. Whereas most of the cochleae show pathology of several different cell types, a sufficient number show losses that involve predominantly a single cell type, which permits the following deductions: (1) focal lesions of the organ of Corti are strongly tonotopic and are responsible for those instances of abrupt pure-tone threshold losses; (2) lesions of the stria vascularis show no tonotopic organization but lead to flat pure-tone threshold losses; and (3) the principal effect of neuronal losses is a diminished capability for word recognition. There is an indeterminate group where no pathologic correlate can be identified by light microscopy.

[Spontaneous cerebrospinal fluid fistula in the tegmen tympani].

The author's experience with spontaneous cerebrospinal fluid (CSF) otorrhea from the tympanomastoid tegmen is based on the management of two clinical cases, the temporal bone histopathological findings in a third case suffering fatal meningitis and a review of the literature. Characteristically, the disorder occurs in otherwise healthy ears and is the consequence of embryogenic faults in the dura mater and adjacent tegmen. After years of exposure to physiologically normal CSF pressures, these faults may fistulize into the tympanomastoid compartment.

Pathology of prelingual profound deafness: magnitude of labyrinthitis fibro-ossificans.

Quantitative histologic studies were performed on 15 temporal bones from eight adult persons who were known to have prelingual bilateral profound hearing loss. The pathologic changes are characterized by severe degeneration of the structures of the cochlear duct, often with degeneration of the vestibular sense organs, causing a reparative host response that features osteoneogenesis and fibrous proliferation followed by retrograde neuronal degeneration. The pathology is consistent with meningogenic bacterial or viral labyrinthitis that occurred subclinically or went undiagnosed.

The National Temporal Bone, Hearing, and Balance Pathology Resource Registry.

The National Temporal Bone, Hearing, and Balance Pathology Resource Registry has been established with funding provided by the National Institute on Deafness and Other Communication Disorders of the National Institutes of Health. The registry is meant to serve as a national resource for researchers and the public to stimulate and facilitate human otopathologic research. It will maintain a computerized database of currently active and inactive temporal bone and auditory brain-stem collections throughout the United States.

Cochlear pathology in presbycusis.

A survey of the temporal bone collection at the Massachusetts Eye and Ear Infirmary reveals 21 cases that meet the criterion for the clinical diagnosis of presbycusis. It is evident that the previously advanced concept of four predominant pathologic types of presbycusis is valid, these being sensory, neural, strial, and cochlear conductive. An abrupt high-tone loss signals sensory presbycusis, a flat threshold pattern is indicative of strial presbycusis, and loss of word discrimination is characteristic of neural presbycusis.

Myths in neurotology.

For many diseases the etiology of disordered function is unknown and therapy is either symptomatic or empirical. The empirical approach to therapy is acceptable to many physicians, particularly if a treatment can be based on some attractive, although unproven, concept of pathogenesis. A hypothetical explanation of disordered function can become widely popular in spite of a serious lack of scientific support.

Bilateral acoustic neurofibromatosis (neurofibromatosis 2): a disorder distinct from von Recklinghausen's neurofibromatosis (neurofibromatosis 1).

A case report of bilateral acoustic neurofibromatosis (NF-2) is presented with temporal bone histopathology. The distinct clinical and genetic features of NF-2 are reviewed in the context of recent data that show it to be a disorder distinct from classic von Recklinghausen's disease (NF-1).

Autosomal dominant sensorineural hearing loss. Pedigrees, audiologic findings, and temporal bone findings in two kindreds.

We report the clinical and otopathologic findings in three persons from two kindreds affected with adult-onset autosomal dominant progressive sensorineural hearing loss. The primary pathologic change is a deposit of acid polymucosaccharide ground substance in the cribrose areas; in the spiral ligament, limbus, and spinal lamina of the cochlea; and in the stroma of the maculae and cristae. These deposits obstruct the channels that accommodate the dendritic nerve fibers to the auditory and vestibular sense organs.

The anterior inferior cerebellar artery in the internal auditory canal.

