The Genetics of Mitochondrial Disease by Ryan L. Davis, Carolyn M. Sue

By Ryan L. Davis, Carolyn M. Sue

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With eventual brainstem compression headaches, ataxia, hypesthesia of trigeminal nerve, and facial and lower cranial nerve deficits occur. 1,2 Tumor growth is 1–3 mm per year, and up to 30% show no growth, whereas 22% grow more than 2 mm per year. 3 Tumor measurements are based on the CPA three-axis component with additional mention of extent of IAC involvement and total tumor volume. Diagnostic tests include audiometry. There is usually an asymmetric sensorineural hearing loss with disproportionate decrease in speech discrimination.

2. Engineering controls: develop ways to isolate the source of noise (enclosures, barriers, distance) from susceptible individuals. 3. Administrative controls: determine ways to diminish noise intensity and exposure at work. 4. Worker education: proper training must be provided for employees and management to understand the risks of noise exposure, with retraining on an annual basis. 5. Hearing protection devices: mandatory when noise averages more than 90 dB during an 8-hour day. Ear plugs (premolded or custom made), canal caps, and ear muffs all reduce noise by 15–30 dB if continuously used and properly fitted.

The tinnitus and HL is usually high frequency, irreversible, and cumulative dose related. Vestibular side effects are less frequent and less severe. Pathology is attributed to iron-induced free radicals, which injure the organ of Corti at the basal turn of the cochlea. Concurrent therapy with fosfomycins may prevent the toxic effects on the otic capsule. 10,11 Numerous other agents may be ototoxic to varying degrees. Most commonly encountered are caffeine, nonsteroidal anti-inflammatory medications, nicotine, alcohol, industrial solvents, iodine, and some metals.

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