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Causes of Sensorineural Deafness
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This has many causes Explosions or intense, sustained noise in certain industrial settings or from gun blasts or even rock music may result in a high-tone sensorineural hearing loss Certain antimicrobial drugs (namely, the aminoglycoside group and vancomycin) damage cochlear hair cells and, after prolonged use, can result in severe hearing loss If these drugs have been used to treat bacterial meningitis, it may be dif cult to determine whether the antibiotic or the infection is the cause A variety of other commonly used drugs are ototoxic, usually in a dose-dependent fashion (see Nadol) Quinine and acetylsalicylic acid may impair sensorineural function transiently The common high-frequency sensorineural type of hearing loss in the aged (presbycusis) is probably due to neuronal degeneration, ie, progressive loss of spiral ganglion neurons (Suga and Lindsay) The cochlea of a neonate may have been damaged in utero by rubella in the pregnant mother Mumps, acute purulent meningitis (particularly from Pneumococcus and Haemophilus), or chronic infection spreading from the middle to the inner ear may cause nerve deafness in childhood The meningeal infection spreads along the cochlear aqueduct, a structure that connects the cerebrospinal uid (CSF) space with the perilymph of the cochlea Measles vaccination, Mycoplasma pneumoniae infection, and scarlet fever are sometimes associated with acute deafness with or without vestibular symptoms It is uncertain whether the deafness in these cases is due to direct infection or represents an autoimmune reaction directed to the inner ear Also, the inner ear contains melanocytes, and their involvement in Vogt-Koyanagi-Harada disease adds dysacusis, tinnitus, and sensorineural deafness to the usual manifestations of vitiligo of the eyebrows, iridocyclitis, retinal depigmentation, and recurrent meningitis Meningeal hemosiderosis, a rare process that results from repeated bouts of subarachnoid hemor-
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rhage, also causes eighth nerve damage and deafness, presumably as a toxic effect of iron deposition in the meninges Episodic deafness in one ear, even without vertigo, proves in most instances to be Meniere disease (see further on) Of equal ` concern to neurologists is the onset in an adult of sudden and permanent unilateral hearing loss without vertigo Little is known about the pathogenesis of this syndrome A vascular causation (occlusion of the auditory artery or its cochlear branch or presumed arterial spasm in the course of migraine) has been postulated, on uncertain grounds We do not know how to interpret the ndings of DeFelice and colleagues as well as others, who report that the posterior communicating arteries are absent in a disproportionate number of patients with sudden hearing loss A few cases have complicated herpes zoster and mumps parotitis, but aside from these there is no proven relationship to the usual viral respiratory infections In a prospective study of 88 cases of acute sensorineural hearing loss, two-thirds recovered their hearing completely within a few days or a week or two (Mattox and Simmons) In the remaining patients, recovery was much slower and often incomplete; in this latter group, the hearing loss was predominantly for high tones and in some cases was associated with varying degrees of vertigo and hypoactive caloric responses The sudden onset of bilateral sensorineural hearing loss has been reported to follow cardiopulmonary bypass surgery and has been ascribed to microemboli Less often, such an event follows general anesthesia for nonotologic surgery (Evan et al); the pathogenesis is quite obscure None of the currently popular therapeutic agents such as histamine, calcium channel blockers, anticoagulants, carbogen inhalation, and steroids seem to affect the outcome of sudden unilateral or bilateral deafness without vertigo Nonetheless, corticosteroids are often prescribed Otologists have described a progressive sensorineural type of hearing loss as a late manifestation of congenital syphilis, allegedly occurring despite prior treatment with adequate doses of penicillin It has been claimed that the long-term administration of steroids may be useful in such cases The pathologic basis of the hearing loss has not been determined and the causal relationship to congenital syphilis remains to be established The auditory nerve may be involved by tumors of the cerebellopontine angle or by mycotic, lymphomatous, carcinomatous, tuberculous, or other types of chronic meningitis and rarely in sarcoidosis Lymphomatous meningitis has a particular predilection to cause unilateral hearing loss; we have seen several such cases in which no other cranial nerves were in ltrated Carcinomatous meningitis may do the same but almost always in the context of other cranial and spinal nerve palsies (see Chap 31) Of the solid tumors, the ones that involve the auditory nerve most frequently are schwannomas, neuro bromas, meningiomas, dermoids, and metastatic carcinoma In central neuro bromatosis (type II), the involvement by acoustic neuromas is often bilateral Unilateral deafness may also result from demyelinative plaques, infarction, or tumor involving the cochlear nerve bers or nuclei in the brainstem Rarely, deafness is the result of bilateral lesions of the temporal lobes (Chap 22) The condition called pure word deafness is also due to left temporal lobe disease; despite normal pure-tone perception and audiometry and normal brainstem auditory evoked potentials, spoken words cannot be understood This condition is discussed in Chap 23 Hereditary Deafness (Table 15-1) A large number of genetically determined syndromes that feature a neural or conductive type of deafness some congenital and others having their onset in childhood or early adult life have come to light (see articles by Tekin
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