This is a malignant tumor of the outer ear, middle ear, or inner ear that may infiltrate locally or metastasize. Cancers may be lethal by invading nearby normal tissues or by spreading (metastasis) to sites distant from the place of origin. Malignant growths may also arise secondary to invasion of the external auditory canal, the external ear and from scalp or parotid neoplasms. Some tumors can arise from the vestibular division of the VIII cranial nerve; however these are usually benign. As these tumors increase in size, they may involve the internal auditory meatus and spread into the cerebellopontine angle and compress the cerebellum (division of the brain) and brainstem.

The cause of the neoplasm is unknown. However, most are either squamous cell or basal cell carcinomas. Chemicals, viruses, radiation, familial predisposition, lifestyle, or a combination of factors may cause an unregulated, disorganized increase in cells into cancerous new growths. External and internal risk factors such as chemicals, radiation, viruses, age, gender, race, and genetic factors may contribute to the patient's predisposition to cancer.

Signs and symptoms of the condition may include: hearing loss, tinnitus, unsteadiness, and vertigo (see Diagnostic Code: 6204 Peripheral vestibular disorders).

Diagnostic tests include a comprehensive general and head and neck physical examination; otoscopy; audiologic assessment; magnetic resonance imaging (MRI); computed tomography (CT) scan; x-rays; biopsy; electronystagmography (ENG); and caloric stimulation test.

Radiation therapy and surgical excision (simple to complex) with reconstruction if needed.

Residuals may include: loss of hearing, vertigo, and tinnitus.

• This disease, (condition), only in its active form , has a 3 year presumptive period and shall be granted service connection although not otherwise established as incurred in or aggravated by service if manifested to a compensable degree within the applicable time limits under 38 CFR 3.307 following service in a period of war or following peacetime service on or after January 1, 1947, provided the rebuttable presumption provisions of 38 CFR 3.307 are also satisfied. [38 CFR 3.309 [Disease subject to presumptive service connection], 38 CFR 3.309(a). [chronic disease].
• This condition shall be service connected when meeting the criteria as set forth in 38 CFR 3.311 [Claims based on exposure to ionizing radiation], and 38 CFR 3.311(b) [Initial review of claims] provided the provisions of 38 CFR 3.311(b)(5)(iv) have been met. A claim under 38 CFR 3.311 that involves radiation exposure requires an opinion by the C&P Service before any decision may be made by a Regional Office to grant or deny benefits.
• May be entitled to special monthly compensation where the veteran has a single service-connected disability rated as 100% with additional service-connected disability or disabilities independently ratable at 60% or more, which are separate and distinct from the 100% service-connected disability and involves different anatomical segments or bodily systems. See  38 CFR 3.350(i)(1) – Total plus 60% and – Special Monthly Compensation (SMC).
• If the veteran was exposed to an herbicide agent during active military, naval, or air service, this disease shall be service connected if the requirements of 38 CFR 3.307(a)(6) are met even though there is no record of such disease during service, provided further that the rebuttable presumption provisions of 38 CFR 3.307(d) are also satisfied [38 CFR 3.309(e) Disease subject to presumptive service connection].
• Compensation is payable for the combinations of service-connected and nonservice-connected disabilities specified in (38 CFR 3.383(a)(3))(see below) as if both disabilities were service-connected, provided the nonservice-connected disability is not the result of the veteran's own willful misconduct.
• Hearing impairment in one ear compensable to a degree of 10 percent or more as a result of service-connected disability and hearing impairment as a result of nonservice-connected disability that meets the provisions of 38 CFR 3.385 in the other ear.

Notes

• A rating of 100% shall continue beyond the cessation of any surgical radiation treatment, antineoplastic chemotherapy or other therapeutic procedure. Six months after discontinuance of such treatment, the appropriate disability rating shall be determined by mandatory VA examination. Any change in evaluation based on that or any subsequent examination shall be subject to the provisions of 38 CFR 3.105(e). If there has been no local recurrence or metastasis, rate on residuals.