Menière's disease as a manifestation of vestibular ganglionitis.

OBJECTIVE: To present morphologic evidence of viral-induced vestibular nerve pathology in Menière's Disease (MD). MATERIAL STUDIED: Twelve temporal bones (TB) from 8 patients with the clinical symptoms of MD. RESULTS: There was endolymphatic hydrops (EH) and perilymphatic fibrosis in 10 of the 12 TB from MD patients. Of the 10 TB with EH of the pars inferior, 3 also contained outpouchings in the pars superior (utricle and canals), and 3 showed apical spiral ganglion cell loss. Focal vestibular nerve axonal degeneration was observed in all but one TB. CONCLUSION: Morphologic changes in TB of patients with MD, and clinical observations in patients with recurrent vestibulopathy, support the concept that the pathologic mechanism responsible for auditory and vestibular symptoms in MD may be reactivation of a latent viral vestibular ganglionitis.

Adult spontaneous cerebrospinal fluid otorrhea: diagnosis and management.

OBJECTIVE: To describe demographic, radiologic, and surgical features in adult patients with spontaneous cerebrospinal fluid otorrhea (SCSFO). STUDIED: Review was made of office and hospital charts of 21 patients with SCSFO and 2 patients with spontaneous CSF rhinorrhea, all of which were repaired successfully from 1989 to 1998. METHOD: Radiologic examples of the structure responsible for SCSFO and rhinorrhea are used to illustrate the changes essential for diagnosis. RESULTS: The responsible lesion for SCSFO and rhinorrhea in the adult are arachnoid granulations (AG) or villi, which do not reach a venous lumen and are aberrantly distributed in areas of the anterior, middle, and posterior cranial fossae that are in proximity to the middle ear/mastoid space, ethmoid, and sphenoid sinuses. The ages of the 21 patients ranged from 38 to 83 years (mean 63 years) with all but one older than 50 years. The sex ratio was 14 women to 7 men; the CSF leak was right sided in 13 and left sided in 8 patients. Eighteen of the SCSF leaks were located in the middle cranial fossa surface of the temporal bone (TB) while two were on the posterior fossa border of the TB. The middle fossa leaks were managed by craniotomy and repair with fascia, whereas the posterior fossa defects were obliterated by adipose tissue inserted through an intact canal wall mastoidectomy. The most common radiologic finding on computerized tomography (CT) was a soft tissue mass adjacent to a tegmen bone defect. The posterior fossa AG created an erosion of cortical and trabecular bone in the mastoid compartment. Spontaneous CSF rhinorrhea in two patients also radiologically appeared as soft tissue mass adjacent to bone erosion in the sphenoid and ethmoid sinuses. These also represent aberrant AGs, which are responsible for CSF rhinorrhea in later life. CONCLUSIONS: The demographic, radiologic, and pathologic findings in this series of 21 TB and 2 paranasal sinus SCSF leaks support the concept that the responsible lesions are AGs that are aberrantly located adjacent to pneumatized parts of the skull. Because these AGs enlarge with age, they may erode through the bony confines of the TB and sinuses and present as SCSFO or rhinorrhea in middle and old age.

Evidence for laryngeal paralysis in cricoarytenoid joint arthritis.

OBJECTIVE/HYPOTHESIS: To demonstrate denervation atrophy of laryngeal muscles in a case of gout involving the cricoarytenoid joint. METHODS: The posterior cricoarytenoid (PCA) and arytenoideus (A) muscles from a 72-year-old man with extensive gout were compared with those from a normal adult larynx (age and sex unknown) using stereologic techniques for changes in muscle composition and fiber diameter. RESULTS: The PCA and A muscles in the gout specimen contained changes Indicative of muscle degeneration. In the PCA the volume fraction (VF) of intact muscle was 0.30, of degenerating muscle 0.13, and of fat 0.16. A normal PCA had a VF for intact muscle of 0.64 and 0 for degenerating muscle and fat. Similar changes were seen in the gout A muscle but were not measured. Muscle fiber diameters in the gout PCA (1,024 fibers) showed a significantly higher atrophy and hypertrophy factor than the normal PCA (1,255 fibers). The variability coefficient in the gout PCA (487) was almost double that in the normal PCA (290). Although muscle fiber diameters were not measured in the A muscle in gout, variability in fiber size was seen. CONCLUSIONS: The pattern and magnitude of muscle fiber degeneration in the PCA and A muscles from a larynx with gout fixation of the cricoarytenoid joint indicate neural degeneration. Since similar changes were not found in the thyroarytenoid (TA) and lateral cricoarytenoid (LCA), the neuropathy is selective for the posterior branch of the recurrent laryngeal nerve. This neuropathy is likely responsible for vocal cord adduction (stridor) and incomplete closure of the posterior commissure (aspiration) associated with acute cricoarytenoid arthritis. In chronic cricoarytenoid joint arthritis, ankylosis of the joint space maintains the adducted cord position.

