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Autoimmune vestibulo-cochlear disorders.

Autoimmune vestibulo-cochlear disorders (AVCD) represent a group of syndromes with overlapping clinical features, manifesting as sensorineural hearing loss, often associated with vertigo, tinnitus, and aural fullness, and believed to be caused by an autoimmune mechanism. Although definitive evidence of a classic "autoimmune process" is still lacking, substantial indirect evidence has accumulated to strongly indicate such a pathogenesis. Rapidly progressing AVCD is analogous to rapidly progressive glomerulonephritis in that inner ear inflammation progresses to severe, irreversible damage within 3 months of onset (and often much more quickly). Thus patients with rapidly progressive AVCD are treated with a sense of urgency. Prompt treatment with corticosteroids and other antirheumatic/immunosuppressive agents can preserve hearing and vestibular functions. We are not aware of any randomized controlled clinical trials evaluating the efficacy of antirheumatic/immunosuppressive agents in AVCD. In this article we review reports of various therapies that have been tried in this condition and our experience of etanercept therapy in AVCD.

Antirheumatic Agents↗

Consequences to hearing during the conservative management of vestibular schwannomas.

OBJECTIVE: To estimate the risk of loss of serviceable hearing during the conservative management of vestibular schwannomas. STUDY DESIGN: Retrospective case review. METHODS: Twenty-five patients with a radiological diagnosis of unilateral vestibular schwannoma were managed conservatively for a mean duration of 43.8 months (range, 12-194 mo). The pure-tone average (PTA) (0.5, 1, 2, and 3 kHz) and speech discrimination scores (SDS) were measured at regular intervals throughout the entire duration of follow-up. Serviceable hearing was defined using two criteria: 70% SDS/30 dB PTA (the 70/30 rule) and 50% SDS/50 dB PTA (the 50/50 rule). The size and growth rate of tumors were determined according to the American Academy of Otolaryngology-Head and Neck Surgery guidelines (1995). Intervention was recommended if there was evidence of continuous or rapid radiological tumor growth, and/or increasing symptoms or signs suggestive of tumor growth. RESULTS: The risk of loss of serviceable hearing for the total group was 43% using the 70/30 rule and 42% using the 50/50 rule. Tumor growth was considered significant (> 1 mm) in 8 tumors (32%) and nonsignificant in 17 (68%). The risk of loss of serviceable hearing for the tumor-growth group was 67% using the 70/30 rule and 80% using the 50/50 rule. In contrast, the risk of loss of serviceable hearing for the no tumor-growth group was 25% using the 70/30 rule and 14% using the 50/50 rule. No audiological factors predictive of tumor growth were identified. CONCLUSIONS: There is a significant risk of loss of serviceable hearing during the conservative management of vestibular schwannomas. This risk appears to be greater in tumors that demonstrate significant growth.

Adult↗

A critical look at vestibular dysfunction in learning-disabled children.

The purpose of this study was to investigate the vestibular dysfunction hypothesis in learning disabilities. The data indicated that there was no significant difference in vestibular function between normal and learning-disabled children; that there was no significant correlation between vestibular function and academic achievement; and that there was no significant educational relevance in categorizing learning-disabled children according to vestibular dimensions.

Achievement↗

The effect of various topical antibiotic and antibacterial agents on the middle and inner ear of the guinea-pig.

The topical application of antibiotics and anti-inflammatory agents is used to reduce inflammation of the middle ear mucosa and irradicate infection. Many of the antibiotic and anti-inflammatory preparations so used contain compounds known to be or are potentially ototoxic. Eighteen antibiotic, antibacterial, antifungal and anti-inflammatory compounds and four commonly used solvents were screened for the absence of ototoxicity and inflammation to the middle ear mucosa. All compounds were injected intra-tympanically and sensory hair cells loss recorded graphically. Inflammation of the middle ear mucosa was assessed macroscopically. Of the 18 compounds only three, penicillin, carbenicillin and nystatin were free of hair cell toxicity and inflammatory effects on the middle ear mucosa. Only one of the commonly used solvents was free of side effects.

Animals↗

Small cerebellar strokes may mimic labyrinthine lesions.

Thirty nine cases of cerebellar infarct and haemorrhage were seen over a period of 3 years. Of these, 69% had no impairment of consciousness. Six cases had nystagmus and gait ataxia as their only abnormal signs. Small cerebellar strokes may present with only vertigo, unsteady gait, and unidirectional nystagmus, thus mimicking labyrinthine lesions. Care in managing vertigo attacks in patients with risk factors for cerebrovascular disease appears to be warranted.

Adult↗

Management of superior canal dehiscence syndrome with extensive skull-base deficiency.

Superior canal dehiscence syndrome is a recently described condition resulting in noise- or pressure-induced vertigo. We review the case of a 50-year-old woman who presented with debilitating pressure and noise-induced vertigo as well as a low-frequency conductive hearing loss. Imaging was consistent with superior semicircular canal dehiscence syndrome. An extradural middle fossa approach was used to approach the dehiscent superior canal. Intraoperatively, our patient was found to have extensive idiopathic skull base dehiscence of the temporal floor. Middle ear and mastoid mucosa was exposed with focal areas of dura prolapsed into the mastoid cavity. Because of these findings, temporalis fascia and bone pate were used to cover the dehiscent canal as well as a large area of the temporal floor. Additionally, a temporalis muscle flap was rotated between the dura and the dehiscent temporal floor to reconstruct the middle fossa skull base and prevent encephalocele.

Bone Diseases↗

Hearing loss of a central type secondary to anoxic anoxia.

The effect of anoxic anoxia on the threshold sensitivity and amplitude of the responses from the auditory cortex, inferior colliculus and cochlea to acoustic stimuli in guinea pigs was studied. Decay of the amplitude of the responses from the auditory cortex and the inferior colliculus occurs faster and is more severe than that of the cochlea. Recovery of the amplitude of the responses is slower at the auditory cortex and the inferior colliculus than at the cochlea. Loss of auditory threshold sensitivity in anoxic anoxia is most prominent at the auditory cortex. The loss of sensitivity at the inferior colliculus is the next most severe. The loss of sensitivity at the cochlea is negligible. The relative vulnerability of the central auditory pathway to anoxic anoxia as compared to the end organ is demonstrated.

Animals↗

Antibody activity in perilymph from rats with type II collagen-induced autoimmune inner ear disease.

Rats were immunized with native type II bovine collagen to induce autoimmune inner ear disease, and the antibody activity in the inner ear fluid was measured. Antibody activity against type II collagen was detected in the perilymph at about 10% to 20% of serum level. Thus, the presence of antibody against type II collagen in inner ear fluid may imply an immune injury mechanism in inner ear diseases.

Animals↗