It has been proposed that compression of the auditory and vestibular nerve trunks by vascular loops might be the cause of otherwise unexplained hearing loss, tinnitus, and vertigo, as well as Meniere's disease. We studied the human temporal bone histological collection at the Massachusetts Eye and Ear Infirmary to determine whether audiovestibular symptoms could be correlated with the presence of the anterior inferior cerebellar artery within the internal auditory canal. Anterior inferior cerebellar artery loops were found within the internal auditory canals of 12.

Blockage of longitudinal flow in endolymphatic hydrops.

The external shunt operation on the endolymphatic sac is based on the concept that it will drain excess endolymph and thus arrest the progression of endolymphatic hydrops. We performed histological studies on 46 temporal bones from 13 cases of bilateral and 20 cases of unilateral Meniere's disease to evaluate the status of the pathways of longitudinal flow of endolymph to the sac. The endolymphatic ducts were blocked in 8 specimens (17%), the endolymphatic sinuses in 9 (19.

Delayed endolymphatic hydrops and its relationship to Meniére's disease.

Delayed endolymphatic hydrops (EH) can be characterized as having ipsilateral and contralateral types. They are similar in that both have early and late phases of otologic symptoms and that the early phase is a profound hearing loss in one ear. The late phases differ, however, in that the ipsilateral type develops the symptoms of EH (episodic vertigo) in the deaf ear and the contralateral type develops the symptoms of EH (fluctuating hearing loss and/or episodic vertigo) in the hearing ear.

In search of cochlear morphologic correlates for tinnitus.

A correlative study was made of the cochlear pathologic features existing in the temporal bones of 83 subjects with a clinical history of tinnitus and 33 without tinnitus. None of 24 types of pathologic change assessed by light microscope occurred in more than 40% of tinnitus cases; nor was the prevalence of any of these pathologic changes significantly greater in subjects with tinnitus compared with control subjects. For five types of morphologic alteration the prevalence was significantly greater among control subjects compared with tinnitus cases, but this is interpreted cautiously because of the challenge in retrospectively selecting an appropriate control group.

Pathology of peripheral vestibular disorders in the elderly.

A clinical classification of vertigo commonly seen in the elderly and caused by peripheral vestibular disorders is illustrated by case reports and temporal bone histopathology. The classification includes inducible transient vertigo and noninducible protracted vertigo. The peripheral vestibular pathology includes abnormalities of sensory, neural, and mechanical structures and is often asymmetric.

Temporal bone findings in a case of bilateral Menière's disease treated by parenteral streptomycin and endolymphatic shunt.

A patient with bilateral Menière's disease who had progressive hearing loss and intractable vertigo was treated at ages 60 and 62 with parenteral streptomycin to ablate vestibular function, and at age 74 by a left endolymphatic shunt procedure. He was confined to a wheelchair because of ataxia from age 75 until the time of his death at age 81. Both temporal bones show congenitally hypoplastic endolymphatic sacs and severe endolymphatic hydrops consistent with Meniere's disease.

In search of pathologic correlates for hearing loss and vertigo in Paget's disease. A clinical and histopathologic study of 26 temporal bones.

Mixed sensorineural and conductive hearing loss is a common clinical manifestation of Paget's disease of the temporal bone, and while there are numerous clinical and pathologic reports on the condition, none have identified a consistent pathologic explanation for the hearing loss. We performed histologic studies on 26 temporal bones exhibiting Paget's disease from 16 persons, of whom 7 had audiometric testing performed. Contrary to common opinion, the conductive hearing loss is not caused by ossicular fixation; in fact, no cause could be found in the seven ears with documented conductive hearing losses.

The pathology of peripheral vestibular disorders in the elderly.

We describe a clinical classification of vertigo commonly seen in the elderly and caused by peripheral vestibular disorders. The classification includes inducible transient vertigo and noninducible protracted vertigo. The peripheral vestibular disorder includes abnormalities of sensory, neural, and mechanical structures and is often asymmetric.

Congenital aural atresia.

This report is based on 25 years' experience in the surgical management of 69 ears with congenital aural atresia. There were 7 ears with meatal atresia, 11 with partial atresia, 50 with total atresia, and 1 with hypopneumatic total atresia. Meatoplasty was performed in 7 ears, canaloplasty was performed in 22 ears, canaloplasties with strust in 8 ears, and mastoidectomy with stapediopexy was performed in 26 ears.