Pseudoepitheliomatous hyperplasia versus squamous cell carcinoma of the external auditory canal.

Four case reports are presented to demonstrate the clinical and histopathologic similarity of pseudoepitheliomatous hyperplasia (PH) to squamous cell carcinoma (SCC) in the external auditory canal (EAC). In all four cases the original report of SCC on a biopsy specimen of an EAC lesion was corrected on review to PH. In one patient conservative management resulted in resolution of the EAC lesion. A second patient underwent radiation therapy and partial temporal bone resection with no SCC found in the surgical specimen. A third patient's ear canal had healed with conservative treatment and repeated biopsy revealed no malignancy. After a 6-year symptom-free interval, she developed invasive SCC with bone involvement that required surgery and radiation treatment. A fourth patient underwent a sleeve resection of the skin of the EAC that proved to be PH, and no evidence of SCC was found. A thoughtful clinical history, careful physical examination, response to conservative treatment, and close communication with the pathologist should be exercised in the evaluation of EAC lesions.

Comparison of labyrinthectomy and vestibular neurectomy in the control of vertigo.

One hundred twenty-six patients who were treated with labyrinthectomy (81 patients) or vestibular neurectomy (45) between the years 1979 and 1994 were reviewed. The cause for vertigo in 124 of the 126 patients was Meniere's disease (89 patients), labyrinthitis (15), delayed endolymphatic hydrops (8), vestibular neuritis (7), and failed labyrinthectomy (5). In the remaining 2 patients, a normal labyrinth was sacrificed to fistulize a petrous apex cyst. Both procedures were equally effective in relieving vertigo (labyrinthectomy 98.8%; neurectomy 97.8%), but the length of hospitalization, length of disability before return to work, and cost were twice as great with vestibular neurectomy than with labyrinthectomy. More patients exhibited prolonged ataxia following neurectomy (5 patients) than after labyrinthectomy (2). Vestibular neurectomy was associated with several complications: reversible facial paresis (15 patients), meningitis (1), cerebrospinal fluid leak (1), and epidural hematoma (1). Labyrinthectomy was complicated by postoperative hyponatremia in 1 patient. Selective vestibular neurectomy preserved hearing in 32 (82%) of 39 patients. Criteria for recommending either ablation procedure are discussed. The incidence of sequential involvement of the contralateral ear was 1.5%.

Effect of singular neurectomy on the caloric response.

PURPOSE: This study was performed to determine the contribution of the posterior semicircular canal to the vestibulo-ocular responses (VOR) from caloric stimulation of the labyrinth. MATERIALS AND METHODS: Seven consecutive patients with disabling benign paroxysmal positional vertigo, which was treated with singular neurectomy, were tested before and after surgery with bithermal stimulation of the operated and nonoperated ears. RESULTS: There was a significant (P = .0156) decrease in the VOR from stimulation of the operated ear following singular neurectomy. The nonoperated ear showed a variety of changes that may have resulted from compensatory mechanisms and/or utricular dysfunction. CONCLUSIONS: We conclude that the posterior canal together with the lateral and superior canals are responsible for the VOR following caloric stimulation of the labyrinth.

Singular neurectomy in the management of paroxysmal positional vertigo.

Paroxysmal positional vertigo is one of the common causes of episodic vertigo seen in neurotologic practice. It is thought to be caused by high specific gravity deposits in the cupula (cupulolithiasis) or membranous posterior semicircular canal (canalithesis) as a result of trauma, aging, viral insult, or surgical insult to the inner ear. The vast majority (90%) of patients undergo spontaneous resolution or cure by positioning maneuvers designed to dislodge the otoconial debris. The very small number of patients with disabling chronic paroxysmal positional vertigo (more than 1 year duration) can be effectively relieved of the positional vertigo (95%) by selective transection of the nerve supply to the posterior canal sense organ (singular neurectomy) through a tympanotomy approach using local anesthesia. The risk of sensorineural hearing loss is 3%.

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. When the increments of threshold loss present a gradually decreasing linear distribution pattern on the audiometric scale and have no pathologic correlate, it is speculated that the hearing loss is caused by alterations in the physical characteristics of the cochlear duct, and the loss is identified as cochlear conductive presbycusis. It is clear that many individual cases do not separate into a specific type but have mixtures of these pathologic types and are termed mixed presbycusis. About 25% of all cases of presbycusis show none of the above characteristics and are classified as indeterminate presbycusis.